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Case Report: An Unusual Case of Subacute Sclerosing Panencephalitis with Distinctive Clinical and Neuroimaging Features
ABSTRACT.
Subacute sclerosing panencephalitis (SSPE) is a relentlessly progressive brain disorder with invariable mortality. Subacute sclerosing panencephalitis is common in measles-endemic areas. We report an unusual SSPE patient with distinctive clinical and neuroimaging features. A 9-year-old boy came with a 5-month history of spontaneously dropping objects from both hands. Subsequently, he developed mental decline, a loss of interest in his surroundings, decreased verbal output, and inappropriate crying and laughing along with generalized periodic myoclonus. On examination, the child was akinetic mute. The child demonstrated intermittent generalized axial dystonic storm with flexion of upper limbs, an extension of lower limbs, and opisthotonos. Dystonic posturing was more dominant on the right side. Electroencephalography revealed periodic discharges. Cerebrospinal fluid antimeasles IgG antibody titer was markedly elevated. Magnetic resonance imaging revealed marked diffuse cerebral atrophy, and periventricular T2/fluid-attenuated inversion recovery hyperintensity. T2/fluid-attenuated inversion recovery images also revealed multiple cystic lesions present in the region of periventricular white matter. The patient was given a monthly injection of intrathecal interferon-α. The patient is currently continuing in the akinetic-mute stage. In conclusion, in this report, we described an unusual case of acute fulminant SSPE in which neuroimaging demonstrated unusual multiple small discrete cystic lesions in the cortical white matter. The pathological nature of these cystic lesions currently is not clear and needs to be explored.
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Author Notes
Authors’ addresses: Ravindra Kumar Garg, Shweta Pandey, Harish Nigam, D. B. Keerthiraj, Imran Rizvi, Neeraj Kumar, Ravi Uniyal, Hardeep Singh Malhotra, and Praveen Kumar Sharma, Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India, E-mails: garg50@yahoo.com, shwetapandey@kgmcindia.edu, harish.nigam007@gmail.com, dbkeerthiraj@gmail.com, imranrizvi09@gmail.com, drneeraj2903@gmail.com, ravi.sun.uniyal@gmail.com, drhsmalhotra@gmail.com, and pspgimer@gmail.com.