Objective: To highlight atypical presentation of SSPE and to create high index of awareness about phenotypic spectrum of the disease especially in developing countries.

Background: Subacute Sclerosing Panencephalitis is a neurodegenerative disorder of brain by persistent infection with aberrant measles virus. The disease usually presents with progressive cognitive decline, behavioural changes, myoclonus and seizures.Diagnosis is established by periodic high amplitude complexes in EEG and raised titres of anti measles antibody in CSF. We present 4 atypical cases of SSPE who deviated from usual clinical presentation mimicking movement disorders.

Methods: Case 1. 18 year old girl presented with upper limb onset progressive generalized dystonia, gait ataxia, spastic speech and progressive cognitive decline. At the onset of illness she had short lasting jerky movements which subsided later on. Case 2. 16 year old boy presented with slowly progressive balancing problems and constant rocking movements of the trunk. He also had progressive cognitive subnormality. Case 3. 20 years old young man had slowly progressive spastic hemidystonia syndrome involving left upper limb. Case 4. 22 year old young man presented with speech problems in the form of spastic dysarthria, freezing of gait and action induced orofacial dyskinesias

Results: MRI brain of all 4 patients showed T2 hyperintensities in subcortical white matter. EEG revealed periodic high amplitude (200-500MV) complexes in 4-10 seconds interval, non specific generalised slowing, frontal rhythmic delta activities. All the 4 patients were subjected to various investigations including viral markers and autoimmune profile. All the patients showed raised anti measles antibody IgG concentration ranging from 10-54ug/dl in CSF (1:4).

Conclusions: We present 4 cases of SSPE whose clinical presentation deviated from classical picture. Dystonia, freezing of gait, speech problem, action induced dyskinesia are rarely reported in SSPE. We opine that SSPE is a great mimicker and index of suspicion should be high in appropriate clinical setting

References: 1. Atypical presentation of adult-age onset subacute sclerosing panencephalitis. Vilas D, Becerra JL, Lozano M, Soriano A, Matas L, Martro E, Dávalos A.—Rev Neurol 2012 Jan 1;54(1):60-1. 2. Adult onset subacute sclerosing panencephalitis. Report of two cases with atypicalpresentation:Valenzuela V1, Miranda M, Aguilera L, Fabres L, Galdames D.—Rev Med Chil 1999 May;127(5):589-94.

« Back to 2018 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/atypical-presentation-in-4-patients-of-subacute-sclerosing-pan-encephalitis-sspe-widening-phenotypic-horizon/