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

Propriospinal myoclonus: clinical reappraisal and review of literature.

Author information

1
From the Department of Neurology and Clinical Neurophysiology (S.M.A.v.d.S., J.H.T.M.K., T.v.d.E., A.-F.v.R.), Academic Medical Center, Amsterdam, the Netherlands; Sobell Department of Motor Neuroscience and Movement Disorders (R.E., C.C., M.J.E., K.P.B.), University College London (UCL) Institute of Neurology, London; Clinical Neurophysiology (R.E., C.C.), National Hospital for Neurology and Neurosurgery, London; Nuffield Department of Clinical Neurosciences (P.B.), University of Oxford, John Radcliffe Hospital, Oxford, UK; and the Department of Neurology (M.A.J.T.), University Medical Center Groningen, University of Groningen, the Netherlands.
2
From the Department of Neurology and Clinical Neurophysiology (S.M.A.v.d.S., J.H.T.M.K., T.v.d.E., A.-F.v.R.), Academic Medical Center, Amsterdam, the Netherlands; Sobell Department of Motor Neuroscience and Movement Disorders (R.E., C.C., M.J.E., K.P.B.), University College London (UCL) Institute of Neurology, London; Clinical Neurophysiology (R.E., C.C.), National Hospital for Neurology and Neurosurgery, London; Nuffield Department of Clinical Neurosciences (P.B.), University of Oxford, John Radcliffe Hospital, Oxford, UK; and the Department of Neurology (M.A.J.T.), University Medical Center Groningen, University of Groningen, the Netherlands. M.A.J.de.Koning-Tijssen@umcg.nl.

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.

PMID:
25305154
PMCID:
PMC4240434
DOI:
10.1212/WNL.0000000000000982
[Indexed for MEDLINE]
Free PMC Article

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