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

Autoimmune basal ganglia encephalitis causes neurological symptoms such as parkinsonism associated with basal ganglia lesions. Here, we report a case of autoimmune basal ganglia encephalitis without retinal lesions or malignancy harboring anti-recoverin antibodies. The patient was a 67-year-old Japanese woman who developed anorexia, parkinsonism, and disturbance of consciousness 7 days before admission. Brain magnetic resonance imaging showed hyperintense bilateral basal ganglia lesions on fluid-attenuated inversion recovery images. 18F-fluorodeoxyglucose-positron emission tomography showed no malignancy in the trunk, and dopamine transporter single-photon emission computed tomography with dopamine transporters revealed reduced radiotracer uptake in the basal ganglia. Further, anti-recoverin IgG antibodies were detected in serum immunoblot. Based on the clinical and imaging findings, the patient was diagnosed with autoimmune basal ganglia encephalitis with anti-recoverin antibodies and administered high-dose immunoglobulins (HD-IVIG), which led to an improvement in clinical symptoms. Anti-recoverin antibodies are paraneoplastic antibodies that explicitly bind to Ca2+-binding proteins in the retina and cause retinopathy. This pathological sequence is defined as cancer-associated retinopathy (CAR). However, in our case, autoimmune basal ganglia encephalitis developed without CAR syndrome or malignancy. Clinicians should be aware of the possibility of autoimmune basal ganglia encephalitis showing anti-recoverin antibodies but no CAR syndrome or malignancy, which should be treated with HD-IVIG therapy.

DOI： 10.1016/j.ensci.2021.100382

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

PURPOSE: In patients with Wallenberg's syndrome who present with body lateropulsion (BL), whether the center of pressure (COP) position and velocity characterize postural dysregulation is unknown. We measured time-course changes in COP parameters in three BL patients. METHODS: Three patients with acute Wallenberg's syndrome presented with BL. COP was measured for time-course changes during first standing and every week thereafter. COP positions, which indicate the deviation in the center of gravity, were calculated. COP velocities associated with dynamic movements of the center of gravity were analyzed separately for the BL and non-BL sides. RESULTS: All patients showed that COP position shifted to the BL side in first standing and changed to the center over time. COP velocities to the BL side were fast in first standing. Two of the three patients had significantly faster COP velocities to the BL side than to the non-BL side (p < .05), and one did not. In all three cases, the faster COP velocities to the BL side decreased significantly after 2 weeks compared to the initial standing position (p < .001). The change seemed to be related to the time when independent walking became possible. CONCLUSIONS: Fast COP velocity to the BL side might reflect postural dysregulation in patients with BL. These findings might be useful information for devising effective rehabilitation in patients with BL.

DOI： 10.1080/09593985.2021.1990448

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This report describes a 59-year-old woman who presented with progressive encephalomyelitis with rigidity and myoclonus (PERM)-like symptoms and severe dysautonomia, including orthostatic hypotension, sinus bradycardia, dysuria, and prolonged constipation. Her neurological symptoms improved after immunotherapy, but the dysautonomia persisted. Anti-ganglionic acetylcholine receptor (gAChR) α3 subunit antibodies, which are frequently identified in patients with autoimmune autonomic ganglionopathy, were detected in the pre-treatment serum. The central distribution of the nicotinic acetylcholine receptors, a target of anti-gAChR antibodies, and immunotherapeutic efficacy observed in this case indicate that anti-gAChR α3 subunit antibodies are associated with the PERM-like features accompanied by autonomic manifestations.

DOI： 10.2169/internalmedicine.6419-20

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

DOI： 10.1111/cen3.12656

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

Since autonomic dysfunction is closely associated with autoimmune encephalitis (AE), the objective of this study was to determine the autonomic symptoms and the prevalence of anti-α3 subunit of the ganglionic-type nicotinic acetylcholine receptor (gAChRα3) antibodies in the patients with AE. We reviewed the clinical features of 19 AE patients, and specifically analyzed sera for anti-gAChRα3 antibodies using the luciferase immunoprecipitation system (LIPS) assay. Cardiovascular autonomic symptoms were found to be common in patients with AE, and hypersalivation was seen only in patients with NMDAR encephalitis. LIPS detected anti-gAChRα3 antibodies in the sera from patients with AE (5/29, 26%). This study is the first to demonstrate that clinical characteristics including autonomic symptoms of AE patients with seropositivity for gAChR autoantibodies. It will be important to verify the role of gAChR antibodies in autonomic dysfunction and brain symptoms to clarify the pathogenesis of AE.

DOI： 10.1016/j.jneuroim.2020.577399

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Authorship：Lead author   Language：Japanese   Publisher：一般社団法人日本神経学会  

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Authorship：Lead author   Language：English  

Severe neurologic complications following epidural and spinal anesthesia rarely occur. Transverse myelitis has been reported as a rare complication of epidural or spinal anesthesia. We report a case of longitudinally extensive transverse myelitis and an isolated pontine lesion, which responded to immunotherapy. The patient was a 31-year-old pregnant woman who underwent elective cesarean section under epidural and spinal anesthesia. Though the insertions of the epidural and spinal catheters were smooth, she experienced back pain and transient hearing loss during epidural anesthesia. Postoperatively, she exhibited severe motor weakness in both lower extremities, neuralgia below the level of Th10 dermatome, and urinary retention. Magnetic resonance imaging showed longitudinally extensive transverse myelitis from T6 to T10 with a ring-shaped enhanced lesion and an isolated pontine lesion. These findings on magnetic resonance imaging were suggestive of autoimmune diseases such as neuromyelitis optica. The patient was diagnosed with an immunoreactive disease triggered by epidural or spinal anesthesia and was administered high-dose methylprednisolone, which led to the improvement in clinical symptoms. Clinicians should be aware of the possibility of the development of longitudinally extensive transverse myelitis and isolated pontine lesions after cesarean section under epidural and spinal anesthesia.

DOI： 10.1016/j.ensci.2020.100264

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

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2020&ichushi_jid=J01550&link_issn=&doc_id=20201110260013&doc_link_id=1390286426516089856&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1390286426516089856&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

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

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2020&ichushi_jid=J01550&link_issn=&doc_id=20201008400009&doc_link_id=1391130851446196096&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1391130851446196096&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

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

Nested polymerase chain reaction (PCR) testing of cerebrospinal fluid (CSF) has higher diagnostic sensitivity with regard to tuberculous meningitis (TBM) than conventional methods. Herein we describe the autopsy case of a 70-year-old woman with TBM that could not be diagnosed via nested PCR in CSF, even though it was performed three times. The clinical course, magnetic resonance imaging results, and elevated adenosine deaminase levels in CSF were consistent with TBM. We also performed a brain biopsy from the thickened leptomeninges of the patient, which showed granulomatous leptomeningitis consistent with TBM. However, we were not able to identify tuberculous bacilli by the acid-fast bacterial staining, single PCR test, and culture of the biopsy preparations. We finally diagnosed TBM in this case by the positive results of both the fourth PCR test and culture of her CSF, which were taken 7 days before her death. This case suggests that even the combination of repetitive nested PCR in CSF and brain biopsy lacks adequate sensitivity to exclude TBM in some patients.

DOI： 10.1016/j.jns.2020.116968

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Authorship：Lead author   Language：Japanese   Publisher：(株)医学書院  

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2020&ichushi_jid=J04871&link_issn=&doc_id=20200529420012&doc_link_id=10.11477%2Fmf.1416201559&url=https%3A%2F%2Fdoi.org%2F10.11477%2Fmf.1416201559&type=%88%E3%8F%91.jp_%83I%81%5B%83%8B%83A%83N%83Z%83X&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00024_2.gif

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

DOI： 10.2169/internalmedicine.3295-19

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Cladophialophora bantiana (C. bantiana) is a life-threatening melanized mycelial fungus causing brain abscess. C. bantiana is usually observed in tropical countries, including India. We report a Japanese case of C. bantiana presenting with myelitis mimicking neuromyelitis optica (NMO) and brain abscess. A 73-year-old man was administered prednisolone (30 mg/day) for antineutrophil cytoplasmic antibody (ANCA)-related vasculitis 100 days before admission. He had right side-dominant paraplegia and sensory loss in the right leg. T2-weighted spinal cord MRI revealed longitudinal high-intensity signals at the T7 to T12 levels. A ring-enhancing lesion at the T10 level was detected on gadolinium (Gd)-enhanced MRI. He was tentatively diagnosed with NMO, and steroid pulse therapy was performed. One month later, an abscess at the right cerebropontine angle was noted on Gd-enhanced brain MRI. Two months later, several subcutaneous intramuscular tumors were detected. Based on the morphological study of the cultured organelle obtained by tumor enucleation and the internal transcribed spacer sequence of ribosomal RNA, the pathogen was identified as C. bantiana. Although he received liposomal amphotericin B treatment, the patient died of respiratory insufficiency. C. bantiana infection should be considered in patients with myelitis presenting with longitudinal lesions and CNS abscess in an immunocompromised state.

DOI： 10.1016/j.jns.2018.12.013

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

PURPOSE: The aim of this study was to evaluate the clinical utility of arterial spin labeling (ASL) magnetic resonance imaging (MRI) for the detection of cerebellar hypoperfusion in patients with spinocerebellar degeneration (SCD). METHODS: Regional cerebral blood flow (CBF) were obtained from ASL and 123I-IMP single-photon emission computed tomography (SPECT) images by volume-of-interest analysis in patients with SCD (n = 16). Regional CBF were also measured by ASL in age-matched controls (n = 19) and by SPECT in separate controls (n = 17). The cerebellar CBF values were normalized to the CBF values for the whole gray matter (nCBF) in ASL and SPECT. RESULTS: The mean cerebellar nCBF measured by ASL was lower in patients with SCD (0.70 ± 0.09) than in the controls (0.91 ± 0.05) (p < 0.001), which was consistent with the comparison using SPECT (0.82 ± 0.05 vs. 0.98 ± 0.05, p < 0.001). The cerebellar nCBF measured by ASL significantly correlated with that determined by SPECT in patients (r = 0.56, p < 0.001). CONCLUSIONS: ASL imaging showed decreased cerebellar blood flow, which correlated with that measured by SPECT, in patients with SCD. These findings suggest the clinical utility of noninvasive MRI with ASL for detecting cerebellar hypoperfusion in addition to atrophy, which would aid the diagnosis of SCD.

DOI： 10.1016/j.jns.2018.09.007

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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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Event date： 2024

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Event date： 2024

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Event date： 2023.9

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Venue：東京  

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Venue：ハイブリッド開催（金沢で発表）  

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Event date： 2023.7

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Venue：名古屋  

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Event date： 2023.5 - 2023.6

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Venue：千葉  

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Venue：千葉  

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Venue：千葉  

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Venue：千葉  

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Venue：千葉  

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Event date： 2023.5 - 2023.6

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Venue：千葉  

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Event date： 2023.5 - 2023.6

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Venue：千葉  

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Event date： 2023.5 - 2023.6

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Venue：千葉  

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Event date： 2023.5 - 2023.6

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Venue：千葉  

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Event date： 2023.5 - 2023.6

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Venue：千葉  

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Event date： 2023.3

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Venue：ハイブリッド開催（WEBで発表）(金沢）  

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Event date： 2022.11

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Venue：東京  

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Venue：福島  

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Venue：山梨  

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Event date： 2022.7

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Venue：完全WEB開催  

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Event date： 2022.6

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Venue：金沢  

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Event date： 2022.5

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Venue：ハイブリッド開催（東京で発表）  

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Event date： 2022.5

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Venue：ハイブリッド開催（WEBで発表）（東京）  

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Event date： 2022.5

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Venue：ハイブリッド開催（WEBで発表）（東京）  

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Event date： 2022.5

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Venue：ハイブリッド開催（東京で発表）  

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Venue：ハイブリッド開催（WEBで発表）（東京）  

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Event date： 2022.5

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Venue：ハイブリッド開催（東京で発表）  

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Event date： 2022.5

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Venue：ハイブリッド開催（WEBで発表）（東京）  

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Venue：ハイブリッド開催（京都で発表）  

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Event date： 2022.3

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Venue：完全WEB開催  

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Event date： 2022.3

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Venue：完全WEB開催  

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Event date： 2022.3

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Venue：完全WEB開催  

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Venue：完全WEB開催  

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Venue：ハイブリッド開催（福井で発表）  

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Event date： 2022

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Event date： 2021.11

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Venue：ハイブリッド開催（WEBで発表）（東京）  

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Event date： 2021.10

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Venue：完全WEB開催  

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Event date： 2021.5

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Venue：ハイブリッド開催（WEBで発表）（京都）  

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Event date： 2021.5

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Venue：ハイブリッド開催（WEBで発表）（京都）  

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Event date： 2021.5

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Venue：ハイブリッド開催（WEBで発表）（京都）  

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Event date： 2021.3

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Venue：完全WEB開催  

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Venue：完全WEB開催  

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Venue：完全WEB開催  

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Venue：福井  

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Venue：福井  

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Event date： 2020.10

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Venue：完全WEB開催  

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Event date： 2020.10

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Venue：完全WEB開催  

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Event date： 2020.8 - 2020.9

Language：English  

Venue：ハイブリッド開催（発表なし誌上掲載のみ）（岡山）  

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Event date： 2020

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Event date： 2019.1

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Event date： 2016.9

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