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
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Article info
Publication history
Accepted:
July 26,
2001
Received in revised form:
July 23,
2001
Received:
May 31,
2001
Identification
Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
