Severe myoclonic epilepsy in infancy: evolution of electroencephalographic and clinical features.

P. J. Wang, P. C. Fan, W. T. Lee, C. Young, C. C. Huang, Y. Z. Shen

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

Since 1987, we have diagnosed 10 patients, 4 males and 6 females, aged 2-11 years at the last evaluation, who all met the following criteria of severe myoclonic epilepsy in infancy (SMEI): generalized or unilateral long-lasting febrile clonic seizures in the first year of life; the subsequent appearance of myoclonic seizures and other types of seizure (partial seizures, atypical absences and convulsive status epilepticus); and neuropsychological deterioration for a certain period. Family histories of epilepsy and febrile seizures could be traced in 1 and 3 cases, respectively. None of them had previous personal history of brain insult. Electroencephalographic (EEGic) recordings in febrile seizure stage were normal; and continuous prophylaxis with phenobarbital failed to prevent the recurrence of febrile seizures. EEG studies in myoclonic stage showed generalized spike-and-waves, polyspike-and-waves, focal abnormalities and/or photosensitivity. The seizures were highly resistant to antiepileptic drugs. Our experiences suggested that comedication of valproic acid, clonazepam and carbamazepine may be most effective in treatment of the diverse seizures including myoclonic seizures, myoclonic-tonic-clonic seizures, atypical absences and partial seizures. Myoclonic seizures and atypical absences diminished in parallel to a clear-cut decrease in generalized abnormalities on EEG in 4 cases aged more than 7 years. However, the partial seizures, secondarily generalized seizures and status epilepticus were still present. Further investigations should aim to identify the underlying etiology and to search more effective treatment.

Original languageEnglish
Pages (from-to)428-432
Number of pages5
JournalActa Paediatrica Sinica
Volume37
Issue number6
Publication statusPublished - Nov 1996
Externally publishedYes

Fingerprint

Myoclonic Epilepsy
Seizures
Febrile Seizures
Status Epilepticus
Absence Epilepsy
Electroencephalography
Clonazepam
Carbamazepine
Valproic Acid
Phenobarbital
Anticonvulsants
Epilepsy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Severe myoclonic epilepsy in infancy : evolution of electroencephalographic and clinical features. / Wang, P. J.; Fan, P. C.; Lee, W. T.; Young, C.; Huang, C. C.; Shen, Y. Z.

In: Acta Paediatrica Sinica, Vol. 37, No. 6, 11.1996, p. 428-432.

Research output: Contribution to journalArticle

Wang, PJ, Fan, PC, Lee, WT, Young, C, Huang, CC & Shen, YZ 1996, 'Severe myoclonic epilepsy in infancy: evolution of electroencephalographic and clinical features.', Acta Paediatrica Sinica, vol. 37, no. 6, pp. 428-432.
Wang, P. J. ; Fan, P. C. ; Lee, W. T. ; Young, C. ; Huang, C. C. ; Shen, Y. Z. / Severe myoclonic epilepsy in infancy : evolution of electroencephalographic and clinical features. In: Acta Paediatrica Sinica. 1996 ; Vol. 37, No. 6. pp. 428-432.
@article{ed5fa2d3a00c4bad842a7e9b0e89dd42,
title = "Severe myoclonic epilepsy in infancy: evolution of electroencephalographic and clinical features.",
abstract = "Since 1987, we have diagnosed 10 patients, 4 males and 6 females, aged 2-11 years at the last evaluation, who all met the following criteria of severe myoclonic epilepsy in infancy (SMEI): generalized or unilateral long-lasting febrile clonic seizures in the first year of life; the subsequent appearance of myoclonic seizures and other types of seizure (partial seizures, atypical absences and convulsive status epilepticus); and neuropsychological deterioration for a certain period. Family histories of epilepsy and febrile seizures could be traced in 1 and 3 cases, respectively. None of them had previous personal history of brain insult. Electroencephalographic (EEGic) recordings in febrile seizure stage were normal; and continuous prophylaxis with phenobarbital failed to prevent the recurrence of febrile seizures. EEG studies in myoclonic stage showed generalized spike-and-waves, polyspike-and-waves, focal abnormalities and/or photosensitivity. The seizures were highly resistant to antiepileptic drugs. Our experiences suggested that comedication of valproic acid, clonazepam and carbamazepine may be most effective in treatment of the diverse seizures including myoclonic seizures, myoclonic-tonic-clonic seizures, atypical absences and partial seizures. Myoclonic seizures and atypical absences diminished in parallel to a clear-cut decrease in generalized abnormalities on EEG in 4 cases aged more than 7 years. However, the partial seizures, secondarily generalized seizures and status epilepticus were still present. Further investigations should aim to identify the underlying etiology and to search more effective treatment.",
author = "Wang, {P. J.} and Fan, {P. C.} and Lee, {W. T.} and C. Young and Huang, {C. C.} and Shen, {Y. Z.}",
year = "1996",
month = "11",
language = "English",
volume = "37",
pages = "428--432",
journal = "Pediatrics and Neonatology",
issn = "1875-9572",
publisher = "臺灣兒科醫學會",
number = "6",

}

TY - JOUR

T1 - Severe myoclonic epilepsy in infancy

T2 - evolution of electroencephalographic and clinical features.

AU - Wang, P. J.

AU - Fan, P. C.

AU - Lee, W. T.

AU - Young, C.

AU - Huang, C. C.

AU - Shen, Y. Z.

PY - 1996/11

Y1 - 1996/11

N2 - Since 1987, we have diagnosed 10 patients, 4 males and 6 females, aged 2-11 years at the last evaluation, who all met the following criteria of severe myoclonic epilepsy in infancy (SMEI): generalized or unilateral long-lasting febrile clonic seizures in the first year of life; the subsequent appearance of myoclonic seizures and other types of seizure (partial seizures, atypical absences and convulsive status epilepticus); and neuropsychological deterioration for a certain period. Family histories of epilepsy and febrile seizures could be traced in 1 and 3 cases, respectively. None of them had previous personal history of brain insult. Electroencephalographic (EEGic) recordings in febrile seizure stage were normal; and continuous prophylaxis with phenobarbital failed to prevent the recurrence of febrile seizures. EEG studies in myoclonic stage showed generalized spike-and-waves, polyspike-and-waves, focal abnormalities and/or photosensitivity. The seizures were highly resistant to antiepileptic drugs. Our experiences suggested that comedication of valproic acid, clonazepam and carbamazepine may be most effective in treatment of the diverse seizures including myoclonic seizures, myoclonic-tonic-clonic seizures, atypical absences and partial seizures. Myoclonic seizures and atypical absences diminished in parallel to a clear-cut decrease in generalized abnormalities on EEG in 4 cases aged more than 7 years. However, the partial seizures, secondarily generalized seizures and status epilepticus were still present. Further investigations should aim to identify the underlying etiology and to search more effective treatment.

AB - Since 1987, we have diagnosed 10 patients, 4 males and 6 females, aged 2-11 years at the last evaluation, who all met the following criteria of severe myoclonic epilepsy in infancy (SMEI): generalized or unilateral long-lasting febrile clonic seizures in the first year of life; the subsequent appearance of myoclonic seizures and other types of seizure (partial seizures, atypical absences and convulsive status epilepticus); and neuropsychological deterioration for a certain period. Family histories of epilepsy and febrile seizures could be traced in 1 and 3 cases, respectively. None of them had previous personal history of brain insult. Electroencephalographic (EEGic) recordings in febrile seizure stage were normal; and continuous prophylaxis with phenobarbital failed to prevent the recurrence of febrile seizures. EEG studies in myoclonic stage showed generalized spike-and-waves, polyspike-and-waves, focal abnormalities and/or photosensitivity. The seizures were highly resistant to antiepileptic drugs. Our experiences suggested that comedication of valproic acid, clonazepam and carbamazepine may be most effective in treatment of the diverse seizures including myoclonic seizures, myoclonic-tonic-clonic seizures, atypical absences and partial seizures. Myoclonic seizures and atypical absences diminished in parallel to a clear-cut decrease in generalized abnormalities on EEG in 4 cases aged more than 7 years. However, the partial seizures, secondarily generalized seizures and status epilepticus were still present. Further investigations should aim to identify the underlying etiology and to search more effective treatment.

UR - http://www.scopus.com/inward/record.url?scp=0030281736&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0030281736&partnerID=8YFLogxK

M3 - Article

C2 - 9074279

AN - SCOPUS:0030281736

VL - 37

SP - 428

EP - 432

JO - Pediatrics and Neonatology

JF - Pediatrics and Neonatology

SN - 1875-9572

IS - 6

ER -