Spasms in clusters in epilepsies other than typical West syndrome

Yoko Ohtsuka, Katsuhiro Kobayashi, Tatsuya Ogino, Eiji Oka

Research output: Contribution to journalArticle

27 Citations (Scopus)

Abstract

Although spasms in clusters are one of the major characteristics of West syndrome (WS), there are a significant number of patients who show spasms in clusters but do not fit the standard pattern of WS. It is possible to divide these atypical cases into the following three groups. Group 1: refractory epilepsies beginning in early infancy, associated with atypical electroencephalographic (EEG) features; Group 2: generalized epilepsies with spasms in clusters at ages of 2-3 years or above; and Group 3: localization-related epilepsies with spasms in clusters. Ictal clinical and EEG findings of spasms in clusters in these atypical patients and also those in WS are similar. Patients in Group 1 often suffer from Aicardi syndrome, cortical malformations, early myoclonic encephalopathy and Ohtahara syndrome. Most patients in Group 2 suffer from Lennox-Gastaut syndrome and other generalized epilepsies such as severe epilepsy with multiple independent spike foci. A significant number of them had a history of WS. Small number of patients in Group 2 can be diagnosed as having late-onset WS or long-lasting WS. In Groups 1 and 3 patients, cortical mechanisms play a critical role in their pathophysiology. The presence of older patients with spasms in clusters might indicate not only developing process of the brain but also some selective dysfunction of the brain plays an important role in the occurrence of spasms in clusters. Investigations on these atypical patients can help the understanding of pathophysiological mechanisms of WS and its related epileptic syndromes.

Original languageEnglish
Pages (from-to)473-481
Number of pages9
JournalBrain and Development
Volume23
Issue number7
DOIs
Publication statusPublished - 2001

Fingerprint

Infantile Spasms
Spasm
Epilepsy
Generalized Epilepsy
Aicardi Syndrome
Partial Epilepsy
Brain
Stroke

Keywords

  • Atypical West syndrome
  • Cortical malformations
  • Hypsarrhythmia
  • Infantile spasms
  • Lennox-Gastaut syndrome
  • Periodic spasms

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Neurology

Cite this

Spasms in clusters in epilepsies other than typical West syndrome. / Ohtsuka, Yoko; Kobayashi, Katsuhiro; Ogino, Tatsuya; Oka, Eiji.

In: Brain and Development, Vol. 23, No. 7, 2001, p. 473-481.

Research output: Contribution to journalArticle

Ohtsuka, Yoko ; Kobayashi, Katsuhiro ; Ogino, Tatsuya ; Oka, Eiji. / Spasms in clusters in epilepsies other than typical West syndrome. In: Brain and Development. 2001 ; Vol. 23, No. 7. pp. 473-481.
@article{65fc8c0bde6c4a0994a007fc2198bd1f,
title = "Spasms in clusters in epilepsies other than typical West syndrome",
abstract = "Although spasms in clusters are one of the major characteristics of West syndrome (WS), there are a significant number of patients who show spasms in clusters but do not fit the standard pattern of WS. It is possible to divide these atypical cases into the following three groups. Group 1: refractory epilepsies beginning in early infancy, associated with atypical electroencephalographic (EEG) features; Group 2: generalized epilepsies with spasms in clusters at ages of 2-3 years or above; and Group 3: localization-related epilepsies with spasms in clusters. Ictal clinical and EEG findings of spasms in clusters in these atypical patients and also those in WS are similar. Patients in Group 1 often suffer from Aicardi syndrome, cortical malformations, early myoclonic encephalopathy and Ohtahara syndrome. Most patients in Group 2 suffer from Lennox-Gastaut syndrome and other generalized epilepsies such as severe epilepsy with multiple independent spike foci. A significant number of them had a history of WS. Small number of patients in Group 2 can be diagnosed as having late-onset WS or long-lasting WS. In Groups 1 and 3 patients, cortical mechanisms play a critical role in their pathophysiology. The presence of older patients with spasms in clusters might indicate not only developing process of the brain but also some selective dysfunction of the brain plays an important role in the occurrence of spasms in clusters. Investigations on these atypical patients can help the understanding of pathophysiological mechanisms of WS and its related epileptic syndromes.",
keywords = "Atypical West syndrome, Cortical malformations, Hypsarrhythmia, Infantile spasms, Lennox-Gastaut syndrome, Periodic spasms",
author = "Yoko Ohtsuka and Katsuhiro Kobayashi and Tatsuya Ogino and Eiji Oka",
year = "2001",
doi = "10.1016/S0387-7604(01)00289-3",
language = "English",
volume = "23",
pages = "473--481",
journal = "Brain and Development",
issn = "0387-7604",
publisher = "Elsevier",
number = "7",

}

TY - JOUR

T1 - Spasms in clusters in epilepsies other than typical West syndrome

AU - Ohtsuka, Yoko

AU - Kobayashi, Katsuhiro

AU - Ogino, Tatsuya

AU - Oka, Eiji

PY - 2001

Y1 - 2001

N2 - Although spasms in clusters are one of the major characteristics of West syndrome (WS), there are a significant number of patients who show spasms in clusters but do not fit the standard pattern of WS. It is possible to divide these atypical cases into the following three groups. Group 1: refractory epilepsies beginning in early infancy, associated with atypical electroencephalographic (EEG) features; Group 2: generalized epilepsies with spasms in clusters at ages of 2-3 years or above; and Group 3: localization-related epilepsies with spasms in clusters. Ictal clinical and EEG findings of spasms in clusters in these atypical patients and also those in WS are similar. Patients in Group 1 often suffer from Aicardi syndrome, cortical malformations, early myoclonic encephalopathy and Ohtahara syndrome. Most patients in Group 2 suffer from Lennox-Gastaut syndrome and other generalized epilepsies such as severe epilepsy with multiple independent spike foci. A significant number of them had a history of WS. Small number of patients in Group 2 can be diagnosed as having late-onset WS or long-lasting WS. In Groups 1 and 3 patients, cortical mechanisms play a critical role in their pathophysiology. The presence of older patients with spasms in clusters might indicate not only developing process of the brain but also some selective dysfunction of the brain plays an important role in the occurrence of spasms in clusters. Investigations on these atypical patients can help the understanding of pathophysiological mechanisms of WS and its related epileptic syndromes.

AB - Although spasms in clusters are one of the major characteristics of West syndrome (WS), there are a significant number of patients who show spasms in clusters but do not fit the standard pattern of WS. It is possible to divide these atypical cases into the following three groups. Group 1: refractory epilepsies beginning in early infancy, associated with atypical electroencephalographic (EEG) features; Group 2: generalized epilepsies with spasms in clusters at ages of 2-3 years or above; and Group 3: localization-related epilepsies with spasms in clusters. Ictal clinical and EEG findings of spasms in clusters in these atypical patients and also those in WS are similar. Patients in Group 1 often suffer from Aicardi syndrome, cortical malformations, early myoclonic encephalopathy and Ohtahara syndrome. Most patients in Group 2 suffer from Lennox-Gastaut syndrome and other generalized epilepsies such as severe epilepsy with multiple independent spike foci. A significant number of them had a history of WS. Small number of patients in Group 2 can be diagnosed as having late-onset WS or long-lasting WS. In Groups 1 and 3 patients, cortical mechanisms play a critical role in their pathophysiology. The presence of older patients with spasms in clusters might indicate not only developing process of the brain but also some selective dysfunction of the brain plays an important role in the occurrence of spasms in clusters. Investigations on these atypical patients can help the understanding of pathophysiological mechanisms of WS and its related epileptic syndromes.

KW - Atypical West syndrome

KW - Cortical malformations

KW - Hypsarrhythmia

KW - Infantile spasms

KW - Lennox-Gastaut syndrome

KW - Periodic spasms

UR - http://www.scopus.com/inward/record.url?scp=0034782754&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0034782754&partnerID=8YFLogxK

U2 - 10.1016/S0387-7604(01)00289-3

DO - 10.1016/S0387-7604(01)00289-3

M3 - Article

VL - 23

SP - 473

EP - 481

JO - Brain and Development

JF - Brain and Development

SN - 0387-7604

IS - 7

ER -