Most scalp neuralgias are supraorbital or occipital. Although they have been considered idiopathic, recent studies revealed that some were attributable to mechanical irritation with the peripheral nerve of the scalp by superficial anatomical cranial structures. Supraorbital neuralgia involves entrapment of the supraorbital nerve by the facial muscle, and occipital neuralgia involves entrapment of occipital nerves, mainly the greater occipital nerve, by the semispinalis capitis muscle. Contact between the occipital artery and the greater occipital nerve in the scalp may also be causative. Decompression surgery to address these neuralgias has been reported. As headache after craniotomy is the result of iatrogenic injury to the peripheral nerve of the scalp, post-craniotomy headache should be considered as a differential diagnosis.

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Clinical manifestations of 16 patients with spontaneous spinal epidural hematoma were presented. We examined the point similar to that of stroke. During the initial visit of our hospital, the patients showed the hemiplegia in 10 cases (62.5%), Horner syndrome in 4 cases (25%), the painless onset in 1 case (6.3%). And one case showed the impairment of consciousness due to vagal reflex in severe pain onset, which was similar to those of subarachnoid hemorrhage. MRI images are useful to confirm the diagnosis. The frequent site of hematoma was the lower cervical spinal cord. The oval shaped hematomas shifted to the left or right in spinal canals, compressed spinal cords in axial image, which was a cause of hemiplegia. Many cases developed during active periods, and the hemorrhage might be relevant to oral antithrombotic agent, C hepatitis, and chronic renal failure. Rapidly progressive cases were indications for emergency surgery, but conservative therapy is also possible and was better prognosis.

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The frequency and clinical characteristics of intractable hiccups due to the medullary infarction is unknown. The aim of this study was to identify the lesions of hiccups using by brain MRI. Ninety acute medullary patients admitted to our stroke center within 14 days of stroke onset between April 2004 and August 2012 were retrospectively studied. We evaluated clinical characteristics and the frequency of the patients causing intractable hiccups among acute medullary stroke patients. We divided the patients into two groups, intractable hiccups group, and not hiccups group. Of 90 patients, five (5.5%) had intractable hiccups. Hiccups group had more frequently involved right middle medullary lesion than not hiccups group (hiccups group vs. not hiccups group; (60% vs. 4%, p < 0.001). In 16 cases reported the lesion of intractable hiccups, the right middle medullary lesion was 11 cases. We suspected that the lesion of the intractable hiccups was associated with the right middle medullary.

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A-47-year-old woman was admitted to our hospital because of eruption, severe arthritis, myalgia, pharyngalgia and the elevation of serum creatine kinase. She was diagnosed with dermatomyositis based on a biopsied specimen and clinical symptoms. Serum anti CADM-140 antibody was detected by immunoprecipitation. Pulse therapy with methylprednisolone and cyclophophamide could transiently improve myalgia and so on, but she died of rapidly progressive interstitial lung disease. Autopsy findings demonstrated diffuse alveolar damage and alveolar hemorrhage. It is said that patients with anti CADM-140 antibody show poor muscle symptoms and alveolar hemorrhage has been described very rarely. This patient is the rare case of dermatomyositis with anti CADM-140 antibody developing severe muscle symptoms, pharyngalgia and aleveolar hemorrhage in autopsy findings.

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A 71-year-old woman developed advanced thermal hypoalgesia, bathyesthesia, and significant sensory ataxia 1 year ago. She also had difficulty maintaining a sitting posture. Patchy and reduced thermal nociception corresponding to a dermatome was found in her four extremities and trunk. On the basis of several tests, she was diagnosed with ataxic sensory neuronopathy due to dorsal root ganglionitis associated with Sjögren’s syndrome. Generally, dorsal root ganglionitis associated with Sjögren’s syndrome is refractory. After treatment with simple plasmapheresis, she was able to maintain a sitting posture. Finally, her symptoms stabilized after the inclusion of oral D-penicillamine to her treatment regimen. Although the clinical course was observed for about one year, we report this case because of its valuable finding, i.e., her symptoms improved after simple plasmapheresis and oral administration of D-penicillamine.

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Multiple mitochondrial DNA (mtDNA) deletions usually occur secondarily to a mutation in one of the enzymes involved in mtDNA maintenance, such as polymerase γ, which is encoded by the nuclear polymerase γ1 gene (POLG1) and POLG2. Patients with multiple mtDNA deletion disorders show clinical heterogeneity of symptoms, in addition to usually seen progressive external ophthalmoplegia (PEO). We conducted clinical, histological and genetic analyses of two affected sisters in a family with the autosomal dominant inheritance pattern of PEO. A 73-year-old woman (patient 1) with congenital hypogonadism and PEO developed L-dopa responsive parkinsonism about the age of 60. Neurological examination revealed mild proximal muscle weakness and polyneuropathy too. Her 69-year-old sister (patient 2) also showed PEO, parkinsonism and polyneuropathy. Histopathological studies of biopsied muscle specimens from patient 1 revealed numerous ragged red fibers as well as fibers with increased succinate dehydrogenase activity and decreased cytochrome c oxidase activity. Multiple mtDNA deletions were detected, both by Southern blot and long-range PCR assays of total DNA from the biopsied muscle specimens. A systemic mutational analysis in both sisters revealed a heterozygous p.Y955C (c.2864A>G) mutation in POLG1. This is the first Japanese family identified with this mutation. We reviewed cases with this mutation highlighting a wide phenotypic spectrum of this disorder.

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We report two cases of stroke associated with the use of finasteride at 1 mg/day, which is approved in Japan for the treatment of male-pattern hair loss. The first case involved a 35-year-old male taking 1 mg of finasteride daily for 6 months to prevent male-pattern hair loss. He was taken to a hospital and later admitted to our hospital owing to headache and seizures. Brain computed tomography (CT) images showed a low-density area in the right frontal lobe. CT venography (CTV) revealed sinus thrombosis and he was treated with an anticoagulant. As the headache gradually subsided, medications were tapered and terminated 10 months later when venous flow to the sagittal sinus and left transverse sinus was confirmed to be recanalized. The second case involved a 41-year-old male taking 1 mg of finasteride and 6 mg of minoxidil daily for 1 year for male-pattern hair loss. He started having headaches and was admitted to our hospital when diffusion-weighted images of brain magnetic resonance imaging (MRI) showed a high-intensity area in the left parietotemporal lobe. He was treated with antiplatelet and anticoagulation medicines. The Japan Pharmaceutical and Medical Devices Agency (PMDA) has reported 14 cases of thrombosis in patients taking finasteride in Japan; 4 cases of stroke (our 2 cases and 2 reported by PMDA), 6 cases of myocardial infarction, and 4 cases of other thrombotic diseases. Increases in estrone and estradiol levels in prostate cancer patients and controls receiving 5 mg of finasteride have been reported. Gynecomastia has also been reported as one of the adverse effects of finasteride at 1 mg or 5 mg daily. Taken together, we assume that the increases in estrone and estradiol levels induced by finasteride lead to thrombosis development.

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A 73-year-old man was admitted to our hospital because of a decrease in spontaneity. His medical history included two stroke episodes, probably related to hypertension. Brain MRI on admission demonstrated acute infarction in the right caudate nucleus and left putamen. Intravenous infusion of a low molecular-weight heparin added to oral antiplatelets was started. Following admission, he developed a low grade fever and severe inflammatory reaction. The focus of infection was not evident, and none of the antibiotics tried were effective. Ten days after admission, he developed right hemiparesis, and an additional brain MRI showed new multiple infarctions. We also determined the presence of a high MPO-ANCA titer (57 EU), and we diagnosed the patient’s condition to be ANCA-associated vasculitis (AAV). Steroid therapy improved his inflammatory reaction and stroke recurrence was not observed. We suggest that vasculitis should be considered as a potential risk factor for repeated small infarctions with fever of unknown origin, especially those of perforating artery territories.

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A 73-year-old man with recurrent periodic paralytic episodes lasting for two weeks each admitted to our hospital because of the leg weakness and the elevated value of serum creatine kinase. On admission, weakness in the proximal legs and mild eye lid myotonia were noted. Needle electromyography revealed abundant myotonic discharges. The prolonged exercise test showed a continuous reduction of compound muscle action potentials in the abductor digiti minimi muscle. Direct sequencing of SCN4A in the proband showed a G-to-A alteration at position 4774 that results in a change of 1592^nd methionine to valine (M1592V). Cosegregation regarding the M1592V mutation and paralytic phenotype in this family was confirmed. Two cardinal features in this family were longer paralytic episodes compared to classical hyperkalemic/normokalemic periodic paralysis and the normal potassium value during the paralytic episodes. This study together with antecedent reports indicates that M1592V mutation shares a much greater clinical diversity ranging from congenital paramyotonia to periodic paralysis with a longer duration.

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A 39-year-old woman developed right hemiparesis in a few days. Magnetic resonance images revealed cerebral infarction in the territory of the left lenticulostriate artery, and MR angiography showed severe stenosis of the middle and anterior cerebral arteries and moderate one of the vertebral arteries. Bilateral and symmetric T₁ hyperintensity in the pulvinar (T₁-weighted imaging-pulvinar sign; “T₁ pulvinar sign”) was detected, which is recognized as a key imaging of Fabry disease. The α-galactosidase A gene analysis, however, showed no mutation. Although specific physical symptoms were solely short stature and oligomenorrhea, the diagnosis of Turner syndrome was confirmed by the chromosome analysis which showed mosaicism of 45XO and 46X,r(X) (60%:40%). To our knowledge, this is the first report of Turner syndrome with “T₁ pulvinar sign”.

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[in Japanese]

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[in Japanese]

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