Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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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

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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

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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

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Language：Japanese   Publisher：(一社)日本神経学会  

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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

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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

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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

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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

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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

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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

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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

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Language：Japanese   Publisher：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)

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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

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

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

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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

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Language：Japanese   Publisher：(有)科学評論社  

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Foreign accent syndrome (FAS) is a rare speech disorder characterized by the emergence of a foreign accent. To date, more than a hundred cases of FAS have been reported, and the impression of accent change is regarded to be the result of a combination of segmental deficits (i.e., phonetic distortions and phonemic paraphasias) and supra-segmental changes (i.e., stress, pitch, or rhythm variation). The most common etiology of FAS is stroke, followed by other causes. Various lesion locations have been identified to cause FAS. Owing to various heterogeneous etiologies and lesion locations, it remains controversial whether there is enough consistency or universality to treat FAS as a "syndrome".

DOI： 10.11477/mf.1416201748

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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

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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

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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

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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

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Language：Japanese   Publisher：(株)中外医学社  

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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

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A 24-year-old Japanese man exhibited slowly progressive gait disturbance from childhood to young adulthood. Physical and physiological examinations showed the involvement of both upper and lower motor neurons, fulfilling the diagnostic criteria for amyotrophic lateral sclerosis (ALS). Mild cognitive impairment and subclinical sensory involvement were also observed. A genetic analysis revealed novel compound heterozygous mutations, c.767C>T (p.Thr256Ile) and c.800A>G (p.Asp267Gly), in the vaccinia-related kinase 1 gene (VRK1). This is the first report of a Japanese patient with a motor neuron disease phenotype caused by VRK1 mutations. This diagnosis should be considered in atypical cases of juvenile-onset and slowly progressive types of motor neuron disease.

DOI： 10.2169/internalmedicine.2126-18

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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

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Language：Japanese   Publisher：医歯薬出版(株)  

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Essential hypersomnia (EHS) is a lifelong disorder characterized by excessive daytime sleepiness without cataplexy. EHS is associated with human leukocyte antigen (HLA)-DQB1*06:02, similar to narcolepsy with cataplexy (narcolepsy). Previous studies suggest that DQB1*06:02-positive and -negative EHS are different in terms of their clinical features and follow different pathological pathways. DQB1*06:02-positive EHS and narcolepsy share the same susceptibility genes. In the present study, we report a genome-wide association study with replication for DQB1*06:02-negative EHS (408 patients and 2247 healthy controls, all Japanese). One single-nucleotide polymorphism, rs10988217, which is located 15-kb upstream of carnitine O-acetyltransferase (CRAT), was significantly associated with DQB1*06:02-negative EHS (P = 7.5 × 10-9, odds ratio = 2.63). The risk allele of the disease-associated SNP was correlated with higher expression levels of CRAT in various tissues and cell types, including brain tissue. In addition, the risk allele was associated with levels of succinylcarnitine (P = 1.4 × 10-18) in human blood. The leading SNP in this region was the same in associations with both DQB1*06:02-negative EHS and succinylcarnitine levels. The results suggest that DQB1*06:02-negative EHS may be associated with an underlying dysfunction in energy metabolic pathways.

DOI： 10.1038/s10038-018-0518-8

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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

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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

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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

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Language：English   Publishing type：Research paper (scientific journal)  

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

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Language：Japanese   Publisher：(株)中外医学社  

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Language：Japanese   Publisher：日本神経心理学会  

&lt;p&gt;The foreign accent syndrome (FAS) is a rare speech disorder characterized by the emergence of foreign accent. Until now, more than 100 cases of FAS have been reported and the impression of accent change is regarded to be the result of a combination of segmental deficits (i.e., phonetic distortions and phonemic paraphasias), and supra-segmental changes (i.e., stress, pitch, or rhythm variation known as disprosody). The most common etiology for FAS involves a stroke, followed by other causes. As for the anatomical substrate for FAS, most of the cases involve the lesion in the left frontal lobe, especially in the left pre-central gyrus. However, various lesions were reported to develop FAS, including the right hemisphere, the brain stem, and the cerebellum. Because of such a heterogeneous etiology and lesion location, it is controversial whether there is consistency or universality enough to treat it as a &quot;syndrome&quot;.&lt;/p&gt;&lt;p&gt;Here, we reviewed the literature of FAS with a neurogenic origin to elucidate the characteristics and mechanism of FAS. As a result, we suggested that the Japanese FAS cases could be classified into two subtypes, i.e., the &quot;English accent type&quot; and the &quot;Chinese/Korean accent type&quot; depending on the types of speech error. In addition, the lesion network mapping analysis using the lesions of the previous FAS cases without aphasia suggested that the common disrupted functional network might be localized to the middle portion of the precentral gyrus, known as the larynx / phonation area.&lt;/p&gt;

DOI： 10.20584/neuropsychology.17025

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Language：Japanese   Publisher：認知神経科学会  

DOI： 10.11253/ninchishinkeikagaku.20.89_2

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Language：Japanese   Publisher：認知神経科学会  

&lt;p&gt;The purpose of neuropsychology is to clarify the structure and function of human psychological phenomena by observing injured neural system, and to treat the patients with cognitive dysfunctions. Even in the subacute or chronic phase, detailed neuropsychological evaluation is helpful to determine treatment policy for the stroke patient and to reduce the burden of the family care. Moreover, neuropsychology can greatly contribute to the proposal and verification of new hypotheses in brain science. In combination with neuroimaging and neurophysiological methods which have been greatly advanced in recent years, neuropsychology will continue to contribute to the progression of brain science.&lt;/p&gt;

DOI： 10.11253/ninchishinkeikagaku.20.8

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Language：Japanese   Publisher：(有)科学評論社  

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Language：Japanese   Publisher：一般社団法人 日本高次脳機能障害学会  

&lt;p&gt;&amp;ensp;&amp;ensp;Perseveration is frequently observed in aphasic patients and it can be an obstruction for aphasic evaluations and rehabilitations. In this study, we employed a one-month training program to reduce perseveration for an aphasic patient who showed perseverations as a main complaint throughout the 6-month conventional aphasic therapies. The training program was made based on the error-control method of the Treatment of Aphasic Perseveration (TAP) . Additionally, we studied the underlying mechanism for the recovery of perseverations by qualitative analysis of perseverative errors during the TAP program. As a result, the perseveration had been remarkably reduced and naming scores had been improved even in the un-trained word-set. Furthermore, the word-expressive function in daily life had also been improved after training. That is to say, the effect of the training had generalized in every day life. Also, qualitative analysis of perseverative errors showed that TAP program had effect on reducing immediate type of perseveration.&lt;/p&gt;

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Language：Japanese   Publisher：一般社団法人 日本高次脳機能障害学会  

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Language：Japanese   Publisher：(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)

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：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

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Language：Japanese   Publisher：日本神経心理学会  

&lt;p&gt;Reading and writing is one of the most important invension in the history of mankind, and has played a central role in the dissemination of culture and the development of civilization. Therefore, an influence of aquired reading and writing disorders (i.e., alexia and agrapihia) has become more serious in the modern society. It is well known that the patient with the lesion that involves the posterior part of the left middle frontal gyrus, tradittionally known as the &quot;Exner&#039;s area&quot;, sometimes develops pure agraphia, and this brain lesion has been described as a major &quot;writing center&quot;. Although the role of this region is still unknown, recent clinical and neuroimaging studies suggested that this area should have a crucial roles in writing processes, e.g., the conversion of graphemic representations to motor commands, the orthographic working-memory, or the interface between orthographic represanetation and generation of motor commands. Here, we review the historical bakground and the recent findings of reading and writing disorders due to the left frontal lobe lesions. Especially, we focus on the relationship between agraphia and Exner&#039;s area.&lt;/p&gt;

DOI： 10.20584/neuropsychology.32.4_278

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Language：Japanese   Publisher：一般社団法人 日本高次脳機能障害学会  

&lt;p&gt;&amp;ensp;&amp;ensp;The opportunity of using a personal computer is increasing in everyday life and, accordingly, the impact of typing disorder on our social activities is becoming a serious issue. To investigate the neural substrate of typewriting and to detect the crucial lesions for typewriting disorder, we have intensively examined a case with isolated typing impairment, and conducted a functional magnetic resonance imaging (fMRI) study in healthy volunteers.〈Case〉A 78-year-old right-handed man, who suffered from stroke, showed isolated typewriting disorder without aphasia, apraxia, hemispatial neglect, or sensory-motor deficit. The history of present illness, the MRI findings, and the detailed neuropsychological examinations suggested that the typing impairment was attributable to disturbances of the phoneme-grapheme conversion and the graphemic buffer resulted from the left posterior part of the left superior/middle frontal lesion.〈fMRI study〉To investigate the neural substrate of typewriting, we conducted a fMRI study in 16 healthy skillful touch typists. As a result, two brain regions were activated during both the typing and the writing tasks: the left superior parietal lobule ranging to the left supramarginal gyrus, and the left premotor cortex. Furthermore, direct comparison between the typing and the writing task revealed greater activation of left posteromedial intraparietal cortex in the typing task.&lt;/p&gt;&lt;p&gt;&amp;ensp;&amp;ensp;It is suggested that typewriting is a complex cognitive process that involves multiple brain regions including the writing centers and the left posteromedial intraparietal cortex. Accordingly, it is supposed that the different brain lesions might cause different types of typing disorders. Further study is needed to establish the symptomatology and the training methods for typewriting disorders.&lt;/p&gt;

DOI： 10.2496/hbfr.36.392

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Language：Japanese   Publisher：(有)科学評論社  

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Language：English   Publisher：PUBLIC LIBRARY SCIENCE  

DOI： 10.1371/journal.pone.0137265

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Language：English   Publisher：PUBLIC LIBRARY SCIENCE  

DOI： 10.1371/journal.pone.0137265

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：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

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Language：Japanese   Publisher：(有)科学評論社  

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Language：Japanese   Publisher：(有)科学評論社  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：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

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Language：Japanese   Publisher：(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)

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Language：Japanese   Publisher：(株)中外医学社  

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Language：Japanese   Publisher：(有)科学評論社  

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Language：English   Publisher：WILEY-BLACKWELL  

DOI： 10.1002/mds.25296

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Language：Japanese   Publisher：医歯薬出版(株)  

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：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.

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Total pages：vi, 202p   Language：Japanese  

CiNii Books

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Language：Japanese   Publisher：(一社)日本高次脳機能学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：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)

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本臨床神経生理学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：(有)科学評論社  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本臨床神経生理学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本高次脳機能障害学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：(株)へるす出版  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本臨床神経生理学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経治療学会  

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Language：English   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：日本神経眼科学会  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経治療学会  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：認知神経科学会  

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Language：Japanese   Publisher：日本神経心理学会  

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Language：Japanese   Publisher：日本神経心理学会  

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Language：Japanese   Publisher：(株)日本臨床社  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経治療学会  

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Language：Japanese   Publisher：日本神経免疫学会  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：日本神経眼科学会  

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Language：Japanese   Publisher：Movement Disorder Society of Japan (MDSJ)  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：日本神経心理学会  

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Language：Japanese   Publisher：(一社)日本核医学会  

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Language：Japanese   Publisher：学研メディカル秀潤社 ; 1981-  

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Language：Japanese   Publisher：(一社)日本高次脳機能障害学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：日本神経感染症学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：日本神経免疫学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本高次脳機能障害学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本認知症学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：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

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：日本音声言語医学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：日本神経心理学会  

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Language：Japanese   Publisher：(一社)日本神経学会  

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Language：Japanese   Publisher：日本神経心理学会  

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Language：English   Publishing type：Research paper, summary (international conference)   Publisher：SAGE PUBLICATIONS LTD  

Web of Science

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Event date： 2023.5 - 2023.6

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Event date： 2023.3

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