Common variable immunodeficiency (CVID) is a primary immunodeficiency.1,2 The autoimmune diseases associated with CVID commonly affect the hematologic, dermatologic, and gastrointestinal systems.3–6 Neurologic autoimmune disorders associated with CVID are seen infrequently.7 We describe the first case of CVID associated with stiff-person syndrome (SPS), a neurologic autoimmune disorder. SPS is an extremely rare autoimmune neuromuscular disorder with a prevalence of 1–2 per million and an incidence of 1 case per million per year.
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