TI: Bilateral periventricular nodular heterotopia with mental retardation and syndactyly in boys: a new X-linked mental retardation syndrome.
AU: Dobyns-WB; Guerrini-R; Czapansky-Beilman-DK; Pierpont-ME; Breningstall-G; Yock-DH Jr; Bonanni-P; Truwit-CL
AD: Department of Neurology, and Institute of Human Genetics, University of Minnesota Medical School, Minneapolis, USA.
SO: Neurology. 1997 Oct; 49(4): 1042-7
ISSN: 0028-3878
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: Bilateral periventricular nodular heterotopia (BPNH) is a recently recognized malformation of neuronal migration, and perhaps proliferation, in which nodular masses of gray matter line the walls of the lateral ventricles. Most affected individuals have epilepsy and normal intelligence with no other congenital anomalies. A striking skew of the sex ratio has been observed because 31 of 38 probands have been female, and one gene associated with BPNH was recently mapped to chromosome Xq28. We report three unrelated boys with a new multiple congenital anomaly-mental retardation syndrome that consists of BPNH, cerebellar hypoplasia, severe mental retardation, epilepsy, and syndactyly. Variable abnormalities included focal or regional cortical dysplasia, cataracts, and hypospadius. We hypothesize that this syndrome involves the same Xq28 locus as isolated BPNH, and we review the expanding number of syndromes associated with BPNH.
MESH: Adolescence-; Brain-Diseases-diagnosis; Brain-Diseases-genetics; Child,-Preschool; Magnetic-Resonance-Imaging; Syndactyly-pathology; Syndrome-
MESH: *Cerebral-Ventricles; *Choristoma-genetics; *Linkage-Genetics; *Mental-Retardation-genetics; *Periaqueductal-Gray; *Syndactyly-genetics; *X-Chromosome
TG: Case-Report; Human; Male
PT: JOURNAL-ARTICLE; REVIEW; REVIEW-OF-REPORTED-CASES
AN: 97479606
UD: 9801
SB: AIM
MEDLINE EXPRESS (R) 10/97-1/98 2 of 48
TI: Identification of a duplication of Xq28 associated with bilateral periventricular nodular heterotopia.
AU: Fink-JM; Dobyns-WB; Guerrini-R; Hirsch-BA
AD: Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis 55455, USA.
SO: Am-J-Hum-Genet. 1997 Aug; 61(2): 379-87
ISSN: 0002-9297
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: Bilateral periventricular nodular heterotopia (BPNH) is a malformation of neuronal migration and is characterized by nodules of heterotopic gray matter lining the lateral ventricles of the brain. The majority of BPNH patients are female and have epilepsy as a sole clinical manifestation of their disease. Familial BPNH has been mapped to Xq28 by linkage analysis. A multiple congenital anomaly-mental retardation syndrome (BPNH/MR) was recently delineated in three unrelated boys with BPNH, cerebellar hypoplasia, severe mental retardation, epilepsy, and syndactyly. High-resolution chromosome analysis revealed a subtle abnormality of Xq28 in one of the boys with BPNH/MR syndrome. FISH with cosmids and YACs from Xq28 further characterized this abnormality as a 2.25-3.25-Mb inverted duplication. No abnormality of Xq28 was detected by G-banding or FISH in the other two boys. These data support the linkage assignment of BPNH to band Xq28 and narrow the critical region to the distal 2.25-3.25 Mb of Xq28.
MESH: Brain-Diseases-embryology; Cell-Movement; Cerebellum-abnormalities; Cerebral-Ventricles; Child,-Preschool; Chromosome-Aberrations; Chromosome-Banding; Epilepsy-genetics; In-Situ-Hybridization,-Fluorescence; Inversion-Genetics; Linkage-Genetics; Mental-Retardation-genetics; Neurons-physiology; Syndactyly-genetics; Syndrome-
MESH: *Brain-Diseases-genetics; *Cerebral-Cortex; *Choristoma-genetics; *Sex-Chromosome-Abnormalities-genetics; *X-Chromosome-genetics
TG: Female; Human; Support,-Non-U.S.-Gov't; Support,-U.S.-Gov't,-Non-P.H.S.
PT: JOURNAL-ARTICLE
AN: 97456429
UD: 9712
MEDLINE EXPRESS (R) 10/97-1/98 3 of 48
TI: Periventricular heterotopias display cortical degenerative neuropathology.
AU: Joseph-JT
AD: Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA.
SO: Neurology. 1997 Sep; 49(3): 884-7
ISSN: 0028-3878
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: This report describes beta-amyloid plaques in periventricular heterotopias in two elderly women. The neuropathologic features of the heterotopias recapitulated those of nearby neocortex but were distinct from those of adjacent caudate nucleus. In response to the factors important in plaque and tangle formation, the heterotopias biologically behave like neocortex and unlike adjacent striatum, further supporting the morphologic evidence that these tissues represent heterotopic cortex.
MESH: Aged-; Alzheimer's-Disease-pathology; Caudate-Nucleus-pathology; Immunoenzyme-Techniques; Occipital-Lobe-pathology
MESH: *Cerebral-Cortex-pathology; *Cerebral-Ventricles-pathology; *Choristoma-pathology; *Dementia-pathology
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 97450374
UD: 9712
SB: AIM
MEDLINE EXPRESS (R) 10/97-1/98 4 of 48
TI: A systematic approach for interpreting MR images of the seizure patient.
AU: Bronen-RA; Fulbright-RK; Kim-JH; Spencer-SS; Spencer-DD
AD: Department of Diagnostic Radiology, Yale University School of Medicine, New Haven, CT 06520-18042, USA.
SO: AJR-Am-J-Roentgenol. 1997 Jul; 169(1): 241-7
ISSN: 0361-803X
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: A systematic approach needs to be used to review MR scans in epilepsy patients to avoid the common pitfalls engendered by the subtle nature of many epileptogenic lesions. One should always evaluate the hippocampus regardless of other MR findings to avoid missing dual abnormalities. False-positive and false-negative diagnosis of hippocampal sclerosis can be avoided by evaluating the hippocampus after correcting for head rotation [by assessing the internal auditory canals and atria). Periventricular heterotopia can be successfully diagnosed by systematically studying the periventricular regions, especially those adjacent to the atria of the lateral ventricles. Gray matter lateral to the ventricles (excluding the caudate nucleus) is always an abnormal finding. Sulcal and cortical morphologic abnormalities are particularly difficult to diagnose unless a high index of suspicion is maintained. Cortical thickening is indicative of a developmental anomaly and should be screened in an organized manner. Because epilepsy is generally a cortical process, one must search for subtle cortical abnormalities, including focal atrophic abnormalities and lesions without mass effect. Diligence will offer its own rewards.
MESH: Adult-; Epilepsy-diagnosis; Epilepsy-etiology; Epilepsy-surgery
MESH: *Brain-pathology; *Epilepsy-pathology; *Magnetic-Resonance-Imaging
TG: Female; Human; Male
PT: JOURNAL-ARTICLE
AN: 97351251
UD: 9711
SB: AIM
MEDLINE EXPRESS (R) 10/97-1/98 5 of 48
TI: Tuberous sclerosis in a 20-week gestation fetus: immunohistochemical study.
AU: Park-SH; Pepkowitz-SH; Kerfoot-C; De-Rosa-MJ; Poukens-V; Wienecke-R; DeClue-JE; Vinters-HV
AD: Department of Pathology (Neuropathology), UCLA School of Medicine 90095-1732, USA.
SO: Acta-Neuropathol-Berl. 1997 Aug; 94(2): 180-6
ISSN: 0001-6322
PY: 1997
LA: ENGLISH
CP: GERMANY
AB: We report an autopsy case of tuberous sclerosis complex (TSC) in a 20-week gestational age female fetus. The brain showed lesions suggestive of early cortical tubers and subependymal hamartomatous nodules. The large cells within these nodular clusters were variably immunoreactive for glial fibrillary acidic protein (GFAP) and vimentin and negative for synaptophysin and neurofilament. Subependymal radial glia expressed both vimentin and GFAP, but subpial radial glia either did not express these markers (in contrast to an age-matched control) or were absent. Tuberin expression was noted in heterotopic neurons in the white matter and brain cells consistent with Cajal Retzius cells in the neocortical molecular layer, very weakly in superficial cortical neurons, neurons in the basal ganglia, Purkinje cells and external granular cells of cerebellum, cranial nerve nuclei neurons, occasional germinal matrix cells, ependymal cells, choroid plexus epithelium, and pituitary gland neuroendocrine cells; it was not seen within the cells of subependymal nodules. The pattern of tuberin immunoreactivity was similar to that which we have observed in older TSC patients. Proliferating cell labeling indexes were comparable in the germinal matrix of the TSC patient and an age-matched control. Abnormal subpial radial glia may be responsible for some of the neuronal migration abnormalities that appear to result in neocortical tubers.
MESH: Brain-pathology; Brain-Chemistry; Fetus-; Gestational-Age; Glial-Fibrillary-Acidic-Protein-analysis; Immunohistochemistry-; Neurofilament-Proteins-analysis; Neurons-chemistry; Neurons-pathology; Pregnancy-; Synaptophysin-analysis; Tuberous-Sclerosis-embryology; Tuberous-Sclerosis-physiopathology; Vimentin-analysis
MESH: *Tuberous-Sclerosis-pathology
TG: Case-Report; Female; Human; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
CN: PO1NS28383NSNINDS
RN: 0; 0; 0; 0
NM: Glial-Fibrillary-Acidic-Protein; Neurofilament-Proteins; Synaptophysin; Vimentin
AN: 97399265
UD: 9711
MEDLINE EXPRESS (R) 10/97-1/98 6 of 48
TI: Recovery of taste aversion learning induced by fetal neocortex grafts: correlation with in vivo extracellular acetylcholine.
AU: Miranda-MI; Lopez-Colome-AM; Bermudez-Rattoni-F
AD: Departamento de Neurociencias, Instituto de Fisiologia Celular, Universidad Nacional Autonoma de Mexico, D.F.
SO: Brain-Res. 1997 Jun 6; 759(1): 141-8
ISSN: 0006-8993
PY: 1997
LA: ENGLISH
CP: NETHERLANDS
AB: Rats showing disrupted taste aversion due to insular cortex lesions, received either homotopic or heterotopic (occipital) cortical fetal brain grafts. Behavioral results showed that the recovery of the ability to acquire conditioned taste aversions induced by fetal grafts depended on post-graft time (45 but not at 15 days) and tissue specificity (homotopic but not heterotopic). In vivo analysis of acetylcholine (ACh) release revealed that only the group receiving homotopic grafts and tested 45 days post graft had a release of ACh after KCl stimulation similar to that in the control group. Furthermore, homotopic grafts and lesioned groups showed significantly weaker specific receptor binding of [3H]L-glutamate compared with controls. These results suggest that ACh is specifically involved in the process of behavioral recovery induced by homotopic cortical transplants.
MESH: Acetylcholine-metabolism; Cerebral-Cortex-metabolism; Conditioning-Psychology-physiology; Extracellular-Space-metabolism; Glutamic-Acid-metabolism; Microdialysis-; Potassium-Chloride-pharmacology; Rats-; Rats,-Wistar; Transplantation,-Heterotopic
MESH: *Avoidance-Learning-physiology; *Cerebral-Cortex-embryology; *Cerebral-Cortex-physiology; *Fetal-Tissue-Transplantation; *Taste-physiology
TG: Animal; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 51-84-3; 56-86-0; 7447-40-7
NM: Acetylcholine; Glutamic-Acid; Potassium-Chloride
AN: 97363579
UD: 9711
MEDLINE EXPRESS (R) 10/97-1/98 7 of 48
TI: Inflammatory cell-derived NO modulates cardiac allograft contractile and electrophysiological function.
AU: Worrall-NK; Pyo-RT; Botney-MD; Misko-TP; Sullivan-PM; Alexander-DG; Lazenby-WD; Ferguson-TB
AD: Department of Surgery, Washington University School of Medicine, St. Louis 63110, USA.
SO: Am-J-Physiol. 1997 Jul; 273(1 Pt 2): H28-37
ISSN: 0002-9513
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: We previously demonstrated that inhibition of inducible nitric oxide (NO) synthase (iNOS) ameliorated acute cardiac allograft rejection. This study used a rat cardiac transplant model to characterize contractile and electrophysiological dysfunction during early acute rejection, further examine the role of NO and iNOS in this process, and determine which cells expressed iNOS during early rejection. During early acute rejection, before significant myocyte necrosis, allograft papillary muscles had reduced tension development and rates of tension development and decline during beta-adrenergic, adenylate cyclase, and calcium stimulation compared with isograft and normals [e.g., tension of 36 (allograft) vs. 73 (isograft) mN/mm2 during calcium stimulation, P < 0.001]. Allografts had resting membrane potential depolarization and reduced action potential amplitude and upstroke velocity. iNOS mRNA was expressed in infiltrating inflammatory cells but not in allograft myocytes, endothelial cells, or isografts. Corticosteroids attenuated allograft contractile and electrophysiological dysfunction and inhibited iNOS enzyme activity. Direct iNOS inhibition with aminoguanidine inhibited NO production and prevented allograft contractile and electrophysiological dysfunction (e.g., tension of 64 mN/mm2 during calcium stimulation, P < 0.001). We conclude that 1) early allograft rejection caused contractile and electrophysiological dysfunction that was largely mediated by iNOS expression in infiltrating inflammatory cells, 2) corticosteroid-mediated amelioration of allograft contractile and electrophysiological dysfunction may reflect inhibition of iNOS, and 3) iNOS inhibition may offer an alternative in management of immune-mediated myocardial dysfunction.
MESH: Action-Potentials-drug-effects; Adenyl-Cyclase-metabolism; Adrenal-Cortex-Hormones-pharmacology; Calcium-pharmacology; Cells,-Cultured; Electrophysiology-methods; Guanidines-pharmacology; Heart-Rate-drug-effects; Heart-Ventricle; Membrane-Potentials-drug-effects; Methylprednisolone-pharmacology; Nitric-Oxide-Synthase-antagonists-and-inhibitors; Papillary-Muscles-drug-effects; Rats-; Rats,-Inbred-ACI; Rats,-Inbred-Lew; RNA-Probes; RNA,-Messenger-biosynthesis; Transcription,-Genetic; Transplantation,-Heterotopic; Transplantation,-Homologous-physiology; Transplantation,-Isogeneic-physiology
MESH: *Heart-Transplantation-physiology; *Myocardial-Contraction; *Nitric-Oxide-physiology; *Nitric-Oxide-Synthase-biosynthesis; *Papillary-Muscles-physiology
TG: Animal; Comparative-Study; In-Vitro; Male; Support,-Non-U.S.-Gov't; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
CN: F32HL09021HLNHLBI; R29HL46387HLNHLBI
RN: EC 1.14.13.39; EC 4.6.1.1; 0; 0; 0; 0; 10102-43-9; 7440-70-2; 79-17-4; 83-43-2
NM: Nitric-Oxide-Synthase; Adenyl-Cyclase; Adrenal-Cortex-Hormones; Guanidines; RNA-Probes; RNA,-Messenger; Nitric-Oxide; Calcium; pimagedine; Methylprednisolone
AN: 97392953
UD: 9711
MEDLINE EXPRESS (R) 10/97-1/98 8 of 48
TI: Afterload mismatch-related problems after "domino" heart transplantation.
AU: Campana-C; Gavazzi-A; Inserra-C; Pederzolli-N; Ponzetta-M; Martinelli-L; Vigano-M
AD: Divisione di Cardiologia, Policlinico S. Matteo, Pavia.
SO: G-Ital-Cardiol. 1997 Jun; 27(6): 533-9
ISSN: 0046-5968
PY: 1997
LA: ENGLISH
CP: ITALY
AB: The heart transplants with domino technique, which uses donor hearts from heart-lung recipients, increases the pool of donors, provides the advantage of shortening the ischemic time and makes suitable hearts for patients with pulmonary hypertension. The present study aimed to characterise the pre- and post-transplant clinical and hemodynamic profiles of patients that underwent domino transplant in Pavia. METHODS: Between 1991 and 1992, 9 heart transplants were performed with the domino procedure at I.R.C.C.S. Policlinico S. Matteo of Pavia. Domino donors (6 with primary pulmonary hypertension, 2 with Eisenmenger's syndrome due to atrial septal defect, 1 with cystic fibrosis) underwent electrocardiographic, echocardiographic, chest roentgenogram studies, and right heart catheterization and coronary angiography (for donor older than 40). Domino recipients, 6 males and 3 females with a mean age of 44 years, had dilated cardiomyopathy (4 cases), coronary artery disease (4 cases) and valvular heart disease (1 case) (group 1). Seven of the 9 cases entered the study; 2 were excluded: one because had undergone heterotopic transplantation, the other had received the heart from another country and therefore the graft had suffered from a very long ischemic time. Controls group consisted of 12 patients who had consecutively undergone cardiac transplantation with non-domino donors during the same period (group 2). Immunosuppression was similar in both groups, and consisted of a combination of cyclosporin A, azathioprine and corticosteroids, plus a 7-day-course of antithymocyte globulin. Hemodynamic and echocardiographic controls were performed at 2, 3 and 4 weeks (short-term control) and at 2 and 6 months (mid-term control) after surgery. RESULTS: Domino donors (39 +/- 12.5 years) had significantly higher mean right ventricular end-diastolic diameter and lower left ventricular diameter than normal mean values. Domino recipients had significantly higher mean pulmonary arteriolar resistances than controls; mean ischemic time was also significantly lower in group 1 than in group 2. Short- and mid-term controls after surgery in group 1 showed persistently higher systemic vascular resistances and pulmonary vascular resistances and lower cardiac output than in group 1. Two patients developed an early and unexpected increase in pulmonary wedge pressure accompanied by severe paroxysmal nocturnal dyspnea and mitral regurgitation. In all cases, the left ventricles were relatively inadequate; the combination of low cardiac output and of high systemic vascular resistances favoured the occurrence of an afterload mismatch condition that was worsened by chronic hypoxia. This condition must be known and expected in these patients after transplantation in order to provide timely and effective treatment to potentially life-threatening left ventricular failure episodes. IN CONCLUSION, the subset of transplanted patients that receives domino donors may develop left-side afterload mismatch which, combined with low cardiac output, with high systemic vascular resistances and with the effects of chronic hypoxia originally suffered by the heart, may cause sudden and unexpected left-side heart failure which has to be timely recognised and managed. Although hemodynamic adaptation of this patients is highly problematic, it does not limit the value of the domino procedure.
MESH: Adult-; Echocardiography-; Follow-Up-Studies; Hemodynamics-physiology; Immunosuppression-; Middle-Age; Reoperation-; Risk-Factors; Ventricular-Function
MESH: *Heart-Transplantation-adverse-effects
TG: Female; Human; Male
PT: CLINICAL-TRIAL; JOURNAL-ARTICLE
AN: 97378425
UD: 9711
MEDLINE EXPRESS (R) 10/97-1/98 9 of 48
TI: Ultrasonographic evaluation of renal size in dogs with acute allograft rejection.
AU: Nyland-TG; Fisher-PE; Gregory-CR; Wisner-ER
AD: Department of Surgical and Radiological Sciences, School of Veterinary Medicine, University of California, Davis 95616, USA.
SO: Vet-Radiol-Ultrasound. 1997 Jan-Feb; 38(1): 55-61
ISSN: 1058-8183
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: The purpose of this study was to determine the best method to ultrasonographically monitor renal size changes associated with acute allograft rejection in dogs. Qualitative changes in renal cortical and medullary echogenicity were also evaluated, although this was not a major focus of the study. Four unrelated, mixed-breed dogs underwent bilateral nephrectomies and heterotopic renal allograft transplantation. Ultrasound examinations of transplanted kidneys were initiated at 3 days after surgery and continued at 2-3 day intervals until death (38 +/- 2 days). Ultrasound measurements of kidney length, width, height, cross-sectional area, and estimated volume were used to assess relative changes in renal size associated with transplantation and rejection. Transplanted kidneys had a rapid increase in volume and cross-sectional area that averaged 103% and 83% above baseline levels, respectively, by 17 days after transplantation. The increased size was attributed to a combination of hypertrophy and acute rejection, the latter of which was confirmed at postmortem. Kidney volume decreased to approximately 35% above baseline volume by day 34 as rejection became more advanced. Qualitative changes associated with rejection included medullary enlargement with decreased echogenicity early in the study, followed by increased cortical thickness and echogenicity with poor cortical medullary definition in the latter stages of the survival period. It was concluded that relative changes in renal allograft size can be easily monitored with ultrasound. In regard to linear measurements, changes in renal width were more pronounced than changes in height or length with acute rejection. Therefore measurements that incorporate the width, namely volume or cross-sectional area, appear to be the most sensitive for monitoring changes in allograft size. Renal cross-sectional area measurements are preferred because they are simple to perform using the automated calculation capability of most newer ultrasound units.
MESH: Acute-Disease; Anatomy,-Cross-Sectional; Creatinine-blood; Dog-Diseases-pathology; Dogs-; Evaluation-Studies; Follow-Up-Studies; Graft-Rejection-pathology; Graft-Rejection-ultrasonography; Graft-Survival; Hypertrophy-; Kidney-Cortex-pathology; Kidney-Cortex-ultrasonography; Kidney-Medulla-pathology; Kidney-Medulla-ultrasonography; Kidney-Pelvis-pathology; Kidney-Pelvis-ultrasonography; Kidney-Transplantation-pathology; Kidney-Transplantation-ultrasonography; Nephrectomy-veterinary; Sensitivity-and-Specificity; Time-Factors; Transplantation,-Heterotopic-veterinary; Transplantation,-Homologous
MESH: *Dog-Diseases-ultrasonography; *Graft-Rejection-veterinary; *Kidney-Transplantation-veterinary
TG: Animal; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 60-27-5
NM: Creatinine
AN: 97381458
UD: 9711
MEDLINE EXPRESS (R) 10/97-1/98 10 of 48
TI: [Femur head necrosis in the adult. Diagnosis, therapy, legal aspects for insurance medicine]
TO: Huftkopfnekrose des Erwachsenen. Diagnostik, Therapie, versicherungsrechtliche Aspekte.
AU: Vogel-HJ; Schwetlick-G
AD: Orthopadischen Klinik der Pfeifferschen Stiftungen, Magdeburg.
SO: Versicherungsmedizin. 1997 Jun 1; 49(3): 82-5
ISSN: 0933-4548
PY: 1997
LA: GERMAN; NON-ENGLISH
CP: GERMANY
AB: Early recognition and adequate therapy of necrosis of the head of the femur in adult patients in increasingly gaining importance since they considerably influence the permanent loss of function and reduction of the earning capacity of people of working age. Presently, MRT examination is the most conclusive method for the early stage of necrosis of the head of the femur. After the introduction of microsurgical methods, new therapeutic measures for avascular necrosis of the head of the femur are available, apart from conventional surgical methods to relieve the necrotic areas and the so-called metabolic irritation drilling. Direct vascular-supply, autologous, heterotopic, corticospongiose transplants in the necrotic head of the femur are suited to re-establish circulation in the necrotic area. This involves a considerable operative scope of work.
MESH: Adult-; Disability-Evaluation; English-Abstract; Femur-Head-pathology; Femur-Head-Necrosis-etiology; Femur-Head-Necrosis-rehabilitation; Magnetic-Resonance-Imaging; Prognosis-
MESH: *Expert-Testimony-legislation-and-jurisprudence; *Femur-Head-Necrosis-diagnosis; *Insurance,-Accident-legislation-and-jurisprudence
TG: Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 97350609
UD: 9710
MEDLINE EXPRESS (R) 10/97-1/98 11 of 48
TI: [Acetabular fractures in elderly persons. Primary endoprosthetic treatment]
TO: Acetabulumfraktur beim alten Menschen. Primare endoprothetische Versorgung.
AU: Hoellen-IP; Mentzel-M; Bischoff-M; Kinzl-L
AD: Abteilung fur Unfall-, Hand- und Wiederherstellungschirurgie, Universitatsklinik Ulm.
SO: Orthopade. 1997 Apr; 26(4): 348-53
ISSN: 0085-4350
PY: 1997
LA: GERMAN; NON-ENGLISH
CP: GERMANY
AB: In the Department of Traumatology at the University of Ulm, 26 elderly patients with displaced acetabular fractures were treated with primary implantation of a hip joint endoprosthesis between 1986 and 1996. The principles of operative therapy are stabilization of the acetabular ring, grafting of acetabular defects with autogenous corticocancellous bone and cranial buttressing with a reinforcing ring. This concept permits immediate mobilization of elderly patients and full weight-bearing and thus results in favorable early postoperative results. Secondary complications typically caused by immobilization can therefore be avoided.
MESH: Acetabulum-surgery; Age-Factors; Aged-; Aged,-80-and-over; Algorithms-; Early-Ambulation; English-Abstract; Hip-Prosthesis; Middle-Age; Ossification,-Heterotopic-radiography; Postoperative-Complications-radiography; Transplantation,-Autologous
MESH: *Acetabulum-injuries; *Bone-Transplantation-methods; *Fractures-surgery
TG: Female; Human; Male
PT: JOURNAL-ARTICLE
AN: 97318177
UD: 9710
MEDLINE EXPRESS (R) 10/97-1/98 12 of 48
TI: A genetic animal model of human neocortical heterotopia associated with seizures.
AU: Lee-KS; Schottler-F; Collins-JL; Lanzino-G; Couture-D; Rao-A; Hiramatsu-K; Goto-Y; Hong-SC; Caner-H; Yamamoto-H; Chen-ZF; Bertram-E; Berr-S; Omary-R; Scrable-H; Jackson-T; Goble-J; Eisenman-L
AD: Department of Neurological Surgery, University of Virginia Health Sciences Center, Charlottesville, Virginia 22908, USA.
SO: J-Neurosci. 1997 Aug 15; 17(16): 6236-42
ISSN: 0270-6474
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: Malformations of the human neocortex are commonly associated with developmental delays, mental retardation, and epilepsy. This study describes a novel neurologically mutant rat exhibiting a forebrain anomaly resembling the human neuronal migration disorder of double cortex. This mutant displays a telencephalic internal structural heterotopia (tish) that is inherited in an autosomal recessive manner. The bilateral heterotopia is prominent below the frontal and parietal neocortices but is rarely observed in temporal neocortex. Neurons in the heterotopia exhibit neocortical-like morphologies and send typical projections to subcortical sites; however, characteristic lamination and radial orientation are disturbed in the heterotopia. The period of neurogenesis during which cells in the heterotopia are generated is the same as in the normotopic neocortex; however, the cells in the heterotopia exhibit a "rim-to-core" neurogenetic pattern rather than the characteristic "inside-out" pattern observed in normotopic neocortex. Similar to the human syndrome of double cortex, some of the animals with the tish phenotype exhibit spontaneous recurrent electrographic and behavioral seizures. The tish rat is a unique neurological mutant that shares several features with a human cortical malformation associated with epilepsy. On the basis of its regional connectivity, histological composition, and period of neurogenesis, the heterotopic region in the tish rat is neocortical in nature. This neurological mutant represents a novel model system for investigating mechanisms of aberrant neocortical development and is likely to provide insights into the cellular and molecular events contributing to seizure development in dysplastic neocortex.
MESH: Cerebral-Cortex; Choristoma-complications; Choristoma-physiopathology; Electroencephalography-; Epilepsy-etiology; Epilepsy-physiopathology; Image-Processing,-Computer-Assisted; Magnetic-Resonance-Imaging; Neural-Pathways-physiology; Prosencephalon-embryology; Prosencephalon-pathology; Prosencephalon-physiopathology; Rats-
MESH: *Choristoma-pathology; *Disease-Models,-Animal; *Epilepsy-pathology; *Rats,-Mutant-Strains
TG: Animal; Female; Male; Support,-Non-U.S.-Gov't; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
CN: NS34124NSNINDS
AN: 97383273
UD: 9710
MEDLINE EXPRESS (R) 10/97-1/98 13 of 48
TI: Internexin, MAP1B, and nestin in cortical dysplasia as markers of developmental maturity.
AU: Crino-PB; Trojanowski-JQ; Eberwine-J
AD: Department of Neurology, University of Pennsylvania Medical Center, Philadelphia 19140, USA.
SO: Acta-Neuropathol-Berl. 1997 Jun; 93(6): 619-27
ISSN: 0001-6322
PY: 1997
LA: ENGLISH
CP: GERMANY
AB: Cortical dysplasias (CD) are characterized histologically by disorganized cortical lamination and abnormally shaped neurons. We hypothesized that neurons within CD have failed to differentiate fully and may express proteins such as cytoskeletal elements characteristic of immature cells. Disrupted expression of certain cytoskeletal proteins, which have been implicated in neuronal polarity, process outgrowth, and migration, could result in disorganized cortical lamination. Thus, we probed two CD subtypes, focal CD (FCD) and hemimegalencephaly (HME), with antibodies specific for cytoskeletal proteins that are developmentally regulated in neural progenitor cells and neurons to define more fully the developmental phenotype of neurons within CD. Microtubule-associated protein 1B (MAP1B) and the intermediate filament (IF) protein nestin are enriched in neural progenitors, whereas MAP2B, phosphorylated and non-phosphorylated forms of medium (NFM) and high (NFH) molecular weight neurofilament (NF) proteins, as well as the light NF subunit (NFL) and IF protein alpha internexin are expressed in developing and mature neurons. Immunolabeling for internexin and MAP1B was more abundant in the most abnormally shaped neurons that populated dysplastic regions than in adjacent regions exhibiting milder cytoarchitectural abnormalities or control cortex. Nestin immunoreactivity was noted in large dysplastic and heterotopic neurons within the deeper cortical layers of CD specimens but not in normal cortex. In contrast, neurons in CD specimens also expressed cytoskeletal markers characteristic of differentiated neurons such as NF subunits and MAP2B. These findings suggest that the cytoarchitectural abnormalities in CD may reflect pathophysiological changes in the developing brain that disrupt expression of several key components of the neuronal cytoskeleton and may contribute to impaired migration of cortical neurons.
MESH: Antibodies,-Monoclonal-immunology; Biological-Markers-analysis; Cell-Differentiation-genetics; Cerebral-Cortex-abnormalities; Child-; Child,-Preschool; Epilepsy,-Partial-genetics; Epilepsy,-Partial-metabolism; Epilepsy,-Partial-pathology; Ki-67-Antigen-immunology; Nerve-Tissue-Proteins-analysis; Proliferating-Cell-Nuclear-Antigen-immunology
MESH: *Carrier-Proteins-analysis; *Cell-Movement-genetics; *Cerebral-Cortex-growth-and-development; *Cerebral-Cortex-metabolism; *Intermediate-Filament-Proteins-analysis; *Microtubule-Associated-Proteins-analysis
TG: Female; Human; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 0; 0; 0; 0; 0; 0; 0; 0; 0; 0; 0
NM: alpha-internexin; microtubule-associated-protein-1B; nestin-protein; Antibodies,-Monoclonal; Biological-Markers; Carrier-Proteins; Intermediate-Filament-Proteins; Ki-67-Antigen; Microtubule-Associated-Proteins; Nerve-Tissue-Proteins; Proliferating-Cell-Nuclear-Antigen
AN: 97338342
UD: 9710
MEDLINE EXPRESS (R) 10/97-1/98 14 of 48
TI: Transplantation of mesencephalic cell suspension in dopamine-denervated striatum of the rat. II. Effects on corticostriatal transmission.
AU: Capozzo-A; Florio-T; Di-Loreto-S; Adorno-D; Scarnati-E
AD: Department of Biomedical Technology, School of Medicine, University of L'Aquila, Italy.
SO: Exp-Neurol. 1997 Jul; 146(1): 142-50
ISSN: 0014-4886
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: The present study has been designed to investigate whether intrastriatal implantation of mesencephalic dopamine (DA)-synthetizing neurons into the striatum (ST) of rats whose substantia nigra (SN) was previously destroyed by 6-hydroxydopamine (6-OHDA) restores the pattern of corticostriatal transmission from the medial prelimbic and sensorimotor cortices. In 6-month-old normal animals electrical stimulation of these two functionally unrelated cortices evoked a short latency and brief excitation in 81.6% of neurons recorded in the dorsolateral ST. This percentage decreased significantly (70.6%) in age-matched animals whose dopaminergic nigrostriatal pathway was unilaterally destroyed by 6-OHDA 3 months before recording. However a significant increase in neurons (36.9%) which could be simultaneously activated from the two cortices in comparison to intact rats was noted. In addition the lesion caused a significant decrease in the threshold current required to evoke activation of striatal neurons from the sensorimotor cortex. The increase in the number of striatal neurons responding simultaneously to cortical stimulations demonstrates that destruction of the dopaminergic nigrostriatal pathway causes a loss of the focusing action of DA on corticostriatal transmission. Transplantation of embryonic mesencephalic neurons appears to reestablish this action since the number of convergent responses was significantly decreased in grafted animals (23.5%) in comparison to denervated (36.9%) and sham-grafted (35.1%) animals. Furthermore, the grafts showed a trend to increase current intensities required to evoke activation of striatal cells from both cortices. The action of grafted mesencephalic neurons over prelimbic and sensorimotor cortical inputs to the dorsal ST could be involved in recovery of grafted animals in the correct execution of complex sensorimotor tasks requiring integration of different cortical signals within the ST.
MESH: Denervation-; Dopamine-physiology; Neurons-transplantation; Oxidopamine-; Rats-; Rats,-Sprague-Dawley; Transplantation,-Heterotopic
MESH: *Brain-Tissue-Transplantation-physiology; *Cerebral-Cortex-physiology; *Corpus-Striatum-physiology; *Fetal-Tissue-Transplantation-physiology; *Mesencephalon-physiology; *Mesencephalon-transplantation; *Neurons-physiology; *Synaptic-Transmission
TG: Animal; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 1199-18-4; 51-61-6
NM: Oxidopamine; Dopamine
AN: 97369259
UD: 9710
MEDLINE EXPRESS (R) 1/97-9/97 15 of 48
TI: Function and organization in dysgenic cortex. Case report.
AU: Preul-MC; Leblanc-R; Cendes-F; Dubeau-F; Reutens-D; Spreafico-R; Battaglia-G; Avoli-M; Langevin-P; Arnold-DL; Villemure-JG
AD: McConnell Brain Imaging Centre, Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University, Quebec, Canada.
SO: J-Neurosurg. 1997 Jul; 87(1): 113-21
ISSN: 0022-3085
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: Cerebral dysgenesis is a subject of interest because of its relationship to cerebral development and dysfunction and to epilepsy. The authors present a detailed study of a 16-year-old boy who underwent surgery for a severe seizure disorder. This patient had dysgenesis of the right hemisphere, which was composed of a giant central frontoparietal nodular gray matter heterotopia with overlying large islands of cortical dysplasia around a displaced central fissure. Exceptional insight into the function, biochemistry, electrophysiology, and histological structure of this lesion was obtained from neurological studies that revealed complementary information: magnetic resonance (MR) imaging, [18]fluoro-2-deoxy-D-glucose positron emission tomography (PET), functional PET scanning, proton MR spectroscopic (1H-MRS) imaging, intraoperative cortical mapping and electrocorticography, in vitro electrophysiology, and immunocytochemistry. These studies demonstrated compensatory cortical reorganization and showed that large areas of heterotopia and cortical dysplasia in the central area may retain normal motor and sensory function despite strikingly altered cytoarchitectonic organization and neuronal metabolism. Such lesions necessitate appropriate functional imaging studies prior to surgery and cortical mapping to avoid creating neurological deficits. Integrated studies, such as PET, 1H-MRS imaging, cortical mapping, immunocytochemistry, and electrophysiology may provide information on the function of developmental disorders of cerebral organization.
MESH: Adolescence-; Brain-surgery; Brain-Diseases-complications; Brain-Diseases-diagnosis; Brain-Mapping; Choristoma-complications; Choristoma-diagnosis; Electroencephalography-; Epilepsy-diagnosis; Epilepsy-etiology; Evoked-Potentials,-Somatosensory; Immunohistochemistry-; Intraoperative-Period; Neurosurgery-; Nuclear-Magnetic-Resonance; Periaqueductal-Gray; Tomography,-Emission-Computed
MESH: *Brain-abnormalities; *Brain-physiopathology
TG: Case-Report; Human; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
AN: 97345785
UD: 9709
SB: AIM
MEDLINE EXPRESS (R) 1/97-9/97 16 of 48
TI: Periventricular nodular heterotopia and intractable temporal lobe epilepsy: poor outcome after temporal lobe resection.
AU: Li-LM; Dubeau-F; Andermann-F; Fish-DR; Watson-C; Cascino-GD; Berkovic-SF; Moran-N; Duncan-JS; Olivier-A; Leblanc-R; Harkness-W
AD: Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, Quebec, Canada.
SO: Ann-Neurol. 1997 May; 41(5): 662-8
ISSN: 0364-5134
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: We describe 5 women and 5 men with periventricular nodular heterotopia and electroclinical features suggestive of temporal lobe epilepsy, who were surgically treated for control of medically refractory seizures. Magnetic resonance imaging revealed bilateral periventricular nodular heterotopia in 7 of the 10 patients. Because of the lack of clear localization, 6 patients were studied with intracranial depth electrode recordings. Seizures were of hippocampal onset (3 patients), regional temporal lobe onset (2 patients), or occipital-temporal onset (1 patient). Anterior temporal lobectomy was performed in 6 patients; selective amygdalohippocampectomy, in 1; and anterior temporal lobectomy plus resection of the heterotopic tissue, in 3. None of the 9 patients followed for more than 12 months postoperatively were seizure free. Two patients were initially seizure free for approximately 18 months, but then seizures recurred. One patient had a major reduction in seizure frequency at a 39-month follow-up after most of the unilateral heterotopic tissue was included in the temporal resection. Temporal resection did not lead to a long-term favorable outcome in this group of patients with periventricular nodular heterotopia and epileptogenic discharges involving the temporal lobe. This suggests a more widespread disorder with epileptogenic activity possibly originating in or near the heterotopic tissue. The clinical and electrographic features of periventricular nodular heterotopia pointing to temporal lobe origin are misleading and temporal resection does not result in long-term cessation of seizures.
MESH: Adult-; Amygdaloid-Body-surgery; Brain-Diseases-pathology; Choristoma-pathology; Follow-Up-Studies; Gliosis-; Hippocampus-pathology; Hippocampus-surgery; Treatment-Outcome
MESH: *Brain-Diseases-surgery; *Choristoma-surgery; *Epilepsy,-Temporal-Lobe-surgery; *Temporal-Lobe
TG: Female; Human; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
AN: 97297897
UD: 9708
MEDLINE EXPRESS (R) 1/97-9/97 17 of 48
TI: Spatial and temporal development of the gliovascular tissue in type II lissencephaly.
AU: Bornemann-A; Aigner-T; Kirchner-T
AD: Institute of Pathology, University of Erlangen, Germany.
SO: Acta-Neuropathol-Berl. 1997 Feb; 93(2): 173-7
ISSN: 0001-6322
PY: 1997
LA: ENGLISH
CP: GERMANY
AB: Type II lissencephaly is a complex cortical malformation in which mesenchymal and central nervous components are intermingled. It is generally believed that the histological pattern is created by migration of heterotopic neuroblasts into the leptomeninges through defects in the superficial basement membrane. Defects of the extracellular matrix have been suggested to be the primary cause of type II lissencephaly. To elucidate the underlying pathogenetic mechanisms, we immunostained extracellular matrix and basement membrane components of the cerebral cortex from six fetal and two infantile brains. We found that the pattern of collagen subtypes I, III and VI was not altered in type II lissencephaly brains when compared to normal controls. As to the pathogenesis of type II lissencephaly, a polymicrogyria-like pattern is created, which results in considerable cortical enlargement. The microgyri do not fuse but remain separated from each other by gliovascular tissue, i.e., leptomeninges which contain astrocytes. At the interface between the enlarged brain surface and the gliovascular tissue, neuronal migration takes place through gaps in the external basement membrane. Thus, the cortical dysplasia encountered in type II lissencephaly is only due to a limited amount to neuronal heterotopia in the leptomeninges.
MESH: Astrocytes-pathology; Basement-Membrane-pathology; Cerebral-Cortex-embryology; Cerebral-Cortex-pathology; Extracellular-Matrix-pathology; Infant-
MESH: *Cerebral-Cortex-abnormalities; *Cerebral-Cortex-blood-supply; *Fetal-Development; *Neuroglia-pathology
TG: Human
PT: JOURNAL-ARTICLE
AN: 97191507
UD: 9708
MEDLINE EXPRESS (R) 1/97-9/97 18 of 48
TI: Bilateral periventricular nodular heterotopia. PET and MRI of a patient with focal seizures.
AU: Haase-CG; Masur-H; Matheja-P; Kuwert-T; Schober-O
AD: Department of Neurology, University Hospital of Munster, Germany.
SO: Clin-Nucl-Med. 1997 Feb; 22(2): 119-20
ISSN: 0363-9762
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
MESH: Adult-; Brain-Diseases-diagnosis; Brain-Diseases-radionuclide-imaging; Cerebral-Ventricles-pathology; Choristoma-diagnosis; Epilepsy,-Partial-diagnosis
MESH: *Cerebral-Cortex; *Cerebral-Ventricles-radionuclide-imaging; *Choristoma-radionuclide-imaging; *Epilepsy,-Partial-radionuclide-imaging; *Magnetic-Resonance-Imaging; *Tomography,-Emission-Computed
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 97183811
UD: 9708
MEDLINE EXPRESS (R) 1/97-9/97 19 of 48
TI: Immunohistochemical expression of cell adhesion molecule L1 in hemimegalencephaly.
AU: Tsuru-A; Mizuguchi-M; Uyemura-K; Becker-LE; Takashima-S
AD: Department of Mental Retardation and Birth Defect Research, National Institute of Neuroscience; NCNP, Tokyo, Japan.
SO: Pediatr-Neurol. 1997 Jan; 16(1): 45-9
ISSN: 0887-8994
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: We demonstrated immunohistochemically an abnormal expression of the neural cell adhesion molecule L1 in 10 developing brains of children with hemimegalencephaly (HM) aged from 36 weeks gestation to 10 years of age, comparing them with 23 controls aged from 13 weeks of gestation to 14 years. There was dense L1 expression in focal regions of the molecular layer beneath leptomeningeal glioneuronal heterotopia, in areas of cerebral cortex with large neurons, and in the disorganized or neuronal heterotopic sites in the white matter in HM. L1 was also heterogeneously enhanced in the abnormal cortex after 1 year of age, suggesting that axonal growth was delayed. These changes persisted into the older age group in the abnormal areas of cortex in HM. The cell bodies of many enlarged neurons in HM were immunopositive for L1, whereas L1 was usually localized to the processes of normal neurons. The delayed L1 immunoreactivity and enlarged L1-immunopositive neurons may be closely related to the pathogenesis of unilateral megalencephaly with cortical dysplasia and heterotopia.
MESH: Adolescence-; Brain-pathology; Brain-Diseases-pathology; Cell-Movement-genetics; Cell-Movement-physiology; Cerebral-Cortex-abnormalities; Cerebral-Cortex-pathology; Child-; Child,-Preschool; Choristoma-genetics; Choristoma-pathology; Dominance,-Cerebral-physiology; Gene-Expression-physiology; Immunoenzyme-Techniques; Infant-; Infant,-Newborn; Neuroglia-pathology; Neurons-pathology; Pregnancy-
MESH: *Brain-abnormalities; *Brain-Diseases-genetics; *Dominance,-Cerebral-genetics; *NCAM-genetics
TG: Female; Human; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 0; 0
NM: L1-antigen; NCAM
AN: 97197341
UD: 9707
MEDLINE EXPRESS (R) 1/97-9/97 20 of 48
TI: Proton spectroscopic imaging at 4.1 tesla in patients with malformations of cortical development and epilepsy.
AU: Kuzniecky-R; Hetherington-H; Pan-J; Hugg-J; Palmer-C; Gilliam-F; Faught-E; Morawetz-R
AD: UAB Epilepsy Center, University of Alabama at Birmingham 35294, USA.
SO: Neurology. 1997 Apr; 48(4): 1018-24
ISSN: 0028-3878
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: We used proton magnetic resonance spectroscopic imaging (MRSI) at 4.1 tesla in patients with malformations of cortical development (MCDs) and epilepsy. We compared the spectroscopic results with normative data using 2 SDs (95% confidence) above normal values for detection of significant abnormalities for creatine-N-acetylated compounds (Cr/NA) ratio and choline-N-acetylated compounds (Cho/NA). The results were correlated with clinical, EEG, and histologic findings. Patients with focal cortical dysplasia showed significant metabolic abnormalities in correspondence with the structural lesions, whereas patients with heterotopia and polymicrogyria demonstrated no subcortical MRSI abnormalities. Significant correlations were found between the metabolic abnormalities and the frequency of seizures but not with the degree of interictal EEG discharges. Quantitative neuronal and glial cell counts revealed no statistically significant correlation between cell loss and the abnormal metabolic ratios in those who underwent surgery. These preliminary findings suggest that MRSI-based metabolic abnormalities in patients with MCDs are variable and are likely to be associated with complex cellular mechanisms involving the regulation of NA, total Cr content, and Cho.
MESH: Acetylation-; Adolescence-; Adult-; Brain-abnormalities; Brain-metabolism; Brain-Neoplasms-diagnosis; Choline-metabolism; Choristoma-diagnosis; Creatine-metabolism; Electroencephalography-; Magnetic-Resonance-Imaging; Middle-Age; Protons-; Reference-Values
MESH: *Cerebral-Cortex-abnormalities; *Cerebral-Cortex-pathology; *Epilepsy-diagnosis; *Nuclear-Magnetic-Resonance
TG: Female; Human; Male; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
CN: RO1NS33919NSNINDS; RO1NS33991NSNINDS; RR07723RRNCRR
RN: 0; 57-00-1; 62-49-7
NM: Protons; Creatine; Choline
AN: 97264021
UD: 9707
SB: AIM
MEDLINE EXPRESS (R) 1/97-9/97 21 of 48
TI: Decreased senile plaque density in Alzheimer neocortex adjacent to an omental transposition.
AU: Relkin-NR; Edgar-MA; Gouras-GK; Gandy-SE; Goldsmith-HS
AD: Department of Neurology and Neuroscience, Cornell University Medical Center, New York, NY 10021, USA.
SO: Neurol-Res. 1996 Aug; 18(4): 291-4; discussion 295-6
ISSN: 0161-6412
PY: 1996
LA: ENGLISH
CP: ENGLAND
AB: Post-mortem studies of the brain of an Alzheimer patient indicate fewer senile plaques in the crests of cortical gyri underneath an omental transposition than in neighboring cortical areas.
MESH: Alzheimer's-Disease-surgery; Amyloid-beta-Protein-metabolism; Astrocytes-metabolism; Astrocytes-pathology; Atrophy-; Cell-Hypoxia; Cerebrovascular-Circulation; Hemosiderin-analysis; Iron-metabolism; Occipital-Lobe-blood-supply; Parietal-Lobe-blood-supply
MESH: *Alzheimer's-Disease-pathology; *Occipital-Lobe-pathology; *Omentum-transplantation; *Parietal-Lobe-pathology; *Transplantation,-Heterotopic
TG: Animal; Case-Report; Human; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 0; 7439-89-6; 9011-92-1
NM: Amyloid-beta-Protein; Iron; Hemosiderin
AN: 97029433
UD: 9707
MEDLINE EXPRESS (R) 1/97-9/97 22 of 48
TI: Dominant X linked subcortical laminar heterotopia and lissencephaly syndrome (XSCLH/LIS): evidence for the occurrence of mutation in males and mapping of a potential locus in Xq22.
AU: des-Portes-V; Pinard-JM; Smadja-D; Motte-J; Boespflug-Tanguy-O; Moutard-ML; Desguerre-I; Billuart-P; Carrie-A; Bienvenu-T; Vinet-MC; Bachner-L; Beldjord-C; Dulac-O; Kahn-A; Ponsot-G; Chelly-J
AD: INSERM U129-ICGM, Faculte de Medecine Cochin, Paris, France.
SO: J-Med-Genet. 1997 Mar; 34(3): 177-83
ISSN: 0022-2593
PY: 1997
LA: ENGLISH
CP: ENGLAND
AB: X linked subcortical laminar heterotopia and lissencephaly syndrome (XSCLH/ LIS) is an intriguing disorder of cortical development, which causes classical lissencephaly with severe mental retardation and epilepsy in hemizygous males, and subcortical laminar heterotopia (SCLH) associated with milder mental retardation and epilepsy in heterozygous females. Here we report an exclusion mapping study carried out in three unrelated previously described families in which males are affected with lissencephaly and females with SCLH, using 38 microsatellite markers evenly distributed on the X chromosome. Most of the X chromosome was excluded and potential intervals of assignment in Xq22.3-q23 or in Xq27 are reported. Although the number of informative meioses did not allow a decision between these two loci, it is worth noting that the former interval is compatible with the mapping of a breakpoint involved in a de novo X;autosomal balanced translocation 46,XX,t(X;2)(q22;p25) previously described in a female with classical lissencephaly. In addition, haplotype inheritance in two families showed a grandpaternal origin of the mutation and suggested in one family the presence of mosaicism in germline cells of normal transmitting males.
MESH: Adult-; Child-; Child,-Preschool; Chromosome-Mapping; Genes,-Dominant; Haplotypes-; Linkage-Genetics; Microsatellite-Repeats; Mutation-; Pedigree-; Syndrome-
MESH: *Cerebral-Cortex-abnormalities; *Epilepsy-genetics; *Mental-Retardation-genetics; *Sex-Chromosome-Abnormalities-genetics; *X-Chromosome-genetics
TG: Case-Report; Female; Human; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
AN: 97227381
UD: 9707
MEDLINE EXPRESS (R) 1/97-9/97 23 of 48
TI: Endometrial pathology and infertility.
AU: Ruiz-Velasco-V; Gonzalez-Alfani-G; Pliego-Sanchez-L; Alamillo-Vera-M
AD: Universidad Nacional Autonoma de Mexico, Mexico City, Mexico.
SO: Fertil-Steril. 1997 Apr; 67(4): 687-92
ISSN: 0015-0282
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: OBJECTIVE: To call attention to endometrial pathologies, which in addition to causing menstrual problems, are a cause of infertility. DESIGN: Controlled clinical study. SETTING: Specialized unit in the management of infertile patients. PATIENT(S): Fifteen infertile women between the ages of 26 and 39 years and suffering infertility from endometrial problems for a period of 4 to 18 years were included in the study. Six patients had primary infertility and nine others had secondary infertility. INTERVENTION(S): Once the endometrial pathology was diagnosed, treatment was initiated according to the type of problem: hysteroscopy, curettage, and hormonal replacement with or without corticoids or antiphymic drugs. MAIN OUTCOME MEASURE(S): Clinical studies, laboratory tests, hormonal serum levels, and endoscopy. RESULT(S): After initiating specific treatment for each of the pathologies, menstruation was re-established in 14 of 15 patients. Nine patients became pregnant (8 of 10 cases with bone, squamous cell, or muscular metaplasia). CONCLUSION(S): Pathological changes of the endometrium are causes of infertility. These problems are not as rare as thought. They must be searched for carefully and diagnosed promptly. The majority carry a good prognosis when adequately treated.
MESH: Adult-; Amenorrhea-diagnosis; Amenorrhea-pathology; Amenorrhea-therapy; Cohort-Studies; Endometrial-Neoplasms-diagnosis; Endometrial-Neoplasms-pathology; Endometrial-Neoplasms-therapy; Endometrium-ultrasonography; Infertility,-Female-diagnosis; Infertility,-Female-therapy; Metaplasia-pathology; Ossification,-Heterotopic-diagnosis; Ossification,-Heterotopic-pathology; Ossification,-Heterotopic-therapy; Treatment-Outcome; Tuberculosis,-Female-Genital-diagnosis; Tuberculosis,-Female-Genital-pathology; Tuberculosis,-Female-Genital-therapy
MESH: *Endometrium-pathology; *Infertility,-Female-etiology
TG: Female; Human
PT: JOURNAL-ARTICLE
AN: 97247061
UD: 9707
MEDLINE EXPRESS (R) 1/97-9/97 24 of 48
TI: Hemimegalencephaly--morphological and immunocytochemical study.
AU: Yasha-TC; Santosh-V; Das-S; Shankar-SK
AD: Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, India.
SO: Clin-Neuropathol. 1997 Jan-Feb; 16(1): 17-22
ISSN: 0722-5091
PY: 1997
LA: ENGLISH
CP: GERMANY
AB: Hemimegalencephaly (HME), a rare congenital abnormality characterized by unilateral enlargement of the cerebral hemisphere, is one of the less common causes of intractable seizures. We report a 6-month-old infant with uncontrolled seizures who was diagnosed to have a large mass lesion based on a CT scan. Postmortem examination revealed left-sided HME with pachygyria, widened cortex, indistinct grey-white junction, and distorted deep nuclear masses. Histological features included loss of cortical lamination, large atypical neurons with argyrophilic accumulations, ballooned cells, neuronal heterotopia, and astrocytosis with dystrophic calcification. The heterotopic neurons in the white matter were present in a radial pattern suggestive of aberrant neuronal migration. Several large neurons were dystrophic with cytoskeletal abnormalities like phosphorylated high molecular weight neurofilament and ubiquitin in the cytoplasm. However, typical neurofibrillary tangles with Congo red and tau positivity were not observed. Synaptophysin labelling was found to be decreased in the cortex, but some of the abnormal neurons had dense perisomatic label. The majority of the balloon cells were astrocytic in origin, being positive for glial fibrillary acidic protein and negative for the neuronal markers. Although the etiology of HME is not known, it provides an opportunity to study anomalous development of the brain and neuronal developmental abnormalities.
MESH: Astrocytes-pathology; Cerebral-Cortex-pathology; Choristoma-pathology; Diagnosis,-Differential; Immunoenzyme-Techniques; Infant-; Neurofilament-Proteins-analysis; Neurons-pathology; Synaptophysin-analysis; Ubiquitin-analysis
MESH: *Cerebral-Cortex-abnormalities; *Dominance,-Cerebral-physiology; *Nerve-Tissue-Proteins-analysis; *Spasms,-Infantile-pathology
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
RN: 0; 0; 0; 0
NM: Nerve-Tissue-Proteins; Neurofilament-Proteins; Synaptophysin; Ubiquitin
AN: 97172305
UD: 9707
MEDLINE EXPRESS (R) 1/97-9/97 25 of 48
TI: [Pathogenesis of the neuronal migration disorder, with special reference to the animal model of prenatal exposure to low-dose ionizing radiation]
AU: Fushiki-S
AD: Department of Dynamic Pathology, Kyoto Prefectural University of Medicine.
SO: No-To-Hattatsu. 1997 Mar; 29(2): 102-7
ISSN: 0029-0831
PY: 1997
LA: JAPANESE; NON-ENGLISH
CP: JAPAN
AB: Cortical malformations such as cortical dysplasia and heterotopia constitute the underlying pathology of epilepsy and mental retardation. It is thus important to elucidate the pathogenesis of these migration disorders from the neuropathological viewpoint based upon animal experiments. In this review, I describe the experiment in which low-dose prenatal X-or gamma-irradiation was performed at the mid-gestational period of mice or rats. Low-dose irradiation as low as 150 mGy induced decelerated migration of cortical neurons during the embryonic period together with a changed pattern of cell adhesion molecule, N-CAM. In addition, the effect of radiation remained at least up until 3-week postnatal as disorganized neuronal allocation with respect to the birthdate. With time of further maturation in the neocortex, however, the architecture, in terms of the pattern of distribution of the labeled neurons, returned closely to that found in non-irradiated control animals. Considering the fact that the number of labeled cells per unit cortical area decreased considerably from 3-week to 8-week postnatal, it is conceivable that apoptotic cell death might have occurred in aberrantly placed neurons. Recent progress of molecular genetical approach to human hereditary neurodevelopmental diseases is briefly reviewed, since it greatly contributes to our understanding on the pathogenesis of neuronal migration disorders.
MESH: English-Abstract; Mice-; Radiation,-Ionizing; Rats-
MESH: *Cerebral-Cortex-abnormalities; *Embryo-radiation-effects
TG: Animal; Human; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE; REVIEW; REVIEW-LITERATURE
AN: 97224803
UD: 9707
MEDLINE EXPRESS (R) 1/97-9/97 26 of 48
TI: Hemimegalencephaly. Histological, immunohistochemical, ultrastructural and cytofluorimetric study of six patients.
AU: Bosman-C; Boldrini-R; Dimitri-L; Di-Rocco-C; Corsi-A
AD: Dipartimento di Medicina Sperimentale e Patologia, Universita La Sapienza, Policlinico Umberto I, Rome, Italy.
SO: Childs-Nerv-Syst. 1996 Dec; 12(12): 765-75
ISSN: 0256-7040
PY: 1996
LA: ENGLISH
CP: GERMANY
AB: Hemimegalencephaly (HME) is an uncommon sporadic nonfamilial congenital dysplastic abnormality of the central nervous system, characterized by enlargement of one cerebral hemisphere, with cranial asymmetry, hemiparesis, epilepsy, and mental retardation. It can occur in isolation or associated with various anomalies, namely skin disorders. The main neuropathologic findings are hemispheric gigantism, macro- and/or micropolygyria, cortical thickening with lack of lamination, blurred boundaries of the gray and white matter, and large ortho- and heterotopic neural cells. The results obtained by morphological investigations carried out on six patients with HME, compared with the findings recorded in similar studies performed on one patient with tuberous sclerosis (TS) and another with pachygyria, allow the authors to (a) confirm the dysplastic nature of HME and its autonomy from TS; (b) demonstrate that ortho- and heterotopic neuronal cells do not differentiate completely during proliferation and migration from the germinal matrix; (c) document, by means of flow cytometric study, a normal euploid DNA content in the enlarged hemisphere, consequently ruling out heteroploidy as a cause of both cell "hypertrophy" and enlargement of the malformed cerebral hemisphere.
MESH: Brain-surgery; Cell-Division-physiology; Cell-Movement-physiology; Cerebral-Cortex-abnormalities; Cerebral-Cortex-pathology; Cerebral-Cortex-surgery; Child-; Child,-Preschool; Diagnosis,-Differential; Dominance,-Cerebral; Flow-Cytometry; Immunoenzyme-Techniques; Infant-; Infant,-Newborn; Mental-Retardation-pathology; Microscopy,-Electron; Neurons-pathology; Tuberous-Sclerosis-pathology
MESH: *Brain-abnormalities; *Brain-pathology
TG: Female; Human; Male
PT: JOURNAL-ARTICLE
AN: 97159790
UD: 9706
MEDLINE EXPRESS (R) 1/97-9/97 27 of 48
TI: Neuronal heterotopias in the developing cerebral cortex produced by neurotrophin-4.
AU: Brunstrom-JE; Gray-Swain-MR; Osborne-PA; Pearlman-AL
AD: Department of Neurology, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
SO: Neuron. 1997 Mar; 18(3): 505-17
ISSN: 0896-6273
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: The marginal zone (MZ) of embryonic neocortex is crucial to its normal development. We report that neurotrophin-4 (but not NT3 or NGF), applied to embryonic rodent cortex in vitro or in vivo, produces heterotopic accumulations of neurons in the MZ. Although heterotopia production is TrkB mediated, BDNF is >10-fold less effective than NT4. Heterotopic neurons have the same birth date and phenotype as normal MZ neurons; they are not the result of NT4-induced proliferation or rescue from apoptosis. We suggest that NT4 causes excess neurons to migrate into the MZ and thus may play a role in normal MZ formation as well as in the pathogenesis of certain human cortical dysplasias.
MESH: Apoptosis-drug-effects; Brain-Derived-Neurotrophic-Factor-pharmacology; Brain-Derived-Neurotrophic-Factor-toxicity; Cell-Division-drug-effects; Cell-Lineage; Cell-Movement-drug-effects; Cerebral-Cortex-embryology; Cerebral-Cortex-pathology; Choristoma-embryology; Choristoma-pathology; Injections,-Intraventricular; Mice-; Mice,-Inbred-C3H; Mice,-Knockout; Morphogenesis-drug-effects; Nerve-Growth-Factors-toxicity; Nerve-Tissue-Proteins-drug-effects; Nerve-Tissue-Proteins-physiology; Organ-Culture; Rats-; Rats,-Sprague-Dawley; Receptors,-Nerve-Growth-Factor-drug-effects; Receptors,-Nerve-Growth-Factor-genetics; Receptors,-Nerve-Growth-Factor-physiology
MESH: *Abnormalities,-Drug-Induced-pathology; *Cerebral-Cortex-drug-effects; *Choristoma-chemically-induced; *Nerve-Growth-Factors-pharmacology; *Neurons-drug-effects
TG: Animal; Human; Support,-Non-U.S.-Gov't; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
RN: 0; 0; 0; 0; 0; 143551-63-7
NM: ciliary-neurotrophic-factor-receptor; Brain-Derived-Neurotrophic-Factor; Nerve-Growth-Factors; Nerve-Tissue-Proteins; Receptors,-Nerve-Growth-Factor; neurotrophin-4
AN: 97234729
UD: 9706
MEDLINE EXPRESS (R) 1/97-9/97 28 of 48
TI: Developmental spectrum of the excitotoxic cascade induced by ibotenate: a model of hypoxic insults in fetuses and neonates.
AU: Gressens-P; Marret-S; Evrard-P
AD: Service de Neurologie Pediatrique, Hopital Robert Debre, Faculte de Medecine Xavier-Bichat, Paris, France.
SO: Neuropathol-Appl-Neurobiol. 1996 Dec; 22(6): 498-502
ISSN: 0305-1846
PY: 1996
LA: ENGLISH
CP: ENGLAND
AB: Ibotenate, a glutamatergic agonist, was used to study the spectrum of excitoxic disturbances at different ages of cerebral development. Cultures of whole mouse embryo were submitted to ibotenate at E8 for 20 h: during the phase of early premigratory differentiation: ibotenate did not induce any detectable histological lesion. During migration of supragranular neurons, newborn hamsters intracerebrally injected at PO with ibotenate display neuronal migration disorders graded from nodular heterotopias to extensive laminar heterotopias mimicking some aspects of lissencephalic and double-cortex syndromes. After completion of neuronal layer V, PO mice injected with ibotenate exhibit laminar neuronal depopulation of layer V-VIa mimicking human microgyria. At P5 in mouse, after completion of neuronal migration of the cortical plate, ibotenate induces neuronal loss in all cortical layers and the formation of porencephalic cysts. This study emphasizes the dramatic role played by glutamate in brain development, in the occurrence of neuronal migration disorders in the cortex, and in grey and white matter damage.
MESH: Cerebral-Ischemia-chemically-induced; Disease-Models,-Animal; Hamsters-; Mice-
MESH: *Animals,-Newborn-physiology; *Anoxia-physiopathology; *Cerebral-Ischemia-pathology; *Embryo-drug-effects; *Ibotenic-Acid-pharmacology; *Receptors,-N-Methyl-D-Aspartate-agonists
TG: Animal
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
RN: 0; 2552-55-8
NM: Receptors,-N-Methyl-D-Aspartate; Ibotenic-Acid
AN: 97157957
UD: 9706
MEDLINE EXPRESS (R) 1/97-9/97 29 of 48
TI: Characterization of neuronal migration disorders in neocortical structures: I. Expression of epileptiform activity in an animal model.
AU: Luhmann-HJ; Raabe-K
AD: Institute of Physiology II, Department of Neurophysiology, Dusseldorf, Germany. luhmann@uni-duesseldorf.de
SO: Epilepsy-Res. 1996 Dec; 26(1): 67-74
ISSN: 0920-1211
PY: 1996
LA: ENGLISH
CP: NETHERLANDS
AB: Hypoxia, ischemia and other forms of brain injury during the pre- and perinatal period may cause neuronal migration disorders which results in irreversible structural modifications. In human neocortex, these malformations have been associated with severe mental retardation, motor dysfunction and the manifestation of therapy-resistant epilepsy. We were interested in analyzing the expression of epileptiform activity in an animal model of neocortical migration disorders. Newborn rats received a focal freeze lesion and were investigated anatomically and in vitro electrophysiologically after survival times of up to five months. Anatomic abnormalities included loss of normal cortical lamination (focal microgyrus) and presence of ectopic cell clusters in layer I and in the white matter (heterotopia). The functional in vitro analyses with eight extracellular recording electrodes revealed a prominent hyperexcitability of the disorganized neocortical network. Electrical stimulation of the afferents elicited epileptiform responses that propagated over > 4 mm in the horizontal direction. In untreated and sham-operated animals, this spread of evoked activity was restricted to 0.5-1 mm. Epileptiform responses were not significantly affected by APV but blocked by NBQX, indicating that AMPA receptors play a prominent role in the generation and propagation of this pathophysiological activity. Our data suggest that the experimentally induced migration disturbances cause long-term structural and/or functional modifications in the neocortical network which may form the basis for the expression of epileptiform activity.
MESH: Animals,-Newborn; Brain-physiopathology; Cerebral-Cortex-physiopathology; Choristoma-pathology; Disease-Models,-Animal; Electroencephalography-; Electrophysiology-; Epilepsy-etiology; Epilepsy-pathology; Rats-; Rats,-Wistar
MESH: *Brain-abnormalities; *Brain-pathology; *Cerebral-Cortex-abnormalities; *Cerebral-Cortex-pathology; *Epilepsy-physiopathology
TG: Animal; Human; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
AN: 97138664
UD: 9706
MEDLINE EXPRESS (R) 1/97-9/97 30 of 48
TI: Neuronal migration disorders and epilepsy: a morphological analysis of three surgically treated patients.
AU: Battaglia-G; Arcelli-P; Granata-T; Selvaggio-M; Andermann-F; Dubeau-F; Olivier-A; Tampieri-D; Villemure-JG; Avoli-M; Avanzini-G; Spreafico-R
AD: Neurological Institute C. Besta, Milan, Italy.
SO: Epilepsy-Res. 1996 Dec; 26(1): 49-58
ISSN: 0920-1211
PY: 1996
LA: ENGLISH
CP: NETHERLANDS
AB: Despite the increasing number of patients affected by neuronal migration disorders (NMDs) recently diagnosed in vivo by means of magnetic resonance imaging (MRI), few detailed data on the correlation between the neuroradiological and the anatomical features in the single NMD case are available. The present paper reports a combined cytoarchitectural and immunocytochemical analysis, by means of antisera recognizing specific neuronal and glial markers, of three MRI diagnosed NMD patients surgically treated for the relief of intractable seizures. The first case was a giant subcortical nodular heterotopia of morphologically normal neurons lacking any type of cortical lamination. The second case was a layered polymicrogyria with an abnormal amount of ectopic neurons in the underlying white matter. The third case was a focal cortical dysplasia characterized by a dramatic disruption of the normal cortical layering associated with marked cytological abnormalities. The present data demonstrate that the macroscopical and microscopical brain abnormalities can be markedly different in different NMD subtypes, and suggest that different anatomical substrates can underlie the intrinsic hyperexcitability of these brain malformations. The relevance of further prospective clinico-morphological studies for a better understanding of the mechanisms determining the development of these brain malformations is underlined.
MESH: Brain-pathology; Brain-surgery; Choristoma-pathology; Epilepsy-pathology; Immune-Sera; Immunohistochemistry-; Magnetic-Resonance-Imaging; Neuroglia-pathology; Neurons-pathology
MESH: *Brain-abnormalities; *Epilepsy-surgery
TG: Case-Report; Female; Human; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 0
NM: Immune-Sera
AN: 97138662
UD: 9706
MEDLINE EXPRESS (R) 1/97-9/97 31 of 48
TI: Total elbow arthroplasty: revision with use of a non-custom semiconstrained prosthesis.
AU: King-GJ; Adams-RA; Morrey-BF
AD: Mayo Clinic, Rochester, Minnesota 55905, USA.
SO: J-Bone-Joint-Surg-Am. 1997 Mar; 79(3): 394-400
ISSN: 0021-9355
PY: 1997
LA: ENGLISH
CP: UNITED-STATES
AB: The results of revision elbow arthroplasty with use of the semiconstrained Mayo-modified Coonrad implant in forty-one patients were reviewed retrospectively. The average duration of follow-up was six years (range, two to thirteen years). At the time of the latest follow-up evaluation, thirty-eight patients were able to perform activities of daily living, one had a stiff elbow because of heterotopic ossification, one had weakness secondary to an injury of the radial nerve, and one had an unstable elbow after removal of the prosthesis because of recurrent aseptic loosening. Fourteen patients sustained either a fracture or a perforation of the cortex at the time of removal of the primary implant. Three of these patients had an injury of the radial nerve; the injury was due to extravasation of the cement from a cortical defect in two of them and was sustained during removal of the cement in one. Eight patients had an intraoperative or postoperative complication that necessitated additional operative intervention. Postoperatively, twenty-two patients had complete relief of pain and sixteen had mild discomfort. Three patients remained disabled: one, because of pain secondary to loosening of the component; one, because of a pre-existing nerve injury; and one, because of the residual effects of an intraoperative injury of the radial nerve. The average Mayo elbow performance score was 87 +/- 16 points at the latest follow-up evaluation, compared with 44 +/- 17 points preoperatively (p < 0.0001). Revision elbow arthroplasty restored function to the patients who had had a failed prosthesis without infection.
MESH: Adult-; Aged-; Elbow-Joint-physiopathology; Elbow-Joint-radiography; Follow-Up-Studies; Joint-Prosthesis-adverse-effects; Middle-Age; Postoperative-Complications; Prosthesis-Design; Range-of-Motion,-Articular; Reoperation-
MESH: *Elbow-Joint-surgery; *Joint-Prosthesis
TG: Female; Human; Male
PT: JOURNAL-ARTICLE
AN: 97223948
UD: 9706
SB: AIM
MEDLINE EXPRESS (R) 1/97-9/97 32 of 48
TI: Morphological study of the brainstem in Fukuyama type congenital muscular dystrophy.
AU: Itoh-M; Houdou-S; Kawahara-H; Ohama-E
AD: Department of Mental Retardation and Birth Defect Research, National Institute of Neuroscience, Tokyo, Japan.
SO: Pediatr-Neurol. 1996 Nov; 15(4): 327-31
ISSN: 0887-8994
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: We have observed sudden clinical death due to Fukuyama-type congenital muscular dystrophy (FCMD). In FCMD, brain abnormalities, such as polymicrogyria, leptomeningeal neuroglial heterotopia and abnormal course of the corticospinal tracts, are well known. We investigated the brainstem of 10 FCMD and 7 control cases. Among the control cases, 5 with Duchenne type muscular dystrophy died of heart failure and 2 died accidental death. In the brainstem, the catecholaminergic neurons characterized by reaction with antiserum to tyrosin hydroxylase showed notable reduction in the reticular formation, vagal nuclei, and nucleus tractus solitarius. Delays or aberrations of neural control may contribute to the pathogenesis of sudden infant death syndrome, and medullary gliosis occurs in the reticular formation of sudden infant death syndrome. The pathogenesis of neurons in the brainstem in FCMD may be similar to that in sudden infant death syndrome. These findings suggest neuronal dysfunction in the brainstem and may be related to respiratory, circulatory, or sleep-wake regulation disorders.
MESH: Adolescence-; Adult-; Catecholamines-physiology; Child-; Gliosis-pathology; Mental-Retardation-genetics; Mental-Retardation-pathology; Motor-Neurons-pathology; Muscular-Dystrophy-genetics; Neuroglia-pathology; Neurons-pathology; Solitary-Nucleus-pathology; Vagus-Nerve-pathology
MESH: *Brain-Stem-pathology; *Death,-Sudden-pathology; *Muscular-Dystrophy-pathology
TG: Female; Human; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 0
NM: Catecholamines
AN: 97127782
UD: 9706
MEDLINE EXPRESS (R) 1/97-9/97 33 of 48
TI: An unusual type of primary cerebral hemihypotrophy with signs of dysfunctional neuronal migration.
AU: Malandrini-A; Lo-Russo-F; Villanova-M; Salvestroni-R; Sicurelli-F; Salvadori-C; Paolozzi-C; Guazzi-GC
AD: Laboratory of Neuropathology, University of Siena, Italy.
SO: Acta-Neuropathol-Berl. 1996 Dec; 92(6): 631-4
ISSN: 0001-6322
PY: 1996
LA: ENGLISH
CP: GERMANY
AB: We describe the neuropathological features of a complex brain malformation characterized by cerebral hemihypotrophy with ipsilateral lissencephaly, periventricular nodular heterotopia and macrogyria. The contralateral hemisphere showed only slight alterations of the gyral pattern and a limited periventricular gray matter heterotopia. The clinical picture of the patient, who died at the age of 15 years, consisted of severe oligophrenia, intractable seizures and left hemiparesis. We discuss the nosological status of this neuronal migration disorder of apparently unknown origin.
MESH: Adolescence-; Cell-Movement; Epilepsy,-Absence-complications; Epilepsy,-Tonic-Clonic-complications; Fatal-Outcome
MESH: *Brain-abnormalities; *Brain-pathology; *Neurons-physiology
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 97119500
UD: 9705
MEDLINE EXPRESS (R) 1/97-9/97 34 of 48
TI: Neuroimaging of focal malformations of cortical development.
AU: Barkovich-AJ; Kuzniecky-RI
AD: Neuroradiology Section, University of California, San Francisco, 94143-0628, USA.
SO: J-Clin-Neurophysiol. 1996 Nov; 13(6): 481-94
ISSN: 0736-0258
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: Neuroimaging is playing an increasingly important role in the evaluation of patients with malformations of cerebral cortical development. In this review, the authors address optimal neuroimaging of cortical malformations using x-ray computed tomography, single-photon-emission computed tomography, positron emission tomography, magnetic resonance imaging, and magnetic resonance spectroscopy. Initially, the authors discuss the strengths and weaknesses of the various imaging techniques. This is followed by a discussion of the clinical and neuroimaging characteristics of several different imaging manifestations of focal malformations of cortical development, including polymicrogyria, focal subcortical heterotopia, schizencephaly, focally thickened gyri, focally irregular gyri, hemimegalencephaly, and transmural dysplasia. The authors intend that, after reading this review, the reader will have a better understanding of the optimal neuroimaging techniques for evaluating these malformations and their many neuroimaging appearances.
MESH: *Cerebral-Cortex-abnormalities; *Cerebral-Cortex-physiopathology; *Magnetic-Resonance-Imaging; *Tomography,-Emission-Computed; *Tomography,-Emission-Computed,-Single-Photon; *Tomography,-X-Ray-Computed
TG: Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 97133223
UD: 9705
MEDLINE EXPRESS (R) 1/97-9/97 35 of 48
TI: [Spectroscopic magnetic resonance in hemimegalencephalus]
TO: Resonancia magnetica espectroscopica en la hemimegalencefalia.
AU: Martinez-Bermejo-A; Pascual-Castroviejo-I; Garcia-Segura-JM; Viano-J; Lopez-Martin-V; Martinez-Fernandez-V; Arcas-J; Tendero-A; Gutierrez-M
AD: Departamento de Neurorradiologia, Clinica Ntra. Sra. del Rosario, Madrid, Espana.
SO: Rev-Neurol. 1996 Dec; 24(136): 1548-51
ISSN: 0210-0010
PY: 1996
LA: SPANISH; NON-ENGLISH
CP: SPAIN
AB: INTRODUCTION: Hemimegalencephalus (HM) is a disorder of cerebral migration characterized by the overdevelopment of one cerebral hemisphere. It is usually associated with pachygyria, gliosis and neurone loss. We present a study using stereoscopic magnetic resonance (SMR) in a case of HM confirmed by the pathologist. CLINICAL CASE: A girl with right HM had hemigeneralized crisis since birth. A selective right temporoccipital cortectomy was done when she was two and a half years old. The resected piece showed thickening and absence of cortical striation, neurone loss, gliosis, giant neurones and heterotopias. After a symptom-free period the crises reappeared as right fronto-parietal epileptiform anomalies. When she was four years old SMR was done to compare this area with the corresponding area of the radiologically normal left hemisphere. Comparative study showed a marked drop in N-acetyl-aspartate (NAA), glutamate (Glu) and Gaba, and increased choline (Col) and inositol (Ino). We found no difference in the creatinine levels. CONCLUSIONS: The histological findings are in concordance with the levels of metabolites found in the affected hemisphere. The drop in NAA and Glu is related to neurone loss and the increase in glial cells, and the increase in Col and Ino with increase in membranes metabolism, as is observed in the gliosis. SMR is an advance in the identification and grading of changes seen on conventional MR, when establishing the prognosis and choice of treatment in HM.
MESH: Brain-physiopathology; Brain-surgery; Child,-Preschool; Electroencephalography-; English-Abstract; Epilepsy-diagnosis; Epilepsy-physiopathology
MESH: *Brain-abnormalities; *Laterality-; *Magnetic-Resonance-Imaging
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 97192383
UD: 9705
MEDLINE EXPRESS (R) 1/97-9/97 36 of 48
TI: Complete regeneration of bone in the baboon by recombinant human osteogenic protein-1 (hOP-1, bone morphogenetic protein-7).
AU: Ripamonti-U; Van-Den-Heever-B; Sampath-TK; Tucker-MM; Rueger-DC; Reddi-AH
AD: Bone Research Laboratory, Medical Research Council/University of the Witwatersrand, Medical School, Johannesburg, South Africa. 177RIPA@chiron.witac.za
SO: Growth-Factors. 1996; 13(3-4): 273-89,color plates III-VIII,pre.bk
ISSN: 0897-7194
PY: 1996
LA: ENGLISH
CP: SWITZERLAND
AB: We examined the efficacy of a single application of recombinant human osteogenic protein-1 (hOP-1, bone morphogenetic protein-7) for its ability to regenerate large calvarial defects in adult male baboons (Papio ursinus). Recombinant hOP-1, in conjunction with baboon or bovine guanidinium-extracted insoluble collagenous bone matrix (0.1, 0.5 and 2.5 mg per g of collagenous matrix as carrier), was implanted in 46 calvarial defects surgically prepared in 14 baboons, whilst 18 defects were implanted with the carrier matrix without hOP-1. Specimens were harvested on d 15, 30, 90 and 365 and subjected to histomorphometry on serial undecalcified sections cut at 7 microm to study the temporal sequence of tissue morphogenesis after the single application of hOP-1. Histological analysis indicated that the induction of new bone formation proceeded from the periphery to the central core of hOP-1 treated specimens after rapid angiogenesis and mesenchymal cell migration in apposition to the collagenous matrix. Whilst chondrogenesis was limited, newly formed bone has already filled with fully differentiated bone marrow elements as early as d 15, even with the 0.1 mg dose of hOP-1. On d 30 and 90, doses of 0.1 and 0.5 mg of hOP-1 showed greater amounts of bone than controls, and on d 90, they induced complete regeneration of the defects. Doses of 2.5 mg hOP-1 per g of matrix induced extensive osteogenesis initially with heterotopic ossification and displacement of the temporalis muscle above the defects. One year after implantation of hOP-1 there was restoration of the internal and external cortices of the calvaria. These results show that hOP-1 induces complete regeneration of calvarial bone in the adult primate, and suggest that the optimal activity of hOP-1 to achieve regeneration is between 100 and 500 microg of hOP-1 per g of matrix. These results in the primate may form the scientific basis for future clinical applications of hOP-1.
MESH: Cattle-; Papio-; Recombinant-Proteins-pharmacology
MESH: *Bone-Morphogenetic-Proteins-pharmacology; *Bone-Regeneration-drug-effects; *Transforming-Growth-Factor-beta-pharmacology
TG: Animal; Human; Male; Support,-Non-U.S.-Gov't; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
CN: DE1071201DENIDR
RN: 0; 0; 0; 0
NM: bone-morphogenetic-protein-7; Bone-Morphogenetic-Proteins; Recombinant-Proteins; Transforming-Growth-Factor-beta
AN: 97078909
UD: 9705
MEDLINE EXPRESS (R) 1/97-9/97 37 of 48
TI: A new family with periventricular nodular heterotopia and peculiar dysmorphic features. A probable X-linked dominant trait.
AU: Musumeci-SA; Ferri-R; Elia-M; Scuderi-C; Del-Gracco-S; Azan-G; Stefanini-MC
AD: Department of Neurology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy.
SO: Arch-Neurol. 1996 Jan; 54(1): 61-4
ISSN: 0003-9942
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: OBJECTIVE: To describe 3 sisters with brain periventricular heterotopia and peculiar dysmorphic features as a probable X-linked dominant trait. DESIGN: Clinical, laboratory, neurophysiological, and brain imaging data were studied. SETTING: Research institute for mental retardation. PATIENTS: The 3 sisters had mental retardation, drug-resistant epilepsy, gray matter heterotopia, and peculiar malformations (low nasal bridge, upslanting palpebral fissures, palpebral edema, attached hypoplastic earlobes, thickened calvaria, rectal fibrovascular polyps, urinary tract anomalies, and increased foot length). The patients were 35, 30, and 25 years old and belonged to a sibship of 6, born of nonconsanguineous healthy parents. CONCLUSION: The 3 patients constitute a well-defined clinical entity not previously described of a probable X-linked dominant nature.
MESH: Adult-; Brain-physiopathology; Brain-Diseases-pathology; Epilepsy-genetics; Linkage-Genetics; Mental-Retardation-genetics; X-Chromosome
MESH: *Brain-abnormalities; *Brain-Diseases-genetics
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 97159074
UD: 9705
SB: AIM
MEDLINE EXPRESS (R) 1/97-9/97 38 of 48
TI: Migration disorders leading to a wide spectrum of brain malformations in a case with multiorganic dysgeneses: A new syndrome?
AU: Dambska-M; Kuchna-I; Bobkiewicz-P; Wisniewski-K
AD: Laboratory of Developmental Neuropathology Medical Research Centre, Polish Academy of Sciences, Warszawa.
SO: Folia-Neuropathol. 1996; 34(1): 11-5
PY: 1996
LA: ENGLISH
CP: POLAND
AB: A case of a preterm infant who died with multiorgan, mainly cerebro-oculo-cutaneous malformations is presented. The brain dysgenesias consist of early disturbances of neuronal migration. They result on appearance of nodular subcortical heterotopias, cortical anomalies including pachy- and polymicrogyria and focal intrusion of numerous abnormally migrating nerve cells into leptomeninges. A various degree of nerve and glial cell maturity was observed within heterotopic tissue. The other malformations include eye, skin and internal organs anomalies. Similarities and differences between our case and another previously described cases were discussed but it seems difficult to include the analysed case into one of the known syndromes.
MESH: Choristoma-; Glial-Fibrillary-Acidic-Protein; Infant,-Newborn
MESH: *Brain-abnormalities; *Cell-Movement
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
RN: 0
NM: Glial-Fibrillary-Acidic-Protein
AN: 97007823
UD: 9704
MEDLINE EXPRESS (R) 1/97-9/97 39 of 48
TI: Neuronal migration disorder presenting with epilepsy: a report of five illustrative cases.
AU: Chee-MW; Chee-TS; Hui-F
AD: Department of Neurology, Singapore General Hospital.
SO: Ann-Acad-Med-Singapore. 1995 Nov; 24(6): 887-90
ISSN: 0304-4602
PY: 1995
LA: ENGLISH
CP: SINGAPORE
AB: Neuronal migration disorders are an uncommon but well-recognised cause of medically intractable epilepsy. The increasing availability of magnetic resonance imaging of the brain has made it possible to diagnose this condition ante-mortem. There are several types of migration disorders, each with different radiological and clinical features. A case each of focal cortical dysplasia, focal subcortical heterotopia, double cortex syndrome, periventricular nodular heterotopia and closed lip schizencephaly is presented to highlight the distinctive features of each entity.
MESH: Adult-; Brain-pathology; Brain-Diseases-diagnosis; Cell-Movement; Cerebral-Cortex-pathology; Cerebral-Ventricles-pathology; Choristoma-complications; Choristoma-diagnosis; Epilepsy-diagnosis; Epilepsy,-Partial-etiology; Epilepsy,-Temporal-Lobe-etiology; Epilepsy,-Tonic-Clonic-etiology; Magnetic-Resonance-Imaging; Middle-Age; Neurons-pathology; Occipital-Lobe-pathology; Septum-Pellucidum-abnormalities; Temporal-Lobe-pathology
MESH: *Brain-abnormalities; *Brain-Diseases-complications; *Epilepsy-etiology
TG: Case-Report; Female; Human; Male
PT: JOURNAL-ARTICLE
AN: 96436117
UD: 9704
MEDLINE EXPRESS (R) 1/97-9/97 40 of 48
TI: Perivascular external granule cells in hereditary cerebellar vermis defect rat: pathogenesis of cerebellar cortical dysplasia.
AU: Kuwamura-M; Yoshida-T; Yamate-J; Kotani-T; Sakuma-S; Tsudzuki-M
AD: Department of Veterinary Pathology, College of Agriculture, Osaka Prefecture University, Japan.
SO: J-Vet-Med-Sci. 1996 Sep; 58(9): 875-9
ISSN: 0916-7250
PY: 1996
LA: ENGLISH
CP: JAPAN
AB: Hereditary cerebellar vermis defect rats (CVD), a new neurological mutant, developed disorganized cerebellar cortical tissues. The postnatal development of the cerebellum in the CVD was examined histologically and immunohistochemically. A main pathological change in the CVD was abnormal perivascular cell aggregations, beginning to be observed from postnatal day 5. Around postnatal day 14, perivascular cells increased in number and many of them showed vigorous bromodeoxyuridine incorporation activities, as seen in the normal external granule cells (EGCs). The perivascular cells and EGCs were strongly positive for low affinity nerve growth factor receptor. These immunohistochemical results revealed that abnormal perivascular cells were heterotopic EGCs. The perivascular cells led to dysplastic abnormalities of lamination and abnormal cell positioning in the CVD. These findings indicate that abnormal perivascular aggregations of EGCs play an important role in the pathogenesis for cerebellar cortical dysplasia of CVD.
MESH: Cerebellar-Cortex-chemistry; Cerebellar-Diseases-etiology; Cerebellar-Diseases-pathology; Hereditary-Diseases-etiology; Hereditary-Diseases-pathology; Immunohistochemistry-; Mutation-; Rats-; Rats,-Inbred-Lew; Receptors,-Nerve-Growth-Factor-analysis; Rodent-Diseases-genetics
MESH: *Cerebellar-Cortex-pathology; *Cerebellar-Diseases-veterinary; *Hereditary-Diseases-veterinary; *Rodent-Diseases-etiology; *Rodent-Diseases-pathology
TG: Animal; Female; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 0
NM: Receptors,-Nerve-Growth-Factor
AN: 97053872
UD: 9704
MEDLINE EXPRESS (R) 1/97-9/97 41 of 48
TI: Arrest of neuronal migration by excitatory amino acids in hamster developing brain.
AU: Marret-S; Gressens-P; Evrard-P
AD: Laboratoire de Neurologie du Developpement, Hopital Robert-Debre, Paris, France.
SO: Proc-Natl-Acad-Sci-U-S-A. 1996 Dec 24; 93(26): 15463-8
ISSN: 0027-8424
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: The influence of the excitotoxic cascade on the developing brain was investigated using ibotenate, a glutamatergic agonist of both N-methyl-D-aspartate (NMDA) ionotropic receptors and metabotropic receptors. Injected in the neopallium of the golden hamster at the time of production of neurons normally destined for layers IV, III, and II, ibotenate induces arrests of migrating neurons at different distances from the germinative zone within the radial migratory corridors. The resulting cytoarchitectonic patterns include periventricular nodular heterotopias, subcortical band heterotopias, and intracortical arrests of migrating neurons. The radial glial cells and the extracellular matrix are free of detectable damage that could suggest a defect in their guiding role. The migration disorders are prevented by coinjection of DL-2-amino-7-phosphoheptanoic acid, an NMDA ionotropic antagonist, but are not prevented by coinjection of L(+)-2-amino-3-phosphonopropionic acid, a metabotropic antagonist. This implies that an excess of ionic influx through the NMDA channels of neurons alters the metabolic pathways supporting neuronal migration. Ibotenate, a unique molecular trigger of the excitotoxic cascade, produces a wide spectrum of abnormal neuronal migration patterns recognized in mammals, including the neocortical deviations encountered in the human brain.
MESH: Alanine-analogs-and-derivatives; Alanine-pharmacology; Animals,-Newborn; Brain-embryology; Brain-growth-and-development; Cell-Differentiation; Cell-Division; Cell-Movement-drug-effects; DNA-analysis; Extracellular-Matrix-drug-effects; Extracellular-Matrix-physiology; Hamsters-; Mesocricetus-; Neurons-cytology; Neurons-drug-effects; Pregnancy-; Receptors,-N-Methyl-D-Aspartate-drug-effects; 2-Amino-5-phosphonovalerate-analogs-and-derivatives; 2-Amino-5-phosphonovalerate-pharmacology
MESH: *Aging-physiology; *Brain-physiology; *Excitatory-Amino-Acid-Agonists-toxicity; *Excitatory-Amino-Acid-Antagonists-pharmacology; *Ibotenic-Acid-toxicity; *Neurons-physiology; *Receptors,-N-Methyl-D-Aspartate-physiology
TG: Animal; Female; Human; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 0; 0; 0; 2552-55-8; 5652-28-8; 6898-94-8; 76726-92-6; 85797-13-3; 9007-49-2
NM: Excitatory-Amino-Acid-Agonists; Excitatory-Amino-Acid-Antagonists; Receptors,-N-Methyl-D-Aspartate; Ibotenic-Acid; 2-amino-3-phosphonopropionic-acid; Alanine; 2-Amino-5-phosphonovalerate; 2-amino-7-phosphonoheptanoic-acid; DNA
AN: 97140353
UD: 9704
MEDLINE EXPRESS (R) 1/97-9/97 42 of 48
TI: Clinical features of neocortical temporal lobe epilepsy.
AU: Pacia-SV; Devinsky-O; Perrine-K; Ravdin-L; Luciano-D; Vazquez-B; Doyle-WK
AD: Department of Neurology, NYU/HJD Comprehensive Epilepsy Center, Hospital for Joint Diseases, New York, NY 10003, USA.
SO: Ann-Neurol. 1996 Nov; 40(5): 724-30
ISSN: 0364-5134
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: Few studies have examined the clinical features of neocortical temporal lobe epilepsy (NTLE) in carefully selected patients. We reviewed records from 21 patients with NTLE, defined by intracranial electroencephalogram (EEG), who have been seizure free for 1 year or more following temporal lobectomy. The mean age of onset at the time of first seizure was 14 years (range, 1-41 years). Febrile seizures were reported in only 2 patients (9.5%). In contrast to prior mesial temporal lobe epilepsy (MTLE) studies, seizure-free intervals between the initial cerebral insult or first seizure and habitual seizures were uncommon. Possible or known risk factors for epilepsy were reported in 13 of 21 patients (62%). Fifteen (71%) patients reported auras, with experiential phenomena being the most common type. Magnetic resonance imaging was normal or nonspecific in 15 patients, revealed mild hippocampal atrophy in 2, tumors in 2, and heterotopic gray matter and hippocampal atrophy in 1, and cortical dysgenesis in 1. Neuropsychological testing showed deficits consistent with the seizure focus in 13 patients (62%), and Wada test showed ipsilateral memory deficits in 10 (48%). The most common behavioral manifestation was a motionless stare at ictal onset (48%). In contrast to prior studies of MTLE, only 1 NTLE patient had frequent independent, contralateral temporal lobe epileptiform spikes on scalp EEG.
MESH: Adolescence-; Adult-; Age-of-Onset; Disease-Free-Survival; Epilepsy,-Temporal-Lobe-pathology; Fever-; Magnetic-Resonance-Imaging; Neuropsychological-Tests; Retrospective-Studies; Risk-Factors; Seizures-
MESH: *Brain-pathology; *Electroencephalography-; *Epilepsy,-Temporal-Lobe-physiopathology; *Epilepsy,-Temporal-Lobe-surgery
TG: Female; Human; Male
PT: JOURNAL-ARTICLE
AN: 97115894
UD: 9703
MEDLINE EXPRESS (R) 1/97-9/97 43 of 48
TI: Neuronal migrational disorders in children with epilepsy: MRI, interictal SPECT and EEG comparisons.
AU: Iannetti-P; Spalice-A; Atzei-G; Boemi-S; Trasimeni-G
AD: Pediatric Department, University La Sapienza, Rome, Italy.
SO: Brain-Dev. 1996 Jul-Aug; 18(4): 269-79
ISSN: 0387-7604
PY: 1996
LA: ENGLISH
CP: NETHERLANDS
AB: Single-photon emission computed tomography (SPECT) is being increasingly used in the investigation of children with epilepsy and may provide insights into congenital malformations. We analyzed the interictal 99Tc-HMPAO-SPECT in a series of seven children with developmental disorders of the neocortex, each of them representing a prototype of cerebral dysgenesis, such as lissencephaly, pachygyria, opercular dysplasia, polymicrogyria, nodular heterotopia and band heterotopia. The patients studied were selected among 22 epileptic children with neuronal migrational disorders (NMDs). Interictal SPECT hypoperfusion was observed in the area homologous to MRI findings in all the examined children. In three patients low perfusion was also present in the opposite hemisphere, probably due to functional involvement or related to an underlying microdysgenesis, not revealed by structural imaging. EEG features were in agreement with low perfusion areas, both anatomically and functionally, in all children. In one patient hypoperfusion area differed from that revealed by MRI and EEG. Ictal SPECT has been considered a useful tool for accurately locating the epileptic focus. Nevertheless, interictal brain perfusion studies, together with proton magnetic resonance spectroscopy, may play an important role in detecting anatomic substrate in developmental disorders of the neocortex.
MESH: Cerebral-Cortex-cytology; Child-; Child,-Preschool; Electroencephalography-; Infant-; Magnetic-Resonance-Imaging; Tomography,-Emission-Computed,-Single-Photon
MESH: *Cell-Movement; *Cerebral-Cortex-abnormalities; *Epilepsy-congenital; *Epilepsy-diagnosis; *Neurons-cytology; *Neurons-physiology
TG: Comparative-Study; Female; Human; Male
PT: JOURNAL-ARTICLE
AN: 97033987
UD: 9703
MEDLINE EXPRESS (R) 1/97-9/97 44 of 48
TI: Modulation of autotransplanted adrenal gland by endothelin-1: a morphological and biochemical study.
AU: Vendeira-P; Neves-D; Magalhaes-MM; Magalhaes-MC
AD: Department of Histology and Embryology, Faculty of Medicine, University of Oporto, Porto, Portugal.
SO: Anat-Rec. 1996 Sep; 246(1): 98-106
ISSN: 0003-276X
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: BACKGROUND: Adrenal gland autotransplantation, a model of cortical tissue regeneration, provides the reconstruction of distinct functional and morphological zonae. A morphological and biochemical study of the adrenal gland of adult male rats after autotransplantation and endothelin-1 (ET-1) administration was made. METHODS: The technique involved bilateral adrenalectomy and placement of pieces of the adrenal gland in a dorsal plane between the skin and muscle. The animals were killed 90 days after the autotransplantation and 1 hr after intravenous ET-1 administration (0.5 microgram/kg body weight). The autotransplanted pieces were removed, fixed, and processed for light and electron microscopic morphologic studies. Trunk blood was collected for steroid assay. RESULTS: Saline-treated control autotransplanted animals showed no remarkable differences in adrenal organization; grafts exhibiting a mass of regenerated cortical tissue were arranged in nests of glandular cells surrounded by a fibrous capsule and intersected by layers of connective tissue. The adrenal medulla was systematically absent. Ultrastructure of ET-1-treated animals revealed an inner area in the graft, consisting mainly of fasciculatalike cells. Cytoplasmic changes were evident, with high variations in mitochondrial size and arrangement. Profiles of smooth endoplasmic reticulum sometimes exhibited evidence of hypertrophy. Glandular cells in the graft outer area (subcapsular) were almost invariably like glomerulosa; however, some of them showed mitochondria with a peculiar arrangement of the cristae. "Hybrid" cells with mitochondria resembling those of the zona reticularis were also observed in the subcapsular environment. ET-1-stimulated animals showed significant increases in plasma corticosterone and aldosterone concentrations. CONCLUSIONS: Endothelin-1, previously reported to stimulate acutely the aldosterone secretion by the adrenal zona glomerulosa in the rat, seems to exert a modulator role on the physiology of adrenal autotransplants, their regeneration and secretion.
MESH: Adrenal-Glands-ultrastructure; Hormones-blood; Injections,-Intravenous; Microscopy,-Electron; Osmolar-Concentration; Rats-; Rats,-Wistar; Transplantation,-Heterotopic
MESH: *Adrenal-Glands-drug-effects; *Adrenal-Glands-transplantation; *Endothelin-1-pharmacology; *Transplantation,-Autologous
TG: Animal; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 0; 0
NM: Endothelin-1; Hormones
AN: 97030900
UD: 9703
MEDLINE EXPRESS (R) 1/97-9/97 45 of 48
TI: Correlations between clinical and neuropathological diagnosis of cortical anomalies in developmentally disabled children.
AU: Wisniewski-KE; Dambska-M
AD: NYS Institute for Basic Research in Developmental Disabilities, Staten Island 10314, USA.
SO: Brain-Dev. 1995; 17 Suppl: 48-54
ISSN: 0387-7604
PY: 1995
LA: ENGLISH
CP: NETHERLANDS
AB: The capabilities and limitations of clinical diagnoses, particularly brain imaging of cortical anomalies, in developmentally disabled children are reviewed. Some aspects of diagnostic problems in generalized cortical dysgeneses, like lissencephaly type I and II, subcortical heterotopias, generalized polymicrogyria, or focal cortical anomalies and primary micrencephalies, are discussed.
MESH: Brain-Diseases-pathology; Cerebral-Cortex-pathology; Child-; Developmental-Disabilities-pathology; Magnetic-Resonance-Imaging; Tomography,-X-Ray-Computed
MESH: *Brain-Diseases-diagnosis; *Cerebral-Cortex-abnormalities; *Developmental-Disabilities-diagnosis
TG: Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 97036925
UD: 9702
MEDLINE EXPRESS (R) 1/97-9/97 46 of 48
TI: Developmental neuropathology and impact of perinatal brain damage. I: Hemorrhagic lesions of neocortex.
AU: Marin-Padilla-M
AD: Department of Pathology, Dartmouth Medical School, Hanover, NH 03755, USA.
SO: J-Neuropathol-Exp-Neurol. 1996 Jul; 55(7): 758-73
ISSN: 0022-3069
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: To establish developmental correlates among perinatal neocortical damage, its impact on the infant developing brain, and its possible role in the pathogenesis of ensuing neurologic sequelae, the neuropathology of acute, subacute (healing), and chronic (repaired) stages of periventricular and layer I (subpial) hemorrhagic lesions have been studied. Thirty-three cases of infants who survived brain damage for hours, days, weeks, months, and/or years and have been studied with the rapid Golgi and other methods. In periventricular hemorrhagic injury: (a) the local destruction of radial glia stop all cellular migration above the lesion; (b) precursor cells already traveling in damaged radial glia also stop their migration, miss their target, and form acquired heterotopias; and, (c) the cytoarchitecture of the overlying and differentiating gray matter may be secondarily altered. In layer I (subpial) hemorrhagic injury: (a) the neocortex external glial limiting membrane is disrupted and must be repaired; (b) its reparation often causes superficial leptomeningeal heterotopias; (c) the cytoarchitecture and intrinsic circuitry of layer I and underlying gray matter are secondarily altered; and, (d) partially damaged (pruning) and/or displaced gray matter neurons undergo post-injury morphologic transformations, atrophy, hypertrophy, and reestablished new "abnormal" connections. These post-injury gray matter cytoarchitectural alterations could eventually play a role in cortical dysfunction and, hence, in the pathogenesis of neurologic sequelae.
MESH: Adolescence-; Cerebral-Cortex-pathology; Cerebral-Hemorrhage-pathology; Cerebral-Hemorrhage-physiopathology; Child-; Child,-Preschool; Diseases-in-Twins; Infant-; Infant,-Newborn; Neuronal-Plasticity; Wound-Healing
MESH: *Brain-Damage,-Chronic-complications; *Cerebral-Cortex-blood-supply; *Cerebral-Hemorrhage-complications; *Developmental-Disabilities-etiology; *Fetal-Diseases; *Infant,-Newborn,-Diseases
TG: Human; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
CN: NS22897NSNINDS
AN: 96268230
UD: 9702
MEDLINE EXPRESS (R) 1/97-9/97 47 of 48
TI: [A case of pachygyria with cystic changes in the periventricular white matter and putamen]
AU: Okumura-A; Hayakawa-F; Kuno-K; Hayakawa-S; Hashizume-Y; Watanabe-K
AD: Department of Pediatrics, Anjo Kosei Hospital, Aichi.
SO: No-To-Hattatsu. 1996 Sep; 28(5): 424-9
ISSN: 0029-0831
PY: 1996
LA: JAPANESE; NON-ENGLISH
CP: JAPAN
AB: We reported a case of type I lissencephaly/pachygyria which had cystic changes in the periventricular white matter and lentiform nuclei. The patient developed neonatal seizures and was referred to Anjo Kosei Hospital His seizures were frequent and refractory to anticonvulsants. His development was severely retarded. CT and MRI revealed cystic changes in the periventricular white matter and lentiform nuclei as well as bilateral diffuse pachygyria and agenesis of corpus callosum. He died of unknown cause at 5 months of age and postmortem examination was performed. Type I lissencephaly/pachygyria, almost complete agenesis of corpus callosum, leptomeningeal glioneuronal heterotopia and hypoplasia of corticospinal tract were seen pathologically. Marked gliosis and CD 68 positive macrophages were found around the cystic lesions in the periventricular white matter and lentiform nuclei, which suggests that these lesions were the secondarily destructive lesion. We considered that these secondary lesions were due to frequent seizures which could cause insufficient supply of blood and glucose in those areas.
MESH: Brain-pathology; Brain-radiography; Electroencephalography-; English-Abstract; Infant,-Newborn; Magnetic-Resonance-Imaging; Seizures-complications; Seizures-pathology; Tomography,-X-Ray-Computed
MESH: *Brain-abnormalities; *Putamen-pathology
TG: Case-Report; Human; Male
PT: JOURNAL-ARTICLE
AN: 96428082
UD: 9702
MEDLINE EXPRESS (R) 1/97-9/97 48 of 48
TI: [Chaos in pathogenesis of brain dysgenesis]
AU: Shimada-M
AD: Department of Pediatrics, Shiga University of Medical Science, Otsu.
SO: No-To-Hattatsu. 1996 Mar; 28(2): 93-101
ISSN: 0029-0831
PY: 1996
LA: JAPANESE; NON-ENGLISH
CP: JAPAN
AB: Various cortical dysplasias, such as agyria-lissencephalia, pachygyria, micropolygyria, neuronal heterotopia and so on, have become relatively common neuropathological findings among the children with intactable epilepsy and mental and/or physical handicap. Together with various environmental factors, gene abnormalities are recently increasing as a cause in various cortical dysplasias. However, details of the pathogenesis still remain unknown. Experimental studies using animal models indicated that inhibition of neuron production, disorders of neuron-glia and neuron-neuron contact, and plastic and unbalanced synaptogenesis subsequent to abnormal neuron production play an important role either separately or in combination in the pathogenesis of various cortical dysplasias.
MESH: Abnormalities-embryology; Abnormalities-genetics; English-Abstract
MESH: *Brain-abnormalities
TG: Animal; Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW-LITERATURE
AN: 97003966
UD: 9702
web contact: pietsch@indiana.edu
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