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Primary progressive multiple sclerosis — SCE Neurology MCQ

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HardMSPrimary progressive multiple sclerosisSCE Neurology

A 32-year-old develops progressive left focal motor seizures, then fixed left hemiparesis and hemianopia over four years. Serial MRI shows advancing atrophy confined to the right hemisphere. A low-positive serum GAD antibody has not been confirmed in CSF. Which diagnosis best accounts for the longitudinal pattern?

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Correct answer: D — Adult-onset Rasmussen encephalitis

Explanation lettering: D = shown as B · B = shown as C · E = shown as D · C = shown as E

E is correct: adult-onset Rasmussen encephalitis produces progressive drug-resistant focal seizures, unilateral neurological deficits and evolving unihemispheric cortical atrophy. A low unconfirmed serum GAD result should not override this phenotype; GAD encephalitis is usually limbic and does not progressively destroy one hemisphere. Dyke-Davidoff-Masson and hemiconvulsion-hemiplegia-epilepsy are static sequelae of earlier cerebral injury. MELAS produces stroke-like lesions that cross vascular territories but not this steadily unilateral atrophic course.

Reference: Adult-onset Rasmussen encephalitis: https://pn.bmj.com/content/25/5/475