The etiology of shoulder-hand syndrome is as yet unknown. We hypothesized that it may be due to damaged unmyelinated fibers in front of the subscapular muscle. We examined the existence of edema and hypersensitivity to pain in the hands of stroke patients during the subacute stage and their relationships to cutaneous temperatures of the index fingertips in 75 hemiplegic patients (23 without edema, 32 with only edema, and 20 with edema plus allodynia). Patients were placed into two groups (comfortable and warm) depending on room temperature (22.2–25.6°C and 25.7–30°C, respectively). Of the patients with hand edema plus allodynia, 75% had a large lesion in the capsula, cortical white matter, and putamen. It was previously reported that the cutaneous temperature of the arm on the paralysis side of patients with lesions of the capsula or putamen was lower than that on the non-paralysis side. In the edema plus allodynia group, the temperature of the index fingertip on the affected side was higher than that of their contralateral fingers; the differences were smaller under warm conditions possibly due to blockade of the sympathetic nerves in the peripheral nerve. By contrast, in patients in the edema group, there were no differences in cutaneous temperatures of their two index fingers. Thus, it appears that patients with mild cases of shoulder-hand syndrome have conduction blocks in the posterior cord of the brachial plexus, while those with severe cases have both conduction blocks and neurogenic inflammation in both the lateral and posterior cords.

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“Dystypia”, characterized by an impairment of typing on a keyboard, is a unique neurobehavioral syndrome. A 77-year-old right-handed woman developed a relatively selective impairment of typing after ischemic stroke. The MRI documented new scattered ischemic lesions in the middle cerebral artery territory of the left hemisphere and an old infarct lesion in the frontal area of the right hemisphere. The standard neuropsychological tests showed no aphasia, normal praxis, intact visuospatial ability, and a mild visual memory disturbance. The detailed analysis documented severe impairment of writing and reading abilities for Romaji (Romanized Japanese), spelled by alphabet letters and the most common way to input Japanese into computers. The writing and reading abilities for other Japanese linguistic modalities such as kanji (morphogram: Chinese character), kana (syllabogram: Japanese proper character), and alphabet letters, were not or minimally impaired. A disturbance of linguistic processing for Romaji may be the main underlying neural mechanism for dystypia in this patient.

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In a 72-year-old female, subacute right hemiplegia and aphasia appeared in late May 2011. The results of hematology, a cerebrospinal fluid test, ¹³F-FDG-PET, and cephalic MRI suggested intravascular/malignant lymphoma. Brain biopsy was performed. Pathological findings did not suggest a malignant tumor. In the perivascular space, the infiltration of neutrophils or histiocytes was observed. The patient was referred to the Department of Neurology. Based on the results of various examinations, infection was ruled out, and steroid therapy was conducted. Marked improvement was achieved. Subsequently, the results of human leukocyte antigen (HLA) typing showed B54/Cw1. As dermal findings were absent, it was impossible to make a definitive diagnosis of neuro-Sweet disease, but the disorder was regarded as a neuro-neutrophilic disease, which is a more comprehensive entity. Few studies have reported brain tissue findings of active neuro-neutrophilic disease. We report the present case, which will contribute to future research.

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A 65-year-old man with left hemiparesis was referred to our hospital by ambulance. Diffusion-weighted magnetic resonance imaging (DWI-MRI) showed a slight hyperintensity area in the right basal ganglion and deep white matter, and brain magnetic resonance angiography (MRA) revealed right middle cerebral artery (MCA) occlusion in the M1 proximal segment. Receiving intravenous rt-PA therapy, the patient showed no neurological improvement. Therefore emergency neuroendovascular revasculization was decided. After the first evacuation of the clot, the occlusion site was partly recanalyzed. However it was re-occluded after a few minutes. Then, mechanical disruption using balloon catheters were added for the occlusion site allowing it to be recanalyzed. After the acute ischemic stroke therapy, the patient was diagnosed as nephrotic syndrome, because his blood chemistry test indicated hypoproteinemia and urine examination showed proteinuria. Renal biopsy confirmed nephrotic syndrome due to AL amyloidosis. Nephrotic syndrome causes hypercoagulability and increases platelet aggregation. Thus we speculated that nephrotic syndrome inhibited the early recanalization in this patient.

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We report the case of an 18-year-old Japanese woman with cobalamin (cbl) C disease. She was born between non- consanguineous parents, and had easy fatigability from a childhood. At 14 years old, she developed renal failure, and had repeated psychosis during 2 years. At 16 old, she developed her gait disturbance and her symptoms fluctuated, but the cause of gait disturbance was unclear. At 18 years old, she was admitted with worsening of gait disturbance. Physical examination revealed spastic paraparesis and bilateral peroneal nerve paralyses. Homocystinuria and methylmalonic aciduria were detected, although serum vitamin B12 was within normal range. Gene mutation analysis revealed Gly147Asp (440G>A) and Trp157Ser (470G>C) in the MMACHC gene as a compound heterozygous mutation. We diagnosed her as having late-onset cbl C disease, and her gait disturbance and renal failure improved after intramuscular hydroxocobalamin administration. Although late-onset cbl C disease is rare in Japan, it an important to consider this congenital disease because symptoms are expected to improve by medical intervention.

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A 61-year-old man noted flu-like symptoms. Not long afterwards, he felt constipation, nausea, and blackout when standing or sitting. His blood pressure was 110/70 mmHg in the supine position. On sitting blood pressure dropped to 73/34 mmHg. Heart rate increased from 65 to 78 beats per minutes. He did not have fever, edema, or skin rash. The remainder of the general medical examination was normal. A neurological examination revealed normal higher mental, and sensori-motor functions. The blood test revealed leukocytosis 7,320/μl, LD 1,426 IU/l, IL-2R 921 U/ml, and CRP 11.5 mg/dl. A whole body CT scan and cranial MR imaging showed no significant change. Thoracic spine MR imaging revealed multiple T₁ low signal small foci in part of the vertebral body suggesting bone metastasis of the tumor. The heart/mediastinum ratio of ¹²³I-meta-iodobenzylguanidine scintigraphy early imaging was 2.42. The nerve conduction study and electrocardiogram coefficient of variation of R-R intervals showed no abnormalities. Two months after the onset of symptoms, he was found to have glove-and-stocking-form muscle weakness and sensory impairment. The nerve conduction study performed four months after the onset revealed a decreased conduction velocity and conduction block suggesting demyelinated nerve. His neurological manifestations progressed subacutely, despite high-dose intravenous immunoglobulin therapy. Five months after the onset, a histopathological diagnosis of T-cell malignant lymphoma was made on a skin biopsy specimen from the facial rash. To summarise, the present case was a rare example of paraneoplastic autonomic neuropathy as the initial clinical feature in association with T-cell malignant lymphoma.

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A previously healthy 63-year-old man presented with a 2-weeks history of diplopia without headache. Neurological examination revealed total external ophthalmoplegia of the left eye and limitation of abduction of the right eye. Initial cranial MRI showed thickening and enhancement of the dura mater only on the anterior cranial fossa but unremarkable on the cavernous sinus. Idiopathic hypertrophic cranial pachymeningitis was diagnosed in the absence of demonstrable underlying infective, neoplastic, or systemic autoimmune disease by his clinical findings, laboratory tests and radiological examinations. Corticosteroid therapy was initiated with methylprednisolone (1,000 mg/day for 3 days), followed by oral prednisolone and tapering off. Eye movements improved with treatment and completely recovered within 4 weeks after starting administration, and cranial MRI at the 15 days after starting treatment showed improvement. We suggest that his ophthalmoplegia was caused by the inflammation of dura on the cavernous sinus beyond the thickening lesion of cranial MRI. In a case of bilateral ophthalmoplegia with or without headache, it is required to examine the dural thickening and enhancement on the anterior cranial fossa by cranial MRI.

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A 44-year-old man with a bilateral hand tremor suffered from a decline in concentration and abnormal vision for several months. He also complained of easily falling down because of muscle stiffness and cramps in his lower limbs. On admission, he demonstrated lower limb stiffness, muscle cramps, diplopia, hyperhidrosis, left upper limb ataxia and dysesthesia in all limbs. Laboratory examination showed a marked elevation in his serum creatine kinase level (26,890 U/l), and needle electromyography demonstrated myokymic discharges in the muscles of his lower extremities. Isaacs’ syndrome was diagnosed based on a positive voltage-gated potassium channel antibody titer of 1,007 pM. Administration of an anticonvulsant (phenytoin, 200 mg/day) did not resolve his symptoms; however, high-dose intravenous methylprednisolone therapy (1 g/day for 3 days) resulted in marked clinical improvement. This case suggests that high-dose intravenous methylprednisolone therapy for Isaacs’ syndrome might be as effective as other immunosuppressive therapies such as plasma exchange or intravenous immunoglobulin.

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A 39-year-old woman initially developed vomiting and intractable hiccup, followed by progressive dysphagia, dysarthria and hypoglossal nerve palsy. She was admitted to our department on the 30th day of illness. MRI-FLAIR images of the brain revealed a hyperintense lesion in the dorsal medulla. A diagnosis of neuromyelitis optica spectrum disorder (NMOSD) was entertained according to the clinical course and the MRI images. The dysphagia was intractable to methylprednisolone pulse therapy, and so a course of plasma exchange therapy was initiated on the 32nd day of illness. After the third plasma exchange, the symptoms began to improve. Thereafter the patient’s serum on admission was reported as positive for anti-aquaporin-4 antibody. Considering the irreversible nature of NMOSD pathology, early initiation of plasma exchange therapy is recommended to minimize the lesion in the case of steroid-refractory NMOSD patients.

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We report the case of a 72-year-old male who presented with the complaints of muscular pain and weakness. The patient showed marked eosinophilia, elevated levels of myogenic enzymes and pathological abnormalities including eosinophil infiltration obtained from the muscle biopsy. Based on these findings, the patient was diagnosed with eosinophilic myositis. During follow-up, left ventricular wall motion abnormalities with transient electrocardiographic abnormalities were identified; these were believed to be concurrent with eosinophilic myocarditis. Further, notable complications included cardiogenic cerebral embolism. Eosinophilic myositis has been found to cause a wide spectrum of complications. Our findings indicate that in cases of suspected eosinophilic myositis, it is crucial to identify myocarditis immediately and to select an anticoagulant therapy to prevent cerebral embolism.

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[in Japanese]

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[in Japanese]

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[in Japanese]

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