Objective: Here we report an interesting case of a young boy who presented to us with rapidly progressive febrile encephalopathy who developed a generalized dystonia, without myoclonus and a normal electroencephalograhy and ultimately succumbed to his illness within 6 weeks of disease onset.

Background: Subacute Sclerosing Encephalitis (SSPE) is a progressive catastrophic neurodegenerative disorder characterized commonly by progressive cognitive decline, behaviour and personality changes, focal or generalized seizures ,myoclonus, ataxia and visual disturbances. Movement disorders like dystonia, parkinsonism, tremor etc are uncommon having been described in isolated case reports or case series.

Method: A seventeen year old teenaged boy,was apparently asymptomatic one month prior to admission. He developed fever followed by irritable behavior, subtle forgetfulness, 2-3 days later . Over the next 3-4 weeks his sensorium progressively declined and he developed focal and generalized seizures with partial response to anti epileptic drugs.During his admission in the ward he developed abnormal dystonic posturing of right > left half of body with posturing of neck and trunk. Over the next 2 weeks, he became mute,unresponsive and ultimately succumbed to his illness within 2 weeks. Routine biochemical parameters, cerebrospinal fluid examination (CSF), EEG , MRI Brain were all normal at this stage. The child was adequately vaccinated. However there was a history suggestive of measles infection at 7 months of age.

Results: CSF antimeasles antibody titre was strongly positive. MRI brain revealed bilateral thalamic and right corona radia hyperintensities.Repeat EEG at this stage was normal. He was started on symptomatic treatment but developed progressive worsening of sensorium with generalized dystonia.

Conclusion: Conclusion: The occurrence of an unexplained movement disorders in a young adult, in the presence of acute to subacute progressively deteriorating sensorium should prompt us to consider SSPE as an etiology.Measles virus infection at a younger age, is a risk factor for fulminant SSPE which is invariably fatal within 6 months as is exemplified by our case.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/rapidly-progressive-fatal-dystonia-in-an-adult/