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November 11, 2014; 83 (20) Views & Reviews

Propriospinal myoclonus

Clinical reappraisal and review of literature

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
First published October 10, 2014, DOI: https://doi.org/10.1212/WNL.0000000000000982
Sandra M.A. van der Salm
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.
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Roberto Erro
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.
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Carla Cordivari
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.
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Mark J. Edwards
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.
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Johannes H.T.M. Koelman
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.
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Tom van den Ende
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.
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Kailash P. Bhatia
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.
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Anne-Fleur van Rootselaar
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.
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Peter Brown
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.
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Marina A.J. Tijssen
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.
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Full PDF
Citation
Propriospinal myoclonus
Clinical reappraisal and review of literature
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
Neurology Nov 2014, 83 (20) 1862-1870; DOI: 10.1212/WNL.0000000000000982

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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.

GLOSSARY

BP=
Bereitschaftspotential;
DTI=
diffusion tensor imaging;
FMD=
functional movement disorders;
PSM=
propriospinal myoclonus;
RLS=
restless legs syndrome

Footnotes

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Received March 4, 2014.
  • Accepted in final form August 11, 2014.
  • © 2014 American Academy of Neurology
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