Rasmussen syndrome in a tropical environment: a study of six (6) observations

Background In sub-Saharan Africa, encephalitis of various etiologies is a major public health problem and Rasmussen syndrome is rarely diagnosed due to under-medicalization. The objective of this study is to describe the clinical and neuroradiological forms, especially since this affection is little known in sub-Saharan Africa as evidenced by the scarcity of publications. Results A retrospective, descriptive and analytical study of six (6) cases of Rasmussen syndrome, shows that it is an unrecognized disease in Africa. Men were more represented with a frequency of 67% with a young age. The clinical picture dominated by 100% seizures, mental deterioration and hemiparesis. The etiology is still questionable, probably autoimmune in our study. Conclusion Rasmussen syndrome accounts for 3% in 219 patients hospitalized for epileptic conditions. This study shows a clinical profile dominated by recurrent epileptic seizures refractory to the drugs Phenobarbital, Valproic Acid and Carbamazepine, the only antiepileptics available in the country. These results are valid for therapeutic and prognostic discussion.

Language after childhood hemispherectomy

ObjectiveTo conduct a systematic review on language outcomes after left and right hemispherectomy in childhood, a surgical procedure that involves removing or disconnecting a cerebral hemisphere.MethodsWe searched MEDLINE, Embase, and PsycInfo for articles published between January 1, 1988, and May 16, 2019. We included (1) all types of observational studies; (2) studies in which hemispherectomy was performed before age 18 years; and (3) studies with standardized scores measuring receptive vocabulary, expressive vocabulary, sentence comprehension, and/or sentence production. We calculated mean z scores after left and right hemispherectomy in the whole group and within etiology-specific subgroups.ResultsOur search identified 1,096 studies, of which 17 were eligible. The cohort added up to 205 individuals (62% left hemispherectomy) assessed 1 to 15 years after surgery. In the left surgery group, all language skills were impaired (z scores <−1.5) except sentence comprehension. In the right surgery group, language performance was in the borderline range (z scores ∼ −1.5). Children with cortical dysplasia showed the worst outcomes irrespective of surgery side (z scores <−2.5). Individuals with left vascular etiology and right-sided Rasmussen syndrome showed the best outcomes.ConclusionEvidence based on the largest patient cohort to date (205 participants) suggests that the risk of language impairment after hemispherectomy is high, with few exceptions. Etiology plays a major role in postsurgical plasticity. We recommend specialist evaluation of language skills soon after surgery to identify intervention targets. Large-scale studies examining outcomes in consecutive cases are still needed.

Immunomodulation with Azathioprine therapy in Rasmussen syndrome: A multimodal evaluation

Objective:To verify safety and efficacy of the corticosteroid-sparing drug Azathioprine (AZA) in Rasmussen syndrome (RS), we retrospectively analyzed a cohort of RS patients recruited in a single pediatric neuroscience center.Methods:We compared outcomes in 30 RS patients who received AZA with 23 patients who were not treated with this drug. We used a multimodal approach to correlate therapy with clinical features (seizures, epilepsia partialis continua [EPC], hemiparesis) and neuroimaging markers of progressive brain atrophy.Results:AZA was well tolerated; only one patient discontinued treatment due to pancytopenia. In 27/30 AZA patients, all of whom were corticosteroid responders, corticosteroid therapy could be weaned or reduced without worsening of seizures in 89%. AZA patients had a lower prevalence of EPC (42% vs. 67% in controls) and hemiparesis (64% vs. 92%, respectively). Cox regression showed for the AZA group compared to controls a delayed time to: 1) EPC (of about 2 years, Exp(B)=0.295, 95%CI[0.108, 0.807];p=0.017), 2) hemiparesis (about one year, Exp(B)=0.315, 95%CI[0.137, 0.724];p=0.007), and 3) surgery (about 2 years, Exp(B)=2.068, 95%CI[1.012, 4.227];p=0.046). However, there were no group differences in cognitive decline over time (IQ change per year) or in hemispheric grey matter atrophy on serial MRI scans.Conclusion:AZA treatment appears to slow clinical progression of Rasmussen syndrome in steroid responders; this will give most advantage in patients in the early stages of the disease in whom surgical decision-making may require further time.Classification of Evidence:This study provides Class III evidence that for pediatric RS patients AZA is well tolerated and slows hemiparesis and appearance of EPC.