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Record 1 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Topiramate: a new antiepileptic drug. AUTHOR(S): Privitera-MD
SOURCE (BIBLIOGRAPHIC CITATION): Ann-Pharmacother. 1997 Oct; 31(10): 1164-73 INTERNATIONAL STANDARD SERIAL NUMBER: 1060-0280 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: OBJECTIVE: To review proposed mechanisms of action, clinical pharmacology, efficacy, safety, and tolerability of the antiepileptic drug (AED) topiramate. METHODS: All available data from the clinical development program--published and unpublished data (provided by investigators or the RW Johnson Pharmaceutical Research Institute)--were analyzed, with emphasis on the results of double-blind, placebo-controlled trials. Data from open-label studies provided information about long-term efficacy and tolerability. FINDINGS: Topiramate is highly effective in the control of previously therapy-resistant partial seizures, with or without secondary generalization. In the refractory adult patient population enrolled in controlled clinical trials, seizures were reduced by 50% or more in 27-47% of patients compared with 0-18% in placebo-treated patients and by 75% or more in 9-36% of the patients compared with 0-9% of those receiving placebo. The most common adverse effects invol! !
ve the central nervous system and are mild to moderate in nature. Adverse effects include somnolence, fatigue, psychomotor slowing, and concentration problems. The currently recommended dosing is lower and titration slower than schedules used in the clinical trials; this may improve the tolerability of topiramate. Topiramate has few interactions with currently available AEDs, but phenytoin concentrations increased in some patients. Topiramate can be used initially as adjunctive therapy. Plasma topiramate concentrations are reduced substantially when used with enzyme-inducing AEDs. Open-label data and a single double-blind trial suggest that topiramate monotherapy may be effective. Open-label data also indicate that topiramate may be effective in generalized seizures of nonfocal origin, including those associated with Lennox-Gastaut syndrome. CONCLUSIONS: The robust clinical effects of topiramate expand the therapeutic options for patients with epilepsy. Controlled clinical tri! !
als are needed to verify initial observations that topiramate may be effective against a broad spectrum of seizure types and to directly compare efficacy and tolerability with other new and standard AEDs. MEDLINE ACCESSION NUMBER: 97478600
Record 2 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Left vagus nerve stimulation in children with refractory epilepsy: an update. AUTHOR(S): Hornig-GW; Murphy-JV; Schallert-G; Tilton-C SOURCE (BIBLIOGRAPHIC CITATION): South-Med-J. 1997 May; 90(5): 484-8 INTERNATIONAL STANDARD SERIAL NUMBER: 0038-4348 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: This report updates previous reports regarding the tolerance and efficacy of periodic vagus nerve stimulation in a group of 19 children with medically and surgically intractable epilepsy. After vagal stimulator implantation, follow-up continued from 2 months to 30 months, with the study period ending in October 1995. Of the 19 patients, 6 (32%) had more than a 90% reduction in the number of monthly seizures, and 10 (53%) had more than a 50% reduction. Global evaluation scores indicated that only 1 patient had deterioration from baseline, 5 had no change, and the remainder had modest to remarkable improvement. All 3 children with unsuccessful corpuscallosotomy had improvement after implantation of the stimulator, and 5 of 6 children with Lennox-Gastaut syndrome had a 90% reduction of seizures. Five patients required fewer antiepileptic medications, and 1 patient had an increase in medication. Adversities included 2 possible wound infections, 1 instance of generator f! !
ailure, and hoarseness during stimulation in all patients. Changing stimulation parameters to increase the rate of stimulation and reduce the interval between stimulations resulted in improved seizure control in 4 of 5 patients. Periodic VNS was well tolerated by these children and may have a role in the management of refractory epilepsy. MEDLINE ACCESSION NUMBER: 97303740
Record 3 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Felbamate.
AUTHOR(S): Bourgeois-BF
SOURCE (BIBLIOGRAPHIC CITATION): Semin-Pediatr-Neurol. 1997 Mar; 4(1): 3-8 INTERNATIONAL STANDARD SERIAL NUMBER: 1071-9091 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: The development and release of felbamate is characterized by several relatively unique features. Felbamate was submitted to innovative and unconventional clinical trials. It was the first antiepileptic drug (AED) to be tested in a double-blind fashion in patients withdrawn from AEDs for presurgical video/electroencephalogram (EEG) monitoring. In addition, felbamate was the first AED to be tested in double-blind monotherapy trials. Felbamate was also the first drug to be tested in a placebo-controlled trial in children with the Lennox-Gastaut syndrome. When it was first released in North America in 1993, felbamate was the first new antiepileptic drug in 15 years. Finally, after 1 year of being very successfully marked as a drug devoid of the adverse effects of other AEDs, felbamate came very close to being completely withdrawn from the market, because of several cases of fatal bone marrow aplesia and liver toxicity. At the present time, the main indication for felbam! !
ate is in children with Lennox-Gastaut syndrome, and similar forms of epilepsy, who failed to respond to other AEDs. MEDLINE ACCESSION NUMBER: 97251688
Record 4 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Callosotomy for intractable epilepsy: overall outcome. AUTHOR(S): Rougier-A; Claverie-B; Pedespan-JM; Marchal-C; Loiseau-P SOURCE (BIBLIOGRAPHIC CITATION): J-Neurosurg-Sci. 1997 Mar; 41(1): 51-7 INTERNATIONAL STANDARD SERIAL NUMBER: 0390-5616 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: The effectiveness of callosotomy to the control of medically intractable epilepsy is still discussed fifty years after the first reported cases. Nevertheless patient selection, type of seizures and epileptic syndromes are now better determined. Atonic and tonic astatic seizures characterized both by clinical and electroencephalographical specific patterns, are the most responsive. A favorable outcome, from > 50% reduction in seizure frequency to a complete cessation, is obtained from 60 to 80% of the patients. For tonic-clonic seizures, favorable outcome fluctuates from 40% to 80% principally according to the extension of the section. Other types of seizures are not indicated for callosotomy even though some improvement may be observed. Symptomatic secondary generalized epilepsy with predominent unilateral lesion and epileptic focus on bifrontal lobe epilepsy are the most suitable indication. True generalized epilepsies are associated with a less favorable outcome. ! !
Indeed, axial spasms, the most frequent type of drop attacks in the Lennox-Gastaut syndrome, probably do not have a cortical origin. Quality of life and social adjustment are assessed from a cohort of 20 cases of anterior two-third callosotomy. Actual benefits are in close connection with both seizure relief and age at operation. To obtain a gain in social independence not only seizure control has to be better but also surgery must be performed sooner. MEDLINE ACCESSION NUMBER: 97419293
Record 5 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: [Topiramate, a new antiepileptic drug] AUTHOR(S): Herranz-JL
SOURCE (BIBLIOGRAPHIC CITATION): Rev-Neurol. 1997 Aug; 25(144): 1221-5 INTERNATIONAL STANDARD SERIAL NUMBER: 0210-0010 LANGUAGE OF ARTICLE: SPANISH; NON-ENGLISH ABSTRACT: OBJECTIVE: The characteristics of the new anti-epileptic drug topiramato (TPM) are described: multiple mechanism of action, favourable pharmaco-kinetics, wide therapeutic range and is relatively well tolerated, Development. Its initial usefulness in patients with partial crises resistant to other anti-epileptic drugs has been shown. TPM is equally effective in primary generalized crises, and in patients with the Lennox-Gastaut syndrome, in whom no problem of tolerance has been seen. CONCLUSION: TPM is a valuable drug for the treatment of epilepsy. MEDLINE ACCESSION NUMBER: 97403733
Record 6 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Mental deterioration in childhood epilepsy. AUTHOR(S): Oka-E; Sanada-S; Asano-T; Ishida-T SOURCE (BIBLIOGRAPHIC CITATION): Acta-Med-Okayama. 1997 Aug; 51(4): 173-8 INTERNATIONAL STANDARD SERIAL NUMBER: 0386-300X LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Mental retardation is detected in 20-30% of children with epilepsy at hospitals specializing in treatment of childhood epilepsy. However, the incidence of mental deterioration in childhood epilepsy is not high. In this study, mental deterioration was found in 52 (1.8%) of the 2,880 children with epilepsy at Okayama University Hospital. The patients showing mental deterioration mostly suffered from specific epileptic syndromes, such as West syndrome, Lennox-Gastaut syndrome, severe myoclonic epilepsy in infancy and epilepsy with continuous spike-waves during slow wave sleep. These types of epilepsy show generalized electroencephalographic (EEG) abnormalities. It is presumed that mental deterioration is caused by the total effects of prolonged diffuse EEG abnormalities and the age of the patients. Antiepileptic drugs exert a relatively minor effect on mental deterioration. MEDLINE ACCESSION NUMBER: 97430866
Record 7 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: [Therapeutic options provided by new antiepileptic drugs] AUTHOR(S): Baulac-M; Arzimanoglou-A; Semah-F; Cavalcanti-D SOURCE (BIBLIOGRAPHIC CITATION): Rev-Neurol-Paris. 1997 Feb; 155(1): 21-33 INTERNATIONAL STANDARD SERIAL NUMBER: 0035-3787 LANGUAGE OF ARTICLE: FRENCH; NON-ENGLISH ABSTRACT: The introduction on the French market of vigabatrin, gabapentin and lamotrigine has considerably diversified our conventional therapeutical schemes in epilepsies, as will be as amplified by the arrivals of topiramate, tiagabine and oxcarbazepine. Compared to the conventional drugs, these new products present more favorable pharmacokinetics, no or very weak interactions and a better tolerability, specially regarding the cognitive field. They should be used according to their spectrum of activity, function of their modes of action. In add-on trials on partial epilepsy patients all these new products have shown efficacy on partial and secondarily generalized seizures. Seizure frequency is reduced by at least 50 p. 100 in 30 to 50 p. 100 of the patients. A substantial number of patients can be rendered seizure-free with vigabatrin. Lamotrigine has a broader spectrum, as it is also efficacious on the different seizure types of generalized, symptomatic or idiopathic epile! !
psies. Main adverse events are non-specific central nervous system disturbances such as dizziness, drowsiness, ataxia, tremor or diplopia. More specifically, vigabatrin may induce weight gain and requires closer supervision in case of psychiatric history; lamotrigine which has also probable antidepressant properties, may induce skin rashs, rarely severe. Further data are needed for gabapentin which is now used at daily dosages which are two to three times those used in the initial studies. Gabamimetic agents may be worsening in some cases of generalized epilepsies, more specially on absence and myoclonic seizures. The most obvious benefits, some patients becoming seizure-free, are obtained in cases of intermediate severity, with a bitherapy including one of these new drugs. Developments in children are often delayed. Nevertheless the prognosis, including cognitive outcome, is considerably improved in infantile spasms with vigabatrin and in Lennox-Gastaut syndrome with lamotrig! !
ine and felbamate, the latter being highly toxic. For the moment in France, authorities have limited the use of all these new antiepileptic drugs to adjunctive therapy in epilepsies resisting to conventional drugs. But recent monotherapy data show similar efficacy with better tolerability. Once the pivotal, controlled studies have enabled to obtain regulatory approval, all these compounds must undergo a large-scale evaluation phase in order to better define dosages, long-term tolerability, indications and eventual contra-indications in the various epileptic syndromes, including children. MEDLINE ACCESSION NUMBER: 97441934
Record 8 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Clinical analysis of 22 infants with afebrile cluster seizures. AUTHOR(S): Li-PH; Chi-CS; Mak-SC; Chen-CH; Yang-MT SOURCE (BIBLIOGRAPHIC CITATION): Chung-Hua-Min-Kuo-Hsiao-Erh-Ko-I-Hsueh-Hui-Tsa-Chih. 1997 May-Jun; 38(3): 203-7 INTERNATIONAL STANDARD SERIAL NUMBER: 0001-6578 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: The inclusion criteria for afebrile cluster seizures in infancy are defined as follows: (1) frequency of afebrile seizures at least 2 episodes within 72 hours; (2) seizure onset between 2 months and 3 years of age; (3) excluding febrile convulsion, central nervous system infections, status epilepticus, well-known epileptic syndromes in infancy (e.g. early myoclonic encephalopathy, early infantile epileptic encephalopathy, benign myoclonic epilepsy, infantile spasms. Lennox-Gastaut syndrome), electrolyte imbalance, watery diarrhea, head injury and intoxication. From 1986 to 1996, retrospectively and prospectively 22 patients were collected who fulfilled the above criteria. Based on whether or not a strong family history was present and a history of mild diarrhea was associated with seizure onset, they were divided into three groups: Group I, benign infantile familial convulsions (4 patients); Group II, cluster seizures with mild diarrhea in infancy (5 patients); Grou! !
p III, cluster seizures without diarrhea in infancy (13 patients). Before seizure onset and during follow-up, all of the patients had normal development. The seizure pattern in all was generalized, most tonic type with duration of seizure less than five minutes in the majority. Recurrence rate was 100% in Group I and no recurrence in Group II. In 16 patients who were seizure-free over 12 months, the duration of persistence varied from 1 day to 8 months, and was shortest in Group II (range, 1 to 3 days). It was concluded that the vast majority of afebrile cluster seizures in infancy are benign in nature. Whether anticonvulsant therapy is justified must be individualized. MEDLINE ACCESSION NUMBER: 97374080
Record 9 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Lamotrigine as add-on therapy in adult patients with refractory epilepsy. AUTHOR(S): Yen-DJ; Yiu-CH; Kwan-SY; Lin-YY; Su-MS SOURCE (BIBLIOGRAPHIC CITATION): Chung-Hua-I-Hsueh-Tsa-Chih-Taipei. 1997 May; 59(5): 303-7 INTERNATIONAL STANDARD SERIAL NUMBER: 0578-1337 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: BACKGROUND: Lamotrigine (LTG), one of the newly developed antiepileptic drugs, is efficacious in treating both partial and generalized seizure disorders including Lennox-Gastaut syndrome. Its effect as an add-on therapy was evaluated in 41 adult Chinese patients with refractory epilepsy. Five of the patients were Lennox-Gastaut syndrome and 36 patients had partial epilepsy. METHODS: We started LTG at 25 mg or 50 mg per day, respectively, depending on whether the patients were simultaneously taking valproate (VPA) or not. Then, LTG was increased in steps to maximal dosage (200 mg or 400 mg per day) within seven weeks and maintained for three months while pre-existing antiepileptic drugs remained unchanged. The efficacy of LTG therapy was assessed by the reduction in the overall seizure frequency. Hematological and biochemical parameters were checked before and after the trial in all patients. RESULTS: In all, 38 patients completed the trial. Twenty-two patients (57.9! !
%) had > or = 50% reduction in seizure frequency, including four patients with Lennox-Gastaut syndrome and 18 patients with partial seizures. Among the patients with partial epilepsy, there was no significant difference in the efficacy of LTG whether the seizures were of temporal or extra-temporal origin (p = 0.577). In addition, the efficacy was not determined by the concomitant use of VPA (p = 0.189). Thirteen patients (31.7%) complained of adverse experiences, usually mild and dose-dependent. LTG had to be discontinued in only two patients (4.9%) due to severe side effects. The change in blood cell counts and biochemistry profiles was not significant in any of the patients. CONCLUSIONS: We conclude that LTG is an efficacious and safe antiepileptic drug for add-on therapy in adult patients with refractory epilepsy. MEDLINE ACCESSION NUMBER: 97391316
Record 10 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Polygraphic characterization of the sleep-epilepsy patterns in a hydranencephalic child with severe generalized seizures of the Lennox-Gastaut syndrome. AUTHOR(S): Velasco-M; Velasco-F; Gardea-G; Gordillo-F; Diaz-de-Leon-AE SOURCE (BIBLIOGRAPHIC CITATION): Arch-Med-Res. 1997 Summer; 28(2): 297-302 INTERNATIONAL STANDARD SERIAL NUMBER: 0188-0128 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: This is the report of a hydranencephalic child with severe generalized seizures of the Lennox-Gastaut Syndrome (LGS) who lacked the development of the entire cerebral hemispheres and had preserved the brain stem, cerebellum, hypothalamus and a portion of the thalamus as evidenced by radiological and/or physiological studies. Conventional polygraphic sleep studies in these patients showed presence of scalp EEG and other peripheral, somatic and vegetative signs characterizing the wakefulness, quiet sleep and active sleep stages. Absence of the vertex waves and disrupted sleep spindles were the major qualitative EEG abnormalities. In contrast, quantitative abnormalities in duration, latency and number of sleep cycles found in this patient were similar to those found in other children with Idiopathic Lennox-Gastaut Syndrome (ILGS). A substantial reduction in the number of interictal EEG spikes and a shortening of the ictal clonic EEG activities without concomitant EMG j! !
erks were the most distinct epileptiform abnormalities in this child. In contrast, his basic polygraphic patterns of the tonic and apneic seizures were similar to those found in other children with ILGS. Data obtained from this child suggest that both the sleep stages and the generalized seizures of the ILGS basically depend more on the integrity of the brain stem than on the telencephalic structures. MEDLINE ACCESSION NUMBER: 97348642
Record 11 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: 'We think your son has Lennox-Gastaut syndrome'--a case study of monosodium glutamate's possible effect on a child. AUTHOR(S): Shovic-A; Bart-RD; Stalcup-AM SOURCE (BIBLIOGRAPHIC CITATION): J-Am-Diet-Assoc. 1997 Jul; 97(7): 793-4 INTERNATIONAL STANDARD SERIAL NUMBER: 0002-8223 LANGUAGE OF ARTICLE: ENGLISH
MEDLINE ACCESSION NUMBER: 97359644
Record 12 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Prospective, open-label, add-on study of lamotrigine in 56 children with intractable generalized epilepsy. AUTHOR(S): Farrell-K; Connolly-MB; Munn-R; Peng-S; MacWilliam-LM SOURCE (BIBLIOGRAPHIC CITATION): Pediatr-Neurol. 1997 Apr; 16(3): 201-5 INTERNATIONAL STANDARD SERIAL NUMBER: 0887-8994 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: The role of lamotrigine (LTG) in childhood epilepsy is emerging. We evaluated the efficacy and adverse effects of LTG in an open, prospective study of 56 children with generalized epilepsies. Six (11%) children became seizure-free, and 24 (43%) had greater than 50% reduction in seizure frequency. LTG was effective against a broad range of generalized seizure types. Three of 15 patients with Lennox-Gastaut syndrome achieved complete seizure control and eight demonstrated 50 to 99% improvement in seizure control. Increase in seizures (7) and rash (5) were the most common side effects. After valproate was discontinued, LTG therapy was resumed, with no recurrence of rash in any patient. This study suggests that LTG may be a useful drug in the treatment of generalized epilepsies in children.MEDLINE ACCESSION NUMBER: 97308283
Record 13 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Community-based study of Lennox-Gastaut syndrome. AUTHOR(S): Heiskala-H
SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1997 May; 38(5): 526-31 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: PURPOSE: Before 1986, the spectrum of childhood epilepsies, including Lennox-Gastaut syndrome (LGS) and Doose syndrome (DS), known collectively as "epilepsia myoclonica astatica," was believed to represent a single disease. More recently, some investigators have considered these syndromes to be parts of a continuum. To clarify these theories, neurobiologic factors of the syndromes were studied to determine which qualities were shared and which were unique. METHODS: A retrospective (1975-1985), community-based (Helsinki metropolitan area and the province of Uusimaa) study was designed to seek children with features of LGS and DS. It was assumed that recall bias and the selection of documented history would be similar throughout the group. Ranks of increasing pathology were assigned to different seizure types, EEG results, and drug treatments. A similar procedure was applied to epidemiologic data. Spearman rank-order correlations were calculated to determine which fea! !
tures correlated with LGS and which correlated with less severe epilepsy. RESULTS: The survey comprised 75 patients with broadly defined LGS. The annual incidence was 2 in 100,000 children aged 0 to 14 years. Prenatal or perinatal abnormalities did not correlate with severity of epilepsy. As compared with the relatively favorable ranks, the severe epilepsy ranks were more often associated with an early onset of epilepsy, an infectious disease at the onset, delayed development before epilepsy, abnormalities in neurologic or neuroradiologic examinations, and a deteriorating course of the condition. CONCLUSIONS: Patients with LGS are more likely than patients with less severe epilepsy to have a younger age at onset of epilepsy, an infection or both, and a deteriorating course of the condition. MEDLINE ACCESSION NUMBER: 97328039
Record 14 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Vigabatrin.
AUTHOR(S): Shields-WD; Sankar-R
SOURCE (BIBLIOGRAPHIC CITATION): Semin-Pediatr-Neurol. 1997 Mar; 4(1): 43-50 INTERNATIONAL STANDARD SERIAL NUMBER: 1071-9091 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Vigabatrin is a structural analogue of gamma amino butyric acid (GABA), which binds irreversibly to GABA-transaminase causing increased brain levels of GABA. It is an important advance in the medical management of children with epilepsy. It appears to be particularly effective in the treatment of infantile spasms, especially when caused by tuberous sclerosis. It is also effective in the treatment of partial seizures and some generalized seizures including those of the Lennox-Gastaut syndrome. However, myoclonic seizures may be made worse by vigabatrin. It is not yet approved for use in the United States but it is approved throughout most of the rest of the world including Canada and Mexico. Release in the United States is expected in the near future. MEDLINE ACCESSION NUMBER: 97251693
Record 15 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Topiramate.
AUTHOR(S): Glauser-TA
SOURCE (BIBLIOGRAPHIC CITATION): Semin-Pediatr-Neurol. 1997 Mar; 4(1): 34-42 INTERNATIONAL STANDARD SERIAL NUMBER: 1071-9091 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Clinical evidence is emerging that topiramate (TPM) may be effective in pediatric epilepsies. Topiramate pharmacokinetics appear to be linear in children when administered in dosages up to 9 mg/kg/d. Mean oral clearance is 44% to 54% higher in children when compared with historical data from adults; steady-state plasma TPM concentrations for the same mg/kg dose are expected to be 33% lower in children. While double-blind, placebo-controlled trials of adjunctive TPM therapy in Lennox-Gastaut syndrome, primary generalized tonic-clonic seizures, and refractory partial-onset seizures in children are ongoing, preliminary results of pilot studies and the open-label extension phases of these double-blind studies suggest TPM may be effective as adjunctive therapy in a broad range of seizure disorders. In the open-label extension phase of a double-blind trial, the proportion of patients with Lennox-Gastaut syndrome experiencing 50% or greater reduction in seizures was 47%. L! !
ikewise, seizures were reduced 50% or more with TPM adjunctive therapy in 64% of children with partial-onset seizures and in 67% of patients with primary generalized tonic-clonic seizures treated with open-label during the extension phase of two separate double-blind studies. Preliminary experience suggests that TPM monotherapy can be successfully substituted for another antiepileptic drug in some children. The results of the well-controlled trials are needed to confirm these preliminary observations of TPM effectiveness in pediatric epilepsies. MEDLINE ACCESSION NUMBER: 97251692
Record 16 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: [Experience with Health Quality of Life Questionnaire for the epileptic child (CAVE)] AUTHOR(S): Casas-Fernandez-C
SOURCE (BIBLIOGRAPHIC CITATION): Rev-Neurol. 1997 Mar; 25(139): 415-21 INTERNATIONAL STANDARD SERIAL NUMBER: 0210-0010 LANGUAGE OF ARTICLE: SPANISH; NON-ENGLISH ABSTRACT: INTRODUCTION: The practical application of a questionnaire designed to evaluate the quality of life of an epileptic child, known as the CAVE test, is described. This is the first scale of its kind. The test consists of 8 parameters: conduct, attendance at school, learning, independence, social relationships, frequency of crises, intensity of crises and parental opinion. Each aspect is given a score, from a minimum of 1 point to a maximum of 5 points. MATERIAL AND METHODS: A total of 203 tests were carried out. 179 of these were considered to be useful. Nine Spanish neuropediatric departments took part in this study. The age limits were from 0 to 14 years old. The best results showed a direct relationship to the children's age, idiopathic etiology and monotherapy as treatment. The quality of life worsened from those with partial simple crises to those with typical absences and generalized tonic-clonic crises. The lowest scores were found in children with infantile sp! !
asms and in those with Lennox-Gastaut syndrome. There was a clear statistical relationship between the highest score obtained and the best clinical condition, with regard to the frequency and intensity of crises. Parental opinion was in keeping with the clinical evaluation of the disorder to a certain extent. CONCLUSION: The study is still in progress, so as to interview 1000 children altogether. Then any modifications considered advisable will be made by the multicentre group of the Sociedad Espanola de Neurologia Pediatrica, and may be validated internationally. MEDLINE ACCESSION NUMBER: 97229331
Record 17 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: [Which drugs should be chosen for the different types of epilepsy?] AUTHOR(S): Armijo-JA
SOURCE (BIBLIOGRAPHIC CITATION): Rev-Neurol. 1997 Mar; 25(139): 356-66 INTERNATIONAL STANDARD SERIAL NUMBER: 0210-0010 LANGUAGE OF ARTICLE: SPANISH; NON-ENGLISH ABSTRACT: The selection of an antiepileptic drug is based primarily on its efficacy. When the efficacy of several antiepileptic drugs is similar, what is frequently observed, their safety, pharmaco-kinetics, and cost should be also considered. Efficacy, dose-dependent and idiosyncratic toxicity, pharmacokinetics features, and interactions of phenobarbital, phenytoin, carbamazepine, valproate, felbamate, gabapentin, lamotrigine and vigabatrin are comparatively analyzed, and drugs of choice for generalized epilepsies and partial epilepsies are proposed. It is concluded that phenobarbital, primidone and phenytoin are being replaced by carbamazepine and valproate as first choice drugs for most types of epilepsies due to a similar (and even better) efficacy, besides a better safety profile and easier dosing. Features of new antiepileptic drugs make they adequate to be used as add-on drugs in treatment-resistant patients: lamotrigine and felbamate are effective in Lennox-Gastaut sy! !
ndrome, and vigabatrin in West syndrome, most of the new antiepileptic drugs are well tolerated and show less interactions than the standard ones. However, the efficacy and safety of the new antiepileptic drugs in monotherapy, and their long-term safety have not been yet established, and they will cost more than the standard drugs. Therefore, the new antiepileptic drugs are currently considered as second choice drugs, although vigabatrin has been proposed as a first choice drug in West syndrome. MEDLINE ACCESSION NUMBER: 97229327
Record 18 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Preliminary observations on topiramate in pediatric epilepsies. AUTHOR(S): Glauser-TA
SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1997; 38 Suppl 1: S37-41 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Preliminary results of studies of topiramate (TPM) in children are now available. In a pharmacokinetic study among 18 male and female children, target daily dosages of up to 9 mg/kg/day were evaluated. TPM pharmacokinetics in children were linear. Mean TPM oral clearance (CL/F) was 44-54% higher in children [depending on concomitant antiepileptic drugs (AEDs)] compared with historical data from adults, and steady-state plasma TPM concentrations for the same mg/kg dose were 33% lower in children compared with historical adult data. In a long-term, open-label pilot study of adjunctive TPM therapy in 18 patients with Lennox-Gastaut syndrome, six of the eight patients (75%) still receiving TPM report a greater than 50% reduction in total seizures, with the best results observed in tonic-atonic, atypical absence, and generalized tonic-clonic seizures. Subsequent large double-blind, placebo-controlled trials of adjunctive TPM therapy in Lennox-Gastaut syndrome and refract! !
ory partial-onset pediatric epilepsy are ongoing, with high percentages of enrolled patients in both studies completing double-blind treatment and entering long-term TPM open extension trials. A small TPM monotherapy substitution trial in children with well controlled partial onset seizures showed that TPM monotherapeutic substitution can be achieved successfully with an acceptable amount of adverse experiences with a weekly increase of 1 mg/kg/day to a maximal dose of 3 mg/kg/day. These preliminary results suggest that TPM may be a useful new AED in pediatric patients with a variety of seizure disorders. MEDLINE ACCESSION NUMBER: 97246720
Record 19 of 44 in MEDLINE EXPRESS (R) 1997
TITLE: Lamotrigine adjunctive therapy in childhood epileptic encephalopathy (the Lennox Gastaut syndrome). AUTHOR(S): Donaldson-JA; Glauser-TA; Olberding-LS SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1997 Jan; 38(1): 68-73 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: PURPOSE: We assessed efficacy and safety of adjunctive lamotrigine (LTG) therapy in patients with the Lennox-Gastaut syndrome (LGS). METHODS: The study was a single-center, retrospective chart review of open-label adjunctive LTG therapy in patients with LGS. Initial LTG dose and titration was dependent on concomitant antiepileptic drugs (AEDs). Efficacy was based on the change in seizure frequency between the initiation of LTG therapy and December 1, 1995 (or LTG discontinuation). Seizure diaries were used to count patient seizures. A secondary evaluation of efficacy was a parental or guardian assessment of the patient's global status. The evaluation of safety involved chart review for treatment-emergent adverse events (AE). RESULTS: Data from 16 LGS patients were analyzed. Fifty-three percent (8 of 15) had a > 50% reduction in seizure frequency with LTG adjunctive therapy. Tonic, atonic, generalized tonic-clonic (GTCS), and atypical absence seizure frequency but no! !
t myoclonic seizure frequency decreased significantly during LTG therapy. Fifty-three percent of the patient's parents (8 of 15) reported that their child's quality of life (QOL) was much or very much improved during the study. The major treatment-emergent AE were infection (50%, 8 of 16) and sleep disturbance (19%, 3 of 16). A rash was noted in 13% (2 of 16) of the patients and resulted in LTG discontinuation in 1. No clinically significant changes were noted in neurologic examination or laboratory tests during the study. CONCLUSIONS: Our results indicate that LTG adjunctive therapy is effective and well tolerated in patients with LGS. MEDLINE ACCESSION NUMBER: 97176647
Record 20 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Can eliminating monosodium glutamate from the diet affect Lennox-Gastaut syndrome? [letter] AUTHOR(S): Auer-RN
SOURCE (BIBLIOGRAPHIC CITATION): J-Am-Diet-Assoc. 1998 Aug; 98(8): 857 INTERNATIONAL STANDARD SERIAL NUMBER: 0002-8223 LANGUAGE OF ARTICLE: ENGLISH
MEDLINE ACCESSION NUMBER: 98376512
Record 21 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Positive and negative psychotropic effects of lamotrigine in patients with epilepsy and mental retardation. AUTHOR(S): Ettinger-AB; Weisbrot-DM; Saracco-J; Dhoon-A; Kanner-A; Devinsky-O SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1998 Aug; 39(8): 874-7 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: PURPOSE: To describe significant positive or negative psychotropic effects of lamotrigine (LTG) observed in epilepsy patients with mental retardation (MR). METHODS: Seven mentally retarded epilepsy patients, [5 with Lennox-Gastaut syndrome (LGS)] who experienced significant behavioral improvements or worsening after addition of LTG to their medication regimen were studied. RESULTS: LTG produced behavioral improvements in 4 patients. Patient 1, a 14-year-old girl, had LTG added to valproate (VPA) and thioridazine, resulting in diminished lethargy, less hyperactivity, and more appropriate speech. In a 17-year-old boy (patient 2) LTG added to VPA, phenytoin (PHT), and gabapentin (GBP) lessened irritability and hyperactivity. In patient 3, a 41-year-old woman, LTG added to PHT, VPA, and carbamazepine (CBZ) diminished lethargy and enhanced her social interactions. In patient 4, a 27-year-old man, LTG monotherapy diminished irritability and hyperactivity. Adverse behavior! !
al effects were noted in 3 patients. In patient 5, a 43-year-old man, LTG added to PHT, phenobarbital (PB), lorazepam, sertraline, and thioridazine produced irritability, hyperactivity, and poor cooperation. In patient 6, a 29-year-old woman, LTG added to VPA produced frequent screaming, temper tantrums, increased rocking movements, and hyperactivity. In patient 7, a 29-year-old man, LTG added to VPA and PHT resulted in severe exacerbation of baseline behaviors, including self-injurious activity, temper tantrums, and failure to obey simple instructions. CONCLUSIONS: In some patients with epilepsy and MR, LTG has significant positive or negative effects on behavior. MEDLINE ACCESSION NUMBER: 98365109
Record 22 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Vagus nerve stimulation in 16 children with refractory epilepsy. AUTHOR(S): Lundgren-J; Amark-P; Blennow-G; Stromblad-LG; Wallstedt-L SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1998 Aug; 39(8): 809-13 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: PURPOSE: Vagus nerve stimulation (VNS) has been reported to produce >90% reduction in the number of seizures in children with intractable epilepsy. These encouraging results need confirmation. METHODS: Sixteen children, 10 boys and 6 girls aged 4-19 years, were treated with VNS (Cyberonics, Webster, TX, U.S.A.) for 12-24 months. Seizure frequency, seizure severity, changes in quality of life (QOL: visual analogue scale), and side effects were recorded. Eight children had partial and 8 had generalized seizures; 4 of the latter had Lennox-Gastaut syndrome (LGS). RESULTS: During the tenth to twelfth month of VNS, 6 of 16 children experienced > or =50% reduction in seizure frequency. One girl became seizure-free. Seizure severity showed an average decrease in the score from 15 to 11. After 10 months of treatment, QOL was estimated to have improved > or =50% in 6 of 16 children. Reduction in seizure frequency, decreased seizure severity, and reported improvement in QOL d! !
id not entirely coincide. Six children experienced hoarseness, 1 had neck pain, 2 had hypersalivation, 2 experienced tiredness, 2 had aspiration episodes during liquid intake, and 6 had electrical transmission problems; in 4 the problem has been surgically corrected. Five stimulators were turned off due to lack of efficacy. CONCLUSIONS: Six of 16 children with refractory epilepsy treated with VNS improved, with a reduction not only in seizure frequency but also in seizure severity and in QOL. MEDLINE ACCESSION NUMBER: 98365099
Record 23 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Treatment of absence status in the Lennox-Gastaut syndrome with propofol. AUTHOR(S): Crouteau-D; Shevell-M; Rosenblatt-B; Dilenge-ME; Andermann-F SOURCE (BIBLIOGRAPHIC CITATION): Neurology. 1998 Jul; 51(1): 315-6 INTERNATIONAL STANDARD SERIAL NUMBER: 0028-3878 LANGUAGE OF ARTICLE: ENGLISH
MEDLINE ACCESSION NUMBER: 98337591
Record 24 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Intractable epilepsy in a population-based series of mentally retarded children. AUTHOR(S): Steffenburg-U; Hedstrom-A; Lindroth-A; Wiklund-LM; Hagberg-G; Kyllerman-M SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1998 Jul; 39(7): 767-75 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: PURPOSE: The characteristics of intractable epilepsy were analyzed in a population-based study of active epilepsy in mentally retarded children aged 6-13 years. METHODS: Diagnostic registers, EEG laboratory registers, and registers for the Education of the Subnormal were searched. Medical files were scrutinized. Clinical examinations and interviews with parents and caregivers or both were performed. EEG recordings, computed tomography (CT) and magnetic resonance imaging (MRI) of the CNS were reevaluated. RESULTS: Forty-five percent (44 of 98) of the children with mental retardation (MR) and active epilepsy had intractable seizures, defined as one or more seizures every day or week. The median age at onset was 0.8 years, as compared with 3.0 years for those with controlled epilepsy. Predictive factors for frequent seizures were the number of seizure types, severe MR, status epilepticus (SE) and tonic seizures. Epileptiform EEG activity was present in 91%, and focal a! !
ctivity in 65%. Brain lesions were detected on CT and MRI in 70%, with generalized lesions in 60%. Concurrent focal epileptiform activity and focal brain lesions on CT/MRI were detected in 26%. The percentages and prevalence rates for infantile spasms (IS) and Lennox-Gastaut syndrome (LGS) were 18% (0.25 in 1,000) and 7% (0.06 in 1,000), respectively. One of 8 children with IS had had previous neonatal seizures, 3 had SE and 1 later developed LGS. CONCLUSIONS: Children with MR and intractable epilepsy have a high frequency of severe MR and additional major neuroimpairments. EEG recordings frequently showed focal changes despite generalized lesions in neuroradiology. MEDLINE ACCESSION NUMBER: 98333981
Record 25 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Topiramate. Clinical profile in epilepsy. AUTHOR(S): Sachdeo-RC
SOURCE (BIBLIOGRAPHIC CITATION): Clin-Pharmacokinet. 1998 May; 34(5): 335-46 INTERNATIONAL STANDARD SERIAL NUMBER: 0312-5963 LANGUAGE OF ARTICLE: ENGLISHABSTRACT: Topiramate, a sulfamate-substituted monosaccharide, is a new antiepileptic drug (AED) approved as adjunctive therapy for partial-onset seizures in adults. Topiramate is rapidly absorbed, has linear pharmacokinetics, minimal protein binding and a long half-life facilitating a twice-daily dosage regimen. Topiramate has little effect on the plasma concentrations of other AEDs with the exception of phenytoin, concentrations of which may increase in some patients when topiramate is added to the therapy. Topiramate metabolism is increased when administered with carbamazepine or phenytoin. In the absence of enzyme-inducing AEDs, topiramate is eliminated primarily by renal excretion, with 50 to 80% of a dose excreted as unchanged topiramate. In 6 double-blind, placebo-controlled trials, topiramate was shown to be well tolerated and effective as adjunctive therapy for partial-onset seizures in adults. Topiramate consistently reduced seizures across all patient groups defined! !
by age, gender and baseline seizure frequency. Adverse effects were generally mild-to-moderate CNS-related effects and often resolved spontaneously or with slowing of topiramate titration and/or reduction of the dosage of concomitant AEDs. Clinical studies are currently evaluating the effectiveness and safety of topiramate as monotherapy and adjunctive therapy in children with partial seizures, in patients with Lennox-Gastaut syndrome and in patients with generalised tonic-clonic seizures of non-focal onset. Preliminary findings suggest that topiramate has a broad spectrum of clinical use. MEDLINE ACCESSION NUMBER: 98254971
Record 26 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: The benign occipital epilepsies of childhood: an overview of the idiopathic syndromes and of the relationship to migraine. AUTHOR(S): Andermann-F; Zifkin-B
SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1998; 39 Suppl 4: S9-23 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Benign occipital epilepsy of childhood is an idiopathic partial epilepsy syndrome with elementary visual symptomatology, frequently associated with other ictal phenomena. Seizures are usually followed by postictal headache and are often associated with interictal occipital rhythmic paroxysmal EEG activity that appears only after eye closure. In some children the ictal visual symptoms or the interictal EEG abnormalities may not be demonstrated. The clinical and/or EEG manifestations of other forms of idiopathic partial or generalized epilepsy may be found in association. Occipital spikes in non-epileptic children with defective vision, occipital slow spike-and-wave found in some patients with the Lennox-Gastaut syndrome, focal epilepsy due to occipital lesions, seizures originating in the temporal lobe secondary to an occipital abnormality, and complicated or basilar migraine must be considered in the differential diagnosis. Early-onset benign occipital epilepsy or s! !
eizure susceptibility syndrome deserves to be considered separately. It has been defined by Panayiotopoulos as consisting of brief, infrequent attacks or prolonged status epilepticus and characterized by ictal deviation of the eyes and/or head and vomiting, occurring in children usually between the ages of 3 and 7 years. Advances in molecular genetics will help decide whether these two disorders are indeed distinct. Benign occipital and benign rolandic epilepsy are commonly associated with migraine. The selective involvement of the occipital lobe in migraine has not been fully explained. The association between benign occipital epilepsy and migraine is likely related to this underlying mechanism as well. The "fixation off" phenomenon or blocking of occipital epileptic discharges by eye opening is not specific to benign occipital epilepsy of childhood and may be found in symptomatic epilepsies as well. Migraine and epilepsy are distinct disorders, both as far as their pathophys! !
iologic mechanisms and clinical symptomatology are concerned. There is however an overlap in some patients and a causal relationship may exist in some, leading to clinically distinct migraine epilepsy syndromes. Here too, clarification of the molecular basis of migraine and of epilepsy will throw light on the nature of the relationship between the two conditions. MEDLINE ACCESSION NUMBER: 98299702
Record 27 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: [Ketogenic diet: efficacy and tolerability in childhood intractable epilepsy] AUTHOR(S): Caraballo-R; Tripoli-J; Escobal-L; Cersosimo-R; Tenembaum-S; Palacios-C; Fejerman-N SOURCE (BIBLIOGRAPHIC CITATION): Rev-Neurol. 1998 Jan; 26(149): 61-4 INTERNATIONAL STANDARD SERIAL NUMBER: 0210-0010 LANGUAGE OF ARTICLE: SPANISH; NON-ENGLISH ABSTRACT: INTRODUCTION: Prospective study to evaluate efficacy of ketogenic diet (KD) in the treatment of children with intractable epilepsies (IE). Tolerability of the KD was also considered. MATERIAL AND METHODS: Criteria for inclusion were: 1. Epilepsy refractary to treatments with antiepileptic drugs (AED) in monotherapy and combining two or three AED. 2. Acknowledgment of blood levels of these drugs in therapeutic range. 3. Absence of liver or kidney disease, metabolic abnormalities, inborn errors of metabolism or other progressive encephalopathies. 4. Family supposedly in economic and psychologic conditions to accept the difficulties of strictly maintaining KD. We used a classic KD following the criteria suggested by the John Hopkins Pediatrics Epilepsy Center. Baseline neurological and physical examination, EEG, blood chemistry including lipid profile were obtained prior to initiative and during the KD. KD efficacy was measured as percent reduction of baseline seizures! !
frequency, considering positive results as reductions of 50% or over. Acceptance of the diet and quality of life were specially considered. Eighteen patients with ages from 2 to 11 years were admitted. Ten of them were males. Diagnosis followed the last Classification of Epileptic Syndromes of the ILAE, and distribution was: Symptomatic partial epilepsies, 8 cases (one had West syndrome at age 5 months); cryptogenic partial epilepsies, 1 case; Lennox-Gastaut syndrome, 2 cases; severe myoclonic epilepsy of infancy, 6 cases and epilepsy with myoclonic astatic seizures 1 case. RESULTS: Four patients were not able to achieve persistent ketosis either due to patient's rejection of KD or to parents non compliance. KD was kept for at least two months before considering failures. Five patients did not show significant improvement and KD was stopped. At present nine patients have been on KD from 6 to 24 months (average 16 months). Four of them showed a 75-100% reduction in seizures fr! !
equency and in three the reduction was of 50-75%. CONCLUSIONS: KD was fairly well tolerated by 14 of 18 children and their families. Fifty percent of the 14 patients complying KD showed significant improvement in seizure frequency and in quality of life. Due to the small number of patients and short follow-up, we can not speculate about results in relation to each epileptic syndrome, neither the risk of late complications. MEDLINE ACCESSION NUMBER: 98194403
Record 28 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Epilepsy in childhood: an audit of clinical practice. AUTHOR(S): Carpay-HA; Arts-WF; Geerts-AT; Stroink-H; Brouwer-OF; Boudewyn-Peters-AC; van-Donselaar-CA SOURCE (BIBLIOGRAPHIC CITATION): Arch-Neurol. 1998 May; 55(5): 668-73 INTERNATIONAL STANDARD SERIAL NUMBER: 0003-9942 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: BACKGROUND: It is not known how many children with epilepsy may not need treatment with antiepileptic drugs (AEDs), how many respond unsatisfactorily to subsequent treatment regimens, and how many achieve "acceptable control" despite lack of remission. METHODS: In a prospective multicenter hospital-based study, 494 children with a broad range of seizure types and types of epilepsy were followed up for at least 2 years. There was no standard treatment protocol. We describe the treatment strategies applied to these children by the neurologists in charge and outcome with respect to remission from seizures. RESULTS: Treatment was initially withheld in 29% of the children, and after 2 years 17% still had not received any AEDs. There were no serious complications caused by withholding treatment. Of the children treated with AEDs, 60% were still using the first AED after 2 years; 80% received monotherapy and 20%, polytherapy. Children with severe symptomatic epilepsies, su! !
ch as the West or Lennox-Gastaut syndrome, received polytherapy early on in the course of treatment. When 3 regimens had failed, the chance of achieving a remission of more than 1 year with subsequent regimens was 10%. Nevertheless, 15 of 50 children receiving AEDs in whom the "longest remission ever" was less than 6 months did achieve acceptable seizure control according to the neurologist in charge of treatment. Hence, of 494 children, only 35 (7%) developed an intractable form of epilepsy, defined as failure to bring seizures under acceptable control. CONCLUSIONS: A substantial percentage of children with new-onset epilepsy did not need treatment with AEDs. Chances of achieving a good outcome declined with subsequent treatment regimens. Not all children with recurrent seizures were suffering from intractable epilepsy; some had achieved acceptable control of seizures. MEDLINE ACCESSION NUMBER: 98266760
Record 29 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: [Diagnostic focus on the child with epilepsy and neuropsychological deterioration] AUTHOR(S): Rodriguez-Barrionuevo-AC; Bauzano-Poley-E; Rodriguez-Vives-MA SOURCE (BIBLIOGRAPHIC CITATION): Rev-Neurol. 1998 Feb; 26(150): 322-30 INTERNATIONAL STANDARD SERIAL NUMBER: 0210-0010 LANGUAGE OF ARTICLE: SPANISH; NON-ENGLISH ABSTRACT: Symptomatic epilepsy secondary to hereditary metabolic or degenerative disorders, is usually associated to neurological deterioration. Though epilepsy by itself does not induce neurological deterioration, we should remind that some epileptics encephalopathies, such as the West or Lennox-Gastaut syndromes, do actually induce limited neurological deterioration. Furthermore, in some forms of complex partial epilepsy, motor problems and behavior disorders can be observed, specially in adolescents with temporary lobe epilepsy. Other forms of epilepsy, such as the atypical benign partial epilepsy or the Landau-Kleffner syndrome, can present a certain degree of cognitive deterioration in the evolution, although they can recover later lost functions, totally or partially. The evolution of some refractory epilepsy, as patients are submitted to a multiple treatments, can make us suspect a degenerative disease. In some cases, the diagnosis of the hereditary metabolic and hered! !
odegeneratives diseases can be made by the characteristics of the seizures but in most cases the diagnosis will be established by the symptoms of the basic disease and the lab data. MEDLINE ACCESSION NUMBER: 98224202
Record 30 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: [Diagnostic focus on the child with generalized seizures] AUTHOR(S): Casas-Fernandez-C
SOURCE (BIBLIOGRAPHIC CITATION): Rev-Neurol. 1998 Feb; 26(150): 311-21 INTERNATIONAL STANDARD SERIAL NUMBER: 0210-0010 LANGUAGE OF ARTICLE: SPANISH; NON-ENGLISH ABSTRACT: We have carried out an overall analysis of the diagnostic approach to generalized epileptic crises in children. It is emphasized that the clinical history is an essential factor in reaching the correct diagnosis. This is inevitably followed by a general and then a specifically neurological physical examination. Four parameters are considered in order to reach an initial diagnosis: a) The probability of the various types of crises in view of the timing of the clinical features. b) Knowledge of the clinical characteristics of the types of epileptic crises which may occur. c) The interrelation of these two parameters, seeking a specific syndrome which would fit the facts. d) Consideration of the most usual etiological factors of the tentative diagnosis. Subsequently the necessary complementary tests are carried out, but these are always based on rational clinical grounds. The commonest clinical and electroencephalic characteristics of generalized epileptic crises (exce! !
pt for myoclonic crises) are discussed. These are neonatal crises, 'absence', generalized tonic-clonic crisis, generalized crises with predisposing activation mechanisms, West's syndrome and the Lennox-Gastaut syndrome. The diagnostic value of other complementary tests in such crises is considered. MEDLINE ACCESSION NUMBER: 98224201
Record 31 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: The efficacy of lamotrigine in children and adolescents with refractory generalized epilepsy: a randomized, double-blind, crossover study. AUTHOR(S): Eriksson-AS; Nergardh-A; Hoppu-K SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1998 May; 39(5): 495-501 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: PURPOSE: We report a double-blind, placebo-controlled crossover study of lamotrigine (LTG) as add-on treatment in therapy-resistant, generalized epilepsy in children and adolescents (n = 30). METHODS: Twenty patients had Lennox-Gastaut syndrome. Each patient acted as his or her own control. LTG and placebo were randomly added to existing antiepileptic medication (AEDs). The LTG dosage was individualized in an open phase preceding the placebo/treatment phase. Patients who responded to LTG in the open phase went on to the double-blind phase. "Responders " were defined as patients with a >50% seizure reduction or less severe seizures or both, or improved behavior or improved motor skills or both. "Nonresponders" were defined as children who showed no positive effects of LTG with plasma levels of < or = 10 microg/ml or children who had adverse events during the open phase. RESULTS: There was a clear statistically significant reduction of seizure frequency in LTG compare! !
d with placebo treatment. None of the children studied showed abnormal biochemical or hematologic findings, or changes in plasma levels of concomitantly administered AEDs. CONCLUSIONS: LTG is a well-tolerated and effective treatment in children with intractable generalized epilepsies, including those with Lennox-Gastaut syndrome. The study design allowed a double-blind placebo-controlled assessment of LTG although the participating children used 19 different AED combinations at entry. MEDLINE ACCESSION NUMBER: 98255640
Record 32 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Topiramate: a new antiepileptic drug. AUTHOR(S): Markind-JE
SOURCE (BIBLIOGRAPHIC CITATION): Am-J-Health-Syst-Pharm. 1998 Mar 15; 55(6): 554-62 INTERNATIONAL STANDARD SERIAL NUMBER: 1079-2082 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: The pharmacology, pharmacokinetics, clinical efficacy, adverse effects, drug interactions, and dosage of topiramate are reviewed. Topiramate is indicated for use in the adjunctive treatment of adult partial-onset epilepsy. A sulfamate-substituted monosaccharide, it is structurally distinct from other antiepileptic agents. Topiramate acts by blocking the spread of seizures. Oral topiramate has high bioavailability and low protein binding, and as monotherapy its half-life permits once- or twice-daily administration. The drug is excreted largely unchanged in the urine. Clinical trials have shown that topiramate is effective as adjunctive therapy in treating adult partial-onset epilepsy with or without secondarily generalized seizures. In adults with refractory partial epilepsy, topiramate has shown efficacy when carbamazepine or phenytoin has failed. Topiramate may also be effective against partial-onset epilepsy and Lennox-Gastaut syndrome in children, but more pediat! !
ric studies are needed. CNS adverse effects are the most common; weight loss and nephrolithiasis have also been reported. The drug does not appear to interact significantly with other antiepileptic agents, but enzyme inducers like phenytoin and carbamazepine can decrease serum topiramate levels by 50%. The initial dosage is 50 mg nightly for seven nights, followed by an increase weekly to 400 mg/day in two divided doses. Topiramate is more costly than other anticonvulsants; however, drug therapy accounts for less than 10% of the total direct cost of epilepsy treatment. Topiramate offers an effective, well-tolerated option in patients with adult partial-onset seizures. MEDLINE ACCESSION NUMBER: 98205975
Record 33 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Pathogenesis of cortical myoclonus studied by magnetoencephalopathy. AUTHOR(S): Mima-T; Nagamine-T; Ikeda-A; Yazawa-S; Kimura-J; Shibasaki-H SOURCE (BIBLIOGRAPHIC CITATION): Ann-Neurol. 1998 May; 43(5): 598-607 INTERNATIONAL STANDARD SERIAL NUMBER: 0364-5134 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Myoclonus-associated cortical activities were studied by simultaneous recording of a magnetoencephalogram and an electroencephalogram in 6 patients with cortical myoclonus due to various causes. Cortical activities were averaged, with respect to the precise onset of the myoclonic jerk, to evaluate the myoclonus-associated cortical magnetic fields. The estimated generator of their earliest peak was localized at the contralateral precentral gyrus in all patients. As judged from the direction of the electrical current, surface positive activity preceding the electromyographic discharge was detected in 3 patients with cortical reflex myoclonus and in 1 patient with possible corticobasal degeneration. In contrast, in the remaining 2 patients (Lennox-Gastaut syndrome and Alzheimer's disease), magnetic fields time-locked to the myoclonic jerk were associated with surface negative activity at the precentral cortex. The present study, applying for the first time an off-line ! !
jerk-locked back-averaging analysis to magnetoencephalography, demonstrated the important role of the precentral cortex in generating spontaneous myoclonus. It is most likely that the differing polarity of the electromagnetic activity reflects the differing activation patterns within the cortical laminar structure in the precentral area, underlying the generation of various types of myoclonus. MEDLINE ACCESSION NUMBER: 98244752
Record 34 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Evidence of late-onset infantile spasms. AUTHOR(S): Bednarek-N; Motte-J; Soufflet-C; Plouin-P; Dulac-O SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1998 Jan; 39(1): 55-60 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: PURPOSE: To underline the unusual but possible occurrence of epileptic spasms (ES) in children >1 year of age. METHODS: Cases in whom onset of spasms occurred after 1 year of age were identified through a retrospective review of the records of all patients referred for ES to the Saint-Vincent de Paul Hopital (Paris) and American Memorial Hospital (Reims) between 1974 and 1994. RESULTS: Eighteen cases were identified among the 734 children referred for ES, 18 cases were identified where spasm onset time ranged from 12 to 38 months of age. In 1/3 of the cases, the diagnosis was suspected from the onset of clinical manifestations; in the remaining 2/3, diagnosis was delayed by a mean 6 months (range, 2-25 months). Neurobehavioral regression affected two-thirds of the patients. Modified hypsarrhythmia was present in 11 patients; all but one exhibited major and diffuse spike- and slow-wave activity. EEG abnormalities were detected in the frontal areas in 11 patients. Spa! !
sms were cryptogenic in 9 patients. Steroids were administered to 13 patients; these controlled the spasms in 6 patients. Outcome was favorable for both seizures; cognition favorable in only 2 of the 18 patients. CONCLUSIONS: Beginning after the first year of life, ES, or late-onset infantile spasms, are distinct from early Lennox-Gastaut syndrome, although etiology, prognosis and treatment are similar to that for the classical infantile spasms. MEDLINE ACCESSION NUMBER: 98237123
Record 35 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10TITLE: Antiepileptic drugs as a cause of worsening seizures. AUTHOR(S): Perucca-E; Gram-L; Avanzini-G; Dulac-O SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1998 Jan; 39(1): 5-17 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: PURPOSE: Although the paradoxical ability of antiepileptic drugs (AEDs) to increase seizure activity has been recognized for decades, the underlying mechanisms are poorly understood and few systematic studies have addressed this problem. This article is intended to provide a critical review of available literature on this topic. METHODS: Information was collected by means of computerized literature searches, screening of journals and textbooks, and consultation with colleagues. Mechanisms which potentially might precipitate underlying drug-induced exacerbation of seizures were considered based on available pharmacologic and clinical knowledge. RESULTS: The reviewed information suggests that a paradoxical increase in seizure frequency may occur as a result of at least two separate mechanisms. The first appears to involve a nonspecific manifestation of drug intoxication; seizure-worsening in this context is usually reversible by dosage reduction or elimination of unne! !
cessary polypharmacy. Conversely, the other mechanism may involve a distinct adverse primary action of the drug in specific seizure types or in syndromic forms. Carabamazepine, in particular, has been reported to precipitate or exacerbate a variety of seizures, most notably absence, atonic, or myoclonic seizures in patients with generalized epilepsies characterized by bursts of diffuse and bilaterally synchronous spike-and-wave EEG activity. Phenytoin and vigabatrin also have been implicated in worsening of seizures, particularly generalized seizures, whereas gabapentin has been associated repeatedly with precipitation of myoclonic jerks. Benzodiazepines occasionally have been reported to precipitate tonic seizures, especially when given intravenously to control other seizure types in patients with Lennox-Gastaut syndrome. Seizure deterioration has been reported also with other drugs; though in most cases evidence is still insufficient for meaningful conclusions to be drawn. C! !
ONCLUSIONS: Drug-induced exacerbation of seizures is a serious and common clinical problem that is often unrecognized or overlooked by the treating physician. Its occurrence appears to be related to three possible causes: an incorrect diagnosis of seizure type or syndromic form, lack of knowledge about certain drugs that are contraindicated in specific types of epilepsies, or to prescription of excessive drug dosages and drug combinations. Further studies are required to evaluate the prevalence of this phenomenon of drug-induced exacerbation of seizures, to investigate its mechanisms in greater detail and to characterize additional prognostic factors that may be used for early identification of patients at risk. MEDLINE ACCESSION NUMBER: 98237117
Record 36 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Prevalence and descriptive epidemiology of Lennox-Gastaut syndrome among Atlanta children. AUTHOR(S): Trevathan-E; Murphy-CC; Yeargin-Allsopp-M SOURCE (BIBLIOGRAPHIC CITATION): Epilepsia. 1997 Dec; 38(12): 1283-8 INTERNATIONAL STANDARD SERIAL NUMBER: 0013-9580 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: PURPOSE: To determine the prevalence and descriptive epidemiology of Lennox-Gastaut Syndrome (LGS) among metropolitan Atlanta children. METHODS: We conducted a population-based study of LGS as part of the Metropolitan Atlanta Developmental Disabilities Study (MADDS) using a multiple-source surveillance system for epilepsy and developmental disabilities. Children were defined as having LGS if they had onset of multiple seizure types before age 11 years, with at least one seizure type resulting in falls, and an EEG demonstrating slow spike-wave complexes (<2.5 Hz). Mental retardation (MR) was not used as a diagnostic criterion. RESULTS: The lifetime prevalence of LGS at age 10 years was 0.26/1,000. Ninety-one percent of those with LGS had MR (IQ < or = 70), and 39% had a history of infantile spasms (IS). A comparison of children with LGS and those with multiple seizure types without slow spike-wave complexes demonstrated that those with LGS were more likely to have MR! !
, history of IS, and multiple disabilities (MR, cerebral palsy, blindness, hearing impairment). Seventeen percent of all children in Atlanta with profound MR (IQ < 20) had LGS. CONCLUSIONS: LGS accounts for only 4% of all childhood epilepsy, yet is a significant contributor to childhood morbidity. MEDLINE ACCESSION NUMBER: 98237635
Record 37 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: [Value of the new anticonvulsants in pediatrics] AUTHOR(S): Kohler-M; Hoffmann-GF
SOURCE (BIBLIOGRAPHIC CITATION): Klin-Padiatr. 1998 Jan-Feb; 210(1): 17-23 INTERNATIONAL STANDARD SERIAL NUMBER: 0300-8630 LANGUAGE OF ARTICLE: GERMAN; NON-ENGLISH ABSTRACT: Within the last years five new antiepileptics have become available in Germany. Vigabatrin is a second choice drug against partial seizures, West syndrome and epilepsies in infant encephalopathy syndromes. Lamotrigine and Gabapentin can be used as add-on therapy in partial seizures in children above 12 years of age Felbamate has a high incidence of severe side-effects like aplastic anemia and liver failure. Therefore it should be restricted to the treatment of Lennox-Gastaut syndrome. Oxcarbazepine is not yet on the German market, but is available by import from Austria. Its therapeutic range is similar to carbamazepine with less side-effects. The new antiepileptics discussed have turned out to be useful additional therapeutics, especially in focal epilepsies. There is, however, still limited experience with these drugs in children. So none can as yet be considered a drug of first choice in any epileptic childhood disorder. The classical antiepileptic drugs remain e! !
ssential in antiepileptic therapy.
MEDLINE ACCESSION NUMBER: 98182768
Record 38 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Epidemiology of epilepsy in childhood: a cohort of 440 consecutive patients. AUTHOR(S): Kramer-U; Nevo-Y; Neufeld-MY; Fatal-A; Leitner-Y; Harel-S SOURCE (BIBLIOGRAPHIC CITATION): Pediatr-Neurol. 1998 Jan; 18(1): 46-50 INTERNATIONAL STANDARD SERIAL NUMBER: 0887-8994 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: This study analyzes the relative frequency and age of onset of the different seizure types in a 20-year cohort of a pediatric neurology outpatient clinic of an urban hospital that serves the majority of the city's population (Tel Aviv Medical Center). Only patients with two or more unprovoked seizures were included. Neonatal seizures were excluded from the analysis. The different seizure types in descending order of frequency were: partial seizures secondarily generalized (20.6%), complex partial seizures (12.5%), West syndrome (9%), simple partial seizures (8.6%), benign rolandic epilepsy of childhood (8%), absence seizures (7%), generalized tonic-clonic seizures (6.6%), generalized tonic seizures (5%), myoclonic seizures (2.2%), benign occipital epilepsy of childhood (2%), mixed type seizures (1.8%), Lennox-Gastaut syndrome (1.5%), juvenile myoclonic epilepsy (0.9%), atypical absence (0.6%), Landau-Kleffner syndrome, Ohtahara syndrome, myoclonic astatic epilepsy, ! !
electrical status epilepticus in sleep and startle epilepsy (0.2% each), and unclassified seizures (12%). The findings of this study confirm that there are more pediatric patients with partial seizures (52%) than primary generalized seizures (33%) and that partial seizures secondarily generalized is the most frequent seizure type in this age group. MEDLINE ACCESSION NUMBER: 98151055
Record 39 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Newer antiepileptic drugs: gabapentin, lamotrigine, felbamate, topiramate and fosphenytoin. AUTHOR(S): Curry-WJ; Kulling-DL
SOURCE (BIBLIOGRAPHIC CITATION): Am-Fam-Physician. 1998 Feb 1; 57(3): 513-20 INTERNATIONAL STANDARD SERIAL NUMBER: 0002-838X LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Twenty-five to 40 percent of patients with epilepsy continue to have seizures despite optimal treatment with traditional antiepileptic drugs. Treatment with standard anticonvulsants such as phenytoin, carbamazepine, valproic acid and phenobarbital is often complicated by side effects and by failure to adequately control seizures. Up to 61 percent of patients with seizures report having side effects with antiepileptic drugs. After a 15-year hiatus since the last new antiepileptic drug was marketed, five new drugs have been approved by the U.S. Food and Drug Administration for the control of seizures. Three of these, gabapentin, lamotrigine and topiramate, are approved for use in adults with partial seizures with or without generalization. Felbamate is approved for the above indication and also for use in children with Lennox-Gastaut syndrome, a rare childhood seizure disorder. Felbamate and lamotrigine have the potential of significant side effects and should be pres! !
cribed by physicians experienced in managing patients with complicated epilepsy. Fosphenytoin is a parenteral prodrug of phenytoin that is more tolerable than parenteral phenytoin. MEDLINE ACCESSION NUMBER: 98136376
Record 40 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Lamotrigine in absence and primary generalized epilepsies. AUTHOR(S): Mikati-MA; Holmes-GL
SOURCE (BIBLIOGRAPHIC CITATION): J-Child-Neurol. 1997 Nov; 12 Suppl 1: S29-37 INTERNATIONAL STANDARD SERIAL NUMBER: 0883-0738 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Although lamotrigine has been approved in the United States as adjunctive therapy for partial seizures in patients older than 12 years, there is increasing evidence that it is just as effective, if not more effective, in the treatment of generalized seizures. A large number of open-label studies and some single-blind data, all using lamotrigine as add-on therapy in patients with previously refractory generalized seizures, are available. Controlled studies, some on newly diagnosed, previously untreated patients with generalized seizures are ongoing. Investigations have demonstrated that patients with the following generalized seizure types improve with lamotrigine add-on therapy: Typical and atypical absence, atonic, generalized tonic-clonic, myoclonic, and clonic seizures. Response rates, defined as the percentage of patients with better than 50% reduction in seizure frequency, have been, depending on seizure type, in the range of 30% to 56%, with 0 to 33% of the pa! !
tients becoming seizure free. The best responses have been noted in typical and atypical absences, and atonic seizures. Children and adults appear to have comparable responses. In addition, add-on studies in patients with specific, previously refractory, epilepsy syndromes have demonstrated that the best improvement in seizure control occurs in patients with petit mal epilepsy, "other symptomatic" generalized epilepsies, and in Lennox-Gastaut syndrome, followed by patients with other myoclonic epilepsies, myoclonic absence and West syndrome. Many previously refractory patients are able to achieve lamotrigine monotherapy. However, patients with nonprogressive myoclonic epilepsy have little, if any, response. Early data from ambulatory encephalographic (EEG) recordings in patients with previously refractory absence seizures, and from controlled studies on patients with newly diagnosed typical absence seizures, appear to confirm the efficacy of lamotrigine in those patients. Cont! !
rolled studies are ongoing in patients with absence seizures, in patients with generalized tonic-clonic seizures, and in patients with Lennox-Gastaut syndrome. Dosing in generalized seizures is similar to that for partial seizures. Because of the shorter half-life of lamotrigine in children, as compared to adults, higher (mg/kg) doses are often needed in young patients. We conclude that lamotrigine is a promising drug for absence and primary generalized seizures in both children and adults. MEDLINE ACCESSION NUMBER: 98090826
Record 41 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Use of lamotrigine in Lennox-Gastaut and related epilepsy syndromes. AUTHOR(S): Dulac-O; Kaminska-A
SOURCE (BIBLIOGRAPHIC CITATION): J-Child-Neurol. 1997 Nov; 12 Suppl 1: S23-28 INTERNATIONAL STANDARD SERIAL NUMBER: 0883-0738 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Lennox-Gastaut syndrome a combination of various generalized seizures including atypical absences and tonic seizures with generalized slow spike waves and mental deterioration, is often difficult to distinguish from a subgroup of myoclonic-astatic epilepsy, other generalized epilepsy syndromes, and various symptomatic generalized epilepsies. Conventional antiepileptic medication is poorly effective in this condition, particularly because various types of seizures respond differently to each given drug. Lamotrigine is effective in the various types of generalized seizures and efficacy has been demonstrated in Lennox-Gastaut syndrome. Given the potential of major mental deterioration within a matter of months in this condition, and the need of slow dose escalation in order to prevent skin rash, lamotrigine should be administered as soon as the diagnosis of Lennox-Gastaut syndrome is suspected. In addition, there is growing evidence that lamotrigine is also most useful! !
in the subgroup of myoclonic-astatic epilepsy beginning in childhood, and that these patients should benefit from the drug like those affected by Lennox-Gastaut syndrome, as soon as the diagnosis is suspected. However, this drug may worsen other cases of myoclonic-astatic epilepsy beginning in infancy. These clinical observations add to the evidence for the need of clear diagnostic work-up before appropriate drug therapy is decided in pediatric epilepsy. MEDLINE ACCESSION NUMBER: 98090825
Record 42 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Lennox-Gastaut syndrome.
AUTHOR(S): Wheless-JW; Constantinou-JE
SOURCE (BIBLIOGRAPHIC CITATION): Pediatr-Neurol. 1997 Oct; 17(3): 203-11 INTERNATIONAL STANDARD SERIAL NUMBER: 0887-8994 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Lennox-Gastaut syndrome (LGS) is one of the intractable epilepsies of childhood that is associated with an epileptic encephalopathy. Although LGS has been accepted as a distinct epilepsy syndrome for the last 30 years, understanding of its pathogenesis is still incomplete. Because this heterogenous entity has many diverse etiologies, some with specific therapy, a complete evaluation is necessary. The natural history is well defined; most children with LGS will ultimately be mentally retarded, will continue to have seizures, and as adults will be dependent for their daily care. Therefore, their only hope is new therapies and advances in our understanding of the pathogenesis of LGS. Several new treatment options have emerged. For the first time in the last 20 years, we have several medications with documented efficacy. In addition, there are effective nonpharmacologic treatments. These treatments offer the potential for improved seizure control, which we hope will hav! !
e impact and lessen the subsequent epileptic encephalopathy. Children with LGS require multidisciplinary assessment and treatment along with vigorous intervention aimed at minimizing their seizures to maximize their potential. Pediatric neurologists should be familiar with the treatments with proven efficacy, including new antiepileptic drugs, and should develop a rational plan of treatment for each child with LGS. MEDLINE ACCESSION NUMBER: 98051061
Record 43 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Lamotrigine for generalized seizures associated with the Lennox-Gastaut syndrome. Lamictal Lennox-Gastaut Study Group. AUTHOR(S): Motte-J; Trevathan-E; Arvidsson-JF; Barrera-MN; Mullens-EL; Manasco-P SOURCE (BIBLIOGRAPHIC CITATION): N-Engl-J-Med. 1997 Dec 18; 337(25): 1807-12 INTERNATIONAL STANDARD SERIAL NUMBER: 0028-4793 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: BACKGROUND: The Lennox-Gastaut syndrome, a severe form of epilepsy that usually begins in early childhood, is difficult to treat. Dose-related drug toxicity is common. METHODS: We conducted a double-blind, placebo-controlled trial of the antiepileptic drug lamotrigine in patients with the Lennox-Gastaut syndrome. Eligible patients had more than one type of predominantly generalized seizure, including tonic-clonic, atonic, tonic, and major myoclonic, and had seizures on average at least every other day. After a 4-week base-line period in which all participants received placebo, we randomly assigned 169 patients (age range, 3 to 25 years) to 16 weeks of lamotrigine (n= 79) or placebo (n=90) in addition to their other antiepileptic drugs. RESULTS: The median frequency of all major seizures changed from base-line levels of 16.4 and 13.5 per week in the lamotrigine and placebo groups, respectively, to 9.9 and 14.2 per week after 16 weeks of treatment (P=0.002). Thirty-th! !
ree percent of the patients in the lamotrigine group and 16 percent of those in the placebo group had a reduction of at least 50 percent in the frequency of seizures (P= 0.01). There were no significant differences between groups in the incidence of adverse events, except for colds or viral illnesses, which was more common in the lamotrigine group (P=0.05). CONCLUSIONS: Lamotrigine was an effective and well-tolerated treatment for seizures associated with the Lennox-Gastaut syndrome. MEDLINE ACCESSION NUMBER: 98051155
Record 44 of 44 in MEDLINE EXPRESS (R) 1998/01-1998/10
TITLE: Topiramate. A review of its pharmacodynamic and pharmacokinetic properties and clinical efficacy in the management of epilepsy. AUTHOR(S): Langtry-HD; Gillis-JC; Davis-R SOURCE (BIBLIOGRAPHIC CITATION): Drugs. 1997 Nov; 54(5): 752-73 INTERNATIONAL STANDARD SERIAL NUMBER: 0012-6667 LANGUAGE OF ARTICLE: ENGLISH
ABSTRACT: Topiramate is a sulphamate-substituted monosaccharide derived from D-fructose and is structurally unrelated to other antiepileptic drugs. It acts by multiple mechanisms that suggest it may be effective in several types of epilepsy. In double-blind placebo-controlled trials, add-on therapy with topiramate 400 to 1000 mg/day reduces the seizure rate by > or = 50% in 35 to 52% of adult patients with resistant partial epilepsy (with or without secondarily generalised seizures) compared with 0 to 19% of placebo recipients; a 200 mg/day dosage was less effective. Topiramate has also been shown to be superior in efficacy to placebo in well controlled trials in patients with generalised tonic-clonic seizures, Lennox-Gastaut syndrome and in paediatric patients with partial epilepsy. Efficacy has been maintained for 7 years and some patients may also be satisfactorily treated with topiramate monotherapy. Further study is needed to follow up on the promising results of topiram! !
ate use in other paediatric epilepsies. Adverse CNS events are the most common untoward effects during topiramate therapy and are most likely to lead to withdrawal of the drug. However, most adverse events are mild to moderate in severity and lessen with continued drug therapy. In clinical trials, most adverse events which were dose limiting or led to discontinuation of treatment occurred during the titration phase. The overall incidence of adverse events may be reduced by slower upward dosage titration. In summary, topiramate appears to be a suitable agent for add-on therapy in adult patients with partial epilepsy. Preliminary reports support the use of add-on topiramate in adults with generalised epilepsy, in childhood epilepsies and in patients with Lennox-Gastaut syndrome, as well as the use of topiramate monotherapy in patients with partial epilepsy. Thus, topiramate can be considered an important new drug for the management of patients with refractory epilepsy. MEDLINE ACCESSION NUMBER: 98024362