Abstract
Aim We aimed to examine the occurrence of cerebral palsy (CP) in children with West syndrome
(WS), to estimate the possible causative factors by analyzing the neuroimaging examinations
of patients, to evaluate their cognitive/motor function and epileptic status and to
compare the prognosis of children with double pathology of WS and CP and of those
without CP.
Methods The clinical and magnetic resonance imaging (MRI) data of 62 patients with West syndrome
were evaluated. A total of 39 of 62 patients (63%) suffered from CP (CP group). The
non-CP group included 23 patients.
Results Abnormal MRI was found in 55/62 (89%) patients. Main anomalies were: brain malformation
(21), hypoxic-ischemic encephalopathy (13), cerebrovascular insult (8), infection
(7), and other anomalies (6). In the CP group, the most common MRI abnormalities included
pre/perinatal hypoxia/ischemia, brain malformation, cerebrovascular insult, and infection.
In the non-CP group, brain malformations were the most frequent. Significantly more
negative MRIs were found in the non-CP group. More than 60% of the patients were severely
cognitively impaired, almost 90% of them had CP. Not only the occurrence of intellectual
disability was lower in the non-CP group, but its severity was milder as well. A total
of 78% of the children with CP had a very severe motor disability. Fifty-four percent
in the CP and 67% in the non-CP group had therapy-resistant epilepsy.
Conclusion WS has an especially unfavorable prognosis: cerebral anomaly was confirmed in 89%
of our patients. CP was present in almost two-thirds of the children with WS, most
of them had severe cognitive and motor deficits.
Keywords
West syndrome - infantile spasm - cerebral palsy - etiology - MRI - walking ability
