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Propriospinal myoclonus: Clinical reappraisal and review of literature.
MedLine Citation:
PMID:  25305154     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
OBJECTIVE: Propriospinal myoclonus (PSM) is a rare disorder with repetitive, usually flexor arrhythmic brief jerks of the trunk, hips, and knees in a fixed pattern. It has a presumed generation in the spinal cord and diagnosis depends on characteristic features at polymyography. Recently, a historical paradigm shift took place as PSM has been reported to be a functional (or psychogenic) movement disorder (FMD) in most patients. This review aims to characterize the clinical features, etiology, electrophysiologic features, and treatment outcomes of PSM.
METHODS: Re-evaluation of all published PSM cases and systematic scoring of clinical and electrophysiologic characteristics in all published cases since 1991.
RESULTS: Of the 179 identified patients with PSM (55% male), the mean age at onset was 43 years (range 6-88 years). FMD was diagnosed in 104 (58%) cases. In 12 cases (26% of reported secondary cases, 7% of total cases), a structural spinal cord lesion was found. Clonazepam and botulinum toxin may be effective in reducing jerks.
CONCLUSIONS: FMD is more frequent than previously assumed. Structural lesions reported to underlie PSM are scarce. Based on our clinical experience and the reviewed literature, we recommend polymyography to assess recruitment variability combined with a Bereitschaftspotential recording in all cases.
Authors:
Sandra M A van der Salm; Roberto Erro; Carla Cordivari; Mark J Edwards; Johannes H T M Koelman; Tom van den Ende; Kailash P Bhatia; Anne-Fleur van Rootselaar; Peter Brown; Marina A J Tijssen
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Publication Detail:
Type:  REVIEW     Date:  2014-10-10
Journal Detail:
Title:  Neurology     Volume:  -     ISSN:  1526-632X     ISO Abbreviation:  Neurology     Publication Date:  2014 Oct 
Date Detail:
Created Date:  2014-10-11     Completed Date:  -     Revised Date:  2014-10-12    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2014 American Academy of Neurology.
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