Preferred Label : Stiff person syndrome;
ICD-11 definition : Stiff person syndrome (SPS) is a rare autoimmune neurological disorder characterized
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 titer 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.;
ICD-11 synonym : stiff man syndrome; Moersch-Woltman syndrome;
Origin ID : 771780430;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Stiff person syndrome (SPS) is a rare autoimmune neurological disorder characterized
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 titer 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.
