A rare presentation of fulminant subacute sclerosing panencephalitis with acute disseminated encephalomyelitis and meningoencephalitis

Abstract

Subacute sclerosing panencephalitis (SSPE) is a rare form of chronic progressive neurodegenerative disorder, caused by persistent central nervous system (CNS) infection by hypermutated strains of Measles virus. It primarily affects children and early adolescent age group. Since there is widespread involvement of CNS, the primary symptoms may start with behavior and personality changes with intellectual involvement followed with myoclonic jerks, convulsion, and hemi or quadriparesis. In later stage, the patient may experience dementia, stupor, coma, or death. However, initial presentation can be variable and atypical, making the diagnosis very challenging in clinical practice. Herein, we report a case of fulminant SSPE in a 13-year-old adolescent male who presented with the features of acute disseminated encephalomyelitis (ADEM) and meningoencephalitis. The patient presented in altered sensorium with the history of poor communications, jerky movement of limbs, and fever for 10–14 days. Preliminary investigations were suggestive of meningoencephalitis and ADEM. After initial course of treatment, the patient did not improve, deteriorated further, and succumbed to his illness, a day before we received the conclusive reports for SSPE. As we experienced from our case report, SSPE can create a lot of diagnostic dilemma to the clinicians in such a fulminant presentation, mainly due to limitation of resources, time, and lack of proper vaccination records. The lack of definitive treatment for SSPE once again proved the proverb that “prevention will always be better than cure.”