Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by degeneration of alpha motor neurons in the spinal cord, resulting in progressive proximal muscle weakness and paralysis. In addition to the three classical SMA types, a new form known as type 0 with intrauterine onset, profound hypotonia and a progressive and early fatal course has been described. Herein we report a case of type 0 SMA with a Dandy Walker variant anomaly, which has not hitherto been reported in the world literature.

Context:There is limited data to support need of lumbar puncture among Indian children aged less than 5 years presenting with a first episode of fever and seizure. Aims: To determine the incidence and clinical predictors of meningitis among children aged 6-60 months presenting with a first episode of febrile convulsion. Settings and Designs: A prospective study was conducted on 35 children (6-60 months) with a first episode of febrile convulsion subjected to lumbar puncture in a tertiary care teaching hospital of North India. Materials and Methods: Clinical characteristics were compared between the two groups: Children with meningitis (n = 17) and children without meningitis (n = 18). Statistical Methods: Multivariate logistic regression was applied to assess the independent predictors of meningitis. Results: A total of 120 children were screened; 35 children subjected to lumbar puncture were finally enrolled. The mean (SD) age of enrolled children was 18.49 (10.79) months. The incidence of meningitis was 48.6% (17/35). Children with meningitis significantly had a higher proportion of children with high grade (temperature >104°F) fever (P = 0.005), received prior antibiotics (P = 0. 041), had lower hemoglobin levels (P = 0.04) and lower blood sugar levels (P = 0.03) as compared to children with no meningitis. On multivariate logistic regression, it was observed that high-grade fever was an independent predictor of meningitis (odds ratio: 0.03 [0.001-0.86] [P = 0.04]). Conclusion: We found that the presence of high-grade fever was an important predictor of meningitis among children aged 6-60 months presenting with a first episode of febrile convulsion.