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 Volume 72, Number 15, April 14, 2009
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NEUROLOGY 2009;72:1301-1309
© 2009 American Academy of Neurology

Propriospinal myoclonus revisited

Clinical, neurophysiologic, and neuroradiologic findings

E. Roze, MD, PhD, P. Bounolleau, MD, D. Ducreux, MD, PhD, V. Cochen, MD, PhD, S. Leu-Semenescu, MD, Y. Beaugendre, MD, M. C. Lavallard-Rousseau, MD, A. Blancher, MD, F. Bourdain, MD, P. Dupont, MD, L. Carluer, MD, L. Verdure, MD, M. Vidailhet, MD and E. Apartis, MD, PhD

From the Federation of Neurology (E.R., M.V.), Department of Rehabilitation (P.D.), Salpêtrière Hospital, AP-HP, Paris; Department of Neurology (P.B.), Val de Grâce Hospital, Paris; Department of Neuroradiology (D.D.), Bicêtre Hospital, APHP, Paris; Department of Neurology (V.C.), Purpan Hospital, Toulouse; Sleep Pathology Unit (S.L.-S.), Salpêtrière Hospital, AP-HP, Paris; Department of Physiology (Y.B., M.C.L.-R., A.B., E.A.), Saint Antoine Hospital, AP-HP, Paris; Department of Neurology (F.B.), Foch Hospital, Suresnes; Department of Neurology (L.C.), Regional University Hospital, Caen; Department of Neurosurgery (L.V.), Charles Nicolle Hospital, Rouen; and INSERM U679 (M.V.), CNRS UMR 7102 (E.R), INSERM U732 (E.A.), Pierre Marie Curie Paris VI University, Paris, France.

Address correspondence and reprint requests to Dr. Emmanuelle Apartis, Service de Physiologie, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris, France Emmanuelle.apartis{at}sat.aphp.fr

Objective: The literature on propriospinal myoclonus (PSM) is poor and there are no systematic reviews of the subject. We sought to clarify the spectrum of PSM.

Methods: We first prospectively investigated all patients seen in our movement disorders clinic with a firm diagnosis of PSM between 2002 and 2007. All had a standardized interview, detailed clinical examination, laboratory investigations, comprehensive neurophysiologic examination, and spinal cord MRI, including diffusion tensor imaging with fiber tracking (DTI-FT). We also collected drug responses. Finally, we conducted a systematic review of the literature.

Results: We enrolled 10 patients meeting the strict criteria for PSM, and also analyzed data on 50 patients from 26 previous reports. PSM occurred predominantly in male and middle-aged patients. The typical clinical picture consisted of myoclonic jerks consistently involving abdominal wall muscles, which worsen in the lying position. A premonitory sensation preceding the jerks and wake-sleep transition phase worsening were frequent. Most patients had a myoclonic generator at the thoracic level, with a myoclonus duration between 200 msec and 2 s. An underlying cause was infrequently found. DTI-FT detected cord abnormalities all of our patients.

Conclusion: The clinico-physiologic spectrum of propriospinal myoclonus (PSM) is homogenous. Involvement of the abdominal wall muscles, worsening in the lying position, premonitory sensation, and wake-sleep transition phase worsening are helpful clinical clues. Diffusion tensor imaging with fiber tracking appears more sensitive than conventional MRI for detecting associated microstructural abnormalities of the spinal cord. Symptomatic treatment of PSM is not straightforward, and clonazepam is reported to be the most effective drug. Zonisamide may be an interesting option.

Abbreviations: DTI-FT = diffusion tensor imaging with fiber tracking; MR-DTI-FT = magnetic resonance diffusion tensor imaging with fiber tracking; PSM = propriospinal myoclonus; SEP = sensory evoked potential.

Supplemental data at www.neurology.org

*These authors contributed equally.

Disclosure: The authors report no disclosures.

Medications: Clonazepam (Rivotril®; Roche, France); zonisamide (Zonegran®; Eisai, France); amitriptyline (Laroxyl®; Roche, France); ropinirole (Requip®; GlaxoSmithKline, France); morphine (Skenan®; Bristol-Myers Squibb, France); baclofen (Lioresal®; Novartis Pharma SAS, France); piracetam (Nootropil®; UCB Pharma, France); primidone (Mysoline®; Zeneca Pharma, France); levetiracetam (Keppra®; UCB Pharma, France); carbamazepine (Tegretol®; Novartis Pharma SAS, France); oxcarbazepine (Trileptal®; Novartis Pharma SAS, France); valproate (Depakine®; Sanofi Aventis, France); topiramate (Epitomax®; Janssen Cilag, France); pregabalin (Lyrica®; Pfizer, France).

Received June 23, 2008. Accepted in final form January 20, 2009.






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