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Stiff-man syndrome: case report

W F Kuhn1, P J Light, S C Kuhn

  • 1Department of Family Medicine, Medical College of Georgia, Augusta, USA.

Academic Emergency Medicine : Official Journal of the Society for Academic Emergency Medicine
|August 1, 1995
PubMed
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Stiff-man syndrome, a rare neurological disorder, causes progressive muscle rigidity and painful spasms. Early recognition and diazepam treatment are crucial for managing this condition, often misdiagnosed as back pain.

Area of Science:

  • Neurology
  • Rare neurological disorders
  • Autoimmune diseases

Background:

  • Stiff-man syndrome (SMS) is a rare neurologic disorder.
  • Characterized by progressive muscle rigidity and painful spasms, primarily affecting the back and proximal extremities.
  • Often associated with diabetes, autoimmune diseases, and cancer.

Observation:

  • A 39-year-old woman presented with a two-year history of leg spasms and severe low back pain, leading to immobility.
  • Symptoms included progressive axial and proximal limb muscle stiffness, lumbar hyperlordosis, and spasms triggered by movement.
  • Neurological examination was normal between spasms.

Findings:

  • Diagnosis of Stiff-man syndrome was supported by clinical presentation.
  • Significant symptom improvement was observed after intravenous diazepam administration.

Related Experiment Videos

  • High-dose oral diazepam is the recommended maintenance therapy.

    • Implications:

    • Highlights the importance of considering Stiff-man syndrome in patients with unexplained back pain and spasms.
    • Emphasizes the diagnostic challenge in emergency departments (EDs).
    • Underscores the efficacy of diazepam in managing Stiff-man syndrome symptoms.