Stiff person syndrome (Q84157): Difference between revisions
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A síndrome da pessoa rígida (SPR) é uma doença neurológica autoimune rara caracterizada por espasmos musculares proximais / axiais dolorosos desencadeados com rigidez que levam a incapacidade progressiva e deformidades da coluna vertebral (por exemplo, lordose lombar exagerada). Há uma predominância do sexo feminino e o diabetes tipo 1 geralmente coexiste. A eletrofisiologia revela hiperexcitabilidade dos neurônios motores espinais. É frequentemente acompanhado por autoanticorpos GAD-65 em títulos elevados, mas outros autoanticorpos neurais foram relatados (por exemplo, anticorpos do receptor de glicina). A SPR pode ser paraneoplásica (classicamente associado ao câncer de mama e anfifisina IgG).A SPR é subdividida em formas clássicas (critérios diagnósticos de Dalkas) e variantes (membro rígido isolado e encefalomielite progressiva com rigidez e mioclonia). | |||
| description / en | description / en | ||
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. | |||
Revision as of 09:31, 15 August 2026
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.
| Language | Label | Description | Also known as |
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| default for all languages | ID_771780430 |
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| English | Stiff person syndrome |
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. |
