Problem West Syndrome (WS) is a severe epileptic disorder. Its aetiology is highly heterogeneous, the treatment is limited and the prognosis depends on the cause. Studies of this disease allowed the establishment of prognosis criteria and optimal therapeutic strategies.
of the work studying the diverse physiopathological, epidemiological, diagnostic, therapeutic and evolutionary aspects of this syndrome and comparing our results with literature to improve our coverage.
Methods We conducted a retrospective study on the observations of 30 children followed for WS in the department of paediatrics of the military hospital of Tunis between 1990 and 2010. The studied variables were: aetiology, treatments and their efficiency as well as neurological, cognitive and behavioural development of children.
Results The age of onset is before the first year in 93.33% of cases. The disease occurs mainly by epileptic spasms and almost all children presented a significant developmental regression. Typical hypsarythmia is found only in 36% of cases. Concerning aetiology, symptomatic forms represent 20% and idiopathic ones 10%. The usual protocol at our department is to start with Vigabatrin-corticosteroid association with 91.6% efficiency. Neurological prognosis is severe with sequelas in 66.66% of cases. Behavioural disorders are also significant because half of children have mental retardation.
Conclusion Despite the existence of more effective molecules, the prognosis of the WS remains severe. Better knowledge of physiopathology, the deferent electroclinical aspects and therapeutic management allow improving the children’s fate.