Stiff person syndrome
Alerts and Notices
Synopsis
Patients present with nonspecific back pain or axial stiffness with or without episodes of severe spasms that resolve over hours to days. Some patients may only present with stiffness in the extremities or facial muscles. Spasms can be precipitated by stress, anxiety, startle responses, stimulation, or emotion. There can be abdominal contractions as well as significant lumbar lordosis. The disorder is associated with diabetes mellitus type 1 and thyroid disease; it rarely occurs as a paraneoplastic syndrome. Prognosis is variable.
Codes
ICD10CM:G25.82 – Stiff-man syndrome
SNOMEDCT:
5217008 – Stiff-man syndrome
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Tetanus – look for trismus and facial spasms early and lack of response to benzodiazepines
- Neuromyotonia
- Hypocalcemia or other electrolyte abnormalities
- Hyperekplexia
- Multiple sclerosis
- Dystonia
- Parkinson disease
- Myoclonic seizures
- Metabolic or congenital myopathies
- Progressive encephalomyelitis with rigidity and myoclonus (PERM)
- Spinocerebellar degeneration
- Neuroleptic malignant syndrome
- Conversion disorder
Best Tests
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Management Pearls
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Therapy
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References
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Last Reviewed:02/24/2019
Last Updated:01/24/2022
Last Updated:01/24/2022