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Book

Stiff Person Syndrome

Alexandra Muranova  1 , Elena Shanina  2
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
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Affiliations
Book

Stiff Person Syndrome

Alexandra Muranova et al.

Excerpt

Stiff person syndrome (SPS) is a rare disorder of the central nervous system characterized by rigidity and stimulus triggered painful muscle spasms of predominantly axial and proximal limb muscles. It was first described in 1956 by Frederick Moersch and Henry Woltman based on a case series of 14 patients with progressive fluctuating tightness of the spinal, abdominal, and thigh muscles. This condition was formerly named stiff-man syndrome and is also known as Moersch-Woltman Syndrome.

The current clinical classification of SPS includes:

  1. Classic SPS

  2. Partial SPS variants

  3. Progressive encephalomyelitis with rigidity and myoclonus (PERM).

Classic SPS is the most common clinical form, present in 70 to 80% of SPS patients. It is associated with anti-glutamic acid decarboxylase (anti-GAD) antibodies. The condition has an insidious onset with gradual worsening over time and often leads to permanent disability and, in some cases, mortality. SPS may coexist with other autoimmune disorders, including Diabetes Mellitus Type 1 (DM-1), autoimmune thyroid disease, pernicious anemia, celiac disease, vitiligo.

Several clinical variants of SPS have been described and include stiff limb syndrome, jerky SPS, cerebellar variant, SPS with epilepsy, and dystonia. The paraneoplastic variant is associated with breast, colon, thyroid, lung malignancies, Hodgkin and non-Hodgkin lymphomas and tends to clinically manifest before cancer itself.

PERM, first described in 1956, is known as SPS-plus syndrome. Patients have the rigidity of axial and limb muscles, diffuse myoclonus in addition to prominent autonomic instability.

There is increasing evidence for immune-mediated etiology of this disorder. Identification of associated antibodies and common comorbidities with other autoimmune diseases and malignancies has been important for a better understanding of disease mechanisms and approaches to treatment.

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Conflict of interest statement

Disclosure: Alexandra Muranova declares no relevant financial relationships with ineligible companies.

Disclosure: Elena Shanina declares no relevant financial relationships with ineligible companies.

References

    1. McKeon A, Robinson MT, McEvoy KM, Matsumoto JY, Lennon VA, Ahlskog JE, Pittock SJ. Stiff-man syndrome and variants: clinical course, treatments, and outcomes. Arch Neurol. 2012 Feb;69(2):230-8. - PubMed
    1. Rakocevic G, Floeter MK. Autoimmune stiff person syndrome and related myelopathies: understanding of electrophysiological and immunological processes. Muscle Nerve. 2012 May;45(5):623-34. - PMC - PubMed
    1. Baizabal-Carvallo JF, Jankovic J. Stiff-person syndrome: insights into a complex autoimmune disorder. J Neurol Neurosurg Psychiatry. 2015 Aug;86(8):840-8. - PubMed
    1. Alexopoulos H, Dalakas MC. Immunology of stiff person syndrome and other GAD-associated neurological disorders. Expert Rev Clin Immunol. 2013 Nov;9(11):1043-53. - PubMed
    1. Hadavi S, Noyce AJ, Leslie RD, Giovannoni G. Stiff person syndrome. Pract Neurol. 2011 Oct;11(5):272-82. - PubMed

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