P20 – 2476: Atypical and fulminant clinical course subacute sclerosing panencephalitis

Abstract

Objective Subacute sclerosing panensefalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. Patient usually have behavioral changes, myoclonus, demantia, visual disturbances, and pyramidal and extrapyramidal sign. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demostration of increased antibody titre againist measles in the plasma and cerebrospinal fluid. Neuroimaging has limited role in the early diagnosis of SSPE. Methods 17 year old girl presented with progressively increasing myoclonic jerks, psychiatric manifestations and cognitive decline for two weeks. There was no fever and vomiting. She had measles at age 1 year. Her developmental milestones were normal. On her examination, patient interact poorly with the examiner and was partial oriented to place or time. She had frequent myoclonic jerks involving limbs, face, head, and gait instability. Tone was increased and deep tendon reflexes were exaggerated in all limbs and bilateral plantar reflexes were extensor. Fundus examination showed papilledema and bilateral macular retinitis. Complete blood count and serum chemistries including lactate and ammonia were normal. Serologies for Herpes simplex virus, and mycoplasma were negative. Cerebrospinal fluid (CSF) examination showed no cells, and normal protein (35.7 mg/dL), and glucose (65 mg/dL). Initial MRI was normal but after 20 days showed nonspesific white matter chances on posterior periventricular area. Electroencephalogram (EEG) showed bilateral symmetrical, syncronous periodic high voltage bursts of polyphasic, steretyped delta waves. The Measles Ig G antibody in CSF was 185194 U/ml, and Ig G index was higher than normal value. Results We diagnosed SSPE to based on clinical feature, typical EEG finding, and laboratory result. Conclusion SSPE is still an important medical problem in the developing countries. So SSPE should be considered when a patient admitted with atypical clinical features like loss of consciousness, acute partial-generalized convulsion, visual loss.