Subacute sclerosing panencephalitis

Case contributed by Ammar Haouimi

Presentation

Primary measles infection at the age of 4 months. Presented with a history of progressive ataxia with seizures.

Patient Data

Age: 3 years
Gender: Male

Low signal T1 and high signal T2 and FLAIR of the brainstem and middle cerebellar peduncles with no enhancement on postcontrast sequences. Restricted diffusion within the lesions of the middle cerebellar peduncles with mild cerebellar volume loss.

Cerebral volume loss with no abnormal signal of the basal ganglia or white matter at the supratentorial level.

Case Discussion

The clinical presentation and the MRI features are most consistent with subacute sclerosing panencephalitis (in this case, the CSF analysis was positive for anti-measles antibodies).

Subacute sclerosing panencephalitis (SSPE), also known as Dawson disease, is a rare chronic, progressive and fatal encephalitis that affects primarily children and young adults, caused by a persistent infection of immune resistant measles virus.

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