Stiff person syndrome (Q84157): Difference between revisions

Stiff person syndrome (SPS) is a rare autoimmune neurological disorder characterised by triggered painful proximal/axial muscle spasms with rigidity which lead to progressive disability and spinal deformities (e.g. exaggerated lumbar lordosis). There is a female predominance and type-1 diabetes usually co-exists. Electrophysiology reveals hyperexcitability of spinal motor neurons. It is frequently accompanied by GAD-65 autoantibodies at high titre but other neural autoantibodies have been reported (e.g. glycine-receptor antibodies). SPS may be paraneoplastic (classically associated with breast cancer and amphiphysin IgG). SPS is subdivided into classic (Dalkas diagnostic criteria) and variant (isolated stiff limb and progressive encephalomyelitis rigidity and myoclonus) forms.