Brain and nerve = Shinkei kenkyū no shinpo
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In this study, we reviewed the findings of diagnostic imaging of cerebral vascular anomalies classified as cerebral cavernous malformation (CCM)/hemangioma, developmental venous anomalies (DVAs)/venous malformation (VM), and capillary telangiectasia. In addition, we described the imaging findings of cavernous sinus hemangioma and orbital hemangioma/vascular anomaly. CCM has 2 forms: a sporadic form characterized by isolated lesions and a familial form characterized by multiple lesions with an autosomal dominant mode of inheritance. ⋯ SWI has improved the diagnosis of microbleeds, brain tumor, cerebrovascular disease, degenerative disease, etc. SWI is superior to GRE T2*-weighted imaging for determining hemosiderin, methohemoglobin, and deoxyhemoglobin contents. The sensitivity of SWI for determining the number of CCM lesions is significantly higher than that of GRE T2*-weighted imaging.
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Gait disturbance, one of the axial symptoms, is caused by various disorders, including basal ganglia disease. Deep brain stimulation (DBS) has widened the spectrum of therapeutic options for patients with gait disturbance due to Parkinson disease and dystonia. In gait disturbance caused by basal ganglia disease, the main targets of DBS are the subthalamic nucleus (STN) and globus pallidus internus (GPi). ⋯ GPi DBS is effective for the treatment of primary segmental or generalized dystonia. The pedunculopontine tegmental nucleus (PPN), which is involved in locomotion, is one of the new targets for treating gait disturbance in Parkinson disease. We review DBS in the treatment of gait disturbance due to Parkinson disease and dystonia.
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In this review study, second-generation antiepileptic drugs (AEDs) (levetiracetam, gabapentin, topiramate, lamotrigine, zonisamide, oxcarbazepine, vigabatrin, pregabalin, rufinamide, tiagabine, lacosamide, and felbamate) and injectable AEDs (levetiracetam, lacosamide, fosphenytoin, lorazepam, and valproic acid) available in North America were compared with those available in Japan. Three second-generation AEDs (gabapentin, topiramate, and lamotrigine) were recently approved in Japan. Levetiracetam is currently under review for approval by the Japanese regulatory agency. ⋯ The effects of this drug are more prolonged than those of diazepam. Intravenous administration of valproic acid is regarded as a new treatment option for patients with status epilepticus, because sedative and negative effects on the cardiorespiratory system of this drug are lesser than those of the traditional injectable AEDs. These novel medications will aid the improvement of the quality of life of epileptic patients through improved seizure control and reduced adverse effects.
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Review
[Stiff-person syndrome and other myelopathies constitute paraneoplastic neurological syndromes].
Stiff-person syndrome (SPS) is an autoimmune neurological disorder characterized by rigidity of the trunk and proximal limb muscles, intermittent superimposed spasms, and increased sensitivity to external stimuli. It has been more than 50 years since Moerch and Woltman reported the first 14 cases with this syndrome. During the last half century, many autoantibodies discovered, such as anti-glutamic acid decarboxylase (GAD), anti-amphiphysin, anti-gephyrin, and anti-gamma-aminobutyric acid A receptor-associated protein (GABARAP) antibodies. ⋯ Although the understanding and treatment of SPS have evolved, the disease remains underdiagnosed. In the past, some patients with SPS have been diagnosed with psychiatric disorders. Therefore, it is important to increase awareness of SPS among practicing physicians.
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Neuromyotonia occurs due to several causes such as hereditary, immune-mediated and degenerative neurological disorders. Isaacs' syndrome (immune-mediated neuromyotonia) is an antibody-mediated potassium channel disorder (channelopathy). Clinical symptoms of Isaacs' syndrome are characterized by muscle cramp, slow relaxation following muscle contraction (pseudomyotonia), and hyperhidrosis; these symptoms are due to hyperexcitability of the peripheral nerve, including autonomic nerve. ⋯ Thus, an increasing number of immune-mediated neurological disorders with anti-VGKC antibodies are being identified. However, except in Morvan's syndrome, it is rare to find symptoms pertaining to involvement of both the peripheral and central nervous system in the same patient with anti-VGKC antibodies. The differences in the pathomechanism of Isaacs' syndrome and limbic encephalitis are still unclear.