A 53-year-old woman was admitted to our hospital with headache and convulsion. Advanced anemia with a Hb level of 3.5g/dl had been detected about a month earlier, and it had been treated by iron administration to achieve a Hb level of 8.9g/dl. The patient developed status epilepticus on admission. The blood pressure was elevated, and brain diffusion weighted imaging and fluid attenuated inversion recovery imaging revealed high intensity areas in the bilateral posterior and parietal lobes, right frontal lobe, and right basal ganglia. The cerebrospinal fluid protein was elevated. The convulsions settled after continuous infusion of thiamylal under mechanical ventilation. Subsequently, the patient became conscious, and the brain MRI abnormalities gradually disappeared. While a number of factors such as hypertension, medication and others have been reported as causes of posterior reversible encephalopathy syndrome (PRES), comparatively rapid anemia correction could also possibly precipitate PRES as like as this case. Thus anemia correction needs to be undertaken carefully.

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We report a 79-year-old man presenting MPO-ANCA associated hypertrophic pachymeningitis and bilateral visual impairment. Two years before, microscopic hematuria and positive MPO-ANCA were indicated, then oral steroids and cyclophosphamide were given as systemic vasculitis. On admission, lateral hemianopsia in the right visual field was documented. Some weeks after admission, he complained of a left-hand side headache, and the visual impairment of a right eye. Brain MRI detected thick dura matter with abnormal enhancement predominantly on the left side of the basal temporal lobe and a tumor-like lesion at the sphenoid sinus near the right cavernous sinus. Multiple scotomas in the left visual field were compatible with ischemic changes caused by MPO-ANCA related vasculitis. On the other hand, the hemianopsia in his right eye was related with a tumor-like lesion. The visual problems showed a favorable response to the steroid pulse therapy. ANCA-positive cases can demonstrate various symptoms including intra-/extra-cranial involvement. Thus, thorough clinical workup is needed to determine the actual site of the lesion when cranial nerve involvement is observed in MPO-ANCA positive hypertrophic pachymeningitis.

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A 55-year-old Japanese female with no family history of neurological disease showed gait disturbance with rigidity and resting tremor. She was initially diagnosed with Parkinson's disease, but neither laterality of symptoms nor levodopa benefit were observed. The parkinsonism and gait freezing progressed unusually slowly and she experienced frequent falls 17 years after the onset of symptoms. When neurologic examination revealed vertical gaze palsy, masked face, bradykinesia and dominant axial rigidity, the diagnosis was modified to progressive supranuclear palsy (PSP). Retrocollis, grasp reflex, and bilateral Babinski's sign developed, but resting tremor disappeared. Gastrostomy was performed 22 years after onset, after which she was still capable of walking with assistance. Tracheotomy was not performed. The patient died of an acute subarachnoid hemorrhage 24 years after onset at the age of 79.
On autopsy, the brain weighed 1,050g and showed frontal lobe atrophy. Coronal cerebral slices showed atrophy of the globus pallidus and subthalamic nucleus. Tegmental atrophy of the brainstem and depigmentation of the substantia nigra were observed. Neuropathologic examination showed severe neuron loss with gliosis in the globus pallidus, subthalamic nucleus, substantia nigra, and tegmentum of the brainstem. The Purkinje neuron layer and cerebellar dentate nucleus showed mild neuron loss. Globose-type neurofibrillary tangles were widespread, particularly in the globus pallidus, subthalamic nucleus, substantia nigra, nucleus of oculomotor nerve, locus ceruleus, and cerebellar dentate nucleus. Glial fibrillary tangles (coiled body and tuft-shaped astrocyte) and argyrophilic threads were also widespread, particularly in the frontal lobe and basal ganglia. Lewy bodies were not observed. Although, the pathologic findings were consistent with PSP, Gallyas-positive and tau-positive structures were generally small in number.
According to the clinicopathological findings, we speculate that this case showed a distinct subtype of PSP with a slowly progressive clinical course and generally mild tau deposition.

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We report a case of paradoxical brain embolism mediated through a pulmonary arteriovenous malformation (PAVM) with hereditary hemorrhagic telangiectasia (HHT). A 25-year-old right handed man was admitted to our hospital after sudden headache and visual field abnormality. In neurologic examinations, he had left superior-quadrantanopsia. Laboratory findings showed iron deficiency anemia. Diffusion weighted images disclosed a high-signal-intensity area in the right occipito-temporal lobe, and intraarterial digital subtraction cerebral angiography revealed occlusion of the right posterior cerebral artery. Transesophageal echocardiography revealed continuous right-to-left shunt. We confirmed his history of spontaneous recurrent epistaxis and the first-degree relatives with epistaxis or PAVM. A contrast enhanced CT scan of the chest revealed a PAVM. The diagnosis of paradoxical brain embolism mediated through the PAVM with HHT was, thus, established. The PAVM was occluded by using embolization coils successfully. In Asian countries, the prevalence of PAVM with HHT is thought to be lower than in European countries. We should carefully take medical and family histories, especially epistaxis, in a young stroke patient.

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We report the case of a 34-year-old woman with cerebral and pulmonary cryptococcosis. After surgery for uterine cervical cancer, chest CT scan indicated a solitary tumor. Cryptococcosis was detected by transbronchial lung biopsy, and brain MRI showed multiple tumors. We diagnosed the patient with cerebral and pulmonary cryptococcosis.
Oral and intravenous antifungal treatments were not effective, and a disturbance of consciousness appeared. We began intraventricular antifungal treatment, and the symptoms improved, with a reduction in the size of multiple lesions. However, the size of the brain lesions increased, and we diagnosed late deterioration of cryptococcosis and corticosteroid response. Because of the refractory clinical course, we examined the Cryptococcus strains from the surgical resected pulmonary lesion and identified Cryptococcus gattii(VG I type). C.gattii occurs predominantly in apparently healthy hosts. An intracranial C.gattii infection is associated with neurological complications and delayed therapeutic response. If cerebral cryptococcosis responds slowly and relatively poorly to antifungal therapy, C.gattii should be considered. Aggressive therapy, including intraventricular therapy and corticosteroids therapy for cryptococcoma, is required.

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Cyclosporine A (CYA) treatment has been reported to be probably useful for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) that is resistant to conventional treatment. Although several studies have shown that appropriate area under the concentration-time curve (AUC) monitoring of CYA levels results in improved outcomes for refractory nephrotic syndrome patients, the importance of using AUC analysis for CIDP remains unclear. In this study, we measured both trough and AUC from 0 to 4 h (AUC_{0-4 h}) levels of CYA in 2 patients with CIDP and compared the findings for the clinical parameters. On the basis of the CYA dosing recommendations for patients with nephrotic syndrome, we used a CYA concentration of 150ng/ml for the trough level and an AUC_{0-4 h} value of 2,500ng/(ml·h). Patient 1 showed a significant increase in grip strength and a prolonged remission period. Patient 2 showed improvement in the modified Rankin scale and manual muscle test (MMT) scores. Monitoring both AUC_{0-4 h} and trough levels of CYA seems to be a better option than monitoring the trough level alone because it leads to improved estimation of the efficacy and safety of CYA treatment in the case of CIDP patients.

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We report a case of a small cortical infarction in the postcentral gyrus that presented an isolated hemicape-like sensory disturbance. A 47-year-old man suddenly developed numbness and paresthesia in the left neck, shoulder, arm, and upper trunk. Examination revealed hypoesthesia to touch and pain in these areas along with a hemicape-like distribution. The sensitivity to cold and vibration was normal, and two-point discrimination and graphesthesia were preserved. The patient had a normal visual field, muscle strength, and reflexes, and there were no neuropsychological deficits. Magnetic resonance imaging (MRI) demonstrated a fresh, small cerebral infarction in the right postcentral gyrus, which was superior medial to the precentral knob. The area of infarction in this patient corresponds well with the area of the upper trunk, neck, head, shoulder, and arm in the sensory homunculus drawn by Penfield and Rassumussen. The spinal MRI was normal. Transesophageal echocardiography disclosed a patent foramen ovale with a right-to-left-shunt. The patient was diagnosed as having acute cerebral infarction, probably due to paradoxical embolism, and was treated with warfarin. A small localized infarct in the postcentral gyrus can present an isolated sensory disturbance with an atypical hemicape-like distribution.

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We present a case of a female patient with reversible cerebral vasoconstriction syndrome (RCVS) arising after receiving subcutaneous injection of human placenta extract. A 44-year-old woman started taking human placenta extract with the aim of improving her menopausal symptoms, fatigue, and beauty. However, 18 days after taking human placenta extract, she had three episodes of thunderclap headache. Repeated cranial CT did not show subarachnoid hemorrhage; CSF examination showed neither xanthochromia nor inflammation. Brain diffusion weighted and FLAIR images were normal. However, magnetic resonance angiography showed multifocal segmental stenosis of the right middle cerebral artery and bilateral anterior cerebral arteries. Follow-up angiography, which performed 12 days after the oncet of thunderclap headache, revealed almost normalized flow in all cerebral arteries; we made a diagnosis of RCVS. She has had no symptoms and signs since the third attack of headache. The only identified etiologic factor was subcutaneous injection of human placenta extract started 18 days prior to onset. This is the first report of RCVS triggered by human placenta extract.

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This report deals with a 46-year-old male with Waldenström's macroglobulinemia (WM), who developed POEMS syndrome four years after diagnosis. The patient was diagnosed with WM, based on the presence of IgM-κ type monoclonal (M) protein and infiltration of lymphoplasmacytic cells identified in bone marrow aspirates. Four years later, the patient presented with progressive weakness and paresthesia of the limb extremities, and he was admitted to our hospital. Physical and neurological examination on admission revealed polyneuropathy, hepatosplenomegaly, hypothyroidism, IgM-κ M protein, leg edema, and cutaneous hyperpigmentation. He fulfilled the diagnostic criteria for POEMS syndrome. Laboratory tests showed normocytic normochromic anemia, elevated erythrocyte sedimentation rate, and increased levels of soluble IL-2 receptor, IL-6 and plasma vascular endothelial growth factor (VEGF). He was started on lenalidomide. After therapy, the leg edema and limb dysesthesia improved, and the VEGF level decreased from 608pg/ml to 380pg/ml. This is a very rare case of POEMS syndrome associated with WM, and is the first case treated with lenalidomide in Japan. VEGF presumably producted WM may be associated with development of POEMS syndrome.

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