Also called West Sd.
(From Dr. West who described it on his own son in the late 19th century.)
1. Seizures of a particular type
2. Psychomotor retardation
3. EEG (Hypsarrhythmia)
1. The Seizures: Sudden, bilateral, symetrical, contractions of
They involve the
Flexors muscles groups (“Syndrome des spasmes en flexions”) also sometime Mixed Flexors/extensors muscles or Extensors muscles groups only (less frequently)
The seizures consist of an abrupt initial contraction followed by more
cry may be heard ; unilaterality or
asymetry are also possible. A laughter, flushing, nystagmus can also be seen.
A key feature is that the seizures are REPETITIVE
every 5 to 30 seconds, there can be repeated sometimes a 100 times.
This type of seizures may lasts 1 - 2 years, up to 5-10 years
2. Psychomotor Retardation/Deterioration: Initially may only be
perceived as simple developmental delay. Often the neurological status is
already abnormal before the onset of Psychomotor Retardation/Deterioration or
delay. Sometimes a loss of social smile, or a loss of interests will lead to the
child being labelled as blind as the presenting complain.
3. The EEG is TYPICAL : There is HYPSARRHYTHMIA. But hypsarhythmia
is not the sole EEG pattern. (NB Hypsarrhythmia is a EEG pattern not to be used
as a synonymous to Infantile Spasms). It is an ICTERICAL pattern observed in the
NB: O. Dulac from Paris (France) has shown the probable
FOCAL onset of the seizures which are very rapidly SECONDARILY
The diagnosis is CLINICAL 1. Spasms
often on awakening.
In the differential diagnosis one must think of
1. Benign Myoclonus of early infancy
2. Early true Myoclonic Epilepsy ( but is briefer more saccadic and not
repeated) 3. The Lennox-Gastaut .
Etiologies: The West syndrome can be symptomatic of a central
nervous system malformation as in the Tuberous Sclerosis condition (TS), or
cryptogenic (the cause remaining then unknown).
Incidence .24 to.42 / 1000.
It occurs in children less than a
year old. Maximum onset is at 3 to7 months
There is a family history of seizures in
6% to 17% of the children.
Treatment: Mogadon (
( Clonazepam )
( Valproic Acid )
in association with Carbamazepine.
to 40 units / day IM. Some 10 to 180 units
2 to 10 mg/kg/day
All these medications have
been shown to have some benefit. Streroids are used for a minimum of 3 to 8
WEEKS up to 6 to 10 months. Other anticonvulsants such as Vigabatrine may be
used much longer.
Usually helps within 1 week, ++ if cryptogenic relapses in 30% after d/c
Mental outcome: almost invariably leads to mental deficiency if
Symptomatic; this outcome is improved to 30-40% incidence of mental
deficiency in case of Cryptogenic West syndrome.
Mortality 20%. Survivors
have sensory defects, CP in 50%
Mental retardation in 80% (severe
THE EARLIER THE INFANTILE SPASMS APPEAR THE LESS FAVORABLE THE PROGNOSIS
ACTH and other steroid medications, used in the
treatment of infantile spasms has major side effects which include:
depression of the immune system
transient brain atrophy
2 infants have been
reported to have developed reversible massive enlargement of both adrenal glands
during treatment with ACTH, one with infantile spasms, and the second with
opsoclonus and cerebellar encephalopathy. The first child was treated with ACTH
80 units IM for 3 weeks followed by 40 units every other day for 14 days. When
the adrenal enlargement was detected, ACTH was tapered off, and the adrenal
enlargement rapidly disappeared. See: Liebling MS; Pediat Radiol 23:454-456, Oct
association seems to be the preferred treatment in case of symptomatic West
particularly when associated with TS. The side effects are infrequent but recent
reports of visual field defects associated with Vigabatrine have made this drug
less widely used.
Vitamin B6 is used
particularly extensively in Japan. There seems to be a similar response rate
than with other therapeutic protocols.