The clinical profile and outcome of children with West syndrome in KwaZulu-Natal Province, South Africa: A 10-year retrospective review

Background. West syndrome (WS) is a rare epileptic encephalopathy of infancy. There is currently no research on the incidence or prevalence of WS in Africa.Methods. We aimed to describe the outcome of children with WS at a quaternary-level hospital in KwaZulu-Natal, South Africa (SA). This was a retrospective chart review conducted on patients diagnosed with WS over a 10-year period. Eight children (males, n=7; African, n=6; Asian, n=2) identified with WS out of 2 206 admitted with epilepsy. The median age (range) at diagnosis was 7.5 (1 - 9) months. The average time between onset of epileptic spasms and diagnosis was 3.1 months.Results. Six patients had abnormal neuroimaging (atrophy (n=2); corpus callosum agenesis (n=2); tuberous sclerosis (n=1); focal dysplasia (n=1)). Drug management included sodium valproate (n=8), topiramate (n=7) and levetiracetam (n=3). Subsequent definitive treatment was intramuscular adrenocorticotrophic hormone (n=3), vigabatrin (n=2) and oral prednisone (n=4). Four (50%) patients had complete seizure remission (neuromigratory disorder (n=2); tuberous sclerosis (n=1); and idiopathic (n=1)) and 4 had partial remission (neonatal complications (n=3); idiopathic (n=1).Discussion. Most of our patients had symptomatic WS, with 50% remission on treatment. Outcomes were poorer in our study when compared with those in published data.Conclusion. Further collaborative studies are still needed to evaluate the true impact and prevalence of WS in SA

Tuberous sclerosis with rhabdomyoma.

Tuberous sclerosis is a neurocutaneous syndrome characterized by abnormalities of both the integument and central nervous system. We present a case of tuberous sclerosis with rhabdomyoma in the heart. This was a 1½-year-old female child with infantile spasms and rhabdomyoma in heart with mother having neurocutaneous markers of tuberous sclerosis. Magnetic resonance imaging brain and electroencephalography findings were consistent with diagnosis.

Pediatric epilepsy -- an Indian perspective.

Prevalence studies from India suggest that epilepsy prevalence is similar to developed nations. Neurocysticercosis (NCC) predominates as an etiology. A large treatment gap is still a public health problem. Benign epilepsies and West syndrome appear to be underrepresented in studies on classification of seizures/syndromes. Febrile seizures prevalence in India is similar to other countries and appear to be as benign. Risk factors of intractable epilepsy (IE) in Indian studies include early age of onset, neurodevelopmental abnormalities and certain seizure types. Perinatal injuries underlie many IE. Many IE are not truly intractable and respond to simple therapeutic measures. The ketogenic diet and surgery are other methods now being used in Indian centers. Neurocysticercosis and neonatal hypoglycemic brain injury, two widely prevalent etiologies are reviewed in detail.