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Kii amyotrophic lateral sclerosis (ALS) is a unique disease that occurs in the southern portion of the Kii Peninsula and exhibits a dual pathology of TAR DNA-binding protein of 43 kDa (TDP-43) proteinopathy and tauopathy. The incidence of ALS in this region was very high in the 1960s, briefly decreased through the 1980s, but began increasing again after 2000 with a change of high-concentration geographic foci. It is unclear, however, whether the unique pathological features have changed along with the incidence changes. This study analyzed postmortem specimens from neuropathologically confirmed Kii ALS cases from the 1970s (n = 4) and those after 1999 (n = 12) from the southern Kii Peninsula or outside of the area. Our results confirm the continued occurrence of Kii ALS after 2000 in the southern Kii Peninsula and the preservation of disease-specific neuronal tau pathology, including the widespread occurrence throughout the brain and spinal cord, sparse neuropil threads, and predominance in superficial layers. Furthermore, we assessed the glial tau pathology of Kii and non-Kii ALS in accordance with the aging-related tau astrogliopathy classification method for the first time and detected a unique brainstem predominant appearance of gray matter aging-related tau astrogliopathy in Kii ALS cases, which may provide clues to pathogenetic mechanisms.
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Esclerose Lateral Amiotrófica , Demência , Transtornos Parkinsonianos , Humanos , Esclerose Lateral Amiotrófica/patologia , Encéfalo/patologia , Demência/patologia , Japão/epidemiologia , Tauopatias/patologia , Proteinopatias TDP-43/patologiaRESUMO
INTRODUCTION: Pareidolias, or visual misperceptions, are a non-motor symptom of Parkinson's disease (PD) with unclear pathophysiology. The noise pareidolia test (NPT) is a tool for screening pareidolias. The usefulness of the NPT in differentiating PD from atypical parkinsonian syndromes (APS) is also unknown. METHODS: We retrospectively investigated 74 patients with PD and 18 patients with APS who took the NPT. Correlations between the number of pareidolic responses, gray matter volume, and cerebral blood flow were also examined in the patients with PD. RESULTS: The median number of pareidolic responses in patients with PD and patients with APS was 0 (interquartile range (IQR): 0-3) and 0 (IQR: 0-1), respectively, and tended to be higher in patients with PD than in those with APS (p = 0.077). It was significantly higher in patients with PD who had hallucinations (2; IQR: 0-9) (p = 0.016). The area under the receiver operating characteristic curve for the number of pareidolic responses in the NPT was 0.62 when used to differentiate PD and APS, and the optimal cutoff number of pareidolic responses was 2/3. Sensitivity and specificity were 25.7% and 100%, respectively. In the PD group, the number of pareidolic responses was correlated with age (r = 0.27; p = 0.021) and the Frontal Assessment Battery (FAB) score (r = -0.34; p = 0.0099). Magnetic resonance imaging showed no significant correlation between the number of pareidolic responses and the volume of focal gray matter. On cerebral hypoperfusion mapping, the left parietal lobe had a significant correlation with the number of pareidolic responses (r = 0.35; p = 0.027). CONCLUSION: The number of pareidolic responses in NPT was suggested to be useful as a red flag to rule out APS in differentiating PD from APS. In PD without dementia, the number of pareidolic responses was associated with reduced blood flow in the left parietal lobe.
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INTRODUCTION: Sarcopenia is a skeletal muscle disease manifesting as low muscle mass and impaired muscle function. It has been reported that sarcopenia correlates with a low quality of life (QOL) and an increased risk of falls in patients with Parkinson's disease (PD). Nevertheless, few studies have investigated the prevalence, impact, and screening methods of sarcopenia in Japanese patients with PD. METHODS: Sarcopenia was diagnosed based on the Asian Working Group for Sarcopenia 2019 consensus. We compared demographic characteristics, severity of PD, levodopa equivalent daily dose, QOL, fatigue, impulsive and compulsive behaviors, body mass index (BMI), calf circumference, skeletal muscle mass index (SMI), handgrip strength, a 4-meter gait speed, a five-time sit-to-stand test (FTSST), short physical performance battery, and SARC-F questionnaire scores between sarcopenia and non-sarcopenia groups. Furthermore, to investigate the best tool for screening sarcopenia in PD, the sensitivity and specificity of calf circumference, handgrip strength, FTSST, and SARC-F questionnaire were compared. RESULTS: The prevalence of sarcopenia in PD was 31.9% (15/47). The sarcopenia group showed significantly higher age (77.3 ± 5.12 versus 70.3 ± 8.17, p = 0.0042), lower BMI (19.3 ± 2.99 versus 23.3 ± 3.18, p = 0.0002), higher rate of decreased calf circumference (86.6% versus 34.3%, p = 0.0013) and SMI (100% versus 6.25%, p < 0.0001), and worse FTSST (15.5 ± 5.57 versus 12.0 ± 4.12, p = 0.0219). The other parameters were not significantly different. Among screening tools, calf circumference had the highest sensitivity (86%) and specificity (65%). All screening tools had higher sensitivity and specificity in men than in women. The SARC-F questionnaire was not useful in distinguishing sarcopenia but was significantly correlated with the Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale Part 3 (r = 0.41, p = 0.0037) and the 39-item Parkinson's Disease QOL Scale (r = 0.71, p < 0.0001). CONCLUSION: This study investigated the characteristics of PD patients with sarcopenia in Japan. Calf circumference was found to be the most useful tool for screening sarcopenia in PD. Handgrip strength and FTSST also showed high sensitivities, particularly in men. Conversely, the SARC-F questionnaire is not suitable for diagnosing sarcopenia in PD.
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BACKGROUND: Impulse control disorders are detrimental neuropsychiatric symptoms of Parkinson's disease. Increased impulsivity is a predisposing factor for impulse control disorders and should therefore be controlled. Recently, mindfulness meditation as a non-drug therapy has been reported to be useful in improving neuropsychiatric symptoms, such as impulsivity. METHODS: We performed a prospective single-arm, open-label pilot trial to investigate the effectiveness of mindfulness meditation to control impulsivity in patients with Parkinson's disease (UMIN clinical trials registry: UMIN000037779). RESULTS: Twenty patients with Parkinson's disease were enrolled in an 8-week mindfulness meditation program. As a primary outcome, we investigated whether the score of the Barratt Impulsiveness Scale (BIS-11) was significantly reduced after the intervention. As an exploratory examination, functional connectivity changes were also assessed by resting-state functional magnetic resonance imaging. After the intervention, the BIS-11 score was decreased from 59.5 [55.6, 63.3] (mean [95% confidence interval]) to 55.2 [50.3, 60.1] (ΔBIS-11: -4.2, [-7.5, -0.9]). Functional connectivity was increased in the default mode network (DMN) at a cluster including the precuneus, posterior cingulate gyrus, and left posterior lobe (false discovery rate-adjusted p [FDR-p] = 0.046) and in the right frontoparietal network (FPN) at the medial frontal lobe (FDR-p = 0.039). CONCLUSIONS: This open-label, single-arm pilot study provided preliminary data for mindfulness meditation to control the impulsivity of patients with PD. A brief mindfulness meditation program may be effective in controlling impulsivity in PD and may change the functional connectivity of the DMN and right FPN.
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Meditação , Atenção Plena , Doença de Parkinson , Humanos , Comportamento Impulsivo , Imageamento por Ressonância Magnética , Meditação/psicologia , Atenção Plena/métodos , Doença de Parkinson/terapia , Projetos Piloto , Estudos ProspectivosRESUMO
A 67-year-old woman with a recurrent history of malignant lymphoma (ML) presented with muscle weakness and paresthesia of the fingertips and feet. Due to the elevated level of serum soluble interleukin-2 receptor and increased 18F-fluorodeoxyglucose uptake in a mediastinal lymph node, neurolymphomatosis was initially suspected. Neurological and electrophysiological examinations were consistent with mononeuropathy multiplex. A diagnosis of neurosarcoidosis was made based on the presence of noncaseating epithelioid granulomas in the mediastinal lymph node, along with the presence of the uveitis, cardiac inflammation, and mononeuropathy multiplex. She was treated with glucocorticoids and azathioprine, and her symptoms disappeared. Sarcoidosis following ML is rare, and since biopsy of nervous systems is often improbable, differentiating neurosarcoidosis and neurolymphomatosis can be difficult as their clinical symptoms can be similar. Clinicians should consider systemic pathological investigations based on 18F-fluorodeoxyglucose positron emission tomography examination in addition to comprehensive evaluation to accurately diagnose neurosarcoidosis.
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Primary phlebitis of the central nervous system (PPCNS) is a rare condition that might be a subset of primary angiitis of the CNS. In this case report, the patient was a 39-year-old man with a 2-week history of anterograde amnesia and abnormal behaviours. Black-blood MRI (BB-MRI) showed contrast enhancement of the left basilar vein and cerebral superficial veins. Angiography showed unremarkable change in arteries. After a thorough differential diagnosis, we diagnosed PPCNS and then administered methylprednisolone pulse and cyclophosphamide pulse. The neuropsychological symptoms and MRI findings gradually improved, and after 2 months, the dose of prednisolone was gradually reduced to 20 mg. No recurrence was observed. This case shows that BB-MRI may be useful for diagnosing PPCNS.
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Flebite , Vasculite do Sistema Nervoso Central , Adulto , Sistema Nervoso Central , Humanos , Angiografia por Ressonância Magnética , Imageamento por Ressonância Magnética , Masculino , Metilprednisolona/uso terapêutico , Flebite/diagnóstico por imagem , Flebite/tratamento farmacológico , Vasculite do Sistema Nervoso Central/diagnóstico por imagem , Vasculite do Sistema Nervoso Central/tratamento farmacológicoRESUMO
Japanese encephalitis (JE) is an acute viral disease caused by the Japanese encephalitis virus (JEV). JEV strains are classified into 5 genotypes (I-V). JEV genotype V strains have never been detected in Japan to date, but they were recently detected in South Korea. In the present analysis, we tried to determine if a JEV genotype V strain caused any JE case in Japan in 2016. Serum and cerebrospinal fluid samples were collected from 10 JE patients reported in Japan in 2016. JEV RNA was not detected in any of the samples. Although JEV is a single-serotype virus, it can be expected that the neutralizing antibody titers against JEV genotype V strains are higher than those against genotype I and III strains in the serum of patients with JE in Japan whose causative JEV was the genotype V strain. The neutralizing antibody titers against the JEV genotype V strain were not higher than those against the genotype I or III strain in any serum samples. Therefore, the evidence that the JEV genotype V strain caused any JE case in Japan in 2016 was absent.