Parkinson disease (PD) is thought to be primarily a disorder of the motor system due to dysfunction of the nigrostriatal dopaminergic system. However, recent studies have revealed that social cognition tasks, such as facial expression recognition, decision-making, and mind-reading, are also impaired in PD. The studies also demonstrated that these impairments can occur due to dysfunctions of mesocorticolimbic dopaminergic system, particularly in the amygdala. Furthermore social cognitive impairments may develop in the early stage of PD. Therefore we have to understand PD as a not only movement but also cognitive disorder.

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We prospectively studied the effects of early statin treatment on stroke-induced changes in the levels of inflammatory biomarkers. Patients admitted within 48 hours after the onset of ischemic stroke were enrolled. They were divided into 2 groups according to their lipid profiles and history of statin treatment. In patients who had received statin treatment prior to admission and those who had abnormal lipid profiles on admission, daily treatment with 10mg atorvastatin was initiated within 48 hours after the onset of stroke (Statin group; n=45). In patients who had normal lipid profiles on admission, statin was not administered for at least 2 weeks after admission (Non-Statin group; n=101). The serum concentrations of interleukin (IL)-6, IL-10, IL-18, matrix metalloproteinase (MMP)-2, MMP-9, and high sensitive C-reactive protein were measured on days 1, 3, 7, and 14. In percentage changes in serially measured circulating IL-6 levels, a significant interaction between group and repeated measures (group X time factor) was demonstrated (p=0.047). Frequency of neurological deterioration episodes (NIHSS score ≥2) during 14 days after admission was lower in the Statin group than in the Non-Statin group, however the difference did not reach statistically significant level (7.9% vs 20.2%, p=0.118). The initiation of usual dose of atorvastatin early after the onset of ischemic stroke significantly decreased the elevation of IL-6 and may protect against the early neurological deterioration. Circulating levels of IL-6 may be one of the candidates for monitoring the acute effects of statin. Further studies wherein IL-6 levels are monitored in larger samples would be feasible for investigating the effect of early treatment with usual dose of atorvastatin on the functional outcome.

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Fast spin-echo (FSE) T₁-weighted magnetic resonance imaging (MRI) at 3T, which was optimized to detect neuromelanin-related contrast (NRC), was applied to quantitative estimation of signal alterations in the substantia nigra pars compacta (SNc) of 72 normal volunteers and 59 patients with Parkinson disease (PD). We examined relationship between NRC in SNc and clinical parameters. The NRC showed significant positive correlation with normal aging and was slightly but significantly higher in women than in men. Significant reduction in the NRC was found in PD as compared with 59 age- and sex-matched normal volunteers. The NRC in PD was negatively and significantly correlated with duration of illness and disease severity assessed by UPDRS and Hoehn & Yahr stage. Significant reduction of the NRC was demonstrated in patients with visual hallucinations as compare with patients without the symptoms. REM sleep behavior disorder also contributed reduction of NRC although it did more mildly than visual hallucination. Anosmia or hyposmia had no statistical relationship with the amount of NRC in PD. The overall visual inspection indicated that the reduction of the NRC in PD should start at the ventrolateral portion of SNc and advance medially. Additionally, we studied dementia with Lewy body disease (DLB). The NRC was reduced more significantly in DLB patients with PD symptoms than in those without them who also showed a significant reduction compared with normal controls. Quantification and distribution of the NRC obtained by 3T MRI was well correlated with pathological findings reported previously and clinical parameters in this study. Visualization and quantification of the NRC provide some parts of clinical and diagnostic information about pathologic condition of SNc.

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A 63-year-old man was admitted to our hospital with cognitive decline. On admission, he had a fever and mild cognitive dysfunction, suggesting chronic meningoencephalitis. Apart from a mild increase in serum C-reactive protein level and marked neutrophilia, laboratory findings were unremarkable. Brain magnetic resonance (MR) imaging showed multiple small T₂-hyperintense lesions in the white matter. Systemic evaluations for infectious organisms, autoantibodies, and malignancy were all negative. For 5 months we conducted therapeutic trials of various antibacterial, antifungal, and antituberculous drugs, but these were completely ineffective, and both meningoencephalitis and inflammatory signs persisted. Repeated brain MRI during the clinical course showed growth of the white matter lesions and progressive cerebral atrophy. C11-methionine positron emission tomography demonstrated a bright focus in the right frontal lobe, and this was biopsied. Key neuropathological findings were neutrophilic infiltration in the subarachnoid space and the frontal lobe without necrotic angiitis. These findings confirmed the diagnosis of neuro-neutrophilic disease, although skin tissue findings characteristic of Sweet disease and a B51, B54, or Cw1 HLA-profile were absent. After intravenous bolus administration of steroid and prolonged oral steroid therapy, fever and inflammatory signs diminished and cognitive symptoms improved.

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Lymphocytic hypophysitis (LYH) is a rare neuroendocrine disorder characterized by autoimmune inflammation of the pituitary gland. Visual disturbance is one of the most common and serious symptoms of LYH. Most of the visual symptoms in LYH are secondary to compression of the optic chiasm and some reports have described direct inflammatory involvement of the optic pathways.
We describe a 30-year-old man with a 9-day history of bilateral blurred vision. Ophthalmic examination demonstrated severely impaired vision without temporal hemianopsia. Hypothyroidism, hypocortisolism, and hypogonadism were detected in laboratory tests. Central diabetes insipidus was diagnosed by a hypertonic saline infusion test. MRI revealed thickening of the pituitary stalk and enlargement of the hypophysis, which was enhanced with gadolinium. High intensity of the posterior lobe was not recognized on T₁-weighted images. These findings established a clinical diagnosis of lymphocytic panhypophysitis. Methylprednisolone pulse therapy was introduced and his visual acuity gradually recovered. The anterior pituitary function improved, but desmopressin was still required.
Pattern-reversal visual evoked potentials (VEP) have been widely used to detect optic nerve lesions caused by multiple sclerosis and brain tumors. However, there have been no previous reports of their usefulness for LYH. The P₁₀₀ latency in our case was slightly prolonged and the amplitude was markedly reduced. These findings are similar to ischemic optic neuropathy and other conditions in which axonal damage is prominent. The prolonged latency and low amplitude on VEP examination in this case showed rapid improvement in parallel with the recovery of visual acuity. Taken together, our case implies the usefulness of pattern-reversal VEP for the diagnosis of optic neuritis in LYH, especially for the evaluation of its pathogenic mechanisms.

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We report a 51-year-old man who was admitted to our hospital due to repeated episodes of syncope associated with generalized myasthenic symptoms. Due to myasthenic symptoms with the presence of anti-AchR antoantibody, he was diagnosed as myasthenia gravis (MG) associated with thymoma. However, Holter ECG showed long pause with maximum R-R interval of 3.8 seconds and paroxysmal atrial fibrillation, indicating the diagnosis of sick sinus syndrome. After pace maker implantation and combination therapy with thymomectomy and steroid administration, no arrhythmia in repeated Holter ECG was found. In addition, an anti-kv1.4 antibody was positive in our case. The involvement of cardiomyopathy in patients with MG has been reported, including the association with sudden death. The anti-kv1.4 antibody was recently identified in cases of myasthenia gravis associated with cardiomyositis. After treatments, no arrhythmia was found in our case. Although the cardiomyopathy was not diagnosed in our case because of lacking of histological confirmation, clinical course associated with positive anti-kv1.4 antibody suggested that the cause of syncope might be immune-related cardiomyopathy. To prevent fatal complication of arrhythmia, appropriate examination and therapy against cardiomyopathy associated with myasthenia gravis should be considered.

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Background: In clinical practice, secondary prevention in patients with ischemic stroke (IS) needs to be continued permanently; however, antithrombotic agents are sometimes stopped by clinicians or the patients themselves. The rate of non-taking oral antithrombotic agents was evaluated in IS patients. Methods: 266 consecutive patients (154 men and 112 women; age, 73.6±11.5 years) with first-ever acute IS were studied. Patients with transient ischemic attack (TIA) were also included. Emboligenic heart diseases, frequency of past stroke, oral antithrombotic agent use just before IS, and secondary prevention were evaluated. Results: The number of past strokes was 0 in 182 cases (68.4%), 1 in 66 cases (24.8%), 2 in 14 cases (5.3%), 3 in 3 cases (1.1%), and 9 in 1 case (0.4%; 3 times with stroke, and 6 times with TIA). There were 42 cases (15.8%) with TIA, 47 (17.7%) with lacunar infarction, 69 (25.9%) with atherothrombotic infarction, 62 (23.3%) with cardioembolic infarction, 23 (8.7%) with other types of infarction, and 23 (8.7%) with stroke of unknown etiology. Although 15-26% of patients with their first IS had taken antithrombotic agents just before IS, about 40% of the patients with a previous IS history were not taking antithrombotic agents just before their recurrent IS. Conclusion: About 40% of the patients with recurrent IS were not taking antithrombotic agents at the time of their recurrent IS; had they been taking antithrombotic agents at the time, the recurrent IS might have been prevented. Clinicians must recognize the importance of antithrombotic agents in patients with IS, and patients must continue to take antithrombotic agents permanently.

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A 57-year-old man presented with acute signs and symptoms mimicking PSP (bradykinesia, supranuclear ocular palsy, dysphagia, neck dystonia, and apraxic gait) on the day after a graft replacement surgery, which was performed for aortic arch aneurysm under deep hypothermic circulatory arrest (rectal temperature, 18°C). Dysphagia improved temporarily, but relapsed after a few months. Symptoms did not change during 2 years of antiparkinsonian drug administration. Brain images obtained before the surgery revealed slight atrophy of the midbrain tegmentum and frontal lobes, but the patient was asymptomatic. No findings of cerebral vascular disease and hypoxic encephalopathy were observed on brain images after the surgery. These clinical features resembling PSP might have been caused by deep hypothermia and the patient's predisposition for PSP. This is the first case report in Japan of a syndrome resembling PSP that occurred after aortic arch replacement under deep hypothermic circulatory arrest.

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We report a 78-year-old woman who had episodes of nonconvulsive status epilepticus (NCSE) with dizziness. At 75 years of age, she had first seizure, but was not well examined. At 78 years of age, she had brief myoclonic jerks of her arms, soon after awakening, in May. She suffered from strong dizziness and was admitted in our hospital at the end of June. The symptoms regressed with bed rest in few days and she was discharged. However, she was admitted again with dizziness in the middle of July. There were no myoclonic jerks of her arms or legs and she could converse and interact normally, but was slightly disoriented (JCS: 2). Blood test, Cerebrospinal fluid analyses and brain MRI were normal. An EEG showed frequent intermittent generalized multiple spikes and slow wave complexes and a 3-4Hz generalized spike and slow wave complexes every 2-4 seconds during whole 20 minutes record. Intravenous injection of 5mg diazepam terminated status immediately. Thereafter, she was treated with sodium valproate (400mg/day). Her symptoms improved, and interictal epileptic discharges extremely decreased. Late-onset NCSE of generalized epilepsy is rare. We discussed this case as an important case for diagnosis of NCSE with subtle symptom of dizziness.

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