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A critical survey of stiff-man syndrome

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      Abstract

      From forty-four cases in the world's literature and one case of our own, diagnostic criteria have been adduced for the stiff-man syndrome in the original sense of Moersch and Woltman [1]. A unique pattern of tonic muscle rigidity emerges, based on clinical and electromyographic findings and objective effects of neuromuscular blocking agents monitored by the electromyograph. These criteria are utilized to establish the validity or probable validity of thirty-four of the forty-five cases and to reject eleven which appear to represent neuraxis disease, myopathy, chronic recurrent tetanus, fibrosis replacement of muscle of obscure origin and other conditions.
      A tentative hypothesis is formulated to account for the state of tonic muscle rigidity of stiff-man syndrome. Persistent extrafusal (α-neurone) muscle contractility is probably maintained by abnormal activity of the γ-motor system, itself possibly subjected to a drive from suprasegmental regions. Painful spasm is due to further intensification of muscle contraction triggered by new trains of enteroceptive, exteroceptive and emotional stimuli directly or indirectly influencing the hyperexcitable α-neurone system. Metabolic factors may also be implicated, as suggested by the presence of a nonglucose reducing substance in the urine and a rise in inorganic serum phosphorus during glycogen deposition. These metabolic deviations and the heightened drive of the γ-motor system need further clarification.
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