Brain and nerve = Shinkei kenkyū no shinpo
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Stiff-person syndrome (SPS) is a disorder characterized by fluctuating muscle rigidity and painful spasms that occur spontaneously or are triggered by diverse stimuli. Partial or segmental forms of the disorder, such as stiff-limb syndrome (SLS) and a more severe disease called progressive encephalomyelitis with rigidity and myoclonus (PERM), are usually considered within the spectrum of SPS. ⋯ Most patients with SPS have a high-titer of antibodies against glutamic acid decarboxylase (GAD), the rate-limiting enzyme for the synthesis of γ-aminobutyric acid (GABA), and up to 15% have antibodies to the glycine receptor α-subunit. This review explains milestones in defining SPS including autoantibodies.
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Recent reserch has successfully identified several autoantibodies in chronic inflammatory demyelinating polyneuropathy (CIDP). Most of those identified were IgG4 antibodies targeting cell adhesion molecules around the node of Ranvier of myelinated fibers. ⋯ Growing evidence has revealed that patients with CIDP with these antibodies are characterized by IVIg-resistance and lack typical demyelinating findings on biopsied nerve fibers, both of which could make diagnosis and treatment difficult without autoantibody measurement. Clinicians should recognize that it is now essential to measure autoantibodies in CIDP and utilize antibody testing.