Matloob Azam, Nasira Bhatti, Jai Krishin, Tabish Hazir.
Subacute Sclerosing Panencephalitis.
J Pak Inst Med Sci Jan ;13(1):618-22.

Clinical features, results of laboratory investigations and course of the disease in children who were diagnosed as waving subacute sclerosing panencephalitis (SSPE) were evaluated It was retrospective analysis of patients admitted between January, 1995 and December, 1997 in Children Hospital, Pakistan Institute of Medical Sciences (PIMS), Islamabad. Children were included whose clinical presentation was consistent with the diagnosis of SSPE and in addition had either typical periodic slow wave complexes on Electroencephalogram (EEG) and or elevated anti-measles antibodies in cerebrospinal fluid (CSF). Fourteen children were diagnosed as SSPE during the study period, nine boys and five girls. Age ranged between four and half years to twelve years with a mean age of eight years at the time of .presentation. Duration of symptoms before presentation varied from two weeks to six months. Myoclonic jerks and losing milestones were most common initial features. Other features included walking difficulty, ataxia, behaviour problems and speech difficulty. Ten children had characteristic suppression-burst pattern and four had encephalopathic picture on EEG. CSF measles antibodies were positive in six. All patients were treated with antiepileptics and five also received antiviral agent amantadine orally. Response to treatment was poor and ultimately all patients were bedridden. Four children died in the ward because of aspiration pneumonia. It is concluded that SSPE is a rare progressive neurodegenerative disease of childhood with poor prognosis. At present no effective therapy is available.

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