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Case Report: An Unusual Case of Subacute Sclerosing Panencephalitis with Distinctive Clinical and Neuroimaging
Ravindra Kumar Garg1, Shweta Pandey1, Harish Nigam1
1Department of Neurology, King George's Medical University Uttar Pradesh, Lucknow, India.
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.
Insights
Subacute sclerosing panencephalitis (SSPE), a fatal measles complication, presented unusually in a child with distinct neuroimaging findings. This case highlights rare cystic lesions in the white matter, prompting further investigation into their pathology.
Area of Science:
- Neurology
- Pediatrics
- Infectious Diseases
Background:
- Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal neurological disorder invariably linked to measles virus infection.
- It is more prevalent in measles-endemic regions and characterized by severe cognitive and motor decline.
Observation:
- A 9-year-old boy presented with a 5-month history of progressive neurological deterioration, including myoclonus, akinetic mutism, and dystonic storms.
- Clinical presentation included inappropriate emotional lability and marked right-sided dystonic posturing.
- Electroencephalography showed periodic discharges, and cerebrospinal fluid revealed elevated anti-measles IgG antibodies.
Findings:
- Neuroimaging demonstrated severe diffuse cerebral atrophy and periventricular white matter abnormalities.
- Unusually, multiple small, discrete cystic lesions were observed in the periventricular white matter on T2/FLAIR MRI sequences.
- The patient received intrathecal interferon-α, but remains in an akinetic-mute state.
Implications:
- This case underscores the diverse clinical and neuroimaging spectrum of SSPE.
- The presence of unique cystic lesions in SSPE warrants further research to elucidate their pathological basis.
- Understanding these atypical features may improve diagnostic accuracy and inform future therapeutic strategies for SSPE.
