Background: Status epilepticus (SE) is a common, life threatening neurologic disorder that is essentially an acute, prolonged epileptic crisis. SE can represent an exacerbation of a pre-existing seizure disorder, the initial manifestation of a seizure disorder, or an insult other than a seizure disorder.Objectives: To study the aetiology, clinical profile, and outcome of SE in pediatric age group.Setting and study design: Prospective study at a tertiary care medical college hospital in Bihar, India. Materials and methods:Study was carried out for a period of one year (from April 2008 to March 2009). Seventy patients of SE in the age group of six month to 12 years were included in the study. Clinical history, general and systemic examination and relevant investigations along with pretested questionnaire were used to categorise different variables. Independent t-test was used for continuous variables and chi-square test for categorical variables.Results: Mean age for the study population was found to be 5.94 years (SD=3.152). Preponderance of male (60%) over female (40%) was observed. Aetiology included Idiopathic (27.14%), remote symptomatic (20%), acute symptomatic (47.14%), febrile (2.86%) and progressive encephalopathy (2.86%) groups. Generalised tonic clonic convulsion (GTC) convulsion was observed in 91.4% of SE patients while 8.6% had partial SE. Eighteen patients (25.7%) had prior history of convulsion whereas 52 patients (74.3%) presented with SE as first episode of convulsion. In our study, mortality rate was found to be 31.4% and acute symptomatic causes were responsible for most of the deaths.Conclusion: SE is a severe life threatening emergency with substantial morbidity and mortality. Patients with younger age and male sex are slightly more vulnerable to develop SE. Longer duration of SE and acute symptomatic aetiologies are independent predictors for poor outcome.

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Hemophilia A and hemophilia B are the most common and serious congenital coagulation factor deficiencies. Intracranial hemorrhages occur in 3-10%. Spinal epidural hematomas are rare. Even with severe hemophilia, only 90% have evidence of increased bleeding by 1 year of age. Only 2% of neonates with hemophilia sustain intracranial hemorrhages. Here we describe a case of a 5-month old boy with positive family history of hemophilia A on his maternal side who was admitted to the hospital because of retention of urine and decreased movement in both lower limbs following history of fall from height (approximately 2 feet high), two days prior to admission. Physical examination showed no skin lesion or hematoma. Neurological examination showed the infant in frog like posture with flaccid paralysis of both lower limbs without loss of sensation. There was associated bladder involvement with a Phantom hernia on the right side of his abdomen. MRI Spine revealed cord contusion in D4-D9 segment with spinal subdural hemorrhage. Coagulation profile was abnormal with a prolonged Activated plasma thromboplastin time. Factor VIII assay revealed a level of 1%. Treatment was conservative, and the infant was given factor VIII replacement. There was remarkable improvement within weeks. Thus, spinal hematomas being rare should still be considered and ruled out for prompt management of cases of suspected hemophilia. This case highlights the importance of a thorough family history which led to the ultimate diagnosis of severe Hemophilia A by coagulation profile and neuroimaging and further confirmed by factor VIII assay.

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