Abstract
The high incidence of infantile spasms (IS) and hypsarrhythmia in tuberous sclerosis
complex (TSC) has long been emphasized but it is now clear that infants with TSC show
clinical and EEG differences from those with classical West syndrome. Seizures at
onset are mainly characterized by partial motor seizures and IS. Subtle partial seizures
may be present in the early neonatal period and may precede the onset of IS. Visual
recording techniques have led to significant progress in the classification of seizures
associated with TSC, demonstrating that they have a focal or multifocal origin in
the vast majority of cases. In most cases, an awake interictal EEG shows focal or
independent multifocal spike and slow-wave activity at onset and later a pseudo-hypsarrhythmic
pattern. Ictal EEG starts with focal spikes originating from the posterotemporal,
or occipital regions followed by a generalized irregular slow transient and an abrupt
diffuse flattening. Although the pathophysiological mechanisms responsible for the
coexistence of partial seizures and IS are still unclear, IS associated with TSC may
be the result of a rapid secondary generalization. The presence of IS due to TSC is
strongly predicted by the cortical tuber count, while the age of onset of seizures
and the age of occurrence of EEG foci depend on the localization of cortical tubers
with an earlier expression of the parieto-occipital than of the frontal regions. Early
recognition of these distinctive features appears worthwhile for therapeutic and prognostic
implications. Despite the efficacy of vigabatrin the prognosis of IS is generally
poor. Studies using combined topographic mapping of EEG, magnetic resonance imaging
and positron emission tomography may provide new strategies for selecting candidates
suitable for surgery.
Keywords
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Article info
Publication history
Accepted:
May 24,
2001
Received in revised form:
May 21,
2001
Received:
May 21,
2001
Identification
Copyright
© 2001 Published by Elsevier Inc.
