NeurologyTier 1Approach to a presentation

Seizures - myoclonic

Red flags

  • Myoclonus + progressive ataxia + cognitive decline -> progressive myoclonic epilepsy (Unverricht-Lundborg, Lafora, MERRF, sialidosis, neuronal ceroid lipofuscinosis)
    • Not benign JME; needs genetic and metabolic workup
  • Myoclonic status epilepticus after cardiac arrest - within 24-48 h, generalised, stimulus-sensitive -> marker of severe hypoxic-ischaemic injury
    • Distinguish from Lance-Adams (post-hypoxic action myoclonus) which appears on awakening and is compatible with good outcome
  • Acute encephalopathy with myoclonus - metabolic (uraemia, hepatic, hyponatraemia), sepsis, drug toxicity, serotonin syndrome, CJD
  • Focal myoclonus (epilepsia partialis continua) -> structural lesion, Rasmussen encephalitis, non-ketotic hyperglycaemia
  • New myoclonus in an older adult with rapid cognitive decline -> CJD, autoimmune encephalitis, paraneoplastic
  • Anti-NMDAR / anti-LGI1 - faciobrachial dystonic seizures, psychiatric prodrome, hyponatraemia (LGI1)
  • Status epilepticus >5 min, or any seizure with airway compromise

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