LE SYNDROME DE WEST - Faculté de Médecine et de Pharmacie ...

SUMMARY
West syndrome, first described by Dr. West in 1841, is a severe epileptic
encephalopathy characterized by specific infant triad: infantile spasms,
hypsarrhythmia and psychomotor regression.
We report a retrospective study analyzing 72 cases of West syndrome seen at
the pediatric neurology consultation of CHU Hassan II of Fez, during a period of five
years from 1 January 2007 to December 31, 2011, in order to study epidemiological
data , clinical, therapeutic, and prognostic features of this syndrome.
The age of onset varied from 1 to 18 months, with an average of 12.26
months, the first spasms occurred during the first year of life in 87% of cases with a
sex ratio of 1 , 88. The average time to diagnosis was 5.36 months. Spasms in
flexion in the most frequent type (79%), organized in bursts at 81% with an average
of 3 to 20 bursts / day. Other neurological signs were reported in 60% of cases. A
microcephaly was present in 30% of cases. Autistic features have been reported in
4% of cases.
The electroencephalogram (EEG) has objectified a typical hypsarrhythmia in
84%of cases. Neuroimaging showed brain atrophy in 24% of cases, and anomalies
for the tuberous sclerosis (TS) in 14% of cases.
West syndrome was symptomatic in 79%, cryptogenic in 15% and idiopathic in
6% of cases. The neonatal anoxic-ischemic encephalopathy has been found in 36%,
neuromeningeal infections in 17.5% and tuberous sclerosis in 12% of cases. Those
are the three most common etiologies of West syndrome.
Sodium Valproate-Clobazam was effective in 5.5%, Vigabatrin in 83% and
steroids in 66.6% of cases.
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