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Clinical features of specific types of myoclonus

Clinical features of specific types of myoclonus
Type Distribution Temporal profile Activation Clinical neurophysiology Anatomic-physiologic classification Clinical-etiologic classification Specific etiology
Hypnic jerks (massive myoclonus of sleep) Generalized Usually single Occur only during sleep or sleep transitions Not studied Presumed subcortical-nonsegmental Physiologic Normal phenomenon
Partial myoclonic jerks (of sleep) Multifocal, distal muscles Usually single Occur only during sleep or sleep transitions Not studied Presumed subcortical-nonsegmental Physiologic Normal phenomenon
Myoclonus-dystonia Neck, trunk, proximal arms Irregular Rest, action, and posture EMG with 50 to 100 ms discharges; EEG and SEP normal Subcortical-nonsegmental Essential Genetically heterogeneous; SGCE, DYT15 mutations
Juvenile myoclonic epilepsy Usually generalized Irregular Occurs from rest EEG with 4 to 6 hertz polyspike and wave discharge Cortical-subcortical Epileptic Genetically heterogeneous; EFHC1 and GABRA1 mutations
Palatal tremor Palate, larynx, face; usually bilateral Rhythmic Persistent, continues during sleep EMG with >100 ms rhythmic discharges Segmental Symptomatic (secondary) palatal tremor Brainstem pathology in the region of the Guillain-Mollaret triangle
Essential palatal tremor Idiopathic
Posthypoxic myoclonus Multifocal and/or generalized Irregular, less often rhythmic Action and reflex EMG discharges <50 ms; cortical EEG correlate grossly time-locked with EEG back-averaging Cortical Symptomatic (secondary) Hypoxic brain injury
Progressive myoclonus epilepsy (PME) and progressive myoclonus ataxia (PMA) Multifocal and/or generalized Irregular Action and reflex EMG discharges <50 ms; cortical EEG correlate grossly time-locked with EEG back-averaging Cortical Symptomatic (secondary) Unverricht-Lundborg disease, Lafora body disease, neuronal ceroid lipofuscinosis, myoclonic epilepsy with ragged red fibers (MERRF), sialidosis
Toxic-metabolic myoclonus Multifocal, sometimes generalized Irregular Rest and action Variable; positive or negative myoclonus; EMG discharges or silence lasting 50 to 200 ms; cortical correlate possible Cortical most common Symptomatic (secondary) Drugs, toxins, organ failure
Neurodegenerative disease Multifocal and worse distally; sometimes generalized Irregular Action most common, then rest and reflex EMG discharges <50 ms; cortical EEG correlate grossly time-locked with EEG back-averaging Cortical Symptomatic Alzheimer disease, dementia with Lewy bodies, Parkinson disease, Huntington disease
Creutzfeldt-Jakob disease Focal, multifocal, or generalized Single, irregular, or rhythmic Rest, action, and/or reflex in response to somatosensory stimuli, particularly loud noise (startle) EEG with periodic synchronous biphasic or triphasic sharp wave complexes; EMG discharge <50 ms Cortical Symptomatic (secondary) Prion disease
EMG: electromyography; EEG: electroencephalography; SEP: somatosensory evoked potential; SGCE: sarcoglycan epsilon; DYT15: dystonia 15, myoclonic; EFHC1: EF-hand domain containing 1; GABRA1: gamma-aminobutyric acid type A receptor subunit alpha1.
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