Abstract
The long-term prognosis of 61 children with West syndrome, 48 alive and 13 dead, was investigated. The follow-up study was made in regard to psychomotor development and clinical seizure.
Fourteen cases (23%) showed normal or borderline mental development and 47 cases (77%) were mentally retarded. Twenty cases (33%) were normal in motor development and 41 cases (67%) were physically handicapped, with tetraplegia, hemiplegia or hypotonia. Twenty three cases (38%) were free from seizures and 38 cases (62%) had seizures of West syndrome, Lennox syndrome and other types of epilepsy. The patients free from seizures had good prognosis of not only mental but also motor development (P<0.001).
The final EEG records were obtained in 52 cases, and 8 cases (15%) showed normal or borderline EEG findings, nineteen cases (37%) showed focal abnormalities and 25 cases (48%) showed centrencephalic abnormalities. There was a significant correlation between the final EEG findings and the above mentioned prognosis. The prognosis was good in the cases of normal, borderline and focal abnormalities, and poor in the case of centrencephalic abnormalities.
Initial EEG findings gave little guide to etiology and final outcome of West syndrome. However, the cases showing centrencephalic abnormalities, even after the therapy for more than 3 months, had a poor final prognosis.
Factors influencing the prognosis of mental development were discussed. Mental prognosis of idiopathic cases was better than that of symptomatic cases (P<0.001). In the symptomatic group, mental prognosis was relatively worse in the cases of early onset under 3 months of age than in the other cases (P<0.005). In the idiopathic group, there was no correlation between the mental prognosis and the age of the onset. Early therapy within 2 months after the onset of the seizures was correlated with good mental prognosis in the idiopathic group (P<0.05). On the other hand, despite the early therapy, almost all patients of symptomatic group showed a subnormal mental development.
There was no significant difference between ACTH therapy and other anticonvulsant therapy for the final outcome, not only in the idiopathic group but also in the symptomatic group. It was not concluded that ACTH therapy should first be chosen in West syndrome.
