記述言語：英語   掲載種別：研究論文（学術雑誌）  

The intestinal microbiota is associated with a variety of diseases, and there are a growing number of research reports on the gut microbiota. In addition, a new technique such as Nanopore sequencing has recently become available, making it easier to conduct research related to the gut microbiota. In this chapter, we introduce a technique used in gut microbiota analysis, from stool collection to sequencing with MinION.

DOI： 10.1007/978-1-0716-3682-4_35

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Neuronal intranuclear inclusion disease (NIID) exhibits diverse clinical manifestations. Our patient was a 64-year-old woman with bilateral ptosis as the chief complaint. She had bilateral miosis, and the pupil was only slightly dilated 60 min after 1% phenylephrine administration, suggesting autonomic dysfunction secondary to preganglionic sympathetic impairment. A head-up tilt test revealed asymptomatic orthostatic hypotension. She was diagnosed with NIID based on a skin biopsy and genetic testing. This study suggests that blepharoptosis is an early manifestation of NIID. Furthermore, patients with suspected NIID should be examined carefully for autonomic dysfunction.

DOI： 10.2169/internalmedicine.2384-23

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Serum growth differentiation factor 15 (GDF-15) levels are elevated in patients with Parkinson's disease (PD) and may help differentiate these patients from healthy individuals. We aimed to clarify whether serum GDF-15 levels can help differentiate PD from atypical parkinsonian syndromes and determine the association between serum GDF-15 levels and clinical parameters. We prospectively enrolled 46, 15, and 12 patients with PD, progressive supranuclear palsy (PSP), and multiple system atrophy (MSA), respectively. The serum GDF-15 level in patients with PD (1394.67 ± 558.46 pg/mL) did not differ significantly from that in patients with PSP (1491.27 ± 620.78 pg/mL; p = 0.573) but was significantly higher than that in patients with MSA (978.42 ± 334.66 pg/mL; p = 0.017). Serum GDF-15 levels were positively correlated with age in patients with PD (r = 0.458; p = 0.001); PSP (r = 0.565; p = 0.028); and MSA (r = 0.708; p = 0.010). After accounting for age differences, serum GDF-15 levels did not differ significantly between patients with PD and MSA (p = 0.114). Thus, age has a strong influence on serum GDF-15 levels, which may not differ significantly between patients with PD and atypical parkinsonian syndromes such as PSP and MSA.

DOI： 10.3390/neurolint15030066

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記述言語：英語  

DOI： 10.1016/j.jns.2023.120549

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Objective Sleep disturbance is a common nonmotor symptom associated with a decreased quality of life in patients with Parkinson's disease (PD). In this study, we evaluated the effects of zonisamide on motor and non-motor symptomology in patients with PD, especially with respect to objective sleep assessments conducted via polysomnography. Methods We conducted a 12-week, open-label study to assess the effects of zonisamide. The patients received 25 mg/day of zonisamide and underwent overnight polysomnography prior to and after 12 weeks of zonisamide treatment. They were assessed for their cognitive function (Mini-Mental State Examination and the Japanese version of the Montreal Cognitive Assessment), gait function (Timed Up-and-Go Test, 10-m Gait Walk Test), Parkinson's symptomology (Movement Disorder Society Revision of the Unified Parkinson's Disease Rating Scale parts 2 and 3), and self-reported sleep (Epworth Sleepiness Score, Parkinson's Disease Sleep Scale-2). Results Six patients completed the study. Polysomnographic data revealed a statistically significant increase in the percentage of time spent in sleep stage N2 (10.8% ±9.2%, p =0.031) and a declining trend in the percentage of time spent in sleep stage N1 (-8.9% ±12.7%, p =0.063). Although none of the patients had sleep stage N3 at baseline, 3 of the 6 patients experienced sleep stage N3 (1.1%-5.4%) after 12 weeks of zonisamide treatment. The other polysomnographic parameters and clinical scores showed no statistically significant differences. Conclusions This preliminary study demonstrated that zonisamide improved objective sleep parameters measured by polysomnography in patients with PD.

DOI： 10.2169/internalmedicine.0037-22

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: Constipation is one of the most frequent non-motor symptoms of Parkinson's disease (PD), and magnesium oxide (MgO) is a frequently used laxative. This study aimed to investigate the effect of concomitant use of MgO on the pharmacokinetics of levodopa preparations in patients with PD. METHODS: We prospectively enrolled 35 patients with PD and compared the pharmacokinetics of levodopa and carbidopa and motor symptoms with and without MgO. The impact of alterations in pH and the addition of MgO on the solubility of levodopa formulations were also evaluated under in vitro conditions. RESULTS: Concomitant use of MgO significantly reduced the maximum plasma concentration of levodopa (Cmax) (from 7.66 ± 3.74 μmol/L to 5.82 ± 3.69 μmol/L; p = 0.006) and area under the plasma concentration-time curve 3 h after drug administration (AUC3h, from 9.64 ± 3.23 μmol·h/L to 7.39 ± 3.15 μmol·h/L; p < 0.001), and further decreased carbidopa Cmax (from 64.02 ± 27.02 ng/mL to 38.83 ± 21.94 μmol/L; p < 0.001) and AUC3h (from 130.58 ± 65.64 ng/mL to 76.48 ± 52.24 ng·h/mL; p < 0.001). The Movement Disorder Society-Sponsored Revision of the Unified Parkinson's Disease Rating Scale part III score also deteriorated significantly (from 30.71 ± 11.34 to 32.06 ± 11.22; p = 0.007). MgO significantly affected the pharmacokinetics of levodopa and carbidopa. This also applied when the findings were analyzed by sex and age. In vitro dissolution experiments revealed a decrease in the relative concentrations of levodopa, carbidopa, and benserazide as the pH increased and in the presence of MgO suspension, with the most prominent impact on benserazide. CONCLUSIONS: Concomitant use of MgO and levodopa should be discouraged to improve levodopa absorption.

DOI： 10.1016/j.prdoa.2023.100227

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: There is some phenotypic overlap between MS, AQP4-IgG positive NMOSD, and MOG-IgG associated disease (MOGAD), and distinguishing a true relapse and a pseudorelapse can be difficult. CSF neopterin, a marker of inflammation-immune-mediated processes in the CNS, may be a useful marker in a wide range of CNS infectious and inflammatory diseases. We compared CSF neopterin levels and other CSF parameters in patients with MS, AQP4-IgG-positive NMOSD, and MOGAD and also investigated whether CSF neopterin levels can distinguish between active and inactive phases of the diseases. METHODS: We retrospectively reviewed the medical records of 22 patients with MS, 18 with AQP4-IgG-positive NMOSD, and five with MOGAD. CSF neopterin concentrations were measured by HPLC with fluorometric detection. RESULTS: CSF neopterin levels at diagnosis were significantly higher in patients with AQP4-IgG-positive NMOSD (52.77 ± 34.56 pmol/mL) than patients with MS (16.92 ± 5.03 pmol/mL, p < 0.001), and tended to be higher in patients with MOGAD (28.87 ± 9.66 pmol/mL) than patients with MS (p = 0.092). ROC analysis revealed that CSF neopterin most accurately discriminated between MS and AQP4-IgG-positive NMOSD (AUC, 0.912; sensitivity, 75.0%; specificity, 100.0%). At diagnosis/relapse and during remission, CSF neopterin most accurately discriminated between the disease phases in patients with MS (AUC, 0.779; sensitivity, 58.1%; specificity, 94.7%) and patients with AQP4-IgG-positive NMOSD (AUC, 0.934; sensitivity, 83.3%; specificity, 94.1%). CONCLUSION: Measurement of CSF neopterin may be useful for differential diagnosis and assessment of disease activity in CNS demyelinating diseases. Further studies with larger cohorts, including comparisons with other biomarkers, are needed to validate the utility of CSF neopterin.

DOI： 10.1016/j.jns.2022.120385

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: Levodopa and carbidopa are reported to be degraded by magnesium oxide (MgO), which is often used as a laxative for patients with Parkinson's disease (PD). Ascorbic acid (AsA) can stabilize levodopa and carbidopa solutions; however, the effect of AsA on the degradation of levodopa and carbidopa induced by MgO has not been fully investigated. METHODS: The effect of AsA was evaluated using in vitro examinations, compared with lemon juice, and by measuring the plasma concentration of levodopa in a patient with PD. RESULTS: In vitro experiments showed that the relative concentrations of levodopa remained almost constant, and the relative concentrations of carbidopa decreased with time with addition of MgO. AsA mitigated this effect in a concentration-dependent manner, whereas the addition of lemon juice caused little change, although the pH decreased to the same extent. The results of levodopa pharmacokinetics of the patient showed that the area under the plasma concentration-time curve values from hour 0 to 8 were 53.00 μmol·h/L with regular administration and 67.27 μmol·h/L with co-administration of AsA. CONCLUSIONS: AsA can mitigate the degradation of carbidopa induced by MgO and may contribute to improving the bioavailability of levodopa in patients with PD.

DOI： 10.1002/brb3.2672

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Amyotrophic lateral sclerosis (ALS) causes deterioration of respiratory function. Muscle weakness of the orbicularis oris interferes with the accurate assessment of respiratory function using spirometry. Reduced forced vital capacity (FVC) is an indicator that helps determine the appropriate timing to provide noninvasive ventilation (NIV) for the survival of ALS patients. We employed ultrasonography to evaluate changes in respiratory function by measuring the thickness of the rectus abdominis (RA) muscle as a possible alternative to spirometry. METHODS: Sixteen subjects with ALS were included in this study. The thickness of RA muscles was measured using ultrasonography, and respiratory fluctuations, such as vital capacity (VC), FVC, FEV1, percentage of predicted VC (%VC), percentage of predicted FVC (%FVC), percentage of predicted FEV1 (%FEV1), and FEV1/FVC, were evaluated using spirometry. RESULTS: Sixteen subjects underwent assessment by ultrasonography. A positive correlation was observed between the percent change in RA muscle thickness evaluated from maximal expiration to maximal inspiration and %VC (P = .001), %FVC (P = .001), FEV1 (P = .009), and %FEV1 (P = .02). CONCLUSIONS: RA ultrasonography was useful for predicting a reduction in VC in subjects with ALS and may help determine the best timing for introducing NIV.

DOI： 10.4187/respcare.09317

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Levodopa-carbidopa intestinal gel (LCIG) therapy is used in advanced Parkinson's disease (PD) and consists of continuous administration of levodopa directly into the jejunum through a percutaneous endoscopic gastro-jejunal (PEG-J) tube. Recently, the metabolism of levodopa by Enterococcus faecalis (E. faecalis) has been reported. Intestinal bacteria can also affect this therapy. OBJECTIVES: To investigate intestinal bacteria and examine its impact on levodopa blood concentration in patients with PD receiving LCIG therapy. METHODS: We enrolled 6 patients receiving LCIG therapy in our department. After PEG-J tube replacement, intestinal bacteria were collected from the tip of the tube and were identified using culture and polymerase chain reaction (PCR) tests. Moreover, the presence of tyrosine decarboxylase, which metabolizes levodopa, was also confirmed by PCR test. The ability of these bacteria to metabolize levodopa was confirmed in vitro. Levodopa blood concentrations were also examined before PEG-J tube replacement. RESULTS: Bacteria were detected in all 6 patients. E. faecalis was present in 4 patients. Moreover, tyrosine decarboxylase was detected in 2 patients. The identified bacteria displayed in vitro metabolization to dopamine in the 4 E. faecalis positive samples. The addition of carbidopa did not inhibit the metabolism of levodopa. However, there was no difference in the mean blood concentration of levodopa, regardless of the presence of E. faecalis. CONCLUSIONS: We found bacteria, including E. faecalis in the PEG-J tube. We observed levodopa metabolism in vitro, but there was no association with levodopa blood concentration. The effect of intestinal bacteria may be limited in patients receiving LCIG therapy.

DOI： 10.1002/mdc3.13417

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Vaccines against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been developed and administered worldwide. There have been reports of neurological adverse events following immunization (AEFIs). We herein report a case of refractory longitudinally extensive transverse myelitis in a 75-year-old Japanese man following the first dose of the BNT162b2 vaccine. The patient developed total sensory loss below the umbilicus and complete paralysis in both legs. Although he was treated with steroid therapy and plasma exchange, his recovery was limited, and severe sequelae remained. Further studies, including large epidemiological studies, are required to understand the association between SARS-CoV-2 vaccines and neurological AEFI.

DOI： 10.2169/internalmedicine.8747-21

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記述言語：日本語   出版者・発行元：日本神経学会  

2020年10月末時点で済生会松山病院脳神経内科を定期受診しているパーキンソン病（Parkinson’s disease，以下PDと略記）患者の情報収集，解析を行った．患者数は187名（男性83名，女性104名），平均年齢73.6歳，平均罹病期間8.9年だった．罹病期間とHoehn & Yahr（HY）stage，抗PD薬の薬剤数は正の相関を認めた．L-ドパ換算用量相当量は罹病期間20年以上，HY 5に至ると減少した．Wearing-off現象，L-ドパ誘発性ジスキネジアは，罹病期間が長い，L-ドパ使用量が高い患者に多かった．PD患者の症状や治療内容について総合的に評価した報告は少なく．本研究により地域中核病院におけるPD患者の臨床像の一端を把握しえた．

DOI： 10.5692/clinicalneurol.cn-001653

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記述言語：英語  

We report the eldest female case of myasthenia gravis (MG) that initially presented with aspiration pneumonia. A 91-year-old female with a high-grade fever and general malaise who had suffered from expectoration for several years was diagnosed with aspiration pneumonia. Thorough medical history taking and physical examination suggested the possibility of MG as a cause of aspiration pneumonia. Positive acetylcholine receptor antibody and waning phenomenon on a nerve conduction study confirmed the diagnosis. Treatment with intravenous immunoglobulin, prednisolone, and pyridostigmine resulted in a rapid improvement. Physicians should always consider the etiology of aspiration pneumonia to prevent further negative events.

DOI： 10.7759/cureus.19268

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: Some patients with Parkinson's disease (PD) undergoing levodopa‒carbidopa intestinal gel (LCIG) treatment experience motor fluctuations in the afternoon. The migrating motor complex, a specific periodic migrating contraction pattern occurring in the stomach and small intestine during the fasting state, can affect drug absorption. We aimed to compare the pharmacokinetic parameters between two conditions (with and without lunch) and assessed the influence of the fasting state on the levodopa pharmacokinetics in LCIG treatment. METHODS: We evaluated the levodopa pharmacokinetics from 12:00 p.m. to 6:00 p.m. in 10 LCIG-treated PD patients in the presence and absence of lunch. RESULTS: The maintenance dose of LCIG correlated strongly with the mean plasma concentration of levodopa in the absence (r = 0.94, coefficient of determination (R2) = 0.89, p < 0.001) or presence of lunch (r = 0.96, R2 = 0.93, p < 0.001). Comparison of the pharmacokinetic parameters revealed that the coefficient of variation was significantly greater in the condition without lunch than in the condition with lunch (p = 0.004): 16.73% (4.88%) without lunch and 9.22% (3.80%) with lunch. There were no significant differences in the mean plasma concentration of levodopa (p = 0.49) and area under the plasma concentration‒time curve (p = 0.27) between the two conditions. CONCLUSIONS: Plasma concentrations of levodopa fluctuated more in patients undergoing LCIG treatment without than with lunch. Our results indicate that a small amount of food intake may be a better corrective approach for worsening of symptoms in the fasting state rather than additional levodopa.

DOI： 10.1016/j.parkreldis.2021.09.001

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記述言語：英語  

DOI： 10.1016/j.parkreldis.2021.04.009

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記述言語：英語  

Dyskinesia is among the most problematic issues for local anaesthesia management in Parkinson's disease (PD) patients. We present a case of a patient with PD who underwent spinal anaesthesia while utilizing dexmedetomidine (DEX) to reduce dyskinesia during urological surgery. DEX stimulates α₂-adrenergic receptors, which works to reduce the dyskinesia in PD patients.

DOI： 10.7759/cureus.13739

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Rheumatoid meningitis (RM) is a rare but treatable central nervous system (CNS) manifestation of rheumatoid arthritis (RA) with various clinical presentations and atypical cerebrospinal fluid (CSF) findings. There are no established biomarkers for RM, making diagnosis a challenge. Herein, we present three cases of RM: two patients with RA diagnosis and one without. CSF analysis showed pleocytosis in only one case. In contrast, CSF neopterin levels were elevated in all three cases and decreased after steroid therapy. This study suggests that CSF neopterin levels may be a useful biomarker for diagnosing and therapeutically monitoring CNS inflammation in patients with RM.

DOI： 10.1016/j.jneuroim.2020.577396

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1002/mdc3.13014

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Duloxetine proved effective for treating pain in people with Parkinson's disease in a single-arm, open-label study. OBJECTIVE: To evaluate the efficacy of duloxetine in a double-blind, randomized, placebo-controlled trial. METHODS: We randomly assigned 46 patients with Parkinson's disease with pain to either the duloxetine 40 mg/day arm or the placebo arm. After 10 weeks, we tested the change from baseline in 24-hour average pain severity measured by a visual analogue scale. RESULTS: We could not confirm the effect of duloxetine on pain. Exploratory analyses indicated that treatment with duloxetine was associated with improved scores on the Unified Parkinson's Disease Rating Scale Part III and 3 domains of the Parkinson's Disease Questionnaire - 39. CONCLUSIONS: The study failed to provide evidence for the use of duloxetine for treating pain in people with Parkinson's disease.

DOI： 10.1016/j.jns.2020.116833

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記述言語：英語  

DOI： 10.1016/j.jns.2020.116855

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DOI： 10.1016/j.parkreldis.2020.05.040

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Springer  

We investigated the relationship between sleep disturbances and 123I-meta-iodobenzylguanidine (MIBG)-uptake during myocardial scintigraphy in patients with Parkinson’s disease (PD). We studied consecutive PD patients who underwent MIBG scintigraphy at our institution. Collected data included Hoehn and Yahr (HY) stage, the heart/mediastinum (H/M) ratio of MIBG scintigraphy, Epworth Sleepiness Scale score, PD Sleep Scale (PDSS)-2 score (total PDSS-2 score and its three subscales: motor problems at night, PD symptoms at night, and disturbed sleep), levodopa daily dose, total levodopa-equivalent daily dose (LEDD), and LEDD of dopamine agonists. We determined the specific binding ratio (SBR) in patients who underwent DaTSPECT to examine the relationship between each sleep disturbance scale and SPECT changes and the other evaluated items. We examined 31 patients with PD. There was a significant negative correlation between the H/M ratio and the score for disturbed sleep. The more the decreased the cardiac MIBG uptake, the higher the score for disturbed sleep (p = 0.040). There was no correlation between the H/M ratio and other sleep scales. The HY stage correlated with the score for PD symptoms at night (p = 0.0244), but not with other sleep scales. Twenty-one patients were evaluated by DaTSPECT
there was no correlation between SBR and any other scores used to evaluate sleep disturbances. The score for disturbed sleep on the PDSS-2 is mainly related insomnia symptoms. Thus, MIBG myocardial scintigraphy detects the severity of sleep disturbance in PD patients.

DOI： 10.1007/s41105-020-00253-1

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Parkinson's disease (PD) is among the most frequently-occurring neurodegenerative diseases in humans. Although mitochondrial dysfunction is suggested to play a central role in PD pathogenesis, few studies have clinically evaluated mitochondrial function in PD patients. We therefore aimed to determine whether mitochondrial function is altered in PD patients by applying two approaches that are normally used for the diagnosis of mitochondrial disorder (MD). METHODS: We measured serum growth differentiation factor 15 (GDF15) levels in 36 PD patients, 30 age-matched healthy controls, and 5 MD patients. Among these, 20 PD patients and 18 healthy controls underwent the lactate stress test. Serum GDF15 levels were evaluated with respect to clinical characteristics. RESULTS: Mean GDF15 levels were significantly higher in the PD (1472 pg/mL, p = .034) and MD (3363 pg/mL, p < .001) groups than in the control group (1093 pg/mL). The lactate stress test exhibited no significant differences between PD patients and controls. Age was identified as an independent factor that correlated with serum GDF15 levels in both groups. In PD patients, there was no significant difference between serum GDF15 levels and other clinical parameters including sex, cognitive function, and lifestyle. CONCLUSION: Serum GDF15 levels, but not lactate levels, were elevated in Japanese patients with PD, thus highlighting the potential association between PD and GDF15.

DOI： 10.1016/j.jns.2019.116616

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Austrian syndrome is a rare condition caused by invasive Streptococcus pneumoniae, comprising a triad of pneumococcal meningitis, endocarditis, and pneumonia. Herein, we report a 59-year-old male patient who presented with fever and tenderness of the right shoulder. Although the initial diagnosis was acromioclavicular joint septic arthritis, the present case showed a reduced level of consciousness, pulmonary infiltrates, cerebral infarcts, and destruction of the mitral valve. This case suggests that acromioclavicular joint arthritis could be an initial presentation of pneumococcal infection inclusive of Austrian syndrome, especially in patients with some risk factors of invasive pneumococcal infections, such as chronic alcoholism.

DOI： 10.1016/j.jiac.2019.06.009

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記述言語：英語  

DOI： 10.1007/s00228-019-02762-6

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記述言語：英語  

DOI： 10.2169/internalmedicine.2416-18

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 20-year-old woman suffered right facial paralysis. The patient showed an abnormality in the perception of speech at an age of 25 years. At an age of 32 years, she developed acute headache and fever. Brain magnetic resonance imaging (MRI) showed an expanded high signal intensity lesion with gadolinium enhancement in the white matter of the left frontal lobe, which was suggestive of tumefactive demyelinating lesion (TDL). A brain tumor was suspected because TDL is a large demyelinating brain lesion mimicking a primary brain tumor. After initiation of steroid therapy, the symptoms and MRI abnormalities improved. At an age of 34 years, she was referred to our hospital with the main complaint of weakness of lips on the left side. Brain MRI showed hyperintense lesions involving the left frontal and the right parietal white matter lobes, and the left ventrolateral pons, which was suggestive of acute disseminated encephalomyelitis (ADEM). Analysis of anti-MOG antibodies identified anti-MOG antibodies both in the serum and in the CSF. Steroid therapy led to complete clinical recovery. MOG antibodies in both serum and CSF were negative six months after the previous measurement. The patient fulfilled the diagnostic criteria for multiple sclerosis (MS) and TDL is one of the rare variants of MS. This study suggests that anti-MOG antibodies can be associated with repetitive encephalitis including TDL and ADEM-like presentation.

DOI： 10.1016/j.msard.2019.03.018

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記述言語：英語  

•LHON cases can show brainstem lesions without visual impairment.•There can be inconsistency between MRI finding and clinical symptom in LHON cases.•Auditory pathways may be often involved in LHON cases.

DOI： 10.1016/j.ensci.2019.01.002

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Objective Assessing daily motor fluctuations is an important part of the disease management for patients with Parkinson's disease (PD). However, the frequent recording of subjective and/or objective assessments is not always feasible, and easier monitoring methods have been sought. Previous studies have reported that the spontaneous eye-blink rate (EBR) is correlated with the dopamine levels in the brain. Thus, the continuous monitoring of the EBR may be useful for predicting the motor status in patients with PD. Methods Electrooculograms (EOGs) were recorded for up to 7.5 hours from three PD patients using a wearable device that resembled ordinary glasses. An receiver operating characteristic (ROC) analysis was performed to compare the ability of the EBR estimates at each time-point (Blink Index) and the plasma levodopa levels to predict the motor status. Results The Blink Index was correlated with the plasma levodopa levels. When an indicator for the first hour of the observation period was included in the model, the Blink Index discerned wearing-off and dyskinesia as accurately as the plasma levodopa level. Conclusion Our study provides preliminary evidence regarding the utility of continuous EBR monitoring for the non-invasive evaluation of the motor status in patients with PD.

DOI： 10.2169/internalmedicine.1960-18

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Myoclonus epilepsy associated with ragged-red fibers (MERRF) is traditionally characterized by myoclonus, generalized epilepsy and ragged-red fibers. We herein report a 42-year-old man who complained of falling after starting running, symptoms resembling those of paroxysmal kinesigenic dyskinesia. He showed only slight muscle weakness of the right quadriceps femoris. Muscle pathology and a genetic analysis identified him as having MERRF with a 8344A>G mtDNA mutation. We diagnosed his symptoms as having been caused by slight quadriceps femoris muscle weakness and exercise intolerance. This case suggests that mitochondrial myopathy should be considered in cases with strong muscle symptoms for muscle weakness.

DOI： 10.2169/internalmedicine.1210-18

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Epidemiological studies have repeatedly reported that increased serum urate level is associated with a slower progress of Parkinson's disease (PD). The urate precursor, inosine, raises the serum urate level and is therefore a candidate for a disease modifying treatment. However, an elevated serum urate level is a risk factor for gout, urolithiasis, and cardiovascular diseases. Although there have been previous clinical studies, the use of inosine in a clinical setting is still limited, and its safety is unclear, especially in an Asian population. METHODS: We conducted a single-arm, single-center clinical trial to assess the safety of inosine for PD patients with relatively low urate levels. After informed consent, 10 subjects were orally administered inosine to maintain a target urate level between 6.0mg/dl and 8.0mg/dl for one year. All adverse effects were recorded and categorized by severity. Also, the efficacy of using inosine to raise the serum urate level was reported. RESULTS: We did not observe any adverse events requiring termination or reduction of the study drug, although uric acid crystalluria was transiently observed in a single subject. An inosine dosage of 1070 (SD=501) mg/day significantly raises the urate level from 3.5 (0.84)mg/dl at baseline to 6.68 (1.11)mg/dl at the 52nd week. CONCLUSIONS: Inosine was safely used for one year and effectively raised urate levels in a small group of subjects. Our study is the first report to use inosine for patients with PD in an Asian population.

DOI： 10.1016/j.jns.2017.10.030

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: Although spinocerebellar ataxia type 2 (SCA2) is classified as hereditary spinocerebellar degeneration, some patients present with parkinsonism before developing cerebellar ataxia. METHODS: 123I-metaiodobenzyl guanidine (123I-MIBG) myocardial scintigraphy and/or dopamine transporter single photon emission computed tomography (DAT SPECT) using 123I-ioflupane (123I-FP-CIT) were performed for the six patients from three SCA2 families. RESULTS: 123I-MIBG myocardial scintigraphy showed reduced cardiac uptake in four of five patients and an association with Lewy body disease was suggested. DAT SPECT showed decreased uptake in the striatum in all four patients who were scanned, including one patient without parkinsonism. When patterns of uptake were compared to those with Parkinson's disease, most of the patients had minimal reduction of uptake in the putamen. CONCLUSION: DAT SPECT is expected to be useful in differentiating SCA2 from Parkinson's disease, making an early diagnosis, and allowing early therapeutic intervention.

DOI： 10.1016/j.parkreldis.2017.08.012

PubMed

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記述言語：英語  

DOI： 10.1002/mds.27109

PubMed

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記述言語：英語   掲載種別：書評論文，書評，文献紹介等   出版者・発行元：ASEAN Neurological Association  

Objective: DNA methyltransferase 1 (DNMT1) is crucial to maintaining methylation during DNA replication and DNA repair. DNMT1 mutations have been identified in two neurological syndromes, including hereditary sensory and autonomic neuropathy type IE (HSAN IE) with dementia and hearing loss and autosomal dominant cerebellar ataxia, deafness and narcolepsy. It is likely that DNMT1 mutations lead to various symptoms of the central and peripheral nervous system. The aim of this study was to examine the clinical characteristics, especially the initial symptoms, in the cases of DNMT1 mutations. Methods: We investigated the clinical manifestation and examination findings of four cases of HSAN IE from one family with the DNMT1 mutation c.1531Y&gt
C (p.Try511His). Results: All four cases exhibited sensory neuropathy, cerebellar ataxia, and hearing loss, all of which were demonstrated by the audiograms. The initial symptoms of the four cases included hearing loss (n=1), gait disturbance (n=1), and depressive mood (n=2). Depressive symptoms are reported in some cases with HSAN IE, however, there are currently no published reports that describe them as primary symptoms. The CSF orexin level was measured in three cases, revealing normal values in two cases and intermediate values in one case, in which the patient exhibited rapid eye movement (REM) sleep behavior disorder. Conclusion: Our findings suggest that in cases with HSAN IE or the DNMT1 mutation, psychiatric symptoms should be taken into account as one of the initial manifestations of the disease.

Scopus

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記述言語：日本語   出版者・発行元：日本神経治療学会  

［目的］Parkinson病に対してL–ドパ持続経腸療法（Levodopa Carbidopa Intestinal Gel：LCIG療法）が承認された．ウェアリングオフの強い症例に対するLCIG療法の効果と課題について血中濃度の検討を含めて報告する．［方法］当院および関連施設でLCIG療法を施行され，血中濃度を評価したParkinson病6例に対して，施行前後においてウェアリングオフの有無と程度，オン時間の変化，ジズキネジア，治療への満足度などを評価し，血中濃度と症状の関連性，課題等について検討した．［結果］オフ時間は短縮した．効果が不十分で頻回の追加投与を行った症例ではジスキネジアが増加し長くなった．投与量を一定としている例では一日の血中濃度はおおむね安定していた．しかしながら，血中濃度が保たれているにも関わらずオフ症状を訴える症例も認められた．また，一日の血中濃度は安定していたが，個体間，また同一症例においても測定日によって血中濃度に変化を認めた．血中濃度の高い例ではジスキネジアが多かった．本人の満足度をもとに持続投与量を設定すると血中濃度が高くなる例があり，血中濃度を確認することで減量することが出来た．［結論］LCIG療法にて血中濃度は一定となったが，血中濃度が一定でもオフを訴える例がみられ，オフの誘因としてL–ドパ濃度以外の要因も示唆された．また，症例ごと，加えて同一症例でも日によって血中濃度に変動が見られた．L–ドパの動態には体重や消化管での吸収・代謝などが関連している可能性があり，投与量の決定には臨床症状とともに，血中濃度のモニタリングが参考となる．

DOI： 10.15082/jsnt.36.2_91

CiNii Research

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記述言語：日本語   出版者・発行元：日本神経治療学会  

Parkinson disease（PD）患者では日中の過度の眠気がみられることがあり，そのリスク因子としてPD治療薬の関与が報告されている．今回，PD患者の内服薬と眠気について調査するため，当科を受診したPD患者に対しEpworth Sleepiness Scale（ESS）を施行し，PD治療薬との関連について検討した．ESSスコアはPD治療薬総量，罹病期間，PD重症度と有意な正の相関を認めた．Dopamine agonist（DA）使用患者はESSスコアがDA未使用患者よりも高かった．原因としてDA使用患者はPD治療薬総量が多く，総量増加に伴うESSスコアの上昇が考えられた．また，突発的睡眠はDA使用患者，未使用患者共にみられ，突発的睡眠を認める患者でESSスコアが高い結果が得られたことから，全てのPD患者で突発的睡眠に注意が必要であり，ESSスコアが突発的睡眠の指標になることが示唆された．

DOI： 10.15082/jsnt.36.3_317

CiNii Research

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記述言語：日本語   出版者・発行元：一般社団法人 日本内科学会  

31歳，女性．以前より細菌性髄膜炎を頻回に再発していた．2015年8月下旬，急性に発熱，意識障害が出現し，精査加療目的にて当院へ入院した．髄液検査などの結果から細菌性髄膜炎を疑い加療を開始したところ，速やかに症状は改善した．頭部造影CT（computed tomography）検査および経鼻ファイバースコープ観察にて上咽頭内に髄膜瘤の遺残を認めた，経鼻式髄膜瘤摘出術を施行したところ，術後から現在まで髄膜炎の再発なく経過している．

DOI： 10.2169/naika.106.1461

CiNii Books

CiNii Research

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その他リンク： http://id.ndl.go.jp/bib/028386206

記述言語：日本語   出版者・発行元：日本臨床社  

CiNii Books

CiNii Research

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その他リンク： https://search.jamas.or.jp/link/ui/2017086842