Stiff-Man Syndrome: Pathogenetic, Nosological and Therapeutic Considerations

  • F. Folli
  • G. Piccolo
Part of the Topics in Neuroscience book series (TOPNEURO)


The acronym SMS (Stiff-Man Syndrome) identifies a syndrome of slowly progressive stiffness involving skeletal muscles (mainly axial) with superimposed muscle spasms. It was first described by Moersch and Woltmann in 1956 [1]. A set of diagnostic criteria was proposed by Gordon et al. [2] and Lorish et al. [3]. Diagnostic criteria included: 1) a prodrome of stiffness and rigidity in axial muscles; 2) a slow progression of stiffness involving proximal limb muscles, making walking difficult; 3) a fixed deformity, usually lordosis, of the spine; 4) the presence of superimposed muscle spasms, often precipitated by external stimuli; 5) normal motor and sensory nerve findings; 6) normal mental status and 7) an EMG finding of continuous motor unit activity (CMUA) at rest, abolished by intravenous diazepam or reduced by orally administered diazepam. It has been recently debated whether SMS is a single disease entity. Thirty-eight reported SMS patients were rexamined [4]; only seven of them (18.4%) fulfilled the Lorish diagnostic criteria [3].


Neurol Neurosurg Psychiatry Therapeutic Consideration GABAergic Drug Palatal Myoclonus Proximal Limb Muscle 
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Copyright information

© Springer-Verlag Italia 2002

Authors and Affiliations

  • F. Folli
    • 1
  • G. Piccolo
    • 2
  1. 1.Unit for Metabolic Diseases and Department of MedicineSan Raffaele Scientific InstituteMilanItaly
  2. 2.Department of Neurology, Fondazione MondinoUniversity of PaviaItaly

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