Journal of Neurology

, Volume 241, Issue 3, pp 157–166 | Cite as

Stiff man syndrome: Clinical and laboratory findings in eight patients

  • H. -M. Meinck
  • K. Ricker
  • P. -J. Hülser
  • E. Schmid
  • J. Peiffer
  • M. Solimena
Original Communication


The clinical, biochemical, neuroimaging and neurophysiological findings of eight patients with stiff man syndroms (SMS) [four of six being tested with autoantibodies against glutamic acid decarboxylase (GAD)] are presented. In two patients (one GAD-positive, one GAD-negative), transient oculomotor disturbances suggested progressive encephalomyelitis with rigidity and myoclonus (PERM) as differential diagnosis. The catalogue of characteristic clinical symptoms of SMS is extended by three new symptoms: (1) an aura-like feeling reported by five patients to precede spontaneous spasmodic attacks; (2) a stereotyped motor pattern seen in seven patients during spasmodic jerks, consisting of brief opisthotonos, stiffening of the slightly abducted legs and inversion of the plantar-flexed feet; (3) a paroxysmal fear when crossing a free space unaided, or even thinking of it. Clinical findings did not enable us to discriminate between patients tested GAD-positive or GAD-negative. Cerebrospinal fluid contained elevated immunoglobulin levels or cell counts, or both, in the majority of patients. Autopsy of one patient revealed scattered lymphocyte cuffs around leptomeningeal, intracerebral and particularly intraspinal vessels, suggesting a mild inflammatory process. Whether SMS and PERM are closely related is discussed; they are possibly both manifestations of a spectrum of encephalomyelopathies having autoimmunity against GABAergic neurons in common.

Key words

Stiff man syndrome Progressive encephalomyelitis Spasms Muscle tone Glutamic acid decarboxylase 


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Copyright information

© Springer-Verlag 1994

Authors and Affiliations

  • H. -M. Meinck
    • 1
  • K. Ricker
    • 2
  • P. -J. Hülser
    • 3
  • E. Schmid
    • 4
  • J. Peiffer
    • 5
  • M. Solimena
    • 6
  1. 1.Abteilung für Klinische NeurophysiologieUniversität HeidelbergHeidelbergGermany
  2. 2.Abteilung für NeurologieUniversität WürzburgWürzburgGermany
  3. 3.Abteilung für NeurologieUniversität UlmUlmGermany
  4. 4.Abteilung für NeurologieBürgerhospitalStuttgartGermany
  5. 5.HirnforschungsinstitutUniversität TübingenTübingenGermany
  6. 6.Department of Cell BiologyYale UniversityNew HavenUSA

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