Naoko Matsui

The University of Tokushima, Tokusima, Tokushima, Japan

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Publications (17)40.33 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: Multifocal motor neuropathy (MMN) is characterized by clinical improvement with intravenous immunoglobulin and the frequent detection of anti-ganglioside antibodies. However, the immunological background of the neuronal damage in MMN is still unclear.
    Journal of the neurological sciences. 08/2014;
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    ABSTRACT: Our objective was to do an epidemiologic survey of patients with multifocal motor neuropathy (MMN) in comparison with those with amyotrophic lateral sclerosis (ALS) in Japan. In this retrospective study, we examined 46 patients with MMN and 1,051 patients with ALS from major neuromuscular centers in Japan from 2005 to 2009. Diagnosis was based on the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) and the revised El Escorial criteria. The efficacy of intravenous immunoglobulin (IVIg) was also taken into consideration in the diagnosis of MMN. The ratio of MMN to ALS patients (0–0.10) varied among the centers, but mostly converged to 0.05. The prevalence was estimated to be 0.29 MMN patients and 6.63 ALS patients per 100,000 population. The frequency of MMN patients was around 1 out of 20 ALS patients, and MMN was possibly underdiagnosed in some centers.
    Muscle & Nerve 03/2014; 49(3):357-61. · 2.31 Impact Factor
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    ABSTRACT: Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the brain caused by the JC virus in immunocompromised patients. We report characteristic features of proton MR spectroscopy, 3-dimensional pseudo-continuous arterial spin labeling imaging, and diffusion tensor imaging in a 53-year-old patient with PML. The utility of multi-modal magnetic resonance techniques for longitudinal monitoring was indicated by their reevaluation over time and consideration of their relation to prognosis.
    Magnetic Resonance in Medical Sciences 01/2014; 13(1):55-59. · 0.75 Impact Factor
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    ABSTRACT: A 36-year-old woman complained of general malaise. She presented with hyponatremia and plasma osmotic pressure was lower than urinary osmotic pressure. In addition, serum antidiuretic hormone level was higher than the measurement sensitivity. She was diagnosed with the syndrome of inappropriate antidiuretic hormone secretion (SIADH). She fell into a coma despite correction of serum sodium level. Brain magnetic resonance imaging (MRI) revealed high signal intensities in the cerebral cortex, striatum, thalamus, hypothalamus, midbrain, and pons in fluid-attenuated inversion recovery images. Spinal MRI revealed a longitudinally extending lesion in the cervical cord. Serum sample was positive for anti-aquaporin-4 antibody, supporting the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) combined with central pontine and extrapontine myelinolysis. In patients with NMOSD, the immune reaction can gradually cause destructive changes of the hypothalamus and lead to unstable ADH secretion in the absence of immunomodulatory treatment.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(7):556-60.
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    ABSTRACT: The thymus is implicated as an organ that contributes to autoimmunity in myasthenia gravis (MG) patients. Hassall’s corpuscles (HCs) are assumed to represent the terminally differentiated stage of medullary thymic epithelial cells (mTECs). By using multicolor immunohistofluorescence analysis, we examined HCs in thymuses that were therapeutically excised from MG (+) and MG (−) patients. We found that the number of HCs per unit area of the thymic medulla was significantly elevated in the thymuses of MG (+) patients with thymic hyperplasia. CCL21 expression increased in the hyperplastic MG thymuses. We speculate that the altered differentiation of mTECs is associated with the thymic hyperplasia and the onset of MG.
    Journal of neuroimmunology 01/2014; · 2.84 Impact Factor
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    ABSTRACT: Myasthenia gravis (MG) is an antibody-mediated, T-cell-dependent autoimmune disease. The symptoms are caused by high-affinity IgG against the muscle acetylcholine receptor (AChR) at the neuromuscular junction. The production of these antibodies in B-cells depends on AChR-specific CD4(+) T-cells and the thymus gland seems to play a significant role in the pathogenesis of MG. Altered thymic T-cell export seems to be associated with a pathological mechanism in myasthenia gravis. Tacrolimus (FK506) has recently been used to treat MG. We examined the effects of tacrolimus on thymic T-cell export in patients with MG. Sixteen patients with nonthymomatous and/or thymectomized MG were treated with oral administrations of tacrolimus. To assess the effect of tacrolimus on the thymic output, we assayed the levels of T-cell receptor excision circle (TREC), a molecular marker of thymus emigrants. T-cell receptor excision circle was not significantly different from those in age-matched controls before tacrolimus therapy, but they were partially decreased 4 months after tacrolimus therapy. T-cell receptor excision circle levels were significantly decreased in the thymomatous group (p < 0.05), but not in the nonthymomatous group. Tacrolimus treatment significantly attenuated TREC levels in cultured CD4(-)CD8(+) cells (p < 0.05), but total cell counts were not significantly changed. These results indicate that TREC levels may become a marker of the curative effect of tacrolimus therapy for thymomatous MG, and that tacrolimus suppresses not only activating T-lymphocytes, but also naïve T-cells.
    Archives of Medical Science 12/2013; 9(6):1090-1096. · 1.89 Impact Factor
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    ABSTRACT: Inflammatory responses in the cerebrospinal fluid (CSF) of patients with sporadic Creutzfeldt-Jakob disease (sCJD) remain elusive. We conducted a case-control study, in which 14 patients with sCJD, 14 with noninflammatory neurological disorders, and 14 with autoimmune encephalitis were enrolled. We used the suspension array system to measure the concentrations of 27 cytokines in CSF. The cytokine titers of the three groups were compared, and the correlation between the relevant cytokine titers and clinical parameters was investigated in the patients with sCJD. Levels of the two cytokines interleukin (IL)-1 receptor antagonist and IL-17 were significantly elevated in the patients with sCJD compared with those in the patients with noninflammatory neurological disorders: IL-17 levels in sCJD were approximately ten times higher than in the noninflammatory neurological disorders (mean, 35.46 vs. 3.45 pg/ml; P < 0.001) but comparable to that in encephalitis (mean, 32.16 pg/ml). In contrast, levels of classical proinflammatory cytokines such as IL-12(p70) and tumor necrosis factor-alpha were increased only in encephalitis. Although not significant, IL-17 titers tended to be higher in the patients with shorter disease duration before CSF sampling (r = -0.452; P = 0.104) and in those with lower CSF total protein concentrations (r = -0.473; P = 0.086). IL-17 is significantly increased in CSF in sCJD, which can be an early event in the pathogenesis of sCJD.
    Journal of Neuroinflammation 11/2013; 10(1):135. · 4.35 Impact Factor
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    ABSTRACT: We present a patient who initially developed diffuse painful muscle cramps who had elevated antibodies against voltage-gated potassium channel (VGKC) complex. Despite the initial improvement after immunomodulatory therapy, she later developed progressive atrophy and weakness of the limb muscles whose electrophysiological finding mimicked that in lower motor neuron disease. A potential causal relationship between anti-VGKC complex antibody-associated hyperexcitability and lower motor axon loss is discussed.
    Neurology and Clinical Neuroscience. 09/2013; 1(5).
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    ABSTRACT: Objective We carried out a retrospective study to define clinical features in a large series of patients with multifocal motor neuropathy (MMN) and to assess the diagnostic spectrum of MMN. Methods The study consisted of 46 patients with MMN between 2005 and 2009 from 19 major neuromuscular centers in Japan. The 2006 European Federation of Neurological Societies (EFNS)/Peripheral Nerve Society (PNS) criteria (hereafter, original criteria) and the efficacy of intravenous immunoglobulin (IVIg) therapy were taken into consideration in the diagnosis of MMN. The main parameters were clinical features and electrophysiological findings. The Japanese MMN Study Group designed a set of recommended criteria to reduce the frequency of underdiagnosis. Furthermore, we verified the diagnostic spectrum of MMN using both the original criteria and the recommended criteria. ResultsClinical features were similar to those of previous studies. A total of 25 of the 46 patients (54.3%) showed conduction block (CB); that is, nearly half of the patients did not satisfy the original criteria. The Japanese MMN Study Group included findings indicative of focal demyelination, namely, activity-dependent CB and asymmetric abnormality of F-waves in the electrophysiological test, in the recommended criteria. By doing so, the diagnostic sensitivity of the recommended criteria was increased by 17.4% compared with that of the original criteria. Conclusions The recommended criteria designed by the Japanese MMN Study Group showed higher diagnostic sensitivity than the original criteria, but no significant difference was found between them. A prospective study using the recommended criteria might reduce the frequency of underdiagnosis in patients with MMN.
    Clinical and Experimental Neuroimmunology. 08/2013; 4(2).
  • Journal of neurology, neurosurgery, and psychiatry 05/2013; 84(10):1177-1180. · 4.87 Impact Factor
  • Naoko Matsui, Ai Miyashiro, Ryuji Kaji
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    ABSTRACT: We surveyed patients with multifocal motor neuropathy (MMN) in comparison with those with amyotrophic lateral sclerosis (ALS) in Japan. This retrospective study consisted of 47 patients with MMN and 1,051 patients with ALS from major neuromuscular centers in Japan from 2005 to 2009. The ratio of MMN to ALS patients (0-0.10) varied among the centers, but mostly converged to 0.05. The prevalence was estimated to be 0.3 cases for MMN and 6.63 cases for ALS per 100,000 persons. Twenty-seven of 47 patients (56.5%) showed conduction block (CB). Of the 45 patients who received intravenous immunoglobulin (MVg), 34(75.6 %) demonstrated clear clinical improvement.
    Nippon rinsho. Japanese journal of clinical medicine 05/2013; 71(5):861-4.
  • Neurology 09/2010; 75(12):1121. · 8.25 Impact Factor
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    ABSTRACT: The thymus has been implicated as a possible site of origin that triggers autoimmunity in myasthenia gravis (MG). Although several groups have suggested that the decrease in the number of regulatory T (Treg) cells contributes to the onset of MG, the exact role of Treg cells in MG remains unclear. To address this point, we examined the number and distribution of Treg cells in a large number of patients with MG. Immunohistofluorescence analysis of Foxp3 along with CD4 and CD8 was performed in thymic sections of MG (+) (n = 24) and MG (-) patients (n = 27). Circulating CD4(+)CD25(+) cells in the peripheral blood of patients with MG (n = 15) and age-matched healthy subjects (n = 15) were also analyzed. Foxp3(+)CD4(+)CD8(-) cells were predominantly found in the thymic medulla and their number declined with age. There was no significant difference in the number or the distribution of Foxp3(+)CD4(+)CD8(-) cells in the thymus between MG (+) and MG (-) patients. The number of circulating CD4(+)CD25(+) cells in the peripheral blood of patients with MG was not significantly altered compared to that in healthy subjects. The cellularity of Treg cells in the thymus and circulation is not diminished in patients with myasthenia gravis.
    Neurology 03/2010; 74(10):816-20. · 8.25 Impact Factor
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    ABSTRACT: We encountered two patients with acute pandysautonomia who subacutely exhibited extensive autonomic dysfunction after antecedent infections. Although these patients had been suffering from autonomic disturbance for several months, they both had a good clinical course after plasma exchange and intravenous immunoglobulin therapy. Thin-layer chromatography (TLC)-immunostaining did not demonstrate any antibodies against gangliosides, but immunoblot analysis showed antibodies against a neuroblastoma cell line, SH-SY5Y, in serum samples. Furthermore, ganglionic acetylcholine receptor autoantibodies were detected in one patient. These findings suggest that neuronal antibodies against the autonomic nervous system play an important role in the pathogenesis of acute pandysautonomia.
    Internal Medicine 01/2010; 49(1):73-7. · 0.97 Impact Factor
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    ABSTRACT: As the number of elderly patients with myasthenia gravis (MG) has recently increased in Europe and the USA, a retrospective survey of Japanese MG patients was conducted in a single neurological centre over several decades. The study consisted of 112 consecutive MG patients with onset of the disease from 1971 to 2006 from an area of approximately 0.8 million inhabitants in Japan. Patients were classified into three subgroups according to age at onset: young onset (39 years old), middle aged onset (40-59 years old) and elderly onset (60 years old). The trends in incidence rate and clinical features were examined: disease severity, seropositivity for antiacetylcholine receptor antibody, occurrence of other autoimmune diseases, occurrence of thymoma and therapeutic response. The onset adjusted age specific average annual incidence per 100,000 of the elderly onset MG patients increased 20-fold from 1981-1990 (0.06; 95% CI 0.00 to 0.36) to 2001-2006 (1.30; 95% CI 0.77 to 2.05). Clinical features of the elderly onset MG patients included low antiacetylcholine receptor antibody titres (mean 24.6 nmol/l), less frequent autoimmune overlaps (8.0%) and nearly no complete stable remission with or without thymectomy. The increasing incidence of elderly onset MG in Japanese patients similar to that reported in Caucasians has been confirmed. The clinical features suggest different immunological backgrounds between young onset and elderly onset MG patients, irrespective of the ethnic background.
    Journal of neurology, neurosurgery, and psychiatry 10/2009; 80(10):1168-71. · 4.87 Impact Factor
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    ABSTRACT: We report a possible case of progressive multifocal leukoencephalopathy (PML) that was attempted to evaluate the pathogenesis by a novel brain MRI techniques. A 72-year-old woman had developed subacute visual disturbance, right hemiparesis and sensory disturbance. Laboratory examinations revealed liver dysfunction and pancytopenia due to liver cirrhosis (type C) and preclinical status of multiple myeloma. Thus, this patient had these two underlying diseases, while anti-HIV antibody was negative. She was suspected with PML by detection of JCV-DNA in cerebrospinal fluid using with PCR. MRI showed multifocal T2-high signals in the bilateral parieto-occipital deep white matter, basal ganglia and right cerebellar hemisphere. No gadolinium enhancement was found. On FLAIR and diffusion weighted images (DWI), the lesion showed hyperintensity. The hyperintense areas on DWI showed various pattern on apparent diffusion coefficient (ADC) and fractional anisotropy (FA). In particular white matter changes, the course of FA reflected the clinical course more than ADC. Proton magnetic resonance spectroscopy (1H-MRS) in deep brain white matter showed ratios of reduced N-acetyl aspartate (NAA) and increased choline (Cho) to creatine. 1H-MRS by chemical shift imaging were undergone three times between 4 and 6 months after the onset. The change of these chemical markers correlated with her clinical course. We conclude that the approach of diffusion tensor imaging (DTI) and 1H-MRS are useful for evaluating neuropathologic observations and clinical course.
    Rinsho shinkeigaku = Clinical neurology 09/2006; 46(8):555-60.
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    ABSTRACT: We describe two cases of dermatomyositis (DM) with nervous system involvement. Polyneuropathy was observed in both patients, and cerebral vasculitis was suspected in one patient. Histological examination of biopsied specimens of skeletal muscles, skin and sural nerves revealed vasculitis. Furthermore, vascular endothelial growth factor (VEGF) and VEGF receptors (VEGFRs) were overexpressed in vasculitic lesions. Although, VEGF and VEGFRs were not detected in biopsied specimens of skeletal muscle from normal subjects, they were observed in one of two patients with DM who did not exhibit neuropathy. These findings suggest the possibility that VEGF overproduction is associated with development of vasculitis in some cases of DM complicated with peripheral neuropathy.
    Internal Medicine 01/2004; 42(12):1233-9. · 0.97 Impact Factor