Does Co-occurred Cerebral Palsy Change the Prognosis of West Syndrome?
Eszter Nagy1, Nelli Farkas2, Katalin Hollódy1
1Department of Pediatrics, Clinical Center, University of Pécs, Medical School, Pécs, Hungary.
Insights
Cerebral palsy (CP) affects nearly two-thirds of children with West syndrome (WS), often leading to severe cognitive and motor deficits. Neuroimaging reveals anomalies in most WS patients, highlighting an unfavorable prognosis.
Area of Science:
- Neurology
- Pediatrics
- Developmental Neuroscience
Background:
- West syndrome (WS) is a severe infantile epilepsy.
- Cerebral palsy (CP) is a common comorbidity in children with neurological disorders.
- Understanding the relationship between WS and CP is crucial for prognosis and management.
Purpose of the Study:
- To determine the incidence of cerebral palsy (CP) in children diagnosed with West syndrome (WS).
- To identify potential causative factors for CP in WS patients through neuroimaging analysis.
- To evaluate cognitive/motor function, epileptic status, and compare prognoses in children with co-occurring WS and CP versus those with WS alone.
Main Methods:
- Retrospective analysis of clinical and magnetic resonance imaging (MRI) data from 62 patients with West syndrome.
- Classification of patients into a CP group (39 patients) and a non-CP group (23 patients).
- Assessment of neuroimaging findings, cognitive and motor function, and epileptic status.
Main Results:
- Cerebral palsy (CP) was diagnosed in 63% of children with West syndrome (WS).
- Abnormalities on MRI were prevalent (89%), with brain malformations, hypoxic-ischemic encephalopathy, and cerebrovascular insults being common in the CP group.
- Children with CP exhibited more severe cognitive and motor impairments compared to those without CP, although epilepsy remained therapy-resistant in a high percentage of both groups.
Conclusions:
- West syndrome (WS) carries a poor prognosis, with nearly two-thirds of affected children developing cerebral palsy (CP).
- The high incidence of CP in WS underscores the importance of early neuroimaging and intervention.
- Severe cognitive and motor deficits are common in children with both WS and CP.
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.
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