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Review
. 2014 Nov 11;83(20):1862-70.
doi: 10.1212/WNL.0000000000000982. Epub 2014 Oct 10.

Propriospinal myoclonus: clinical reappraisal and review of literature

Sandra M A van der Salm  1 , Roberto Erro  1 , Carla Cordivari  1 , Mark J Edwards  1 , Johannes H T M Koelman  1 , Tom van den Ende  1 , Kailash P Bhatia  1 , Anne-Fleur van Rootselaar  1 , Peter Brown  1 , Marina A J Tijssen  2
Affiliations
Review

Propriospinal myoclonus: clinical reappraisal and review of literature

Sandra M A van der Salm et al. Neurology. .

Abstract

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.

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References

    1. Brown P, Thompson PD, Rothwell JC, Day BL, Marsden CD. Axial myoclonus of propriospinal origin. Brain 1991;114:197–214. - PubMed
    1. Brown P, Rothwell JC, Thompson PD, Marsden CD. Propriospinal myoclonus: evidence for spinal “pattern” generators in humans. Mov Disord 1994;9:571–576. - PubMed
    1. Brown P, Thompson PD, Rothwell JC, Day BL, Marsden CD. Paroxysmal axial spasms of spinal origin. Mov Disord 1991;6:43–48. - PubMed
    1. Chokroverty S, Walters A, Zimmerman T, Picone M. Propriospinal myoclonus: a neurophysiologic analysis. Neurology 1992;42:1591–1595. - PubMed
    1. Erro R, Bhatia KP, Edwards MJ, Farmer SF, Cordivari C. Clinical diagnosis of propriospinal myoclonus is unreliable: an electrophysiologic study. Mov Disord 2013;28:1868–1873. - PubMed

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