Rinshō shinkeigaku = Clinical neurology
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Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, immune-mediated polyradiculoneuropathy. The American Academy of Neurology criteria has been used widely in diagnoses, and has generated clinical and pathological information. Recently, the EFNS/PNS criteria revised the concept of conventional "typical CIDP" and "atypical CIDP", with atypical CIDP including five phenotypes: DADS (distal acquired demyelinating symmetric), MADSAM (multifocal acquired demyelinating sensory and motor), focal, pure motor, and pure sensory neuropathy. ⋯ These results suggest that "hybrid therapies", IVIg induction and corticosteroid maintenance, may be effective. A recent study showed that IVIg stabilizes axonal potentials and axonal membranes, and our group showed that juxtaparanodal TAG-1 may be associated with IVIg responsiveness. Although CIDP is a demyelinating disease, the involvement of axon or axon-myelin interactions should be considered.
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Epilepsy is a chronic neurological disorder characterized by recurrent seizures that are caused by abnormal and excessive cerebral neuronal discharges. The clinical symptoms are paroxysmal, and may include impaired consciousness and/or motor, sensory, autonomic, or psychic events. Diagnosis of epilepsy is not always straightforward and clear-cut. ⋯ In real situations, however, it is rare for physicians to actually witness the event of seizure. This lecture provides valuable opportunity to experience "real life" clinical diagnosis of epileptic seizures by showing the video of patient under seizure and EEG data. Representative visual examples of symptoms together with the detailed medical knowledge will greatly enhance the capability of diagnosis, the effectiveness of treatment, and help develop clinical strategies.
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Secondary encephalitis (encephalopathy) causally related with immune response induced by infection, etc., includes encephalitis mediated by innate immunity and adaptive immunity. In the latter, encephalitis mediated by antibodies to molecules at cell-surface of neuron seems to have relatively better outcome. In patients with encephalitis mediated by antibodies to NMDA-type glutamate receptors (NR), mean onset age is 26.5 years old, and the major initial symptoms are limbic symptoms including abnormal behavior, etc. ⋯ In patients with encephalitis mediated by antibodies to voltage-gated potassium channel (VGKC), predominant affection of males are observed, and antibodies to leucine-rich glioma-inactivated 1 (LGI1) and contactin-associate protein (CASR) 2 are found as major epitopes. Patients with Hashimoto encephalitis mediated by antibodies to n-terminal of α-enolase (NAE) have broad spectra of clinical characteristics. Antibodies to TPO can be the marker for diagnosis, and thereafter, confirmation of antibodies to NAE is necessary for definitive diagnosis.
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The success of chronic deep brain stimulation (DBS) and electrical neuro-network modulation (ENM) to address neurological and neuropsychiatric disorders has led the Food and Drug Administration (FDA), and also other worldwide regulatory agencies to grant approval for the use of DBS in specific disorders. In the United States, DBS is FDA approved for the treatment of advanced Parkinson's disease (PD), essential tremor (ET), obsessive compulsive disorder (OCD), and for dystonia. OCD and dystonia have been approved under a mechanism referred to as a humanitarian device exemption (HDE). ⋯ Based on a specific symptom profile, a strategic and personalized medicine approach can be undertaken. The personalized approach will take into consideration the brain target, a unilateral versus a bilateral procedure, and the potential for use of more than one DBS lead per brain hemisphere. Additionally, a personalized approach to DBS will also facilitate improved pre-operative medication adjustments, as well as optimal post-operative medication, behavioral, and device management.
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Intracerebral hemorrhage (ICH) is a common stroke subtype in Japan. Hypertension is the leading cause. Perindopril Protection Against Recurrent Stroke Study (PROGRESS) revealed that blood pressure (BP) lowering could reduce stroke recurrence by 28% (ICH recurrence by 49%). ⋯ Intensive Blood Pressure Reduction in Acute Cerebral Haemorrhage Trial (INTERACT) and Antihypertensive Treatment of Acute Cerebral Hemorrhage (ATACH) showed the feasibility and safety of early rapid BP lowering to 140 mm Hg. INTERACT2 and ATACH II are the randomized trials to compare the guideline-based control (<180 mm Hg) and strict control (<140 mm Hg). We have just started to enroll patients to ATACH II from Japan on February 2012.