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Case Report: An Unusual Case of Subacute Sclerosing Panencephalitis with Distinctive Clinical and Neuroimaging Features

Ravindra Kumar GargDepartment of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India

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Shweta PandeyDepartment of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India

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Harish NigamDepartment of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India

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D. B. KeerthirajDepartment of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India

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Imran RizviDepartment of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India

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Neeraj KumarDepartment of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India

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Ravi UniyalDepartment of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India

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Hardeep Singh MalhotraDepartment of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India

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Praveen Kumar SharmaDepartment of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India

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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

Address correspondence to Ravindra Kumar Garg, Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow 226003, India. E-mail: garg50@yahoo.com

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.

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