記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Pathogenic variants in Gap junction protein beta 1 (GJB1), which encodes Connexin 32, are known to cause X-linked Charcot-Marie-Tooth disease (CMTX), the second most common form of CMT. CMTX presents with the following five central nervous systems (CNS) phenotypes: subclinical electrophysiological abnormalities, mild fixed abnormalities on neurological examination and/or imaging, transient CNS dysfunction, cognitive impairment, and persistent CNS manifestations. CASE PRESENTATION: A 40-year-old Japanese male showed CNS symptoms, including nystagmus, prominent spastic paraplegia, and mild cerebellar ataxia, accompanied by subclinical peripheral neuropathy. Brain magnetic resonance imaging revealed hyperintensities in diffusion-weighted images of the white matter, particularly along the pyramidal tract, which had persisted since childhood. Nerve conduction assessment showed a mild decrease in motor conduction velocity, and auditory brainstem responses beyond wave II were absent. Peripheral and central conduction times in somatosensory evoked potentials elicited by stimulation of the median nerve were prolonged. Genetic analysis identified a hemizygous GJB1 variant, NM_000166.6:c.520C > T p.Pro174Ser. CONCLUSIONS: The patient in the case described here, with a GJB1 p.Pro174Ser variant, presented with a unique CNS-dominant phenotype, characterized by spastic paraplegia and persistent extensive leukoencephalopathy, rather than CMTX. Similar phenotypes have also been observed in patients with GJC2 and CLCN2 variants, likely because of the common function of these genes in regulating ion and water balance, which is essential for maintaining white matter function. CMTX should be considered within the spectrum of GJB1-related disorders, which can include patients with predominant CNS symptoms, some of which can potentially be classified as a new type of spastic paraplegia.

DOI： 10.1186/s12883-024-03823-9

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) is a late-onset, autosomal recessive neurodegenerative disorder caused by biallelic AAGGG/ACAGG repeat expansion (AAGGG-exp/ACAGG-exp) in RFC1. The recent identification of patients with CANVAS exhibiting compound heterozygosity for AAGGG-exp and truncating variants supports the loss-of-function of RFC1 in CANVAS patients. We investigated the pathological changes in 2 autopsied patients with CANVAS harboring biallelic ACAGG-exp and AAGGG-exp. RNA fluorescence in situ hybridization of the 2 patients revealed CCTGT- and CCCTT-containing RNA foci, respectively, in neuronal nuclei of tissues with neuronal loss. Our findings suggest that RNA toxicity may be involved in the pathogenesis of CANVAS. ANN NEUROL 2024;95:607-613.

DOI： 10.1002/ana.26848

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Despite the suggestion that direct compression by granuloma and ischemia resulting from vasculitis can cause nerve fiber damage, the mechanisms underlying sarcoid neuropathy have not yet been fully clarified. METHODS: We examined the clinicopathological features of sarcoid neuropathy by focusing on electrophysiological and histopathological findings of sural nerve biopsy specimens. We included 18 patients with sarcoid neuropathy who had non-caseating epithelioid cell granuloma in their sural nerve biopsy specimens. RESULTS: Although electrophysiological findings suggestive of axonal neuropathy were observed, particularly in the lower limbs, all but three patients showed ≥1 abnormalities in nerve conduction velocity or distal motor latency. Additionally, a conduction block was observed in 11 of the 16 patients for whom waveforms were assessed; five of them fulfilled motor nerve conduction criteria strongly supportive of demyelination as defined in the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guideline for chronic inflammatory demyelinating polyneuropathy (CIDP). In most patients, sural nerve biopsy specimens revealed a mild to moderate degree of myelinated fiber loss. Fibrinoid necrosis was observed in one patient, and electron microscopy analysis revealed demyelinated axons close to granulomas in six patients. CONCLUSIONS: Patients with sarcoid neuropathy may meet the EAN/PNS electrophysiological criteria for CIDP due to the frequent presence of conduction blocks. Based on our results, in addition to the ischemic damage resulting from granulomatous inflammation, demyelination may play an important role in the mechanism underlying sarcoid neuropathy.

DOI： 10.1111/ene.16091

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

This study aimed to test our hypothesis that the cerebellum plays an important role in the generation of the optical-geometric illusion known as the Poggendorff illusion, the mechanism of which has been explained by accumulated experience with natural scene geometry. A total of 79 participants, comprising 28 patients with isolated cerebellar stroke, 27 patients with isolated cerebral stroke and 24 healthy controls, performed Poggendorff illusion tasks and 2 different control tasks. We also investigated core brain regions underpinning changes in the experience of the illusion effect using multivariate lesion-symptom mapping. Our results indicate that patients with isolated cerebellar stroke were significantly less likely to experience the Poggendorff illusion effect than patients with isolated cerebral stroke or healthy controls (74.6, 90.5 and 89.8%, respectively; F(2,76) = 6.675, P = 0.002). However, there were no inter-group differences in the control tasks. Lesion-symptom mapping analysis revealed that the brain lesions associated with the reduced frequency of the Poggendorff illusion effect were mainly centred on the right posteromedial cerebellar region, including the right lobules VI, VII, VIII, IX and Crus II. Our findings demonstrated, for the first time, that patients with cerebellar damage were significantly less likely to experience the Poggendorff illusion effect and that right posteromedial cerebellar lesions played an important role in this effect. These results provide new insight into alterations of a geometric illusion effect in patients with cerebellar disorders and pave the way for future clinical use of the illusion task to detect cerebellar abnormalities.

DOI： 10.1093/braincomms/fcad053

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Buccofacial apraxia (BFA) is associated with nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) as well as with the severity of apraxia of speech (AOS), a core symptom of nfvPPA. However, an association with agrammatism has not been established. The aim of this study was to examine the association between BFA and agrammatism in nfvPPA and to determine differences in atrophic regions in primary progressive aphasia (PPA) with and without BFA. Seventy-four patients with PPA were recruited, including 34, 15, 10, and 15 patients with nfvPPA, semantic variant PPA, logopenic variant PPA, and unclassified PPA, respectively. All patients underwent language examination and BFA evaluations. Voxel-based morphometry (VBM) was performed to determine whether atrophy of a specific lesion correlated with the presence of BFA. BFA was observed in 20 and 3 patients with nfvPPA and unclassified PPA, respectively. In a comparison of patients with nfvPPA with and without BFA, the BFA group showed significantly worse spontaneous speech and writing in the Western Aphasia Battery. The agrammatism ratio or the ratio of agrammatic errors to the total number of particles was higher in the BFA group; however, the severity of prosodic and phonetic components of AOS did not differ between the two groups. VBM showed that the severity of BFA correlated with atrophy of the opercular and triangular areas of the inferior frontal gyrus to a part of the left middle frontal gyrus. BFA has a different anatomical basis from AOS in patients with nfvPPA and that BFA is characterized by more anterior degeneration compared to that of AOS.

DOI： 10.1016/j.cortex.2022.10.010

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 39-year-old man exhibited ocular flutter and cerebellar ataxia following a subacute disturbance of consciousness and partial seizure. He was diagnosed with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy by tissue- and cell-based antibody assays. Brain single-photon emission computed tomography detected a significant increase in blood flow in the fastigial nucleus, a critical region for eye saccade control. Immunotherapies diminished the ocular flutter and reduced hyperperfusion in the fastigial nucleus. This case suggests that autoimmune GFAP astrocytopathy can cause ocular flutter and provides strong imaging evidence supporting the hypothesis that ocular flutter is caused by hyperactivity or disinhibition of the fastigial nucleus.

DOI： 10.1016/j.clineuro.2022.107307

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVE: Simultanagnosia is a rare neuropsychological symptom characterized by difficulty recognizing global structures while preserving perception of local detail. The condition is classified into ventral and dorsal types. Clinical presentation of ventral simultanagnosia includes a reduced ability to recognize multiple visual stimuli rapidly, that is, part-by-part recognition. Here, we report a case of ventral simultanagnosia with a unique presentation; when short-duration visual stimuli were presented, the patient could perform global recognition by improving his part-by-part approach. To investigate the relationship between local and global perception bias and the duration of the present stimulus, we conducted a visual perception test using hierarchically organized Navon figures. METHODS/RESULTS: The patient was a 62-year-old right-handed man who suffered from cerebral infarction in the right occipitotemporal lobe. He had no language dysfunction but exhibited left unilateral neglect, prosopagnosia, and ventral-type simultanagnosia. We conducted a visual perception test using the Navon figures and control figures as a visual stimulus. We randomly presented the figures for intervals of 0.2 or 20 s and let the patient report all the letters (global and/or local element) that he recognized. Global elements of the Navon letter were recognized a rate of 0% and 78.3% at intervals of 20 and 0.2 s, respectively, indicating that shorter presentation made the part-by-part approach less likely to manifest. CONCLUSIONS: We assumed that the simultanagnosia in this case was caused by failure to maintain the initially perceived global information for a long period of time during visual presentation, due to right occipitotemporal damage.

DOI： 10.1093/arclin/acab088

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Guillain-Barré syndrome (GBS) has occasionally occurred in people who have received coronavirus disease 2019 (COVID-19) vaccines. Dysgeusia is rare symptom of GBS. We herein report a rare case of sensory ataxic GBS with dysgeusia just after the second dose of the Pfizer-BioNTech COVID-19 vaccine. Although autoantibodies against glycolipids were not detected, immunotherapy with intravenous immunoglobulin and methylprednisolone pulse therapy effectively ameliorated the symptoms. Our report suggests that the COVID-19 vaccine may induce various clinical subtypes of GBS, including a rare variant with sensory ataxia and dysgeusia.

DOI： 10.2169/internalmedicine.8967-21

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Ultrasonography (US) is a noninvasive and patient-friendly tool for the evaluation of peripheral nerves. In motor neuron diseases, amyotrophic lateral sclerosis (ALS) has been reported to show the atrophy of peripheral nerves on US. However, the US findings are still unclear in spinal and bulbar muscular atrophy (SBMA), an adult-onset lower motor neuron disease caused by an abnormal CAG repeat expansion in the androgen receptor gene. METHODS: We prospectively recruited and evaluated 11 patients with genetically confirmed SBMA and 9 patients with ALS diagnosed according to the revised El Escorial ALS criteria or the Awaji electrodiagnostic criteria. The C5-C7 cervical nerve roots and the median and ulnar nerves were evaluated ultrasonographically. RESULTS: The cross-sectional areas (CSAs) of the C6 and C7 nerve roots, the median nerve in the upper arm and forearm, and the ulnar nerve in the upper arm were smaller in patients with SBMA than those in patients with ALS (p < 0.05), whereas the CSAs of the C5 nerve root and the ulnar nerve in the forearm were not smaller. CONCLUSIONS: US showed that the peripheral nerves in patients with SBMA were thinner than those in patients with ALS despite similar degrees of weakness and motor neuron loss. Possible causes include additional sensory nerve involvement and longer disease duration in patients with SBMA than those in patients with ALS.

DOI： 10.1007/s10072-022-05969-1

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: The basal ganglia and related dopaminergic cortical areas are important neural systems underlying motor learning and are also implicated in impulse control disorders (ICDs). Motor learning impairments and ICDs are frequently observed in Parkinson's disease (PD). Nevertheless, the relationship between motor learning ability and ICDs has not been elucidated. METHODS: We examined the relationship between motor learning ability and gambling propensity, a possible symptom for prodromal ICDs, in PD patients. Fifty-nine PD patients without clinical ICDs and 43 normal controls (NC) were administered a visuomotor rotation perturbation task and the Iowa Gambling Task (IGT) to evaluate motor learning ability and gambling propensity, respectively. Participants also performed additional cognitive assessments and underwent brain perfusion SPECT imaging. RESULTS: Better motor learning ability was significantly correlated with lower IGT scores, i.e., higher gambling propensity, in PD patients but not in NC. The higher scores on assessments reflecting prefrontal lobe function and well-preserved blood perfusion in prefrontal areas were correlated with lower IGT scores along with better motor learning ability. CONCLUSIONS: Our findings suggest that better motor learning ability and higher gambling propensity are based on better prefrontal functions, which are in accordance with the theory that the prefrontal cortex is one of the common essential regions for both motor learning and ICDs.

DOI： 10.1080/13803395.2022.2083083

PubMed

researchmap

記述言語：日本語   出版者・発行元：日本神経心理学会  

Broca野,Wernicke野,角回そして弓状束で構成されるWernicke-Geschwindのモデルは,脳の言語モデルとして広く知られている.しかし,詳細な画像検査に裏打ちされた症例の蓄積と,脳機能画像研究を中心とした脳神経科学の進歩を背景に,Broca野やWernicke野以外の様々な脳領域がヒトの言語活動に関与していることが明らかになっている.さらに近年では拡散MRIを用いた数々の物理モデルの登場により,ヒトの白質線維の走行を詳細に評価することが可能となり,多数の機能領域とそれらを橋渡しする複雑な白質線維から構成されるネットワークとして脳を捉える考え方が主流になりつつある.本章では,こうした古典モデルに代わる新たな言語モデルと,その展望について概説を行う.(著者抄録)

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Anticoagulation therapy, especially using heparin or recently developed oral direct factor Xa inhibitors (DiXals), is recommended as first-line treatment for cancer-related venous thromboembolism (VTE). However, the preventive efficacy of these anticoagulants for cancer-associated ischemic stroke is still unknown. We retrospectively investigated the efficacy of subcutaneous unfractionated heparin (UFH) and DiXals for preventing the recurrence of cancer-associated cryptogenic ischemic stroke with VTE. METHODS: We retrospectively studied consecutive patients with cancer-associated cryptogenic ischemic stroke and comorbid VTE who received subcutaneous UFH or oral DiXaIs at 9 hospitals. RESULT: Fifty-three patients (24 treated with UFH and 29 treated with DiXaIs) were enrolled. Of these, 47 demonstrated systemic metastasis (cancer stage IV). During 30-day follow-up after initiation of anticoagulation therapy, recurrent ischemic stroke was observed in only 1 patient (4%) in the UFH group and in 9 patients (31%) in the DiXal group. The incidence of major bleeding complications was similar between the 2 groups (4% and 10%, respectively). The cumulative risk of ischemic stroke recurrence within 30 days was lower with UFH than with DiXals (competing risk analysis, p = 0.008). In the DiXal group, patients who experienced recurrence showed significantly higher D-dimer levels than those without recurrence. CONCLUSION: In patients with cancer-associated cryptogenic ischemic stroke and comorbid VTE, UFH demonstrated a lower rate of recurrent ischemic stroke than DiXaIs, and there were no differences in bleeding risk between the 2 treatments. D-dimer levels at stroke onset increased the risk of recurrence in the DiXal group but not in the UFH group.

DOI： 10.1016/j.thromres.2021.08.016

PubMed

researchmap

担当区分：筆頭著者   記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Mild cognitive impairment (MCI) in Parkinson's disease (PD) is considered a risk factor for PD with dementia (PDD). Verbal fluency tasks are widely used to assess executive function in PDD. However, in cases of PD with MCI (PD-MCI), the relative diagnostic accuracy of different qualitative verbal fluency measures and their related neural mechanisms remain unknown. OBJECTIVE: This study aimed to investigate the relative diagnostic accuracy of qualitative (clustering and switching) verbal fluency strategies and their correlates with functional imaging in PD-MCI. METHODS: Forty-five patients with PD (26 with MCI and 19 without MCI) and 25 healthy controls underwent comprehensive neurocognitive testing and resting-state functional magnetic resonance imaging. MCI in patients with PD was diagnosed according to established clinical criteria. The diagnostic accuracy of verbal fluency measures was determined via receiver operating characteristic analysis. Changes in brain functional connectivity between groups and across clinical measures were assessed using seed-to-voxel analyses. RESULTS: Patients with PD-MCI generated fewer words and switched less frequently in semantic and phonemic fluency tasks compared to other groups. Switching in semantic fluency showed high diagnostic accuracy for PD-MCI and was associated with reduced functional connectivity in the salience network. CONCLUSION: Our results indicate that reduced switching in semantic fluency tasks is a sensitive and specific marker for PD-MCI. Qualitative verbal fluency deficits and salience network dysfunction represent early clinical changes observed in PD-MCI.

DOI： 10.3233/JPD-202473

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND AND PURPOSE: The presence of hemispatial neglect adversely affects functional outcomes in stroke patients; consequently, it warrants early targeted rehabilitative intervention. Nevertheless, hemispatial neglect in the acute phase of stroke has often been underdiagnosed. In this study, we aimed to detect hemispatial neglect at the bedside in acute stroke patients by measuring eye movements using video-oculography (VOG). METHODS: Forty-seven patients with acute unilateral supratentorial stroke were enrolled. We quantitatively measured horizontal saccade (latency, velocity, and amplitude) and smooth pursuit (gain) at the bedside using VOG and compared these variables with scores on the Behavioral Inattention Test (BIT), a screening battery to assess hemispatial neglect. RESULTS: Contralesional saccade latency, velocity, and amplitude, and ipsilesional smooth pursuit gain were suppressed compared with those in the opposite directions (p = 0.08, 0.02, 0.04, and 0.02, respectively). These directional ocular hypokinesia values correlated with the total BIT score (correlation coefficients -0.53, 0.48, 0.51, and 0.39, respectively). The association was significant even after adjusting for age and stroke severity. CONCLUSIONS: Eye movement measurements performed using VOG significantly correlated with the tendency for hemispatial neglect in acute supratentorial stroke patients. Bedside VOG measurement may be a simple biomarker for detecting hemispatial neglect even in patients in the supine position during the acute phase of stroke.

DOI： 10.1016/j.jns.2021.117442

PubMed

researchmap

記述言語：日本語   出版者・発行元：(有)科学評論社  

researchmap

記述言語：日本語   出版者・発行元：(株)医学書院  

＜文献概要＞Foreign accent syndromeとは「外国語のようだ」という違和感を持つ発話障害を特徴とした症候群である。これまで100例以上の報告がなされ,構音の歪みや音韻性錯語などの分節素の障害,高低・強弱・リズム異常などの超分節素の障害が特徴とされている。脳卒中以外にもさまざまな原因疾患が知られ,その責任病巣も多様である。原因も病巣もさまざまであることから,症候群として扱うほどの一貫性・普遍性があるのかなど,問題が山積している。

DOI： 10.11477/mf.1416201748

PubMed

researchmap

記述言語：英語  

Here we report three cases of anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) mimicking multiple sclerosis in which seropositivity for anti-MOG antibodies occurred during disease-modifying drug dimethyl fumarate (DMF) treatment. These patients developed relapses with anti-MOG antibody seroconversion after switching from fingolimod or steroid pulse therapy to DMF, which was associated with peripheral lymphocyte recovery. MOGAD is considered a humoral immune disease, and DMF reportedly enhances Th2-skewed humoral immune activity. Therefore, we suggest that DMF, but not fingolimod, may exacerbate humoral immune imbalance and enhance autoantibody production, leading to aggravation of MOGAD.

DOI： 10.3389/fimmu.2021.625465

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Aquired apraxia of speech is a disorder that impairs speech production, despite intact peripheral neuromotor function. Its pathomechanism remains to be established. Neurodegenerative lesion models provide an unequalled opportunity to explore the neural correlates of apraxia of speech, which is present in a subset of patients diagnosed with non-semantic variants of primary progressive aphasia. The normalized pairwise variability index, an acoustic measure of speech motor programming, has shown high sensitivity and specificity for apraxia of speech in cross-sectional studies. Here, we aimed to examine the strength of the pairwise variability index and overall word duration (i.e. articulation rate) as markers of progressive motor programming deficits in primary progressive aphasia with apraxia of speech. Seventy-nine individuals diagnosed with primary progressive aphasia (39 with non-fluent variant and 40 with logopenic variant) and 40 matched healthy controls participated. Patients were followed-up annually (range 1-6 years, median number of visits = 2). All participants completed a speech assessment task and a high-resolution MRI. Our analyses investigated trajectories of speech production (e.g. pairwise variablity index and word duration) and associations with cortical atrophy in the patients. At first presentation, word duration differentiated the nonfluent and logopenic cases statistically, but the range of scores overlapped substantially across groups. Longitudinally, we observed progressive deterioration in pairwise variability index and word duration specific to the non-fluent group only. The pairwise variability index showed particularly strong associations with progressive atrophy in speech motor programming brain regions. Of novelty, our results uncovered a key role of the right frontal gyrus in underpinning speech motor programming changes in non-fluent cases, highlighting the importance of right-brain regions in responding to progressive neurological changes in the speech motor network. Taken together, our findings validate the use of a new metric, the pairwise variability index, as a robust marker of apraxia of speech in contrast to more generic measures of speaking rate. Sensitive/specific neuroimaging biomarkers of the emergence and progression of speech impairments will be useful to inform theories of the pathomechanisms underpinning impaired speech motor control. Our findings justify developing more sensitive measures of rhythmic temporal control of speech that may enable confident detection of emerging speech disturbances and more sensitive tracking of intervention-related changes for pharmacological, neuromodulatory and behavioural interventions. A more reliable detection of speech disturbances has relevance for patient care, with predominance of progressive apraxia of speech a high-risk factor for later diagnosis of progressive supranuclear palsy or corticobasal degeneration.

DOI： 10.1093/braincomms/fcab205

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Foreign accent syndrome (FAS) is a rare acquired speech disorder wherein an individual's spoken accent is perceived as "foreign." Most reported cases involve left frontal brain lesions, but it is known that various other lesions can also cause FAS. To determine whether heterogeneous FAS-causing lesions are localized to a common functional speech network rather than to a single anatomical site, we employed a recently validated image analysis technique known as "lesion network mapping." METHODS: We identified 25 published cases of acquired neurogenic FAS without aphasia, and mapped each lesion volume onto a reference brain. We next identified the network of brain regions functionally connected to each FAS lesion using a connectome dataset from normative participants. Network maps were then overlapped to identify common network sites across the lesions. RESULTS: Classical lesion overlap analysis showed heterogeneity in lesion anatomical location, consistent with prior reports. However, at least 80% of lesions showed network overlap in the bilateral lower and middle portions of the precentral gyrus and in the medial frontal cortex. The left lower portion of the precentral gyrus is suggested to be the location of lesions causing apraxia of speech (AOS), and the middle portion is considered to be a larynx-specific motor area associated with the production of vowels and stop/nasal consonants and with the determination of pitch accent. CONCLUSIONS: The lesions that cause FAS are anatomically heterogeneous, but they share a common functional network located in the bilateral posterior region of the frontal lobe. This network specifically includes not only the lower portion of the central gyrus, but also its middle region, which is referred to as the larynx motor cortex and is known to be associated with phonation. Our findings suggest that disrupted networks in FAS might be anatomically different from those in AOS.

DOI： 10.1016/j.nicl.2021.102760

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

We herein report a 33-year-old woman who was an asymptomatic hepatitis B virus (HBV) carrier and presented with distal muscle weakness in the legs and asymmetrical paresthesia in the distal extremities. A nerve biopsy specimen revealed fibrinoid necrosis associated with inflammatory infiltration in the perineural space, and deposition of hepatitis B core antigen and C4d complement was detected in the vascular endothelial cells as well as around the vessels. She was diagnosed with HBV-related vasculitic neuropathy and treated with intravenous immunoglobulin (IVIG). Her symptoms completely subsided after eight weeks. Vasculitic neuropathy rarely develops in the chronic inactive stages of HBV infection. This is the first report of an HBV-inactive carrier with vasculitic neuropathy successfully treated with IVIG.

DOI： 10.2169/internalmedicine.4498-20

PubMed

researchmap

記述言語：日本語   出版者・発行元：(株)中外医学社  

researchmap

記述言語：日本語   出版者・発行元：(株)中外医学社  

researchmap

記述言語：英語  

Chronic tonsillitis has been attracted attention as a source of abnormal immune responses and a possible trigger of autoimmune diseases such as IgA nephritis, IgA vasculitis, palmoplantar pustulosis, psoriasis, rheumatoid arthritis, Behçet's disease, and myositis. Here we present the first report of anti-signal recognition particle antibody-associated necrotizing myopathy (anti-SRP myopathy) with IgA nephropathy and chronic tonsillitis in which the therapeutic response to intravenous immunoglobulin (IVIG) treatment was dramatically improved after tonsillectomy and accompanied by a rapid increase in ΔIgG, defined as the change in serum IgG levels 2 weeks after the start of IVIG treatment relative to pre-treatment levels. Moreover, serum anti-SRP antibody titers became undetectable after tonsillectomy even though the resected tonsils did not produce anti-SRP antibodies. Tonsillectomy should be considered when chronic tonsillitis is observed in patients with autoimmune diseases showing poor response to treatment, including anti-SRP myopathy.

DOI： 10.3389/fimmu.2020.595480

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.2169/internalmedicine.2126-18

PubMed

researchmap

記述言語：英語  

Fingolimod acts as a functional antagonist of the sphingosine-1-phosphate receptor and is widely used for relapsing-remitting multiple sclerosis (MS). Here we report the first case of non-traumatic acute epidural hematoma in a relapsing-remitting MS patient treated with fingolimod. Fingolimod might increase the risk of hemorrhage by enhancing vasospasm and causing vascular disruption. Switching fingolimod to other disease-modifying drugs, including dimethyl fumarate, should be considered when non-traumatic hemorrhage is observed in MS patients.

DOI： 10.3389/fneur.2019.00763

PubMed

researchmap

記述言語：日本語   出版者・発行元：医歯薬出版(株)  

researchmap

記述言語：日本語   出版者・発行元：(有)科学評論社  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1038/s10038-018-0518-8

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Steroid pulse therapy with methylprednisolone (mPSL) succinate ester is the most common treatment for neuromyelitis optica (NMO); no cases of anaphylaxis have been reported to date. Here, we report two cases of anaphylactic shock induced by mPSL pulse therapy in patients with NMO and concurrent systemic lupus erythematosus. Both patients had received several courses of mPSL pulse therapy without any problems previously. Repeated mPSL pulse therapy and comorbid humoral autoimmune disease might increase the risk of anaphylaxis. Corticosteroids without succinate esters should be considered as an alternative therapy to prevent anaphylaxis.

DOI： 10.1177/1352458518763099

PubMed

researchmap

掲載種別：研究論文（学術雑誌）  

Background: Dimethyl fumarate (DMF) was the second oral disease-modifying drug to be approved for multiple sclerosis (MS) in Japan, after fingolimod. Switching from fingolimod to DMF treatment is becoming increasingly common, because DMF has shown a better risk–benefit profile and an equivalent efficacy to fingolimod. Case presentation: We report a 35-year-old woman who was positive for anti-John Cunningham virus antibody and who developed severe rebound relapse of MS after switching from fingolimod to DMF. Five months after starting DMF treatment, she had a severe relapse attack with disseminated lesions in the cerebrum and cervical spinal cord. Furthermore, subsequent relapse attacks occurred with new lesions in the thoracic spinal cord, even during repeated steroid pulse therapies and plasma exchanges. The disease activity finally ceased after natalizumab administration. Conclusions: Switching from fingolimod to DMF carries the risk of MS reactivation and rebound. Natalizumab treatment for a limited period might be recommended to treat MS rebound in anti-John Cunningham virus antibody-positive patients.

DOI： 10.1111/cen3.12470

Scopus

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Although inflammation in the central nervous system is responsible for multiple neurological diseases, the lack of appropriate biomarkers makes it difficult to evaluate inflammatory activities in these diseases. Therefore, a new biomarker reflecting neuroinflammation is required for accurate diagnosis, appropriate therapy, and comprehension of pathogenesis of these neurological disorders. We previously reported that the cerebrospinal fluid (CSF) concentration of lateral olfactory tract usher substance (LOTUS), which promotes axonal growth as a Nogo receptor 1 antagonist, negatively correlates with disease activity in multiple sclerosis, suggesting that variation in LOTUS reflects the inflammatory activities and is a useful biomarker to evaluate the disease activity. To extend this observation, we analyzed the variation of LOTUS in the CSF of patients with bacterial and viral meningitis, which are the most common neuroinflammatory diseases. METHODS: CSF samples were retrospectively obtained from patients with meningitis (n = 40), who were followed up by CSF study at least twice, and from healthy controls (n = 27). Patients were divided into bacterial (n = 14) and viral meningitis (n = 18) after exclusion of eight patients according to the criteria of this study. LOTUS concentrations, total protein levels, and CSF cell counts in the acute and recovery phases were analyzed chronologically. We also used lipopolysaccharide-injected mice as a model of neuroinflammation to evaluate LOTUS mRNA and protein expression in the brain. RESULTS: Regardless of whether meningitis was viral or bacterial, LOTUS concentrations in the CSF of patients in acute phase were lower than those of healthy controls. As the patients recovered from meningitis, LOTUS levels in the CSF returned to the normal range. Lipopolysaccharide-injected mice also exhibited reduced LOTUS mRNA and protein expression in the brain. CONCLUSIONS: CSF levels of LOTUS correlated inversely with disease activity in both bacterial and viral meningitis, as well as in multiple sclerosis, because neuroinflammation downregulated LOTUS expression. Our data strongly suggest that variation of CSF LOTUS is associated with neuroinflammation and is useful as a biomarker for a broader range of neuroinflammatory diseases.

DOI： 10.1186/s12974-018-1084-x

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: In dementia with Lewy bodies (DLB) and Parkinson's disease with dementia (PDD), it is still debated whether white matter hyperintensities (WMH) on MRI reflect atherosclerotic cerebrovascular changes or Alzheimer's disease (AD)-related pathology such as cerebral amyloid angiopathy. To examine AD-related pathology in DLB and PDD, we compared the severity of WMH and medial temporal lobe atrophy among patients with DLB, PDD, non-demented PD (PDND), and AD. METHODS: We retrospectively studied sex- and age-matched outpatients with AD, DLB, PDD, and PDND, as well as subjects without central nervous system disorders as normal controls (n=50 each). All subjects underwent 1.5-T MRI examinations, and WMH detected by T2-weighted images or fluid-attenuated inversion recovery images were semiquantified according to the Fazekas method. Medial temporal lobe atrophy (MTA) was visually assessed by the MTA score. RESULTS: WMH were more prominent in AD, DLB, and PDD patients than in PDND patients and normal controls (NCs). DLB as well as AD showed more severe WMH than PDD. Visual assessment of medial temporal lobe atrophy showed that AD patients had the most severe atrophy, followed by DLB, PDD, and PDND patients, and NC subjects in that order. MTA scores showed significant correlations with WMH severity. CONCLUSION: Our results indicated that DLB was more similar to AD than to PDD in terms of MRI findings, suggesting that WMH in DLB may reflect mainly AD-related pathology rather than atherosclerotic cerebrovascular changes.

DOI： 10.1016/j.jns.2017.12.018

PubMed

researchmap

記述言語：日本語   出版者・発行元：(株)中外医学社  

researchmap

記述言語：日本語   出版者・発行元：日本神経心理学会  

&lt;p&gt;外国語様アクセント症候群（foreign accent syndrome：FAS）とは，同じ母国語を使用する第三者が&quot;外国語のようだ&quot;という違和感を持つような発話障害を特徴とした症候群である．比較的稀な症候ではあるが，これまで100例以上の報告がなされており，発話障害の特徴として，音の高低や強弱，リズム，音調などのイントネーションの異常といった超分節素の障害や，母音・子音変化などの分節素の障害が報告されている．脳卒中以外にも様々な原因疾患で生じることが知られており，その責任病巣については左中心前回など左半球による報告が多いが，右半球や脳幹，小脳病巣での報告もあり多様である．このように，FASは原因も病巣も様々であることから，そもそも&quot;症候群&quot;として扱うほどの一貫性や普遍性があるのか，発語失行（apraxia of speech：AOS）との異同についてなど未解決の問題が山積している．&lt;/p&gt;&lt;p&gt;今回，FASを呈した自験例の紹介と既報告例を振り返ることで，FASの特徴や発現機序などについて考察を行った．特に日本人FAS例は，英語アクセント型と中国・韓国語アクセント型の2つに分類されることが多く，AOSの特徴が目立つ例では，母音や子音の長さの変化を特徴とした英語アクセント型に，AOSの特徴が目立たずピッチの障害が目立つ例は中国・韓国語アクセント型になる可能性が考えられた．&lt;/p&gt;&lt;p&gt;また，失語症を伴わないFAS既報告例の病巣を用いたlesion network mapping解析を行った結果，喉頭の運動野（Larynx/Phonation area）として報告されている中心前回中部が，FASの神経基盤として重要である可能性が示唆された．&lt;/p&gt;

DOI： 10.20584/neuropsychology.17025

researchmap

記述言語：日本語   出版者・発行元：認知神経科学会  

DOI： 10.11253/ninchishinkeikagaku.20.89_2

CiNii Research

researchmap

記述言語：日本語   出版者・発行元：認知神経科学会  

&lt;p&gt;【要旨】 神経心理学とは、損傷された神経過程の観察を通して人間の心理現象の構造・機能を解明し、さらに患者の治療貢献を目指す学問である。症状が比較的安定した亜急性期以降に詳細な神経心理学的評価を行うことは、治療方針の決定や家族の介護を考える上で極めて重要であり、さらに脳卒中診療における神経心理学的診察・評価は、新たな仮説の提唱・検証に大きく貢献することが可能である。&lt;/p&gt;&lt;p&gt; また、近年大きく発展を遂げている脳画像解析や生理学的手法と組み合わせることで、脳卒中研究は、特定病期の病巣-機能という枠組みを超え、発症から慢性期に至るまでのダイナミックな機能ネットワークの変化を的確に捉え、さらにそれを治療へ結びつけるという新たな段階へ移行しつつある。多種多様な研究手法を組み合わせることで、今後も神経心理が脳科学を推進させていくことが期待される。&lt;/p&gt;

DOI： 10.11253/ninchishinkeikagaku.20.8

researchmap

researchmap

記述言語：日本語   出版者・発行元：(有)科学評論社  

researchmap

記述言語：日本語   出版者・発行元：一般社団法人 日本高次脳機能障害学会  

&lt;p&gt;&amp;ensp;&amp;ensp;失語症臨床において保続を観察する機会は多いが, 保続は失語症評価訓練の阻害要因の 1 つとしても知られている。今回, 発症から 6 ヵ月経過し, 保続が主症状の 1 つであった失語症者に対して TAP (Treatment of Aphasic Perseveration) を参考に保続の減少を目的とした訓練を約 1 ヵ月間施行した。訓練手続きは TAP のエラーコントロールメソッドを取り入れ, さらに訓練中に表出された保続の種類の質的な分析を行うことで, 保続に対する TAP の作用機序を明らかにしようと試みた。結果として, TAP による保続の減少は訓練語以外へも汎化し, さらに実生活での言語表出能力をも向上させたことがわかった。 一方, 保続の質的分析の結果からは, TAP は主に直後型保続の減少に寄与していることが明らかとなった。&lt;/p&gt;

researchmap

記述言語：日本語   出版者・発行元：一般社団法人 日本高次脳機能障害学会  

researchmap

記述言語：日本語   出版者・発行元：(株)医学書院  

＜ポイント＞高次脳機能障害は,日常生活に多大な障害をもたらす一方で病識が欠けることもあるため,正しい知識と診察技術をもって,検者が積極的に診察しなければならない.失語症は,自発話,物品呼称,聴理解,復唱,読み,書きを評価し,構音障害などとの鑑別や病型分類を行う.半側空間無視は,線分二等分試験や線分抹消試験,図形の模写試験で検出できる.失行症は,パントマイムや実物品の使用を行わせる.記憶検査は様々あるが,MMSEやHDS-Rの遅延再生課題が簡便に用いられる.(著者抄録)

researchmap

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 HEIDELBERG  

The aim of this study was to analyze the pattern of magnetic resonance diffusion-weighted imaging (DWI) findings in status epilepticus in terms of clinical characteristics. Participants comprised 106 patients with status epilepticus who were admitted to our hospital and underwent DWI. Forty-five patients (42.5 %) showed abnormal findings on DWI and were divided into two groups, comprising 26 patients (24.5 %) with cortex lesions alone and 19 patients (17.9 %) with cortex and pulvinar lesions in the same hemisphere. A long duration of status epilepticus (&gt; 120 min) tended to be more prevalent among patients with cortex and pulvinar lesions (57.9 %) than among patients with cortex lesions alone (30.8 %) by univariate and multivariate analyses. Todd's palsy tended to be more frequent in patients with abnormalities on DWI (24/45, 53.3 %) than in patients with normal DWI (21/61, 34.4 %). Six of the 26 patients with cortex lesions alone (23.1 %) had taken anti-epileptic drugs before the attack compared to none of the 19 patients with both cortex and pulvinar lesions. The trend toward a longer duration of status epilepticus in patients with both cortex and pulvinar lesions favors a spreading pattern of seizure discharge from cortex to pulvinar via cortico-pulvinar pathways, and anti-epileptic drugs might, to some extent, prevent spreading of seizure discharge from cortex to pulvinar. In addition, existence of high-intensity areas on DWI at the onset of epilepsy may be a predictive factor for the occurrence of Todd's palsy.

DOI： 10.1007/s00415-015-7948-4

Web of Science

Scopus

PubMed

researchmap

記述言語：日本語   出版者・発行元：日本神経心理学会  

&lt;p&gt;人類の最も偉大な発明の一つである文字言語は，単なるコミュニケーション手段の一つという枠組みを超え，文化の伝承や文明の発達にこれまで重要な役割を果たしてきた．このような現代社会において，文字言語の障害である失読や失書が日常生活に与える影響は，以前に比べてますます大きなものとなっている．&quot;Exnerの書字中枢&quot;とも呼ばれる左中前頭回後部の損傷により純粋失書が生じることは古くから知られていたが，症例報告の蓄積や脳機能画像研究の結果から，その重要性は近年再認識されている．ところが，複雑な書字過程の中でこの脳領域が担う実際の役割については不明な点が多く，書記素から書字運動への変換プロセスや，書字情報を一時的に保持する作動記憶，書記素表象と書字動作の運動企図のインターフェイスとしての機能など諸説あり，いまだ結論は出ていない．本項では前頭葉損傷による書字障害を中心に，前頭葉病変と読み書き障害について，その歴史的な背景から最近の知見についての概説を行う．&lt;/p&gt;

DOI： 10.20584/neuropsychology.32.4_278

researchmap

記述言語：日本語   出版者・発行元：一般社団法人 日本高次脳機能障害学会  

&lt;p&gt;&amp;ensp;&amp;ensp;コンピュータの急速な普及により, 脳損傷によるタイピング障害が社会生活に与える影響は深刻となりつつある。そこで我々は, 失タイプを呈した自験例の検討と, 健常者 fMRI 研究の結果から, タイピングの神経基盤, 失タイプの責任病巣について検討した。&lt;br&gt;&amp;ensp;&amp;ensp;【症例】78 歳の右利き男性。失語や失行, 半側無視, 運動感覚障害は認めなかったが, 選択的タイプ障害が明らかであった。病歴と脳 MRI, 各種検討課題の結果から, 本例は左中下前頭回後部の脳梗塞により, 音素-書記素変換と書記素バッファに障害を認め, 特に後者が失タイプの原因になっていると考察した。&lt;br&gt;&amp;ensp;&amp;ensp;【fMRI 検討】健常タッチタイピスト16 名を対象に fMRI による, タイプ・書字の神経基盤の検討を行った。その結果, 左上頭頂小葉前部～縁上回, 左上中前頭回後部にタイプ・書字の両課題で有意な賦活を認め, さらに左頭頂間溝 (IPS) 後部内側にタイピングでより強い賦活を認めた。&lt;br&gt;&amp;ensp;&amp;ensp;【考察】タイピングは書字中枢として知られる多くの脳領域や, 左 IPS 後部内側皮質など複数の脳領域が関与する複雑な認知プロセスであり, 障害部位に応じて質の異なる障害が生じる可能性がある。本例のように個々の症例の障害機序を検討していくことで, 失タイプの症候学の確立やリハビリテーションへの発展が今後期待される。&lt;/p&gt;

DOI： 10.2496/hbfr.36.392

researchmap

記述言語：日本語   出版者・発行元：(有)科学評論社  

researchmap

記述言語：英語   出版者・発行元：PUBLIC LIBRARY SCIENCE  

DOI： 10.1371/journal.pone.0137265

Web of Science

PubMed

researchmap

記述言語：英語   出版者・発行元：PUBLIC LIBRARY SCIENCE  

DOI： 10.1371/journal.pone.0137265

Web of Science

PubMed

researchmap

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

Mitochondrial complex III (CIII) deficiency comprises a group of complex and heterogeneous genetic disorders. TTC19 mutations constitute a rare cause of CIII deficiency and are associated with neurological disorders in childhood and adulthood. Herein, we describe a 27-year-old Japanese man with cerebellar ataxia, spastic paraparesis, loss of deep sensation, mild frontal lobe dysfunction and transient psychiatric symptoms. Brain magnetic resonance imaging showed cerebellar atrophy and bilateral high-intensity signals in the inferior olives and regions adjacent to periaqueductal gray matter, on T2-weighted images. On whole-exome sequencing, we detected a novel homozygous frameshift mutation c.157_158dup [p.Pro54Alafs* 48] in TTC19. Mitochondrial enzyme assays confirmed mild impairment of CIII enzymatic activity in lymphoblasts, which was consistent with TTC19-related CIII deficiency. His symptoms and radiological findings demonstrated an early stage or mild form of this disease, and further clarify the characteristics of patients with rare TTC19 mutations.

DOI： 10.1038/jhg.2015.7

Web of Science

Scopus

PubMed

researchmap

記述言語：日本語   出版者・発行元：(有)科学評論社  

researchmap

記述言語：日本語   出版者・発行元：(有)科学評論社  

researchmap

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 HEIDELBERG  

Spinocerebellar degeneration (SCD) is a progressive neurodegenerative disorder in which cerebellar ataxia causes motor disability. There are no widely applicable methods for objective evaluation of ataxia in SCD. An objective system to evaluate ataxia is necessary for use in clinical trials of newly developed medication and rehabilitation. The aim of this study was to develop a simple method to quantify the degree of upper-limb ataxia. Forty-nine patients with SCD participated in this study. Patients were instructed to trace an Archimedean spiral template, and the gap between the template spiral and the drawn spiral (gap area; GA) was measured using Image J software. Ataxia was rated using the Scale for the Assessment and Rating of Ataxia (SARA) and cerebellar volume was evaluated in 37 patients using an axial cross-section of magnetic resonance images that were obtained within 6 months of clinical evaluation. Regression analysis was performed to assess the relation between GA and patient age, disease duration, SARA score, and cerebellar volume. GA was significantly related to total SARA score (r = 0.660, p &lt; 0.001), the posture and gait (r = 0.551, p &lt; 0.001), speech (r = 0.527, p &lt; 0.001), hand movements (r = 0.553, p &lt; 0.001), and heel-shin slide (r = 0.367, p = 0.036) SARA subscores, and cerebellar volume (r = 0.577, p &lt; 0.001) but was not related to patient age (r = 0.176, p = 0.227) or disease duration (r = 0.236, p = 0.103). GA is a simple, useful method to objectively quantify the degree of cerebellar ataxia, especially upper-limb ataxia, and can be widely adopted in various settings, including clinical trials.

DOI： 10.1007/s00415-014-7353-4

Web of Science

Scopus

PubMed

researchmap

記述言語：日本語   出版者・発行元：(株)医学書院  

目的:ぶどう膜炎に多発性脳神経麻痺を合併した眼・神経サルコイドーシス症例の報告。症例:34歳女性が1ヵ月前からの右眼霧視で受診した。矯正視力は右1.2,左1.5で,右眼に前房内炎症があり,両眼に角膜後面沈着物と隅角結節,網膜静脈周囲炎があった。胸部X線検査で肺門リンパ節の腫脹があり,経気管支肺生検でサルコイドーシスと診断された。初診時から嗅覚と味覚障害があり,1ヵ月後から左三叉神経の不全麻痺と右顔面神経麻痺が生じた。嗅神経,視神経,三叉神経,顔面神経,舌咽神経,迷走神経の多発性脳神経麻痺と診断された。ステロイドパルス療法を行い,3日目から味覚と嗅覚障害が回復し,続いて眼底所見が改善した。10ヵ月後にすべての脳神経障害が寛解し,以後8ヵ月後の現在まで,眼炎症と神経症状の再発はない。結論:ステロイドパルス療法で,サルコイドーシスによる神経と眼病変が改善した。(著者抄録)

researchmap

記述言語：日本語   出版者・発行元：(株)中外医学社  

researchmap

記述言語：日本語   出版者・発行元：(有)科学評論社  

researchmap

記述言語：英語   出版者・発行元：WILEY-BLACKWELL  

DOI： 10.1002/mds.25296

Web of Science

Scopus

PubMed

researchmap

記述言語：日本語   出版者・発行元：医歯薬出版(株)  

researchmap

記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：医学書院  

We report a case of brain infarction in the anterior choroidal artery territory accompanied homonymous scotomas. A 59-year-old man with diabetes mellitus felt weakness in his left upper and lower extremities. He was admitted to our hospital with mild hemiparesis on his left side. He noticed a small black spot in the left inferior portion of his visual field
however, this disappeared within one minute. He had no visual defects as assessed by a confrontation test, but a Goldmann visual field test revealed that there were homonymous scotomas in the left inferior portion of the visual field. Brain MRI showed hyperintense signals on diffusion-weighted images in the territory of the right anterior choroidal artery. He was diagnosed as having a brain infarction. The anterior choroidal artery penetrates the lateral geniculate nucleus from the front, and branches of the artery usually supply the medial and lateral parts of the lateral geniculate nucleus. Occlusion of these branches causes the loss of the upper and lower homonymous sectors in the visual field. The present case exhibited homonymous scotomas. We assumed that our patient's homonymous scotomas were a variant form of wedge-shaped visual field deficits often seen in anterior choroidal artery syndrome. On the basis the experience gained in this case, we consider that patients with brain infarction in the anterior choroidal artery territory should undergo ophthalmological examination, even when no visual defects are detected by a confrontation test.

Scopus

PubMed

researchmap

総ページ数：vi, 202p   記述言語：日本語  

CiNii Books

researchmap

記述言語：日本語   出版者・発行元：(一社)日本高次脳機能学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：日本コミュニケーション障害学会  

Foreign accent syndrome(FAS)とは,同じ母国語を使用する第三者が"外国語のようだ"という違和感をもつような発話異常を特徴とした症候群である。これまでに100例以上の報告がなされており,構音の歪みや音韻性錯語などの分節素の障害や,高低・強弱・リズム異常などの超分節素の障害が特徴とされている。脳卒中以外にも様々な原因疾患で生じることが知られており,その責任病巣については左中心前回など左半球による報告が多いが,右半球や脳幹,小脳病巣による報告もあり多様である。このように,FASは原因も病巣も様々であることから,そもそも"症候群"として扱うほどの一貫性や普遍性があるのか,構音障害や発語失行との異同についてなど未解決の問題が山積している。そこで,本講演では主に国内外のFAS既報告例を通して,FASの特徴や発現機序,神経基盤などについて概説を行う。(著者抄録)

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本臨床神経生理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：(有)科学評論社  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本臨床神経生理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本高次脳機能障害学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：(株)へるす出版  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本臨床神経生理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経治療学会  

researchmap

記述言語：英語   出版者・発行元：(一社)日本てんかん学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：日本神経眼科学会  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経治療学会  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：認知神経科学会  

researchmap

記述言語：日本語   出版者・発行元：日本神経心理学会  

researchmap

記述言語：日本語   出版者・発行元：日本神経心理学会  

researchmap

記述言語：日本語   出版者・発行元：(株)日本臨床社  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経治療学会  

researchmap

記述言語：日本語   出版者・発行元：日本神経免疫学会  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：日本神経眼科学会  

researchmap

記述言語：日本語   出版者・発行元：Movement Disorder Society of Japan (MDSJ)  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：日本神経心理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本核医学会  

researchmap

記述言語：日本語   出版者・発行元：学研メディカル秀潤社 ; 1981-  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本高次脳機能障害学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：日本神経感染症学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：日本神経免疫学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本高次脳機能障害学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本認知症学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：東京医学社  

泌尿器科腫瘍でもさまざまなタイプの傍腫瘍性神経症候群(PNS)が報告されているが、なかでも特筆すべきものとして、精巣腫瘍に伴う傍腫瘍性辺縁系脳炎、特に抗Ma2抗体陽性脳炎と抗NMDA受容体脳炎があげられる。これら二つの疾患は若年男性にみられる非ヘルペス性辺縁系脳炎の中でも治療反応性が比較的良好であるため、早期診断による早期治療が、良好な予後を得るためにも極めて重要である。本疾患では初期に精巣腫瘍が検出されないこともしばしばあるため、特徴的な臨床症状を呈し抗体が陽性となった症例では、精巣超音波検査などの画像検査を繰り返し施行することで精巣腫瘍の早期診断に至ることがある。腎細胞癌や前立腺癌にもさまざまなPNSが合併することが知られており、特徴的な臨床症状を認めた場合には、骨盤臓器内の画像検索や各種腫瘍マーカーの検査が必要である。PNSの原因となり得る前立腺小細胞癌では、PSAが高値を呈さないこともあり注意が必要である。(著者抄録)

CiNii Books

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：日本音声言語医学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：日本神経心理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

researchmap

記述言語：日本語   出版者・発行元：日本神経心理学会  

researchmap

記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：SAGE PUBLICATIONS LTD  

Web of Science

researchmap

開催年月日： 2023年5月 - 2023年6月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（指名）  

researchmap

開催年月日： 2023年3月

会議種別：シンポジウム・ワークショップ パネル（指名）  

researchmap

会議種別：シンポジウム・ワークショップ パネル（公募）  

researchmap

記述言語：日本語   会議種別：口頭発表（招待・特別）  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

researchmap

会議種別：公開講演，セミナー，チュートリアル，講習，講義等  

researchmap

会議種別：公開講演，セミナー，チュートリアル，講習，講義等  

researchmap

会議種別：ポスター発表  

researchmap

会議種別：公開講演，セミナー，チュートリアル，講習，講義等  

researchmap

researchmap

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

記述言語：日本語  

researchmap

会議種別：口頭発表（一般）  

researchmap

記述言語：日本語  

researchmap