Abstract
In this brief review, the sleep studies on patients with West syndrome (WS) were summarized.
In addition to the previously reported common finding for sleep in WS – reduction
of the amount of rapid-eye-movement (REM) sleep – weakness of phasic suppression of
chin muscle activity in WS patients has recently been found. The degree of this weakness
is quantified by the phasic inhibition index (PII), which has been found to reflect
a patient's prognosis as to convulsions. PII is proposed to be a useful parameter
for assessing the prognosis of WS. Since the pontine tegmentum is involved in the
production of the REM-related phasic loss of muscle activity in REM sleep, WS patients
are hypothesized to have a functional instability of the pontine tegmentum. After
adrenocorticotropin (ACTH) treatment, PII decreased significantly in all WS patients
examined. Taken together with the effects of corticosteriods on PII, and the incidence
of phasic chin muscle activity in patients with congenital adrenal hyperplasia and
nephrotic syndrome, ACTH is hypothesized to suppress the spasms in WS patients not
only through corticosteroids, but also through a direct action on the pontine tegmentum.
Since PII has been reported to be elevated in patients with an autistic tendency,
the appearance of an autistic tendency is also hypothesized to be involved in the
functional disturbance of the pontine tegmentum.
Keywords
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Article info
Publication history
Accepted:
May 11,
2001
Received in revised form:
May 8,
2001
Received:
April 25,
2001
Identification
Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
