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Lance-Adams Post-Hypoxic Myoclonus — SCE Neurology MCQ

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HardMovement DisordersLance-Adams Post-Hypoxic MyoclonusSCE Neurology

A 55-year-old man presents with action myoclonus affecting both hands, worse with intentional movements. He also has cerebellar ataxia and epilepsy. He had a cardiac arrest 6 months ago with anoxic brain injury. MRI brain shows diffuse cortical atrophy. What is the most likely diagnosis?

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Correct answer: B — Lance-Adams syndrome (post-hypoxic myoclonus)

Lance-Adams syndrome is a chronic post-hypoxic action myoclonus syndrome occurring after recovery from cardiac arrest or other causes of cerebral anoxia. It is characterised by intention/action myoclonus (not present at rest), often with cerebellar ataxia and seizures. It can be severely disabling. Treatment includes levetiracetam, sodium valproate, clonazepam, and piracetam. A: Essential myoclonus is not associated with hypoxia or ataxia. C: CJD has rapid progression and specific MRI changes. D: JME has myoclonic jerks on awakening without prior hypoxic injury. E: OMS has chaotic saccades and an underlying malignancy.

Reference: ABN Myoclonus Guidelines; Lance & Adams Original Description (1963)