The following MEDLINE items were compiled by SilverPlatter and are presented with their generous cooperation and permission. (See SilverPlatter's Worldwide Library for bibliographic search information.)For a non-technical summary of Andermann's syndrome, try here!
Record 1 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Texture analysis and morphological processing of magnetic resonance imaging assist detection of focal cortical dysplasia in extra-temporal partial epilepsy.
AUTHOR: Bernasconi,-A; Antel,-S-B; Collins,-D-L; Bernasconi,-N; Olivier,-A; Dubeau,-F; Pike,-G-B; Andermann,-F; Arnold,-D-L
ADDRESS OF AUTHOR: Department of Neurology and Neurosurgery and Brain Imaging Center, Montreal Neurological Hospital and Institute, McGill University, Montreal, Quebec, Canada.
SOURCE: Ann-Neurol. 2001 Jun; 49(6): 770-5
INTERNATIONAL STANDARD SERIAL NUMBER: 0364-5134
PUBLICATION YEAR: 2001
LANGUAGE: English
COUNTRY OF PUBLICATION: United-States
ABSTRACT: In many patients, focal cortical dysplasia (FCD) is characterized by minor structural changes that may go unrecognized by standard radiological analysis. To increase the sensitivity of magnetic resonance imaging (MRI) for the detection of subtle lesions of FCD, we developed voxel-based image postprocessing methods, including first-order texture analysis and morphological processing modeled on known MRI features of FCD. We selected 16 patients with histologically proven FCD. Image processing features were calculated over a neighborhood for each voxel in the three-dimensional T1-weighted MRI. Three feature maps were generated: (1) gray matter thickness map to model cortical thickening, (2) gradient map to model blurring of the gray matter-white matter junction, and (3) relative intensity map to model the hyperintense signal within the lesion. Two observers detected lesions on conventional MRI in 8/16 and on ratio maps in 14/16 patients. Sensitivity was 87.5% (14/16) for the ratio maps compared to 50% (8/16) for MRI (p < 0.003). Specificity was 95% (19/20) for ratio maps and 100% (20/20) for MRIs. Cohen's kappa was 0.53 for MRIs, indicating moderate agreement, and 0.83 for ratio maps, indicating strong agreement beyond chance between the 2 observers. The image-processing methods developed in this study improve visual detection of FCD, even in cases where no lesion is obvious on MRI. These techniques could increase the number of patients with partial epilepsy who could benefit from surgery.
MAJOR MESH DESCRIPTORS: *Cerebral-Cortex-pathology; *Epilepsy,-Frontal-Lobe-diagnosis; *Epilepsy,-Frontal-Lobe-pathology; *Magnetic-Resonance-Imaging
MINOR MESH DESCRIPTORS: Cell-Movement; Chi-Square-Distribution; Epilepsy,-Frontal-Lobe-physiopathology; Neurons-pathology; Sensitivity-and-Specificity
CHECKTAGS: Human; Support,-Non-U.S.-Gov't
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: pathology; diagnosis; physiopathology
SUBSET: Index-Medicus
UPDATE CODE: 20010628
ACCESSION NUMBER: 21302094
RECORD FEATURES: ABSTRACT (AB)
Record 2 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Entorhinal cortex atrophy in epilepsy patients exhibiting normal hippocampal volumes.
AUTHOR: Bernasconi,-N; Bernasconi,-A; Caramanos,-Z; Dubeau,-F; Richardson,-J; Andermann,-F; Arnold,-D-L
ADDRESS OF AUTHOR: Department of Neurology and Neurosurgery, McGill University, and Montreal Neurological Institute and Hospital, PQ, Canada.
SOURCE: Neurology. 2001 May 22; 56(10): 1335-9
INTERNATIONAL STANDARD SERIAL NUMBER: 0028-3878
PUBLICATION YEAR: 2001
LANGUAGE: English
COUNTRY OF PUBLICATION: United-States
ABSTRACT: OBJECTIVE: To determine whether MRI volumetric measurement of the entorhinal cortex could detect structural damage and lateralize the seizure focus in patients with temporal lobe epilepsy in whom no measurable hippocampal abnormalities were found. BACKGROUND: A reduction in the volume of the entorhinal cortex ipsilateral to the seizure focus in patients with intractable temporal lobe epilepsy and hippocampal atrophy was recently shown. METHODS: MRI volumetric analysis of the entorhinal cortex was performed using a T1-weighted three-dimensional gradient echo sequence in 24 control subjects and 22 patients with temporal lobe epilepsy and normal hippocampal volumes. Thirteen patients underwent surgery, with a mean postoperative follow-up of 36 months. RESULTS: Group analysis (multivariate analysis of variance) showed a reduction in the volume of the entorhinal cortex ipsilateral to the seizure focus in patients with left (p < 0.0001) and right temporal lobe epilepsy (p < 0.0001). Lateralization of the seizure focus could be done in 14 of 22 patients (64%) based on entorhinal cortex volumetry. CONCLUSION: Entorhinal cortex atrophy ipsilateral to the seizure focus supports the presence of structural damage in the mesial temporal lobe in patients with temporal lobe epilepsy and normal hippocampal volumes and emphasizes the participation of the entorhinal cortex in the pathogenesis of this disorder.
MAJOR MESH DESCRIPTORS: *Entorhinal-Cortex-pathology; *Epilepsy,-Temporal-Lobe-pathology; *Hippocampus-pathology
MINOR MESH DESCRIPTORS: Adult-; Amygdala-pathology; Amygdala-physiopathology; Atrophy-etiology; Atrophy-pathology; Atrophy-physiopathology; Entorhinal-Cortex-physiopathology; Epilepsy,-Temporal-Lobe-physiopathology; Gliosis-etiology; Gliosis-pathology; Gliosis-physiopathology; Hippocampus-physiopathology; Magnetic-Resonance-Imaging; Middle-Age; Nerve-Degeneration-etiology; Nerve-Degeneration-pathology; Nerve-Degeneration-physiopathology
CHECKTAGS: Human; Support,-Non-U.S.-Gov't
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: pathology; physiopathology; etiology
SUBSET: Abridged-Index-Medicus; Index-Medicus
UPDATE CODE: 20010628
ACCESSION NUMBER: 21270410
RECORD FEATURES: ABSTRACT (AB)
Record 3 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Lateralizing value of peri-ictal headache: A study of 100 patients with partial epilepsy.
AUTHOR: Bernasconi,-A; Andermann,-F; Bernasconi,-N; Reutens,-D-C; Dubeau,-F
ADDRESS OF AUTHOR: Departments of Neurology and Neurosurgery, McGill University, and Montreal Neurological Institute and Hospital, Quebec, Canada.
SOURCE: Neurology. 2000 Jan 9; 56(1): 130-2
INTERNATIONAL STANDARD SERIAL NUMBER: 0028-3878
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: United-States
ABSTRACT: To determine the lateralizing value of peri-ictal headache, the authors conducted a standardized interview of 100 patients with partial epilepsy, 60 with temporal lobe epilepsy (TLE) and 40 with extratemporal epilepsy (ETE). Peri-ictal headache occurred in 47 of 100 (47%) patients. Peri-ictal headache was more likely to be ipsilateral to the seizure onset in TLE (27 of 30 = 90%) than in ETE (two of 17 = 12%; p< 0.001). For both groups, peri-ictal headache usually conformed to the diagnostic criteria for common migraine (18 of 30 = 60% in TLE; 7 of 17 = 41% in ETE).
MAJOR MESH DESCRIPTORS: *Epilepsies,-Partial-complications; *Epilepsies,-Partial-physiopathology; *Headache-etiology; *Laterality-
MINOR MESH DESCRIPTORS: Adult-
CHECKTAGS: Female; Human; Male; Support,-Non-U.S.-Gov't
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: complications; physiopathology; etiology
SUBSET: Abridged-Index-Medicus; Index-Medicus
UPDATE CODE: 20010628
ACCESSION NUMBER: 21067188
RECORD FEATURES: ABSTRACT (AB)
Record 4 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Evidence for reflex activation of experiential complex partial seizures.
AUTHOR: Martinez,-O; Reisin,-R; Andermann,-F; Zifkin,-B-G; Sevlever,-G
ADDRESS OF AUTHOR: Hospital Britanico de Buenos Aires, Argentina.
SOURCE: Neurology. 2000 Jan 9; 56(1): 121-3
INTERNATIONAL STANDARD SERIAL NUMBER: 0028-3878
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: United-States
ABSTRACT: Reflex activation of seizures by thoughts or mental images is suggested by patients but has not been objectively demonstrated. The authors present a report of a man with experiential complex partial seizures reliably activated by thinking about his family home. During monitoring, such seizures were repeatedly induced in this way. Seizures were refractory to antiepileptic drugs, but ceased after left temporal resection. Pathologic examination showed cortical dysplasia.
MAJOR MESH DESCRIPTORS: *Epilepsy,-Complex-Partial-physiopathology; *Reflex-; *Thinking-
MINOR MESH DESCRIPTORS: Adult-; Electroencephalography-; Epilepsy,-Complex-Partial-diagnosis; Volition-
CHECKTAGS: Case-Report; Human; Male
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: diagnosis; physiopathology
SUBSET: Abridged-Index-Medicus; Index-Medicus
UPDATE CODE: 20010628
ACCESSION NUMBER: 21067191
RECORD FEATURES: ABSTRACT (AB)
Record 5 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Long-term efficacy and safety of piracetam in the treatment of progressive myoclonus epilepsy.
AUTHOR: Fedi,-M; Reutens,-D; Dubeau,-F; Andermann,-E; D'Agostino,-D; Andermann,-F
ADDRESS OF AUTHOR: FRCP(C), Montreal Neurological Institute and Hospital, 3801 University St, Room 127, Montreal, Quebec, Canada H3A 2B4. MIDA@MUSICA.MCGILL.CA
SOURCE: Arch-Neurol. 2001 May; 58(5): 781-6
INTERNATIONAL STANDARD SERIAL NUMBER: 0003-9942
PUBLICATION YEAR: 2001
LANGUAGE: English
COUNTRY OF PUBLICATION: United-States
ABSTRACT: BACKGROUND: Piracetam has been proven to be effective and well tolerated in the treatment of myoclonus in short-term studies. OBJECTIVE: To assess its long-term clinical efficacy, 11 patients with disabling myoclonus due to progressive myoclonus epilepsy were treated with piracetam in an open-label study. METHODS: Neurologic outcome (at the 1st, 6th, 12th, and 18th month of treatment) was assessed by an adjusted sum score of the following 3 indices: motor impairment, functional disability, and global assessment of disability due to myoclonus. Severity of other neurologic symptoms (seizure frequency and severity, dysarthria, and gait ataxia) also was assessed. Treatment with piracetam was initiated at a dose of 3.2 g/d that was gradually increased until stable benefit was noted (maximal dose in the trial was 20 g/d). Concomitant antiepileptic drugs were maintained at their previous dose. RESULTS: Statistically significant improvement in the total rating score was observed after introduction of piracetam at the 1st, 6th, and 12th month of treatment. Severity of other neurologic symptom scores did not improve significantly. Two patients reported drowsiness during the first 2 weeks of treatment. CONCLUSIONS: Piracetam given as add-on therapy seems to be an effective, sustained, and well-tolerated treatment of myoclonus. In patients with progressive myoclonus epilepsy, the efficacy of the drug increased during the first 12 months of treatment and then stabilized.
MAJOR MESH DESCRIPTORS: *Epilepsies,-Myoclonic-drug-therapy; *Nootropic-Agents-adverse-effects; *Nootropic-Agents-therapeutic-use; *Piracetam-adverse-effects; *Piracetam-therapeutic-use
MINOR MESH DESCRIPTORS: Adolescence-; Adult-; Anticonvulsants-therapeutic-use; Disability-Evaluation; Disease-Progression; Dose-Response-Relationship,-Drug; Drug-Therapy,-Combination; Epilepsies,-Myoclonic-physiopathology; Longitudinal-Studies; Nootropic-Agents-administration-and-dosage; Piracetam-administration-and-dosage; Safety-; Treatment-Outcome
CHECKTAGS: Female; Human; Male
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: therapeutic-use; drug-therapy; physiopathology; administration-and-dosage; adverse-effects
CAS REGISTRY NUMBER OR EC NUMBER: 0; 0; 7491-74-9
NAME OF SUBSTANCE: Anticonvulsants; Nootropic-Agents; Piracetam
SUBSET: Abridged-Index-Medicus; Index-Medicus
UPDATE CODE: 20010531
ACCESSION NUMBER: 21244276
RECORD FEATURES: ABSTRACT (AB)
Record 6 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Magnetization transfer ratio is unable to lateralize epileptic foci in patients with temporal lobe epilepsy.
AUTHOR: Li,-L-M; Narayanan,-S; Pike,-G-B; Andermann,-F; Dubeau,-F; Arnold,-D-L
ADDRESS OF AUTHOR: Department of Neurology and Neurosurgery, McGill University and the Montreal Neurological Institute and Hospital, Quebec, Canada.
SOURCE: AJNR-Am-J-Neuroradiol. 2000 Nov-Dec; 21(10): 1853-6
INTERNATIONAL STANDARD SERIAL NUMBER: 0195-6108
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: UNITED-STATES
ABSTRACT: BACKGROUND AND PURPOSE: A preliminary report suggested that magnetization transfer ratio (MTR) was useful to lateralize epileptic foci in patients with refractory temporal lobe epilepsy (TLE). We attempted to confirm this finding in a larger group by investigating the relationship between MTR of mesial temporal structures and seizure lateralization in patients with refractory TLE. METHODS: We compared the MTR of amygdalae and hippocampi of 10 patients with unilateral TLE to values obtained from 10 healthy control participants. RESULTS: Three of 10 patients with TLE had MTR values that were 2 SD below the normal mean; the MTR abnormality was concordant with electroclinical lateralization in only one of the three. CONCLUSION: We conclude that MTR measurements of amygdalae and hippocampi are not useful for lateralization of TLE.
MAJOR MESH DESCRIPTORS: *Epilepsy,-Temporal-Lobe-pathology; *Laterality-; *Magnetic-Resonance-Imaging-methods
MINOR MESH DESCRIPTORS: Adult-; Amygdala-pathology; Case-Control-Studies; Hippocampus-pathology; Middle-Age; Statistics,-Nonparametric
CHECKTAGS: Female; Human; Male; Support,-Non-U.S.-Gov't
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: pathology; methods
SUBSET: Index-Medicus
UPDATE CODE: 20010531
ACCESSION NUMBER: 20560487
RECORD FEATURES: ABSTRACT (AB)
Record 7 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Migraine and the benign partial epilepsies of childhood: evidence for an association.
AUTHOR: Andermann,-F
ADDRESS OF AUTHOR: Montreal Neurological Hospital and Institute, Canada. mida@musica.McGill.ca
SOURCE: Epileptic-Disord. 2000; 2 Suppl 1: S37-9
INTERNATIONAL STANDARD SERIAL NUMBER: 1294-9361
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: France
ABSTRACT: There is an increasing body of evidence to suggest that benign rolandic epilepsy and benign occipital epilepsy of childhood are frequently associated with migraine. This however has not been universally recognized. Clearly further investigation of this association is required. The purpose of this report is to examine the varied diagnostic criteria for common migraine utilized by epileptologists and other neurologists, to review clinical migraine epilepsy relationships, EEG abnormalities in migraine and epilepsy, and the association of seizures with vascular headache in patients with various forms of epilepsy. Hopefully, this will stimulate further research into this intriguing association of two conditions which though they have different pathophysiology frequently coexist.
MAJOR MESH DESCRIPTORS: *Epilepsies,-Partial-complications; *Epilepsies,-Partial-physiopathology; *Migraine-complications; *Occipital-Lobe-physiopathology
MINOR MESH DESCRIPTORS: Adult-; Cerebrovascular-Circulation; Child-; Electroencephalography-; Migraine-physiopathology
CHECKTAGS: Female; Human; Male
PUBLICATION TYPE: Journal-Article; Review; Review,-Tutorial
SUBHEADINGS: complications; physiopathology
SUBSET: Index-Medicus
UPDATE CODE: 20010510
ACCESSION NUMBER: 21155650
RECORD FEATURES: ABSTRACT (AB)
Record 8 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Time course of postoperative recovery of N-acetyl-aspartate in temporal lobe epilepsy.
AUTHOR: Serles,-W; Li,-L-M; Antel,-S-B; Cendes,-F; Gotman,-J; Olivier,-A; Andermann,-F; Dubeau,-F; Arnold,-D-L
ADDRESS OF AUTHOR: Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University, Montreal, Quebec, Canada, H3A 2B4.
SOURCE: Epilepsia. 2001 Feb; 42(2): 190-7
INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580
PUBLICATION YEAR: 2001
LANGUAGE: English
COUNTRY OF PUBLICATION: United-States
ABSTRACT: PURPOSE: To assess the time course of increases in N-acetyl-aspartate/creatine (NAA/Cr), which can be measured using proton MR spectroscopic imaging (1H-MRSI), in patients with intractable nonlesional temporal lobe epilepsy (TLE) after successful epilepsy surgery. METHODS: We performed pre- and postoperative 1H-MRSI in 16 seizure-free (SF) patients and 16 not seizure-free (NSF) TLE patients. We calculated a mixed-design analysis of variance (ANOVA) between SF and NSF groups, ipsi- and contralateral to the side of operation, and pre- and postoperative NAA/Cr measurements. We applied nonlinear regression between pre- and postoperative NAA/Cr differences and the time interval between 1H-MRSI scans to fit a negative exponential model to NAA recovery. RESULTS: Mixed-design ANOVA revealed that (a) postoperative NAA/Cr was significantly higher in SF than in NSF patients (p = 0.02) and that (b) in the SF group, postoperative NAA/Cr values were significantly higher than preoperative values (p < 0.05) and returned to the normal range in most patients. According to our nonlinear regression model, in SF patients, there was a 50% increase relative to preoperative NAA/Cr values after 5.8 months, whereas an improvement of 95% was reached after 25 months. CONCLUSIONS: Our results extend preliminary observations of postoperative NAA recovery of SF patients by characterizing the time course of recovery as an exponential function with a half-time of approximately 6 months. The reversal of neuronal metabolic dysfunction remote from the epileptic focus may underlie the clinical observation of improvement of cognitive dysfunction after successful epilepsy surgery.
MAJOR MESH DESCRIPTORS: *Aspartic-Acid-analogs-and-derivatives; *Aspartic-Acid-analysis; *Epilepsy,-Temporal-Lobe-surgery; *Postoperative-Period; *Temporal-Lobe-chemistry; *Temporal-Lobe-surgery
MINOR MESH DESCRIPTORS: Adolescence-; Adult-; Algorithms-; Analysis-of-Variance; Creatine-analysis; Epilepsy,-Temporal-Lobe-metabolism; Follow-Up-Studies; Laterality-; Linear-Models; Magnetic-Resonance-Spectroscopy-statistics-and-numerical-data; Middle-Age; Regression-Analysis; Time-Factors; Treatment-Outcome
CHECKTAGS: Female; Human; Male; Support,-Non-U.S.-Gov't
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: analogs-and-derivatives; analysis; metabolism; surgery; statistics-and-numerical-data; chemistry
CAS REGISTRY NUMBER OR EC NUMBER: 56-84-8; 57-00-1; 997-55-7
NAME OF SUBSTANCE: Aspartic-Acid; Creatine; N-acetylaspartate
SUBSET: Index-Medicus
UPDATE CODE: 20010329
ACCESSION NUMBER: 21135434
RECORD FEATURES: ABSTRACT (AB)
Record 9 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Intractable epilepsy after a functional hemispherectomy: important lessons from an unusual case. Case report.
AUTHOR: Mittal,-S; Farmer,-J-P; Rosenblatt,-B; Andermann,-F; Montes,-J-L; Villemure,-J-G
ADDRESS OF AUTHOR: Division of Neurosurgery, Montreal Children's Hospital, Quebec, Canada.
SOURCE: J-Neurosurg. 2001 Mar; 94(3): 510-4
INTERNATIONAL STANDARD SERIAL NUMBER: 0022-3085
PUBLICATION YEAR: 2001
LANGUAGE: English
COUNTRY OF PUBLICATION: United-States
ABSTRACT: Residual seizures after functional hemispherectomy occur in approximately 20% of patients with catastrophic epilepsy. These episodes are traditionally attributed to incomplete disconnection, persistent epileptogenic activity in the ipsilateral insular cortex, or bilateral independent epileptogenic activity. The authors report on the case of an 8-year-old boy with an intractable seizure disorder who had classic frontal adversive seizures related to extensive unilateral left hemispheric cortical dysplasia. The initial intervention consisted of extensive removal of the epileptic frontal and precentral dysplastic tissue and multiple subpial transections of the dysplastic motor strip, guided by intraoperative electrocorticography, Subsequently, functional hemispherectomy including insular cortex resection was performed for persistent attacks. After a seizure-free period of 6 months, a new pattern ensued, consisting of an aura of fear, dystonic posturing of the right arm, and unusual postictal hyperphagia coupled with an interictal diencephalic-like syndrome. Electroencephalography and ictal/interictal single-photon emission computerized tomography were used to localize the residual epileptic discharges to deep ipsilateral structures. Results of magnetic resonance imaging indicated a complete disconnection except for a strip of residual frontobasal tissue. Therefore, a volumetric resection of the epileptogenic frontal basal tissue up to the anterior commissure was completed. The child has remained free of seizures during 21 months of follow-up review. Standard hemispherectomy methods provide extensive disconnection, despite the presence of residual frontal basal cortex. However, rarely, and especially if it is dysplastic, this tissue can represent a focus for refractory seizures. This is an important consideration in determining the source of ongoing seizures posthemispherectomy in patients with extensive cortical dysplasia. It remains important to assess them fully before considering their disease refractory to surgical treatment.
MAJOR MESH DESCRIPTORS: *Cerebral-Cortex-abnormalities; *Cerebral-Cortex-surgery; *Cerebral-Decortication; *Epilepsy,-Generalized-surgery; *Postoperative-Complications-pathology
MINOR MESH DESCRIPTORS: Child-; Epilepsy,-Generalized-pathology; Epilepsy,-Generalized-radionuclide-imaging; Magnetic-Resonance-Imaging; Postoperative-Complications-radionuclide-imaging; Seizures-pathology; Seizures-surgery; Tomography,-Emission-Computed,-Single-Photon
CHECKTAGS: Case-Report; Human; Male
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: abnormalities; surgery; pathology; radionuclide-imaging
SUBSET: Abridged-Index-Medicus; Index-Medicus
UPDATE CODE: 20010329
ACCESSION NUMBER: 21130688
RECORD FEATURES: ABSTRACT (AB)
Record 10 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Cortical dysplasias and epilepsy: a review of the architectonic, clinical, and seizure patterns.
AUTHOR: Andermann,-F
ADDRESS OF AUTHOR: Department of Neurology, Montreal Neurological Institute, McGill University, Quebec, Canada.
SOURCE: Adv-Neurol. 2000; 84479-96
INTERNATIONAL STANDARD SERIAL NUMBER: 0091-3952
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: UNITED-STATES
ABSTRACT: Since the nineteenth century, various abnormalities of cortical development resulting from migration defect, disorders of maturation, and disorders of cortical organization were described in brains at autopsy. Cortical dysplasia then was recognized in tissue resected during surgical treatment of patients with intractable epilepsy, but this finding remained largely unappreciated until the development of modern imaging. CT allowed glimpses of the more obvious malformations, but it was the advent of MRI that enabled the recognition and classification of the different types of lesions. In the Taylor type of cortical dysplasia, it became clear that there was a wide range in the severity and, above all, in the extent of the abnormality. The lesions range from small areas, often difficult to identify, to extensive lesions surrounded by a halo or penumbra of presumably less severe, but still clinically significant, structural abnormality. Functional imaging (SPECT, PET, and MRS) have provided additional insights and led to strategies for surgical treatment. Even lesions involving the central strip may at times be successfully resected, but in such patients much depends on the preoperative neurologic status. Recognition of the fact that dysplastic lesions are in themselves epileptogenic has been another milestone in our understanding of these abnormalities. Subcortical heterotopias, in particular periventricular nodular heterotopias, have been recognized as causing intractable epilepsy in some but not in all patients. Surgical approaches to these lesions are now being planned. The hereditary nature of the lesions in some patients has explained the familial occurrence of epilepsy in a number of instances. Generalized epileptic abnormalities and generalized disorders of migration and maturation have been described as band heterotopia or the double-cortex syndrome. Here, too, sex-linked dominant inheritance may occur, and progress has been made in our understanding of the mechanisms of these genetically determined lesions. Focal resection in patients with band heterotopia, however, has been of little value in the small number of patients in whom it has been carried out. Cortical malformations due to disorganization, occurring later in intrauterine life, are represented by micropolygyria. These lesions are often bilateral and perisylvian, but at times they are unilateral and in some patients may be occipital or frontal. Several syndromes have emerged, the most common being the one characterized by severe pseudobulbar palsy and mild pyramidal deficit (31). In some patients with such cortical abnormalities, particularly those with micropolygyria, the epilepsy may not be intractable, and full control may be obtained by medical treatment (32). Interesting and important clinical features of patients with bilateral perisylvian polymicrogyria were described by Guerrini et al. (33) and Caraballo et al. (34). In some patients who develop a secondary generalized electrographic abnormality and drop attacks early in the first decade, there is eventual improvement and cessation of the epileptic abnormality toward the end of the first decade or somewhat later. These investigators stressed that callosotomy should be considered with caution in patients with micropolygyria and this electroclinical pattern. Hypothalamic hamartomata and the associated epileptic syndrome have been better understood in recent years. Despite the risks of surgery, resection of the lesion offers hope of improvement in seizure control and of the often extremely severe behavioral abnormalities. On the other hand, patients with small lesions leading only to a "need to laugh" without more overt epileptic or behavioral manifestations are now being recognized. Finally, initial investigations have begun to uncover the transmitter abnormalities in patients with cortical dysplasia. (ABSTRACT TRUNCATED)
MAJOR MESH DESCRIPTORS: *Brain-abnormalities; *Epilepsy-physiopathology
MINOR MESH DESCRIPTORS: Epilepsy-pathology
CHECKTAGS: Human
PUBLICATION TYPE: Journal-Article; Review; Review,-Tutorial
SUBHEADINGS: abnormalities; pathology; physiopathology
SUBSET: Index-Medicus
UPDATE CODE: 20010322
ACCESSION NUMBER: 20543383
RECORD FEATURES: ABSTRACT (AB)
Record 11 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Sensorimotor organization in patients who have undergone hemispherectomy: a study with (15)O-water PET and somatosensory evoked potentials.
AUTHOR: Bernasconi,-A; Bernasconi,-N; Lassonde,-M; Toussaint,-P-J; Meyer,-E; Reutens,-D-C; Gotman,-J; Andermann,-F; Villemure,-J-G
ADDRESS OF AUTHOR: Montreal Neurological Hospital and Institute, Department of Neurology and Brain Imaging Center, McGill University, Quebec, Canada.
SOURCE: Neuroreport. 2000 Sep 28; 11(14): 3085-90
INTERNATIONAL STANDARD SERIAL NUMBER: 0959-4965
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: ENGLAND
ABSTRACT: To identify cortical structures that subserve residual motor and sensory function in patients with congenital hemiparesis due to a porencephalic cyst, we examined, using [(15)O]H2O, PET and somatosensory evoked potentials (SEPs) in three patients with left-sided hemiparesis who had undergone hemispherectomy. Motor stimulation of the affected hand produced ipsilateral activation in the premotor area in all patients, the SMA in two patients, and SII in two patients. Vibrotactile stimulation resulted in activation of the ipsilateral SII in all subjects. Median nerve stimulation of the affected hand produced ipsilateral long-latency SEPs in fronto-centro-parietal areas, whereas stimulation of the non-affected hand produced normal early cortical potentials in the contralateral hemisphere. Our results suggest that residual function in the paretic hand is warranted through non-primary motor and sensory areas, and higher order associative areas in the intact hemisphere.
MAJOR MESH DESCRIPTORS: *Cerebral-Decortication; *Laterality-physiology; *Motor-Cortex-physiopathology; *Nerve-Regeneration-physiology; *Neuronal-Plasticity-physiology; *Recovery-of-Function-physiology; *Somatosensory-Cortex-physiopathology
MINOR MESH DESCRIPTORS: Adult-; Electric-Stimulation; Evoked-Potentials,-Somatosensory-physiology; Mechanoreceptors-cytology; Mechanoreceptors-physiology; Motor-Activity-physiology; Motor-Cortex-abnormalities; Motor-Cortex-surgery; Oxygen-Radioisotopes-diagnostic-use; Physical-Stimulation; Somatosensory-Cortex-abnormalities; Somatosensory-Cortex-surgery; Tomography,-Emission-Computed; Touch-physiology
CHECKTAGS: Female; Human
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: physiology; cytology; abnormalities; physiopathology; surgery; diagnostic-use
CAS REGISTRY NUMBER OR EC NUMBER: 0
NAME OF SUBSTANCE: Oxygen-Radioisotopes
SUBSET: Index-Medicus
UPDATE CODE: 20010222
ACCESSION NUMBER: 20496244
RECORD FEATURES: ABSTRACT (AB)
Record 12 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Rasmussen's syndrome, with particular reference to cerebral plasticity: a tribute to Frank Morrell.
AUTHOR: Andermann,-F; Hart,-Y
ADDRESS OF AUTHOR: McGill University, Montreal Neurological Hospital and Institute, Montreal, Quebec, Canada.
SOURCE: Int-Rev-Neurobiol. 2001; 45173-208
INTERNATIONAL STANDARD SERIAL NUMBER: 0074-7742
PUBLICATION YEAR: 2001
LANGUAGE: English
COUNTRY OF PUBLICATION: United-States
MAJOR MESH DESCRIPTORS: *Encephalitis-pathology
MINOR MESH DESCRIPTORS: Adolescence-; Adult-; Age-of-Onset; Encephalitis-diagnosis; Encephalitis-physiopathology; Encephalitis-therapy; Laterality-physiology
CHECKTAGS: Human
PUBLICATION TYPE: Journal-Article; Review; Review,-Tutorial
SUBHEADINGS: diagnosis; pathology; physiopathology; therapy; physiology
SUBSET: Index-Medicus
UPDATE CODE: 20010222
ACCESSION NUMBER: 21014517
Record 13 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: T2 relaxometry can lateralize mesial temporal lobe epilepsy in patients with normal MRI.
AUTHOR: Bernasconi,-A; Bernasconi,-N; Caramanos,-Z; Reutens,-D-C; Andermann,-F; Dubeau,-F; Tampieri,-D; Pike,-B-G; Arnold,-D-L
ADDRESS OF AUTHOR: Department of Neurology, Neurosurgery and Radiology, McGill University, Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada.
SOURCE: Neuroimage. 2000 Dec; 12(6): 739-46
INTERNATIONAL STANDARD SERIAL NUMBER: 1053-8119
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: UNITED-STATES
ABSTRACT: In unselected patients with intractable temporal lobe epilepsy (TLE), approximately 15% do not have detectable hippocampal atrophy on MRI. The purpose of this study was to evaluate whether T2 relaxometry can identify hippocampal pathology and lateralize the epileptic focus in patients with intractable TLE, who do not demonstrate hippocampal atrophy on volumetric MRI (MRIV). We selected 14 patients with unilateral TLE who had unilateral atrophy and 11 patients with unilateral TLE who had no evidence of atrophy on MRIV. Images were acquired on a 1.5 T MR scan using a dual echo sequence with 23 contiguous oblique coronal slices in all patients and in 14 healthy subjects. Fitting a single exponential decay equation to the imaging data generated T2 maps. Averages of six slices containing the head, body, and tail of the hippocampus were used to calculate hippocampal T2 relaxation times (HT2). The epileptic focus was defined by history, video-EEG, and surgical response. All TLE patients with hippocampal atrophy and 9/11 (82%) patients with normal MRI had abnormally high HT2 ipsilateral to the epileptic focus. Bilateral abnormal HT2 were found in 6/14 (43%) of patients with unilateral hippocampal atrophy and 2/11 (18%) of patients with normal MRI. However, this increase was always greater ipsilateral to the epileptic focus. Qualitative hippocampal pathology showed gliosis and neuronal loss in 10/14 operated patients with hippocampal atrophy on MRIV and in 5/7 operated patients with normal MRI. In conclusion, hippocampal T2 mapping provides evidence of hippocampal damage in the majority of patients with intractable TLE who have no evidence of atrophy on MRI and can correctly lateralize the epileptic focus in most patients. Copyright 2000 Academic Press.
MAJOR MESH DESCRIPTORS: *Dominance,-Cerebral-physiology; *Epilepsy,-Temporal-Lobe-diagnosis; *Hippocampus-pathology; *Magnetic-Resonance-Imaging
MINOR MESH DESCRIPTORS: Adult-; Atrophy-; Brain-Mapping; Epilepsy,-Temporal-Lobe-physiopathology; Gliosis-diagnosis; Gliosis-physiopathology; Hippocampus-physiopathology; Middle-Age; Nerve-Degeneration-diagnosis; Nerve-Degeneration-physiopathology; Reference-Values; Temporal-Lobe-pathology; Temporal-Lobe-physiopathology
CHECKTAGS: Female; Human; Male; Support,-Non-U.S.-Gov't
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: physiology; diagnosis; physiopathology; pathology
SUBSET: Index-Medicus
UPDATE CODE: 20010202
ACCESSION NUMBER: 20565173
RECORD FEATURES: ABSTRACT (AB)
Record 14 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Vezatin, a novel transmembrane protein, bridges myosin VIIA to the cadherin-catenins complex.
AUTHOR: Kussel-Andermann,-P; El-Amraoui,-A; Safieddine,-S; Nouaille,-S; Perfettini,-I; Lecuit,-M; Cossart,-P; Wolfrum,-U; Petit,-C
ADDRESS OF AUTHOR: Unite de Genetique des Deficits Sensoriels, CNRS URA 1968 and Unite des Interactions Bacteries-Cellules, Institut Pasteur, 25-28 rue du Dr Roux, 75724 Paris cedex 15, France.
SOURCE: EMBO-J. 2000 Nov 15; 19(22): 6020-9
INTERNATIONAL STANDARD SERIAL NUMBER: 0261-4189
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: ENGLAND
ABSTRACT: Defects in myosin VIIA are responsible for deafness in the human and mouse. The role of this unconventional myosin in the sensory hair cells of the inner ear is not yet understood. Here we show that the C-terminal FERM domain of myosin VIIA binds to a novel transmembrane protein, vezatin, which we identified by a yeast two-hybrid screen. Vezatin is a ubiquitous protein of adherens cell-cell junctions, where it interacts with both myosin VIIA and the cadherin-catenins complex. Its recruitment to adherens junctions implicates the C-terminal region of alpha-catenin. Taken together, these data suggest that myosin VIIA, anchored by vezatin to the cadherin-catenins complex, creates a tension force between adherens junctions and the actin cytoskeleton that is expected to strengthen cell-cell adhesion. In the inner ear sensory hair cells vezatin is, in addition, concentrated at another membrane-membrane interaction site, namely at the fibrillar links interconnecting the bases of adjacent stereocilia. In myosin VIIA-defective mutants, inactivity of the vezatin-myosin VIIA complex at both sites could account for splaying out of the hair cell stereocilia.
MAJOR MESH DESCRIPTORS: *Cadherins-metabolism; *Cytoskeletal-Proteins-metabolism; *Membrane-Proteins-chemistry; *Membrane-Proteins-metabolism; *Myosin-chemistry; *Myosin-metabolism
MINOR MESH DESCRIPTORS: Alternative-Splicing; Amino-Acid-Sequence; Cadherins-chemistry; Cell-Line; Cytoskeletal-Proteins-chemistry; Deafness-genetics; Deafness-metabolism; Hair-Cells-metabolism; Intercellular-Junctions-metabolism; Macromolecular-Systems; Membrane-Proteins-genetics; Mice-; Molecular-Sequence-Data; Mutation-; Myosin-genetics; Protein-Binding; Protein-Structure,-Tertiary
CHECKTAGS: Animal; Human; In-Vitro; Support,-Non-U.S.-Gov't
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: chemistry; metabolism; genetics
CAS REGISTRY NUMBER OR EC NUMBER: 0; 0; 0; 0; 0; 0; 138930-21-9
NAME OF SUBSTANCE: Cadherins; Cytoskeletal-Proteins; Macromolecular-Systems; Membrane-Proteins; Myosin; myosin-VIIa; CAP102
SUBSET: Index-Medicus
UPDATE CODE: 20010104
ACCESSION NUMBER: 20532496
RECORD FEATURES: ABSTRACT (AB)
Record 15 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Sturge-Weber syndrome: indications and results of surgery in 20 patients.
AUTHOR: Arzimanoglou,-A-A; Andermann,-F; Aicardi,-J; Sainte-Rose,-C; Beaulieu,-M-A; Villemure,-J-G; Olivier,-A; Rasmussen,-T
ADDRESS OF AUTHOR: Service of Child Neurology and Metabolic Disorders, University Hospital Robert Debre, University Hospital Enfants Malades, Paris, France. alexis.arzimanoglou@rdb.ap-hop-paris.fr
SOURCE: Neurology. 2000 Nov 28; 55(10): 1472-9
INTERNATIONAL STANDARD SERIAL NUMBER: 0028-3878
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: UNITED-STATES
ABSTRACT: OBJECTIVE: To discuss the indications and timing for resective surgery in patients with Sturge-Weber syndrome (SWS) and medication-resistant epilepsy. BACKGROUND: SWS that causes epilepsy severe enough to merit surgery is rare. Because of the variable natural history of the disease, it is difficult to establish clear-cut indications for surgery and prospective studies are not feasible. Attitudes of clinicians and surgeons remain variable. METHODS: The authors assessed the presurgical epilepsy profile, criteria for surgery, monitoring techniques, and the postoperative outcome of epilepsy in all patients with SWS consecutively admitted between 1972 and 1990 to two referral centers (Paris and Montreal) and underwent surgery for intractable seizures. RESULTS: All 20 patients had a minimal postoperative follow-up of 4 years and all but one are still followed by one of the authors. One patient had a callosotomy, five underwent hemispherectomy, and 14 had cortical resection. Despite variability in the age at onset of seizures (range: 2 months to 12 years), age at operation (range: 8 months to 34 years) and surgical methods, almost all patients benefited from surgery. Visually guided complete resection of the pial angioma and underlying cortex, whenever possible, seemed sufficient; results were no better with intraoperative corticography. In children with previous hemiparesis, hemispherectomy proved particularly effective: all five became seizure free. None of the patients showed any aggravation of cognitive impairment following surgery; none of those who were operated on early presented with severe mental retardation, and 13 of 20 became seizure free. CONCLUSION: Although the natural history of SWS is imperfectly known, increasing duration of seizures and of postictal deficits, increase in atrophy or of calcified lesions or both, are indicative of its progressive nature. Despite the expected heterogeneity that renders formal comparison of the various approaches difficult, the current study provides new evidence to support early surgery in patients with SWS and drug-resistant epilepsy. The authors' results suggest that lesionectomy is a good approach, provided that the pial angioma is unilateral and the resection can be complete.
MAJOR MESH DESCRIPTORS: *Sturge-Weber-Syndrome-physiopathology; *Sturge-Weber-Syndrome-surgery
MINOR MESH DESCRIPTORS: Child-; Child,-Preschool; Infant-; Sturge-Weber-Syndrome-radiography; Tomography,-X-Ray-Computed
CHECKTAGS: Female; Human; Male
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: physiopathology; radiography; surgery
SUBSET: Abridged-Index-Medicus; Index-Medicus
UPDATE CODE: 20010104
ACCESSION NUMBER: 20547729
RECORD FEATURES: ABSTRACT (AB)
Record 16 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: An fMRI study of the lateralization of motor cortex activation in acallosal patients.
AUTHOR: Reddy,-H; Lassonde,-M; Bemasconi,-N; Bemasconi,-A; Matthews,-P-M; Andermann,-F; Amold,-D-L
ADDRESS OF AUTHOR: Centre for Functional Magnetic Resonance Imaging of the Brain, Department of Clinical Neurology, University of Oxford, John Radcliffe Hospital, UK.
SOURCE: Neuroreport. 2000 Aug 3; 11(11): 2409-13
INTERNATIONAL STANDARD SERIAL NUMBER: 0959-4965
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: ENGLAND
ABSTRACT: Transcranial magnetic stimulation (TMS) studies have suggested that callosal afferents may mediate inhibition of the ipsilateral motor cortex (IMC) during unilateral hand movements. To test this concept, we used fMRI to determine whether acallosal patients have increased IMC activation with either complex or simple unilateral finger movements. Neither the localization of motor cortical regions activated, the volumes of activation, or the relative hemispheric lateralization of activations were different between the patients and normal controls. The potential callosal inhibitory pathway identified by TMS therefore does not appear to contribute to the interhemispheric suppression of physiologically relevant activations in the motor cortex as measured by fMRI.
MAJOR MESH DESCRIPTORS: *Corpus-Callosum-abnormalities; *Fingers-physiology; *Laterality-physiology; *Motor-Cortex-physiology; *Movement-physiology; *Neural-Inhibition-physiology; *Neural-Pathways-abnormalities
MINOR MESH DESCRIPTORS: Adult-; Brain-Mapping; Corpus-Callosum-anatomy-and-histology; Corpus-Callosum-physiology; Fingers-innervation; Magnetic-Resonance-Imaging; Middle-Age; Motor-Cortex-anatomy-and-histology; Neural-Pathways-anatomy-and-histology; Neural-Pathways-physiology; Neuronal-Plasticity-physiology; Neuropsychological-Tests; Psychomotor-Performance-physiology; Recovery-of-Function-physiology
CHECKTAGS: Female; Human; Male; Support,-Non-U.S.-Gov't
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: abnormalities; anatomy-and-histology; physiology; innervation
SUBSET: Index-Medicus
UPDATE CODE: 20010104
ACCESSION NUMBER: 20397610
RECORD FEATURES: ABSTRACT (AB)
Record 17 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Clinical findings, imaging characteristics and outcome in catastrophic post-encephalitic epilepsy.
AUTHOR: Trinka,-E; Dubeau,-F; Andermann,-F; Bastos,-A; Hui,-A; Li,-L-M; Kohler,-S; Olivier,-A
ADDRESS OF AUTHOR: Montreal Neurological Institute and Hospital, McGill University, Montreal, Quebec, Canada.
SOURCE: Epileptic-Disord. 2000 Sep; 2(3): 153-62
INTERNATIONAL STANDARD SERIAL NUMBER: 1294-9361
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: FRANCE
ABSTRACT: OBJECTIVES: The aim of this study is to characterize the clinical features and prognostic factors for intractable, post-encephalitic epilepsy. METHODS: We studied retrospectively 42 patients (26 men) evaluated between 1982 and 1999. MRI, neuropsychological findings, interictal and ictal scalp EEG were reviewed for all patients. Fifteen patients had additional stereo EEG (SEEG) studies. RESULTS: The mean age at encephalitis was 17 years (SD = 15.5); etiology was identified in 18 patients. During the acute illness, 79% had status epilepticus (SE) or recurrent seizures and 76% were in coma; mean Glasgow outcome scale (GOS) was 3.6 (SD = 0.8). The mean latency to seizure onset was 0.8 years (SD = 1.9). The majority (72%) presented with complex partial seizures with or without secondary generalization. According to interictal epileptiform findings and the predominant seizure onset pattern as found on scalp EEG, patients were unilateral temporal (UTLE) = 8, bilateral temporal (BTLE) = 12, and extratemporal/multifocal or generalized (ETMFE) = 22 patients. MRI atrophy and/or signal changes were unilateral temporal in 7 (18%), bilateral temporal in 5 (13%), multilobar/diffuse in 20 (51%), and absent in 7 (18%). ANOVA revealed significant differences in mean GOS between UTLE versus BTLE and ETMFE (4.7 versus 3.2 versus 3.6; p < 0.0001), but not in age at encephalitis. Latency to the first unprovoked seizure was shorter in patients with ETMFE compared to UTLE and BTLE (p < 0.01). Surgery was performed in 24 patients. The best outcome was obtained in UTLE (7/8 class I and II). In the others, outcome was poor in the majority (13/16 class III and IV). CONCLUSION: There is a small subgroup of patients with postencephalitic UTLE for whom the outcome is favorable. The majority of our patients had multifocal seizure onset with BTLE and ETMFE, and poor outcome after surgical treatment.
MAJOR MESH DESCRIPTORS: *Electroencephalography-; *Encephalitis,-Viral-diagnosis; *Epilepsies,-Partial-diagnosis; *Epilepsy,-Temporal-Lobe-diagnosis
MINOR MESH DESCRIPTORS: Adolescence-; Adult-; Aged-; Atrophy-; Brain-Mapping; Child-; Child,-Preschool; Corpus-Callosum-pathology; Corpus-Callosum-physiopathology; Corpus-Callosum-surgery; Dominance,-Cerebral-physiology; Encephalitis,-Viral-physiopathology; Encephalitis,-Viral-surgery; Epilepsies,-Partial-physiopathology; Epilepsies,-Partial-surgery; Epilepsy,-Temporal-Lobe-physiopathology; Epilepsy,-Temporal-Lobe-surgery; Follow-Up-Studies; Magnetic-Resonance-Imaging; Middle-Age; Psychosurgery-; Temporal-Lobe-pathology; Temporal-Lobe-physiopathology; Temporal-Lobe-surgery
CHECKTAGS: Female; Human; Male
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: pathology; physiopathology; surgery; physiology; diagnosis
SUBSET: Index-Medicus
UPDATE CODE: 20001205
ACCESSION NUMBER: 20478095
RECORD FEATURES: ABSTRACT (AB)
Record 18 of 18 in MEDLINE(R) on CD 2001/01-2001/06
TITLE: Unconventional myosin VIIA is a novel A-kinase-anchoring protein.
AUTHOR: Kussel-Andermann,-P; El-Amraoui,-A; Safieddine,-S; Hardelin,-J-P; Nouaille,-S; Camonis,-J; Petit,-C
ADDRESS OF AUTHOR: Unite de Genetique des Deficits Sensoriels, CNRS URA 1968, 25 rue du Dr. Roux, Institut Pasteur, 75724 Paris cedex 15, France.
SOURCE: J-Biol-Chem. 2000 Sep 22; 275(38): 29654-9
INTERNATIONAL STANDARD SERIAL NUMBER: 0021-9258
PUBLICATION YEAR: 2000
LANGUAGE: English
COUNTRY OF PUBLICATION: UNITED-STATES
ABSTRACT: To gain an insight into the cellular function of the unconventional myosin VIIA, we sought proteins interacting with its tail region, using the yeast two-hybrid system. Here we report on one of the five candidate interactors we identified, namely the type I alpha regulatory subunit (RI alpha) of protein kinase A. The interaction of RI alpha with myosin VIIA tail was demonstrated by coimmunoprecipitation from transfected HEK293 cells. Analysis of deleted constructs in the yeast two-hybrid system showed that the interaction of myosin VIIA with RI alpha involves the dimerization domain of RI alpha. In vitro binding assays identified the C-terminal "4.1, ezrin, radixin, moesin" (FERM)-like domain of myosin VIIA as the interacting domain. In humans and mice, mutations in the myosin VIIA gene underlie hereditary hearing loss, which may or may not be associated with visual deficiency. Immunohistofluorescence revealed that myosin VIIA and RI alpha are coexpressed in the outer hair cells of the cochlea and rod photoreceptor cells of the retina. Our results strongly suggest that myosin VIIA is a novel protein kinase A-anchoring protein that targets protein kinase A to definite subcellular sites of these sensory cells.
MAJOR MESH DESCRIPTORS: *Cyclic-AMP-Dependent-Protein-Kinases-metabolism; *Myosin-metabolism
MINOR MESH DESCRIPTORS: Binding-Sites; Escherichia-coli; Mice-; Myosin-analysis; Protein-Binding; Substrate-Specificity
CHECKTAGS: Animal; Human; Support,-Non-U.S.-Gov't
PUBLICATION TYPE: Journal-Article
SUBHEADINGS: metabolism; analysis
CAS REGISTRY NUMBER OR EC NUMBER: 0; 0; EC 2.7.10.-
NAME OF SUBSTANCE: Myosin; myosin-VIIa; Cyclic-AMP-Dependent-Protein-Kinases
SUBSET: Index-Medicus
UPDATE CODE: 20001103
ACCESSION NUMBER: 20449047
RECORD FEATURES: ABSTRACT (AB)