Stiff-Person syndrome
Stiff-Person syndrome Causes
Three autoantibodies is linked with the occurrence of stiff-person syndrome. The idiopathic form is most often associated with glutamic acid decarboxylase antibodies; the paraneoplastic form, with amphiphysin antibodies; while, gephyrin antibodies is associated with stiff-person syndrome.
Stiff-Person syndrome Definition
Stiff person syndrome is a unique neurological condition which affects the peripheral inhibition via central mechanisms and inhibit central gamma-aminobutyric acid (GABA) systems.
Stiff-Person syndrome Diagnosis
To arrive at a diagnosis for stiff-person syndrome, laboratory tests, and other special tests, such as anti-GAD antibodies, anti?pancreatic islet cell antibodies and anti-amphiphysin antibodies, are preliminarily performed. MRI or CT scanning of the brain and chest, EMG, EEG, and lumbar puncture, may confirm the diagnosis and rule out other neurological disorder.
Stiff-Person syndrome Symptoms and Signs
Stiff-person syndrome is characterized by muscle rigidity that waxes and wanes with concurrent spasms. Stiff-person syndrome usually starts in the axial muscles, and extends to the proximal limb muscles.
Stiff-Person syndrome Treatment
Stiff-person syndrome requires medical treatment which may involve either baclofen, benzodiazepine, or tizanidine. Intrathecal baclofen therapy, plasmapheresis, intravenous immunoglobulin, physical therapy and occupational therapy are other treatment options. For symptoms like anxiety and depression, psychiatric consultation may be helpful.