Genetic Study of Infantile Spasms with Hyp s arrhythmia

Fleiszar, Kathy A.; Daniel, William L.; Imrey, Peter B.

doi:10.1111/j.1528-1157.1977.tb05587.x

Cited by 25 publications

(9 citation statements)

References 16 publications

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“…A familial predisposition to epilepsy is not uncommon among children with CP and epilepsy (Degen et al 1972, Fleiszar et al 1977, Aksu 1989, and in the present study the predisposition was twice as frequent as in patients without detectable organic causes of the epilepsy. These results underline the significance of genetic predisposition, not only in primary generalized epilepsies, but also in epilepsy with morphological organic brain lesions.…”

Section: Discussionsupporting

confidence: 63%

Nature And Prognosis Of Seizures In Patients With Cerebral Palsy

Aksu

1990

Develop Med Child Neuro

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SUMMARY The nature, course and prognosis of seizures were investigated in patients with epilepsy and cerebral palsy (CP). 174 children with epilepsy were investigated: 57 with CP and 117 who were otherwise neurologically normal. Epilepsy in patients with CP was characterized by early age at onset, generalized focal and multifocal seizures and a poorer prognosis. The presence of cerebral palsy requires differential consideration of the severity of epilepsies. This may be of appreciable importance for the prognostic evaluation of anticonvulsant therapy or the course of epilepsy. RÉSUMÉ Nature et pronostic des crises comitiales chez les sujets avec épilepsie et infirmité motrice cérébral La nature, l'évolution et le pronostic des crises comitiales ont été analysés chez des patients présentant á la fois épilepsie et infirmité motrice cérébrale (IMC). 174 enfants avec épilepsie ont été étudiés: 57 d'entre eux avaient une IMC et 117 n'avaient aucun autre trouble en dehors de la comitialité. L'épilepsie chez les sujets avec IMC était caractérisée par un début á un âge précoce, des crises focales généralisées ou multi‐focales et un mauvais pronostic en ce qui concernait l'évolution des crises. L'infirmité motrice cérébrale donnent un critére de gravité aux épilepsies. Cela peut être d'une importance considérable pour l'évaluation pronostique d'un traitement anticonvulsif au cours de l'evolution d'une épilepsie. ZUSAMMENFASSUNG Art und Prognose von Anfällen bei Patienten mit Epilepsie und Cerebralparese Bei Patienten mit Epilepsie und Cerebralparese (CP) wurden Art, Verlauf und Prognose von Anfällen untersucht. In der Studie waren 174 Kinder mit Epilepsie: davon hatten 57 eine CP und 117 waren sonst neurologisch unauffällig. Die Epilepsie bei Patienten mit Cerebralparese ist durch ein frühes Auftreten, generalisierte fokale und multifokale Anfälle und eine schlechtere Prognose des Anfallsleidens charakterisiert. Die Cerebralparese erlaubt eine andere Einschätzung des Schweregrades von Epilepsien. Dies mag für die prognostische Beurteilung der antikonvulsiven Therapie oder den Verlauf einer Epilepsie von Bedeutung sein. RESUMEN Naturaleza y pronóstico de las convulsiones en niños con epilepsia y parálisis cerebral Se investigó la naturaleza, curso y pronóstico de las convulsiones en pacientes con epilepsia y parálisis cerebral (PC) Se investigaron 174 niños: 57 con PC y 117 que eran neurológicamente normales. La epilepsia en los pacientes con PC se caracterizó por un inicio precoz, crisis generalizadas focales y multifocales y un pronóstico mós pobre por Jo que hace a la alteración convulsiva. La Parólisis Cerebral da una consideración diferencial a la gravedad de las epilepsias. Esto puedo serde importancia apreciable para establecer el pronóstico de la terapia anticonvulsiva o el curso de la epilepsia.

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Section: Discussionsupporting

confidence: 63%

Nature And Prognosis Of Seizures In Patients With Cerebral Palsy

Aksu

1990

Develop Med Child Neuro

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“…Fukuyama (1960) reported that 15.6% of cases had a family history of epilepsy or other convulsive disorders in first, second and third degree relatives. In his genetic study of infantile spasms, Fleizar (1977) reported a high incidence of epilepsy in third degree relatives of infants with infantile spasms. Degen (1977) also noted a high rate of hereditary afflication of convulsive disorders in West syndrome (13.5%) compared with the control group (0.6%), emphasizing the important role of genetic factors even in those types of seizures which were considered predominantly symptomatic.…”

Section: Discussionmentioning

confidence: 97%

Infantile spasms: Etiological factors, clinical aspects, and long term prognosis in 200 cases

et al. 1981

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Abstract. The etiological factors, clinical aspects and long term prognosis were studied in 200 patients with infantile spasms. Forty-eight (24.0%) died and the rest were aged 6 years or more at the time of final follow-up. In 73 (36.5%) the etiology was prenatal, in 44 (22.0%) perinatal, and in 17 (8.5%) postnatal: 18 cases (9.0%) were cryptogenic. The remaining 4.8 (24.0%) patients were doubtful cases. The mortalily of the pre-and perinatal cases at 35.6% and 34.1% respectively was significantly higher than that in the other etiologic groups (P< 0.001).With regard to the onset of spasms, these were very early in the pre-and perinatal groups, whereas in the postnatal group the onset was late. The onset in the doubtful and cryptogenic groups was in between. Some 44.4% of the cryptogenic cases showed normal mental and physical development after the age of 6 years, whereas less than 10% of the prenatal and perinatal group did so, Almost all of the doubtful cases became mentally subnormal.The incidence of a family history of epilepsy or other convulsive disorders in the first, second and third degree relatives was highest in the cryptogenic cases (40.0%), and lowest in the perinatal cases (9.3%) (P<0.01). The incidence of laughing attacks was highest in the postnatal cases (42.9%), against no such attacks in cryptogenic cases (P<0.01). The attacks seemed to be linked with organic brain lesions. Among various factors related to the etiology of infantile spasms, genetic propensity seemed especially important in cryptogenic cases.

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“…In a study of 77 WS probands, the observed rate of WS was 7.4 ? 5.2% for first-degree relatives, 2.8 k 2.8% for second-degree relatives, and 0 for thirddegree relatives (Fleiszar et al, 1977). The investigators suspected a polygenic mode of inheritance interacting with environmental factors.…”

Section: Discussionmentioning

confidence: 97%

Genetic Predisposition to West Syndrome

et al. 1993

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To determine the recurrence risk of West syndrome (WS), we studied the familial antecedents of consecutively referred patients. Among siblings, there was an increased incidence of WS but not of febrile convulsions. Familial incidence of epilepsy was intermediate between the epileptic and nonepileptic control groups. When cases resulting from a genetically determined disease were excluded, incidence of epilepsy among siblings was similar to that in normal controls. Five of the 11 familial cases of WS were due to an identifiable cause: twin pregnancy, tuberous sclerosis, and recurrent maternal toxemia. In 4 of the remaining families, the clinical picture included spasms, erratic myoclonus, and postnatal microcephaly, suggestive of a previously unidentifiable progressive encephalopathy. Therefore, when identifiable familial diseases were excluded, the recurrence risk was < 1%.

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Genetic Study of Infantile Spasms with Hyp s arrhythmia

Cited by 25 publications

References 16 publications

Nature And Prognosis Of Seizures In Patients With Cerebral Palsy

Nature And Prognosis Of Seizures In Patients With Cerebral Palsy

Infantile spasms: Etiological factors, clinical aspects, and long term prognosis in 200 cases

Genetic Predisposition to West Syndrome

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