Abstract
ObjectiveTo determine the contribution of electrophysiologic testing in the diagnosis and anatomical classification of myoclonus.MethodsParticipants with a clinical diagnosis of myoclonus were prospectively recruited, each undergoing a videotaped clinical examination and battery of electrophysiologic tests. The diagnosis of myoclonus and its subtype was reviewed after 6 months in the context of the electrophysiologic findings and specialist review of the videotaped clinical examination.ResultsSeventy-two patients with myoclonus were recruited. Initial clinical anatomical classification included 25 patients with cortical myoclonus, 7 with subcortical myoclonus, 2 with spinal myoclonus, and 15 with functional myoclonic jerks. In 23 cases, clinical anatomical classification was not possible because of the complexity of the movement disorder. Electrophysiologic testing was completed in 66, with agreement of myoclonus in 60 (91%) and its subtype in 28 (47%) cases. Subsequent clinical review by a movement disorder specialist agreed with the electrophysiologic findings in 52 of 60; in the remaining 8, electrophysiologic testing was inconclusive.ConclusionsElectrophysiologic testing is an important additional tool in the diagnosis and anatomical classification of myoclonus, also aiding in decision-making regarding therapeutic management. Further development of testing criteria is necessary to optimize its use in clinical practice.
Highlights
Participants with a clinical diagnosis of myoclonus were prospectively recruited, each undergoing a videotaped clinical examination and battery of electrophysiologic tests
Further development of testing criteria is necessary to optimize its use in clinical practice
The demographic details and clinical characteristics of this cohort are summarized in table 2 and table e-1, respectively
Summary
Participants with a clinical diagnosis of myoclonus were prospectively recruited, each undergoing a videotaped clinical examination and battery of electrophysiologic tests. The diagnosis of myoclonus and its subtype was reviewed after 6 months in the context of the electrophysiologic findings and specialist review of the videotaped clinical examination. Electrophysiologic testing The standardized electrophysiologic protocol included an initial polymyography, with participants excluded at this stage if the myoclonus was too subtle to adequately perform the assessment. For those meeting electrophysiologic criteria for myoclonus, further investigations included EEG-EMG backaveraging (if >25 jerks) or coherence analysis (if jerk frequency was >3 Hz). Where possible, those with CM and SCM underwent testing for SSEPs Those with CM and SCM underwent testing for SSEPs (figure e-1, http://links.lww. com/WNL/A164)
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