ABSTRACT
To determine the clinical and demographic characteristics of children diagnosed with Subacute sclerosing panencephalitis [SSPE]. Case series. The Aga Khan University Hospital, Karachi, from January 2000 to June 2012. A retrospective analysis was done, regarding medical charts of 43 children under the age of 16 years with a discharge diagnosis of SSPE. Demographic and clinical characteristics were recorded. Results were expressed as percentages. Most of the 43 patients were male [72%]. The average age at presentation was 8.7 years with average duration of symptoms being 100.6 days. History of measles was present in 17 patients [39.5%]. All children had seizures at presentation and 65% had cognitive impairment. Most patients required poly therapy for control of seizures. Sodium valproate was the most commonly used anti-epileptic agent; Isoprinosine was tried in 22 [51%] patients. CSF for antimeasles antibodies was positive in approximately 86% of the 40 [93%] children. EEG showed burst suppression pattern in 36 [83.7%] cases. Forty-two patients [97.6%] were discharged home in a vegetative state. SSPE is progressive neurodegenerative disorder. It can be prevented by timely immunization against measles. Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis. Most patients experience a gradual but progressive decline in motor and cognitive functions
ABSTRACT
Status epilepticus [SE] is a common, life-threatening neurologic disorder. The exact incidence is not known. The frequency of occurrence of status epilepticus is 17-23 per 100, 000 persons per year, with the higher incidences occurring in developing countries. In children the mortality from SE ranges from 3-10%. To describe the etiology, clinical profile and immediate-outcome of children with status epilepticus at a Tertiary Care Hospital in Karachi. This is a descriptive and retrospective cohort study on all children admitted in our institution with diagnosis of status epileptics [ICD 9 code 3453]. Demographic, pertinent clinical variables and outcomes were collected on structured questionnaire. During the study period, fifty patients were identified. Mean age was 51 months [age range was 1-168 months with SD +/- 41 months]. There were 29 male: and 21 female. Generalized tonic clonic seizures were the most common [86%] form of seizures observed. Twenty- five [50%] patients were newly diagnosed with no prior history of seizure. Acute febrile illness or infections were the most common etiology [52%]. No biochemical abnormalities were observed in our study. Abnormal EEG was reported in 62% of patients. CSF abnormalities were observed in 22%. Minimum 2 and maximum 8 anti-epileptic drugs were used [mean= 4.33]. The most commonly parenteral drugs included phenytoin, phenobarbitone, levetiracetam and valproic acid. Thirty- one [62%] patients required continuous midazolam infusion. In majority [44%], status was controlled after more than 60mins. Thirty-three patients [66%] required PICU admission for seizure control. Thirty one [62%] required mechanical ventilation, twenty five [50%] required inotropic support. Mean PICU stay duration was 3.89 days [range=1-15 days]. Survival rate was 92%. The cause of death [n=4] was related to underlying systemic illness. No complications were observed in forty-one [82%] patients. In our report, young children had a high incidence of SE and the most common etiology was acute febrile illness. Majority of cases were refractory SE although the mortality rate was low