Myoclonus Phenomenology Atlas (Part 4): Progressive Myoclonic Epilepsies and Genetic Myoclonus Disorders
Video 10.62 (C10c62): Reticular Reflex Myoclonus (Posthypoxic)
Proximal positive and negative myoclonus with severe action-induced disability.
Video 10.63 (C10c63): Chronic Lance-Adams Syndrome
Residual action myoclonus with cerebellar signs and touch-responsive gait instability.
Video 10.64 (C10c64): Severe Mixed Cortical and Subcortical Posthypoxic Myoclonus
Profound action myoclonus causing near-complete functional dependence.
Video 10.65 (C10c65): Alcohol-Responsive Posthypoxic Myoclonus
Severe action and negative myoclonus dramatically improving with alcohol and sodium oxybate.
Video 10.66 (C10c66): Severe Lance-Adams Syndrome Responsive to Sodium Oxybate
Extreme truncal and appendicular myoclonus improved by oxybate and GPi DBS.
Video 10.67 (C10c67): Posthypoxic Myoclonus Improved by Sodium Oxybate
Action and gait-limiting myoclonus with acute improvement after treatment.
Video 10.68 (C10c68): Severe Task-Induced Posthypoxic Myoclonus
Target-dependent action myoclonus preventing standing and purposeful hand use.
Video 10.69 (C10c70): Postanoxic Myoclonus with Dystonia
Action myoclonus and dystonia interfering with writing, pouring, and coordination.
Video 10.70 (C10c71): Severe Postanoxic Myoclonus
Action, dystonic, positive, and negative myoclonus causing assisted ambulation.
Video 10.71 (C10c72): EPM1 (Unverricht-Lundborg Disease)
Generalized action myoclonus with excellent response to levetiracetam.
Video 10.72 (C10c73): Advanced EPM1
Myoclonus, dysarthria, cerebellar signs, and treatment-resistant epilepsy.
Video 10.73 (C10c74): MERRF Syndrome
Positive and negative myoclonus worsened by action and standing.
Video 10.74 (C10c75): NUS1-Related Myoclonus Syndrome
Task-specific action myoclonus with preserved gait and cognition.
Video 10.75 (C10c76): Sialidosis Type I
Action myoclonus associated with cherry-red macular spots.
Video 10.76 (C10c77): Sialidosis Type I with Ocular Motor Abnormalities
Nystagmus, mild myoclonus, and wide-based gait.
Video 10.77 (C10c78): Severe Sialidosis Type I
Stimulus-sensitive intention myoclonus and asterixis severely affecting fine motor tasks.