Rinshō shinkeigaku = Clinical neurology
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Over the last few years, various autoantibodies against cell surface or intracellular antigens were identified in association with several forms of encephalitis, i.e. autoimmune encephalitis. Immunoprecipitaion and sequence analysis of the target protein (proteomics) provided the identification of the antigens corresponding to autoantibodies in autoimmune encephalitis. Appropriate preparation of antigens (synthesized peptides, or recombinant proteins prepared in E.coli or cultured mammalian cells) and assay systems (immunoblot, ELISA, immunoprecipitation or cell-based assay) should be selected for detection of each autoantibodies. ⋯ Cerebellar ataxia is a common form of autoimmune encephalitis (cerebellits). The autoimmune cerebellar ataxia consists of paraneoplastic ataxia (anti-Yo etc.), anti-GAD-autoantibodies associated ataxia, gluten ataxia (anti-gliadin) and ataxic form of Hashimoto's encephalitis (anti-NAE). The early and accurate diagnosis of autoimmune encephalitis is important because most patients show responses to immunotherapy.
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We present a case of a patient with reversible cerebral vasoconstriction syndrome (RCVS) triggered by nicotine patches. A-50-year-old woman had no medical history and no regular medication. She smoked 20 cigarettes a day for 30 years. ⋯ Follow-up MRA, 37 days after the first episode, showed improvement of PCA stenosis. We diagnosed her as reversible cerebral vasoconstriction syndrome (RCVS) due to nicotine patches. It is important to recognize nicotine patches as a trigger of RCVS.
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Anti-glycine receptor (anti-GlyR) antibodies were first reported in 2008 in a case of progressive encephalomyelitis with myoclonus and rigidity (PERM), which is a variant of stiff-person syndrome (SPS). After that, the antibodies have been studied extensively. At least 40 patients have been reported or presented until May 2013. ⋯ Twenty-one patients (75%) treated with immunotherapy or thymectomy improved, but two of six patients without immunotherapy died or developed cardiac arrest. The clinical features suggested that antibody-mediated inhibition of the GlyR on the brainstem nuclei or spinal inhibitory interneurons may cause continuous firing of α motor neurons and paroxysmal excessive response to a variety of afferent impulses, leading to increased stiffness, brainstem signs, trismus, myoclonus, painful spasms or hyperekplexia. Phenotype associated with the anti-GlyR antibodies may be broader than previously thought, but among those PERM is the most common phenotype.
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Review
Mechanism of action for deep brain stimulation and electrical neuro-network modulation (ENM).
Deep brain stimulation (DBS) has become an important treatment option for carefully screened medication resistant neurological and neuropsychiatric disorders. DBS therapy is not always applied deep to the brain; does not have to be applied exclusively to the brain; and the mechanism for DBS is not simply stimulation of structures. The applications and target locations for DBS devices are rapidly expanding, with many new regions of the brain, spinal cord, peripheral nerves, and muscles now possibly accessed through this technology. We will review the idea of "electrical neuro-network modulation (ENM)"; discuss the importance of the complex neural networks underpinning the effects of DBS; discuss the expansion of brain targets; discuss the use of fiber based targets; and discuss the importance of tailoring DBS therapy to the symptom, rather than the disease.