Publications by authors named "Yasuhisa Sakurai"

42 Publications

Asymmetric Bálint's syndrome with multimodal agnosia, bilateral agraphesthesia, and ineffective kinesthetic reading due to subcortical hemorrhage in the left parieto-occipito-temporal area.

Neurocase 2020 12 26;26(6):328-339. Epub 2020 Oct 26.

Department of Neurology, Mitsui Memorial Hospital , Tokyo, Japan.

We report a patient with asymmetric Bálint's syndrome (predominantly right-sided oculomotor apraxia and simultanagnosia and optic ataxia for the right hemispace), and multimodal agnosia (apperceptive visual agnosia and bilateral associative tactile agnosia) with accompanying right hemianopia, bilateral agraphesthesia, hemispatial neglect, global alexia with unavailable kinesthetic reading, and lexical agraphia for kanji (Japanese morphograms), after hemorrhage in the left parieto-occipito-temporal area. The coexistence of tactile agnosia, bilateral agraphesthesia, and ineffective kinesthetic reading suggests that tactile-kinesthetic information can be interrupted because of damage to the fiber connection from the parietal lobe to the occipito-temporal area, leading to these tactually related cognitive impairments.
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http://dx.doi.org/10.1080/13554794.2020.1831546DOI Listing
December 2020

Antibodies to the α3 subunit of the ganglionic-type nicotinic acetylcholine receptors in patients with autoimmune encephalitis.

J Neuroimmunol 2020 12 21;349:577399. Epub 2020 Sep 21.

Department of Molecular Neurology and Therapeutics, Kumamoto University Hospital, Kumamoto, Japan. Electronic address:

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.
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http://dx.doi.org/10.1016/j.jneuroim.2020.577399DOI Listing
December 2020

Dorsal type letter-by-letter reading accompanying alexia with agraphia due to a lesion of the lateral occipital gyri.

Neurocase 2020 10 17;26(5):285-292. Epub 2020 Aug 17.

Department of Neurology, Mitsui Memorial Hospital , Tokyo, Japan.

We report a patient with alexia with agraphia accompanied by letter-by-letter reading after hemorrhage in the left middle and inferior occipital gyri that spared the angular gyrus and the fusiform gyrus. Kanji (Japanese morphograms) and kana (Japanese phonetic writing) reading and writing tests revealed that alexia with agraphia was characterized by kana-predominant alexia and kanji-predominant agraphia. This type of "dorsal" letter-by-letter reading is discernable from conventional letter-by-letter reading that is observed in pure alexia in that (1) kinesthetic reading is less effective, (2) kana or literal agraphia coexists, and (3) fundamental visual discrimination is nearly normal.
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http://dx.doi.org/10.1080/13554794.2020.1803922DOI Listing
October 2020

Selective impairment of On-reading (Chinese-style pronunciation) in alexia with agraphia for kanji due to subcortical hemorrhage in the left posterior middle temporal gyrus.

Neurocase 2020 08 16;26(4):220-226. Epub 2020 Jul 16.

Department of Neurology, Mitsui Memorial Hospital , Tokyo, Japan.

We report a patient with alexia with agraphia for kanji after hemorrhage in the left posterior middle temporal gyrus. The results of single-character kanji reading and two-character - (Chinese-style pronunciation), - (native Japanese pronunciation), and Jukujikun (irregular ) reading word tests revealed that the patient could not read kanji characters with -reading but read the characters with -reading. We consider that this -reading alexia was caused by disconnection between the posterior inferior temporal cortex (orthographic lexicon) and the posterior superior temporal gyrus (phonological lexicon), and preserved - and Jukujikun-reading was realized by bypassing the orthography-to-phonology route by the semantic route.
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http://dx.doi.org/10.1080/13554794.2020.1788608DOI Listing
August 2020

Progressive Supranuclear Palsy with Wall-Eyed Bilateral Internuclear Ophthalmoplegia Syndrome: Authors' Second Case.

Case Rep Neurol 2019 May-Aug;11(2):205-208. Epub 2019 Jul 10.

Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan.

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome has previously been reported in only 2 patients with progressive supranuclear palsy (PSP). Herein, we report a third case of WEBINO syndrome with PSP. The patient was an 81-year-old man who had experienced gradually increasing gait disturbance and occasional falls since the age of 78 years. At 80 years of age, he presented with cognitive impairment, parkinsonism, and oculomotor abnormalities. The oculomotor abnormalities consisted of vertical gaze palsy and loss of eye convergence. Brain magnetic resonance imaging demonstrated marked atrophy of the midbrain. He was diagnosed with PSP. At the age of 81 years, he presented with alternating extropia in his forward gaze and adduction paresis and outward nystagmus of the abducted eye in his horizontal gaze, both of which were compatible with WEBINO syndrome. Previously, we reported the first case of PSP with WEBINO syndrome, and another group recently reported a second case. In light of the previous cases and the present case, WEBINO syndrome in PSP should not be considered extremely rare. Furthermore, WEBINO syndrome has not been reported in other neurodegenerative disorders, which suggests that it might be a useful and specific diagnostic finding in PSP.
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http://dx.doi.org/10.1159/000501394DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6738207PMC
July 2019

Kanji (Morphogram) and Kana (Phonogram) Problem in Japanese Alexia and Agraphia.

Authors:
Yasuhisa Sakurai

Front Neurol Neurosci 2019 30;44:53-63. Epub 2019 Apr 30.

Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan,

The kanji and kana (or kanji vs. kana) problem in the Japanese language denotes the dissociation between kanji (morphograms) and kana (phonograms) in reading/comprehension and writing. Since paragraphia of kana in a patient with amyotrophic lateral sclerosis was first reported in 1893, kanji-kana dissociation has been the central topic in Japanese aphasiology. Recent advancements in lesion-to-symptom analyses and functional imaging studies have identified some areas whose damage causes dissociative disturbances of reading or writing between kanji and kana. That is, (1) angular alexia with agraphia causes kanji agraphia; alexia of kana with an angular gyrus lesion is the result of a damage to the middle occipital gyrus; (2) alexia with agraphia for kanji is caused by a posterior inferior temporal cortex (mid-fusiform/inferior temporal gyri; visual word form area) lesion, whereas pure agraphia for kanji is caused by a posterior middle temporal gyrus lesion; and (3) pure alexia, particularly for kanji, results from a mid-fusiform gyrus lesion (Brodmann's Area [BA] 37), whereas pure alexia for kana results from a posterior fusiform/inferior occipital gyri lesion (BA 18/19).
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http://dx.doi.org/10.1159/000494952DOI Listing
August 2019

Rapidly progressive miliary brain metastasis of lung cancer after EGFR tyrosine kinase inhibitor discontinuation: An autopsy report.

Neuropathology 2019 Apr 13;39(2):147-155. Epub 2019 Mar 13.

Department of Pathology, Mitsui Memorial Hospital, Tokyo, Japan.

Miliary brain metastasis is a rare type of brain metastasis, in which carcinoma cells disseminate to numerous foci confined to Virchow-Robin/subpial spaces. Symptoms usually progress within several months, and magnetic resonance imaging (MRI) shows multiple small contrast-enhancing lesions. We report an autopsy case of a patient who rapidly deteriorated within a week due to miliary brain metastasis after epidermal growth factor receptor tyrosine kinase inhibitor (EGFR-TKI) discontinuation, without contrast-enhancing lesions on MRI. A 74-year-old woman was diagnosed with stage IV lung adenocarcinoma with EGFR L868R mutation 2 years before presentation. Gefitinib, an EGFR-TKI was started. After 7 months, multiple new punctate contrast-enhancing lesions in the cerebral cortex appeared. After switching to another EGFR-TKI, erlotinib, these lesions disappeared. One year later, erlotinib was discontinued because of disease progression in the lung and docetaxel was initiated. Sixteen days later, cognitive decline appeared which rapidly progressed to bedridden state in 4 days. MRI showed multiple cortical small fluid-attenuated inversion recovery high intensity lesions which lacked contrast enhancement. The patient exhibited a state of akinetic mutism within a few days, and died 52 days after the appearance of neurological symptoms. The rapid progression indicated disease flare after EGFR-TKI discontinuation. Autopsy revealed numerous foci of metastasis in the cerebral cortex, basal ganglia, thalamus, and cerebellum, in which cancer cells were mostly confined to the Virchow-Robin/subpial spaces. These pathological findings were compatible with previous reports of miliary brain metastasis. Recent reports suggest that early disseminated cancer cells can survive for a long time and even remain after chemotherapy in supportive niches, and Virchow-Robin spaces are the niches in the brain. Our case suggests that these cancer cells may rapidly proliferate as a withdrawal burst after discontinuation of molecular targeted drugs, and show pathological findings of miliary brain metastasis.
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http://dx.doi.org/10.1111/neup.12542DOI Listing
April 2019

Inflammatory myopathy with myasthenia gravis: Thymoma association and polymyositis pathology.

Neurol Neuroimmunol Neuroinflamm 2019 03 24;6(2):e535. Epub 2018 Dec 24.

Department of Neurology (N.U., K.T., C.I., A.U., A.K., T.T., J.S.), Graduate School of Medicine, University of Tokyo; Division of Neurology (M.K., K.K.), Department of Internal Medicine, National Defense Medical College, Saitama; Division of Neurology (Y. Shiio), Tokyo Teishin Hospital; Department of Neurology (Y. Sakurai), Mitsui Memorial Hospital; Department of Neurology (Y.H.), Teikyo University School of Medicine; and Department of Neurology (K.Y., S.N.-E.), Fukushima Medical University, Japan.

Objective: To provide evidence that idiopathic inflammatory myopathy (IM) with myasthenia gravis (MG) frequently shows thymoma association and polymyositis (PM) pathology and shares clinicopathologic characteristics with IM induced by immune checkpoint inhibitors (ICIs).

Methods: We analyzed the clinicopathologic features of 10 patients with idiopathic IM and MG identified in 970 consecutive patients with biopsy-proven IM.

Results: Seven patients (70%) had thymoma. IM and MG were diagnosed with more than 5-year time difference in 6 thymomatous patients and within 1 year in 1 thymomatous and 3 nonthymomatous patients. Seven thymomatous patients showed rhabdomyolysis-like features with respiratory failure (4/7), dropped head (3/7), cardiac involvement (2/7), and positive anti-acetylcholine receptor (anti-AChR) and anti-titin antibodies (7/7 and 4/6, respectively) but rarely showed ocular symptoms (2/7) or decremental repetitive nerve stimulation (RNS) responses (1/7) at IM diagnosis. Three nonthymomatous patients showed acute cardiorespiratory failure with rhabdomyolysis-like features (1/3), positive anti-AChR and anti-titin antibodies (3/2 and 2/2, respectively), and fluctuating weakness of the skeletal muscle without ocular symptoms (3/3). Muscle pathology showed a PM pathology with infiltration of CD8-positive CD45RA-negative T-lymphocytes (9/9), scattered endomysial programmed cell death 1 (PD-1)-positive cells (9/9), and overexpression of programmed cell death ligand 1 (PD-L1) on the sarcolemma of muscle fibers around the infiltrating PD-1-positive cells (7/9).

Conclusion: Rhabdomyolysis-like features, positive anti-AChR antibody without decremental RNS responses, and PD-L1 overexpression are possible characteristics shared by ICI-induced IM. Frequent thymoma association in patients with idiopathic IM and MG may suggest thymoma-related immunopathogenic mechanisms, including dysregulation of the immune checkpoint pathway.
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http://dx.doi.org/10.1212/NXI.0000000000000535DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340335PMC
March 2019

An Autopsy Case of Familial Neuronal Intranuclear Inclusion Disease with Dementia and Neuropathy.

Intern Med 2018 Dec 10;57(23):3459-3462. Epub 2018 Aug 10.

Department of Neurology, Graduate School of Medicine, The University of Tokyo, Japan.

Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease with marked variety in its clinical manifestations. While characteristic neuroimaging and skin biopsy findings are important clues to the diagnosis, autopsy studies are still important for confirming the exact disease features. We herein report the case of a patient who received an antemortem diagnosis of familial NIID with dementia-dominant phenotype that was later confirmed by an autopsy. Our report is the first to document a case of autopsy-confirmed NIID involving both cognitive impairment and sensorimotor neuropathy.
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http://dx.doi.org/10.2169/internalmedicine.1141-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306544PMC
December 2018

Repetitive Discharge in a Case of Isaacs Syndrome with Burning Sensation.

Intern Med 2018 09 27;57(17):2597. Epub 2018 Apr 27.

Department of Neurology, Mitsui Memorial Hospital, Japan.

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http://dx.doi.org/10.2169/internalmedicine.0392-17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6172557PMC
September 2018

Frontal Phonological Agraphia and Acalculia with Impaired Verbal Short-Term Memory due to Left Inferior Precentral Gyrus Lesion.

Case Rep Neurol 2018 Jan-Apr;10(1):72-82. Epub 2018 Mar 14.

Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan.

We report a patient with phonological agraphia (selective impairment of kana [Japanese phonetic writing] nonwords) and acalculia (mental arithmetic difficulties) with impaired verbal short-term memory after a cerebral hemorrhage in the opercular part of the left precentral gyrus (Brodmann area 6) and the adjacent postcentral gyrus. The patient showed phonemic paragraphia in five-character kana nonword writing, minimal acalculia, and reduced digit and letter span. Mental arithmetic normalized after 8 months and agraphia recovered to the normal range at 1 year after onset, in parallel with an improvement of the auditory letter span score from 4 to 6 over a period of 14 months and in the digit span score from 6 to 7 over 24 months. These results suggest a close relationship between the recovery of agraphia and acalculia and the improvement of verbal short-term memory. The present case also suggests that the opercular part of the precentral gyrus constitutes the phonological route in writing that conveys phonological information of syllable sequences, and its damage causes phonological agraphia and acalculia with reduced verbal short-term memory.
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http://dx.doi.org/10.1159/000487849DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5903121PMC
March 2018

Asymmetric oculomotor apraxia, optic ataxia, and simultanagnosia with right hemispatial neglect from a predominantly left-sided lesion of the parieto-occipital area.

Cogn Neuropsychiatry 2018 01 2;23(1):1-14. Epub 2017 Dec 2.

a Department of Neurology , Mitsui Memorial Hospital , Tokyo , Japan.

Introduction: Bálint's syndrome involves bilateral damage to the parieto-occipital area. The extent of the effect of unilateral damage on the Bálint's triad (oculomotor apraxia, optic ataxia, and simultanagnosia) remains unknown.

Methods: We examined a 63-year-old, right-handed woman who developed right hemianopia, oculomotor apraxia, optic ataxia, simultanagnosia, and hemispatial neglect (HSN) for the right after a cerebral infarction, with detailed neuropsychological tests, magnetic resonance imaging, and single photon emission computed tomography (SPECT).

Results: Neuropsychological examination showed that oculomotor apraxia, optic ataxia, and simultanagnosia were more pronounced in the right hemi-space, probably due to the limited eye movement in the right visual field, whereas HSN was restricted to the right hemi-space. Diffusion-weighted MR images revealed hyperintensity in the left parieto-temporo-occipital region, and several spotty areas of the bilateral frontal and parietal subcortical regions. SPECT revealed hypoperfusion in the left parieto-occipital region and frontal operculum and small areas of the right superior parietal lobule.

Conclusions: The case suggests that asymmetric (more pronounced in the right hemi-space) oculomotor apraxia, optic ataxia, and simultanagnosia occur in an extensive lesion of the left parieto-occipital cortices. Although HSN is not a prerequisite for simultanagnosia, the coexistence of HSN aggravates simultanagnosia in the hemi-space opposite the lesion.
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http://dx.doi.org/10.1080/13546805.2017.1407304DOI Listing
January 2018

Progressive Bálint's Syndrome in a Patient Demonstrating Dementia with Lewy Bodies.

Intern Med 2017 1;56(11):1421-1424. Epub 2017 Jun 1.

Department of Psychiatry, Mitsui Memorial Hospital, Japan.

We herein report a 65-year-old man demonstrating dementia with Lewy bodies who first presented with Bálint's syndrome. Two years later, a mild cognitive impairment was noted. From three years after onset, he developed progressive parkinsonism, visual hallucination, and autonomic dysfunction, in line with the deterioration of the cognitive function. Single photon emission computed tomography with a Tc-ethylcysteinate dimer performed two years after onset revealed hypoperfusion in the restricted area of the bilateral superior parietal lobule, which extended to the lateral occipital cortices within two years. It is suggested that the pathological process can extend from the parietal to occipital lobes.
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http://dx.doi.org/10.2169/internalmedicine.56.7005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5498210PMC
November 2017

[Brodmann Areas 39 and 40: Human Parietal Association Area and Higher Cortical Function].

Authors:
Yasuhisa Sakurai

Brain Nerve 2017 Apr;69(4):461-469

Department of Neurology, Mitsui Memorial Hospital.

The anatomy and function of the angular gyrus (Brodmann Area 39) and supramarginal gyrus (Brodmann Area 40) are described here. Both gyri constitute the inferior part of the parietal lobe. Association fibers from the angular gyrus project to the dorsolateral prefrontal cortex via the superior longitudinal fasciculus (SLF) II/arcuate fasciculus (AF), whereas those from the supramarginal gyrus project to the ventrolateral prefrontal cortex via SLF III/AF. Damage to the left angular gyrus causes kanji agraphia (lexical agraphia) and mild anomia, whereas damage to the left supramarginal gyrus causes kana alexia (phonological dyslexia) and kana agraphia (phonological agraphia). Damage to either gyrus causes Gerstmann's syndrome (finger agnosia, left-right disorientation, agraphia and acalculia) and verbal short-term memory impairment. "Angular alexia with agraphia" results from damage to the middle occipital gyrus posterior to the angular gyrus. Alexia and agraphia, with lesions in the angular or supramarginal gyrus, are characterized by kana transposition errors in reading words, which suggests the impairment of sequential phonological processing.
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http://dx.doi.org/10.11477/mf.1416200765DOI Listing
April 2017

Cancer association as a risk factor for anti-HMGCR antibody-positive myopathy.

Neurol Neuroimmunol Neuroinflamm 2016 Dec 7;3(6):e290. Epub 2016 Oct 7.

Department of Neurology and Anti-aging Medicine (M.K., K. Kaida), National Defense Medical College, Saitama; Department of Neurology (A.H., K.T., C.I., N.U., A.K., S.K., S.T., J.S.), Graduate School of Medicine, The University of Tokyo; Department of Neurology (M.H.M., Y.U.), Toranomon Hospital; Division of Rheumatology (Y.M.), Department of Internal Medicine, Showa University School of Medicine, Tokyo; Pulmonary Medicine and Clinical Immunology (K. Kurasawa), Dokkyo Medical University, Tochigi; Department of Diagnostic and Interventional Radiology (H.S.), Osaka City University, Graduate School of Medicine; Department of Neurology (M.S.), Teikyo University School of Medicine; Department of Neurology (A.C.), Kyorin University; Department of Neurology (Y. Shiio), Tokyo Teishin Hospital; Division of Neurology (Y. Sakurai), Mitsui Memorial Hospital, Tokyo; Department of Neurology (T.I.), Tsukazaki Memorial Hospital, Hyogo; and AXA Department of Health and Human Security, Graduate School of Medicine (M.I.), The University of Tokyo, Japan.

Objective: To show cancer association is a risk factor other than statin exposure for anti-3-hydroxy-3-methylglutaryl coenzyme A reductase autoantibody-positive (anti-HMGCR Ab+) myopathy.

Methods: We analyzed the clinical features and courses of 33 patients (23 female and 10 male) with anti-HMGCR Ab+ myopathy among 621 consecutive patients with idiopathic inflammatory myopathies.

Results: Among the 33 patients, 7 (21%) were statin-exposed and 26 were statin-naive. In relation with cancer, there were 12 patients (statin-exposed, n = 4) with cancers detected within 3 years of myopathy diagnosis (cancer association), 3 patients (all statin-naive) with cancers detected more than 3 years before myopathy diagnosis (cancer history), 10 cancer-free patients followed up for more than 3 years (all statin-naive), and 8 patients without cancer detection but followed up for less than 3 years (statin-exposed, n = 3). Therefore, 12 patients with cancer association (36%) formed a larger group than that of 7 statin-exposed patients (21%). Among 12 patients with cancer association, 92% had cancer detection within 1 year of myopathy diagnosis (after 1.3 years in the remaining patient), 83% had advanced cancers, and 75% died of cancers within 2.7 years. Of interest, 1 patient with cancer history had sustained increase in creatine kinase level over 12 years from cancer removal to the development of weakness.

Conclusions: Patients with cancer association formed a large group with poor prognosis in our series of patients with anti-HMGCR Ab+ myopathy. The close synchronous occurrence of cancers and myopathies suggested that cancer association is one of the risk factors for developing anti-HMGCR Ab+ myopathy.
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http://dx.doi.org/10.1212/NXI.0000000000000290DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5056647PMC
December 2016

High PR3-ANCA positivity in a patient with chronic inflammatory demyelinating polyneuropathy.

eNeurologicalSci 2017 Mar 5;6:4-5. Epub 2016 Oct 5.

Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan.

Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) is reported to be highly specific to vasculitis compared to myeloperoxidase (MPO)-ANCA. We report a case of a 19-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) with high PR3-ANCA positivity. The patient responded well to intravenous immunoglobulin plus oral steroid, and showed no signs of systemic vasculitis during the subsequent 10 months of follow-up. Our present case suggests that CIDP may accompany high PR3-ANCA levels, which should be differentiated from axonal neuropathy due to vasculitis.
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http://dx.doi.org/10.1016/j.ensci.2016.10.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5721574PMC
March 2017

Anti-TIF1-γ antibody and cancer-associated myositis: A clinicohistopathologic study.

Neurology 2016 07 24;87(3):299-308. Epub 2016 Jun 24.

Objective: We aimed to analyze the clinical and histopathologic features of cancer-associated myositis (CAM) in relation to anti-transcriptional intermediary factor 1 γ antibody (anti-TIF1-γ-Ab), a marker of cancer association.

Methods: We retrospectively studied 349 patients with idiopathic inflammatory myopathies (IIMs), including 284 patients with pretreatment biopsy samples available. For the classification of IIMs, the European Neuromuscular Center criteria were applied. Patients with CAM with (anti-TIF1-γ-Ab[+] CAM) and without anti-TIF1-γ-Ab (anti-TIF1-γ-Ab[-] CAM) were compared with patients with IIM without cancers within and beyond 3 years of myositis diagnosis.

Results: Cancer was detected in 75 patients, of whom 36 (48%) were positive for anti-TIF1-γ-Ab. In anti-TIF1-γ-Ab(+) patients with CAM, cancers were detected within 1 year of myositis diagnosis in 35 (97%) and before 1 year of myositis diagnosis in 1. All the anti-TIF1-γ-Ab(+) patients with CAM satisfied the dermatomyositis (DM) criteria, including 2 possible DM sine dermatitis cases, and were characterized histologically by the presence of perifascicular atrophy, vacuolated fibers (VFs), and dense C5b-9 deposits on capillaries (dC5b-9). In contrast, 39 anti-TIF1-γ-Ab(-) patients with CAM were classified into various subgroups, and characterized by a higher frequency of necrotizing autoimmune myopathy (NAM). Notably, all 7 patients with CAM classified into the NAM subgroup were anti-TIF1-γ-Ab(-) and exhibited no dC5b-9 or VFs.

Conclusions: CAM includes clinicohistopathologically heterogeneous disease entities. Among CAM entities, anti-TIF1-γ-Ab(+) CAM has characteristically shown a close temporal association with cancer detection and the histopathologic findings of dC5b-9 and VFs, and CAM with NAM is a subset of anti-TIF1-γ-Ab(-) CAM.
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http://dx.doi.org/10.1212/WNL.0000000000002863DOI Listing
July 2016

Letter by Terao et al Regarding Article, "Damage to the Left Precentral Gyrus Is Associated With Apraxia of Speech in Acute Stroke".

Stroke 2016 Apr 10;47(4):e74. Epub 2016 Mar 10.

Department of Neurology, Fukushima Medical University, Fukushima, Japan.

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http://dx.doi.org/10.1161/STROKEAHA.116.012755DOI Listing
April 2016

Recurrent painful ophthalmoplegia in a patient with diabetes mellitus: Is ophthalmoplegia associated with diabetes mellitus?

Cephalalgia 2016 Dec 28;36(14):1397-1398. Epub 2016 Jan 28.

Department of Neurology, Mitsui Memorial Hospital, Japan.

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http://dx.doi.org/10.1177/0333102416629240DOI Listing
December 2016

Ventral simultanagnosia and prosopagnosia for unfamiliar faces due to a right posterior superior temporal sulcus and angular gyrus lesion.

Neurocase 2016 24;22(1):122-9. Epub 2015 Jul 24.

a Department of Neurology , Mitsui Memorial Hospital , Tokyo , Japan.

We report a patient with ventral simultanagnosia, prosopagnosia for "unfamiliar faces" (dorsal prosopagnosia), spatial agraphia, and constructional disorder, particularly on the left spatial side, due to a lesion in the right posterior superior and middle temporal gyri and angular gyrus. The patient showed impairment of fundamental visual and visuospatial recognition, such as in object size, configuration, and horizontal point location, which probably underlay the mechanism of simultanagnosia and prosopagnosia. This case also suggests that the coexistence of simultanagnosia and prosopagnosia results from a right hemispheric insult, and damage to the temporoparietal area interrupts the incorporation of spatial information into object recognition. This disconnection of information flow, together with impaired object recognition per se, may impair the parallel processing of multiple objects, leading to object-by-object or part-by-part recognition.
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http://dx.doi.org/10.1080/13554794.2015.1066827DOI Listing
September 2016

Anti-glutamate ∊2 receptor antibody-positive and anti-N-methyl-d-aspartate receptor antibody-negative lobar encephalitis presenting as global aphasia and swallowing apraxia.

Case Rep Neurol 2014 Sep-Dec;6(3):291-6. Epub 2014 Dec 24.

National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan.

Background: Little is known about the difference between anti-N-methyl-D-aspartate receptor (NMDAR) antibody-positive encephalitis and anti-glutamate receptor (GluR) antibody-positive encephalitis.

Objectives: To characterize anti-GluR antibody-positive encephalitis.

Methods: We report a 33-year-old man with nonparaneoplastic anti-GluR ∊2, ζ1 and δ2 antibody-positive and anti-NMDAR antibody-negative encephalitis, using neuropsychological tests and imaging studies including magnetic resonance imaging and single photon emission computed tomography (SPECT) with a (99m)Tc-ethylcysteinate dimer.

Results: The patient exhibited global aphasia and swallowing apraxia (inability to transfer food to the pharyngeal cavity without sialorrhea). He was treated with 3 courses of corticosteroid pulse therapy and had recovered markedly 3 weeks after onset. Magnetic resonance diffusion-weighted images revealed hyperintensity in the bilateral frontal and left parietal cortices. Seven months later, a small area of hyperintensity in the left supramarginal gyrus remained. SPECT revealed hypoperfusion in extensive regions of the bilateral frontal lobes and left supramarginal gyrus. Thirteen months later, blood flow reduction was restricted to diffuse areas in the frontal lobes.

Conclusions: Frontal lobar encephalitis without medial temporal involvement, marked cognitive impairment with a relatively preserved level of consciousness, and a favorable response to corticosteroid therapy, with nearly reversible cortical damage, may characterize anti-GluR antibody-positive encephalitis.
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http://dx.doi.org/10.1159/000371442DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4307010PMC
February 2015

[Norman Geschwind: career and works].

Authors:
Yasuhisa Sakurai

Brain Nerve 2014 Nov;66(11):1327-36

Department of Neurology, Mitsui Memorial Hospital.

Norman Geschwind was an outstanding American neurologist. He first majored in psychology and then medicine at Harvard University. After graduation, he received neurological training at Beth Israel Hospital, the National Hospital at Queen Square in London, and Boston City Hospital, under the guidance of Denny-Brown, Charles Symonds, and Fred Quadfasel. He later moved to the Boston Veterans Administration Hospital, and established the Boston University Aphasia Research Center with Edith Kaplan. This center played a cardinal role in aphasia research in the United States. In 1969, Geschwind became a Professor at Harvard Medical School. Disconnection syndromes, which made Geschwind's name famous worldwide in the field of neurology, are reviewed here in regard to pure alexia, conduction aphasia, pure word deafness, isolation of the speech area, and apraxia, with reference to the Wernicke-Geschwind model. Other contributions by Geschwind are also described, such as behavioral changes in temporal lobe epilepsy (Geschwind syndrome) and cerebral lateralization, including the Geschwind-Galaburda hypothesis.
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http://dx.doi.org/10.11477/mf.1416200037DOI Listing
November 2014

Delayed leukoencephalopathy after carbon monoxide poisoning presenting as subacute dementia.

Intern Med 2014 15;53(13):1441-5. Epub 2014 Jun 15.

Department of Neurology, Mitsui Memorial Hospital, Japan.

We herein report the case of a 65-year-old woman who presented with the subacute onset of dementia and subsequently developed abnormal behavior and a gait disturbance. Her condition transiently improved; however, within one month, she became drowsy and poorly responsive, with limb chorea and urinary incontinence. Her history of frequently using charcoal led us to diagnose her with carbon monoxide (CO) poisoning. The findings of this case and a literature review suggest that subacute dementia due to CO poisoning recovers late, after a year or more, in patients above sixty years of age, and both hyperbaric oxygen and corticosteroid pulse therapy should be considered in such cases, even after one month.
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http://dx.doi.org/10.2169/internalmedicine.53.2132DOI Listing
May 2015

Impaired laryngeal voice production in a patient with foreign accent syndrome.

Neurocase 2015 4;21(3):289-98. Epub 2014 Mar 4.

a Department of Neurology , Mitsui Memorial Hospital , Tokyo , Japan.

We report a Japanese-speaking monolingual woman who developed foreign accent syndrome (FAS) following an infarction in the precentral and premotor cortices (Brodmann Area 6) at and around the inferior frontal sulcus. Her speech sounded Chinese or Korean to our bilingual coauthor who speaks Chinese and Japanese. Quantitative acoustic analyses of words and sentences showed that pitch (fundamental frequency variation) and intensity variances appeared lowered and fully voiced glottal pulses were reduced. These findings suggest laryngeal dysfunction that contributes to the unusual speech production in a case of FAS. This may be caused by damage to a restricted area of the motor and premotor cortices that controls laryngeal function.
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http://dx.doi.org/10.1080/13554794.2014.892622DOI Listing
December 2015

Progressive apraxic agraphia with micrographia presenting as corticobasal syndrome showing extensive Pittsburgh compound B uptake.

J Neurol 2013 Aug 16;260(8):1982-91. Epub 2013 Apr 16.

Department of Neurology, Mitsui Memorial Hospital, 1, Kanda-Izumi-cho, Chiyoda-ku, Tokyo 101-8643, Japan.

A 65-year-old woman developed progressive apraxic agraphia, characterized by poorly formed graphemes, a kanji (Japanese morphograms) recall impairment, relatively preserved oral spelling of kanji characters, and incorrect stroke sequences on writing accompanied by micrographia over a 3-year period. She also showed minor degrees of rigidity, limb-kinetic apraxia, and ideomotor apraxia of the left hand. Although asymmetric rigidity and limb-kinetic apraxia strongly suggested corticobasal degeneration, (11)C-Pittsburgh compound B positron emission tomography (PiB-PET) showed the predominantly right-sided accumulation of amyloid β in the cortices and striatum. (18)F-fluoro-deoxy-glucose PET and single photon emission computed tomography with a (99m)Tc-ethylcysteinate dimer (ECD-SPECT) also revealed predominantly right-sided hypometabolism and hypoperfusion in the primary sensorimotor cortex, posterior cingulate gyrus, temporoparietal cortices, frontal cortices, thalamus, and basal ganglia, a pattern characteristic of both corticobasal degeneration and Alzheimer's disease. The findings suggest that progressive apraxic agraphia with micrographia presenting as corticobasal syndrome can show an Alzheimer's disease pathology. It is also suggested that ideomotor apraxia of the left hand can occur without a callosal lesion, and is caused by hypometabolism or hypoperfusion in the right frontal and parietal cortices, as revealed by PET and SPECT.
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http://dx.doi.org/10.1007/s00415-013-6908-0DOI Listing
August 2013

[Lesion localization of non-aphasic alexia and agraphia].

Authors:
Yasuhisa Sakurai

Rinsho Shinkeigaku 2011 Aug;51(8):567-75

Department of Neurology, Mitsui Memorial Hospital.

The author reviews the lesion localization of non-aphasic alexia and agraphia and proposes a new classification of alexia and agraphia on this basis. The newly proposed alexia and agraphia are pure alexia for kana (Japanese phonograms), or more generally pure alexia for letters, caused by a lesion in the posterior occipital area (posterior fusiform/inferior occipital gyri), and pure agraphia for kanji (Japanese morphograms) caused by a lesion in the posterior middle temporal gyrus and also a lesion restricted to the angular gyrus. In addition, the anatomical lesions presumably responsible for the parietal apraxic agraphia, frontal pure agraphia and thalamic agraphia are discussed.
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http://dx.doi.org/10.5692/clinicalneurol.51.567DOI Listing
August 2011

Isolated thalamic agraphia with impaired grapheme formation and micrographia.

J Neurol 2011 Aug 6;258(8):1528-37. Epub 2011 Mar 6.

Department of Neurology, Mitsui Memorial Hospital, 1, Kanda-Izumi-cho, Chiyoda-ku, Tokyo 101-8643, Japan.

Two patients with isolated thalamic agraphia are described. Both showed kanji (Japanese morphograms) agraphia due to impaired character recall, grapheme deformity and micrographia (progressive reduction in character size during writing) after a lesion that involved the ventral lateral and ventroposterolateral nuclei. Single photon emission computed tomography with a (99m)Tc-ethylcysteinate dimer revealed hypoperfusion in the left precentral gyrus (Brodmann Area 6) and anterior supramarginal gyrus in both. Six months later, the extent of blood flow reduction decreased in the supramarginal gyrus in both patients and the precentral gyrus in patient 1. By this time, the writing impairment improved to nearly the normal range. Our study suggests that kanji agraphia (corresponding to lexical agraphia in Western countries) with poor grapheme formation and micrographia arises from a lesion in the ventral lateral and ventroposterolateral nuclei in the left thalamus. The accompaniment of poor grapheme formation and micrographia may reflect disruption of the cortico-subcortical motor circuit involving the putamen, thalamus, premotor cortex and sensorimotor cortex. It is also suggested that multiple cortical sites can be a target for secondary dysfunction that yields agraphia in a thalamic lesion, and that the recovery of reduced cortical blood flow does not always proceed in parallel with that of agraphia.
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http://dx.doi.org/10.1007/s00415-011-5981-5DOI Listing
August 2011

Alexia and agraphia with lesions of the angular and supramarginal gyri: evidence for the disruption of sequential processing.

J Neurol Sci 2010 Jan 6;288(1-2):25-33. Epub 2009 Nov 6.

Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan.

Objective: To determine the features of alexia or agraphia with a left angular or supramarginal gyrus lesion.

Methods: We assessed the reading and writing abilities of three patients using kanji (Japanese morphograms) and kana (Japanese syllabograms).

Results: Patient 1 showed kana alexia and kanji agraphia following a hemorrhage in the left angular gyrus and the adjacent lateral occipital gyri. Patient 2 presented with minimal pure agraphia for both kanji and kana after an infarction in the left angular gyrus involving part of the supramarginal gyrus. Patient 3 also showed moderate pure agraphia for both kanji and kana after an infarction in the left supramarginal and postcentral gyri. All three patients made transposition errors (changing of sequential order of kana characters) in reading. Patient 1 showed letter-by-letter reading and a word-length effect and made substitution errors (changing hiragana [one form of kana] characters in a word to katakana [another form of kana] characters and vice versa) in writing.

Conclusion: Alexia occurs as "angular" alexia only when the lesion involves the adjacent lateral occipital gyri. Transposition errors suggest disrupted sequential phonological processing from the angular and lateral occipital gyri to the supramarginal gyrus. Substitution errors suggest impaired allographic conversion between hiragana and katakana attributable to a dysfunction in the angular/lateral occipital gyri.
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http://dx.doi.org/10.1016/j.jns.2009.10.015DOI Listing
January 2010

Intramedullary spinal cord abscess treated with antibiotic therapy--case report and review.

Neurol Med Chir (Tokyo) 2009 Jun;49(6):262-8

Department of Medicine, Mitsui Memorial Hospital, Tokyo, Japan.

A 58-year-old man presented with an intramedullary spinal cord abscess (ISCA) manifesting as posterior neck pain, gait disturbance, and urinary retention, and transverse myelopathy 1 week later. Magnetic resonance imaging showed the ISCA at the C7 to T1 levels. He was treated under a diagnosis of cryptogenic ISCA with high-dose ampicillin and third- or fourth-generation cephalosporins, which resulted in complete recovery after 2 months. Review of the literature between January 1998 and August 2007 identified 26 cases of ISCA, including our patient. We also identified two additional nonsurgically treated ISCA patients reported between 1977 and 2007. The most common presentation was motor deficits in all patients, followed by fever, pain, and bladder dysfunction. The mortality rate was 1 of 26 patients, and neurological sequelae were observed in 15 of the 25 surviving patients. There was no significant difference in the frequency of neurological sequelae between surgically and nonsurgically treated patients. Mean length of the abscess in the surgically treated group was significantly larger than that in the medically treated group (5.8 vs. 2.2 vertebral bodies). All three nonsurgically treated patients with neurological sequelae had anaerobic infections and received antibiotic therapy later and for shorter periods than those with complete neurological recovery. Antibiotic treatment is comparable to surgery plus antibiotic treatment. Early broad-spectrum high-dose ampicillin and third-generation cephalosporin, covering Gram-positive, Gram-negative, and anaerobic organisms, should be the first choice of management for patients with ISCA.
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http://dx.doi.org/10.2176/nmc.49.262DOI Listing
June 2009

Agraphia for kanji resulting from a left posterior middle temporal gyrus lesion.

Behav Neurol 2008 ;19(3):93-106

Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan.

Objective: To clarify whether agraphia or alexia occurs in lesions of the left posterior middle temporal gyrus.

Methods: We assessed the reading and writing abilities of two patients with this lesion using kanji (Japanese morphograms) and kana (Japanese syllabograms).

Results: Patient 1 first presented with pure alexia more impaired for kana after an infarction in the left middle and inferior occipital gyri and right basal occipital cortex, and after a second infarction in the left posterior middle temporal gyrus adjoining the first lesion he showed alexia with agraphia for kanji and worsened alexia for kana; kanji alexia recovered over the following six to 10 months. Patient 2 presented with alexia with agraphia for kanji following a hemorrhage in the left posterior middle and inferior temporal gyri, which resolved to agraphia for kanji at two months after onset. Kana nonword reading was also slightly impaired, but became normal by six months post-onset. In both patients, kanji agraphia was mostly due to impaired character recall.

Conclusion: The present patients demonstrate that damage to the left posterior middle temporal gyrus alone can cause agraphia for kanji. If the adjacent mid fusiform/inferior temporal gyri (Area 37) are spared, the kanji alexia is transient.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5452449PMC
http://dx.doi.org/10.1155/2008/393912DOI Listing
September 2008