Stiff person spectrum disorder: overview with emphasis on head and neck comorbidities

Abstract

Stiff person spectrum disorder (SPSD) is a rare progressive autoimmune neuromuscular syndrome, primarily resulting in severely painful spasms and rigidity of the axial and appendicular musculature. Affected individuals are predisposed to develop an array of other neuropathies, including cerebellar ataxia and seizure activity, ophthalmologic abnormalities, and other autoimmune-based systemic diseases, notably type 1 diabetes mellitus, thyroiditis, pernicious anemia, and malignancy. Limited information exists in the oral medicine literature regarding SPSD. Thus, the objective of this paper is to review the clinicopathologic features of SPSD, with particular emphasis on head and neck involvement. Additionally, clinical guidelines for dental management of affected individuals and a summary of surgical procedures and outcomes performed in the head and neck are provided. (Oral Surg Oral Med Oral Pathol Oral Radiol YEAR;VOL:page range)