Treatment and outcome of myasthenia gravis: retrospective multi-center analysis of 470 Japanese patients, 1999-2000.

Abstract:

:To clarify the current status of treatments and outcomes of patients with myasthenia gravis (MG) in Japan, a total of 470 patients (164 men and 306 women; mean age 41 years) were recruited from 19 Japanese tertiary medical centers in 1999-2000. Thymectomy was performed in 319 (68%) of the patients. Patients who received thymectomy were younger (p = 0.01) and had more severe disabilities (p < 0.01) than patients without thymectomy. Irrespective of receiving thymectomy, most of the patients were administered corticosteroids (64%), other immunosuppressive agents (10%), or cholinesterase inhibitors (86%). Of 395 patients followed up for more than 12 months after treatment (mean 8.0 years), 30% (34% of thymectomized and 21% of non-thymectomized patients) were in remission (no symptoms with/without medication), 34% had only ocular symptoms, and the remaining 35% still had weakness of bulbar or limb muscles at the end of follow-up. The prognosis of MG in Japan was generally favorable, but despite the frequent use of thymectomy and immunosuppressive treatments, approximately one-third of patients still had generalized weakness. More effective or intensive treatments are required to improve the prognosis.

journal_name

J Neurol Sci

authors

Kawaguchi N,Kuwabara S,Nemoto Y,Fukutake T,Satomura Y,Arimura K,Osame M,Hattori T,Study Group for Myasthenia Gravis in Japan.

doi

10.1016/j.jns.2003.09.016

subject

Has Abstract

pub_date

2004-09-15 00:00:00

pages

43-7

issue

1-2

eissn

0022-510X

issn

1878-5883

pii

S0022510X04001819

journal_volume

224

pub_type

杂志文章,多中心研究
  • Pitfalls in the evaluation of isometric strength (TQNE) data in ALS.

    abstract::The phase of rapid reduction of isometric strength in single muscle groups was identified and its slope was calculated in patients studied with the TQNE method. This parameter was studied in the extremities and in respiration in 4 ALS patients and 4 PMA (progressive spinal muscle atrophy) patients. The slopes of the s...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(96)00121-9

    authors: Conradi S,Ronnevi LO

    更新日期:1996-08-01 00:00:00

  • Absence of orbito-ocular signs in dural carotid-cavernous sinus fistula with a prominent anterior venous drainage.

    abstract::A 55-year-old woman developed an intractable right orbitofrontal headache. The symptoms subsided spontaneously 2 months after onset, but diplopia due to right abducens nerve palsy had occurred, and gradually worsened. Orbito-ocular signs were never observed throughout the clinical course. Brain MRI and MR angiography ...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2005.03.037

    authors: Ikeda K,Deguchi K,Tsukaguchi M,Sasaki I,Shimamura M,Urai Y,Touge T,Kawanishi M,Takeuchi H,Kuriyama S

    更新日期:2005-09-15 00:00:00

  • New pathological findings in amyotrophic lateral sclerosis.

    abstract::There have been recent developments in the pathology of sporadic ALS. A new filamentous neuronal inclusion body in ALS detected by immunohistochemical localisation of the protein ubiquitin has been characterised at the light microscopic and ultrastructural level and appears specific for the disease. The molecular comp...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章,评审

    doi:10.1016/0022-510x(94)90175-9

    authors: Lowe J

    更新日期:1994-07-01 00:00:00

  • Experimental ischemic myopathy. Effects of aortic ligation and serotonin.

    abstract::Support for the hypothesis that biogenic amines are involved in the production of muscle diseases comes from a report that rat muscle is damaged by combining distal aortic ligation with serotonin injection. Our studies explore the role of serotonin in the production of the myopathic changes in the aortic ligation-sero...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(79)90005-4

    authors: Kelts KA,Kaiser KK

    更新日期:1979-01-01 00:00:00

  • Frontal-lobe syndrome and psychosis after damage to the brainstem dopaminergic nuclei.

    abstract::A patient developed frontal-lobe syndrome and psychotic symptoms after infarction in the pontomesencephalic junction. Stereotaxic lesion localization on magnetic resonance imaging and statistical analyses of regional cerebral blood flow (rCBF) disclosed an involvement of the rostral brainstem dopaminergic nuclei and h...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2007.04.021

    authors: Nishio Y,Ishii K,Kazui H,Hosokai Y,Mori E

    更新日期:2007-09-15 00:00:00

  • Paraneoplastic neurological syndromes in lung cancer patients with or without onconeural antibodies.

    abstract:BACKGROUND:Paraneoplastic neurological syndromes (PNS) are poorly described in patients without onconeural antibodies and in patients with non-small cell lung cancer (NSCLC). We compared the clinical characteristics of PNS in lung cancer patients with and without onconeural antibodies. METHODS:Medical records from pat...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2014.10.040

    authors: Raspotnig M,Vedeler C,Storstein A

    更新日期:2015-01-15 00:00:00

  • IL-2 and IL-6 secretion in dementia: correlation with type and severity of disease.

    abstract::The production of interleukin-2 (IL-2) and interleukin-6 (IL-6) by peripheral blood mononuclear cells (MNC) was assessed in patients with Alzheimer's disease (AD) who were subdivided into two groups--mild and moderately-severe--according to the severity of the disease, probable vascular dementia (VaD) patients and eld...

    journal_title:Journal of the neurological sciences

    pub_type: 临床试验,杂志文章

    doi:10.1016/0022-510x(95)00016-u

    authors: Huberman M,Sredni B,Stern L,Kott E,Shalit F

    更新日期:1995-06-01 00:00:00

  • Role of Rho-mediated ROCK-Semaphorin3A signaling pathway in the pathogenesis of Parkinson's disease in a mouse model.

    abstract:OBJECTIVE:The present study aims to elucidate the role of Rho-mediated ROCK-Semaphorin3A signaling pathway in the pathogenesis of Parkinson's disease (PD) in a mouse model. METHODS:One-hundred twelve eight-week male C57BL/6 mice were selected. The mouse model of PD was constructed by intraperitoneal injection of MPTP....

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2016.08.061

    authors: Qi L,Tang YG,Wang L,He W,Pan HH,Nie RR,Can Y

    更新日期:2016-11-15 00:00:00

  • Yes/yes head tremor without appendicular tremor after bilateral cerebellar infarction.

    abstract::We report a 67-year-old man who developed yes/yes head tremor without appendicular tremor six weeks after right occipital and bilateral cerebellar infarction. The tremor was resting-postural. Its activity increased with excitement, decreased either after ethanol, lateroflexion or rest and stopped at sleep. Four-vessel...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:

    authors: Finsterer J,Muellbacher W,Mamoli B

    更新日期:1996-08-01 00:00:00

  • Mood disturbances in motor neurone disease.

    abstract::This study explores the severity of depression and anxiety experienced by patients suffering from motor neurone disease and seeks to link this to illness progression, recent life events and personality factors. The 18 patients who participated in the study were assessed for the presence of psychiatric symptoms using t...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(98)00203-2

    authors: Moore MJ,Moore PB,Shaw PJ

    更新日期:1998-10-01 00:00:00

  • Memantine abrogates neurological deficits, but not CNS inflammation, in Lewis rat experimental autoimmune encephalomyelitis.

    abstract::Memantine, a clinically employed drug with N-methyl-D-aspartate (NMDA) receptor antagonistic effects, dose-dependently ameliorates neurological deficits in Lewis rat experimental autoimmune encephalomyelitis (EAE). Interestingly, this therapeutic effect was not due to dampened CNS inflammation, as assessed by immunohi...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(95)00339-4

    authors: Wallström E,Diener P,Ljungdahl A,Khademi M,Nilsson CG,Olsson T

    更新日期:1996-05-01 00:00:00

  • Interictal cortical hyperexcitability in migraine patients demonstrated with transcranial magnetic stimulation.

    abstract::Cortical excitability to magnetic stimulation was investigated interictally in 10 patients with migraine with aura, 10 with migraine without aura and in 10 healthy volunteers. Thresholds, latencies and amplitudes of the magnetic-evoked potentials (MEPs) were measured from threshold to 100% stimulus intensity in 10% st...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(96)00044-5

    authors: van der Kamp W,Maassen VanDenBrink A,Ferrari MD,van Dijk JG

    更新日期:1996-07-01 00:00:00

  • On the development of CNS lesions in natural canine distemper encephalomyelitis.

    abstract::Acute lesions within spinal cord white matter have been studied by light and electron microscopy in 3 dogs suffering from the acute form of canine distemper encephalomyelitis (CDE). Prominent features of these lesions were viral inclusions, giant cell formation, cellular degeneration, myelin breakdown and phagocytic a...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(76)90251-3

    authors: Raine CS

    更新日期:1976-11-01 00:00:00

  • Cognitive conditions of pathologically confirmed dementia with Lewy bodies and Parkinson's disease with dementia.

    abstract::The relationship between dementia with Lewy bodies (DLB) and Parkinson's disease with dementia (PDD) has been insufficiently described, and it is still problematic. Twenty-nine cases of DLB and 10 cases of PDD were investigated in the present study. DLB cases disclosed a significantly older disease onset and shorter d...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(03)00220-x

    authors: Horimoto Y,Matsumoto M,Nakazawa H,Yuasa H,Morishita M,Akatsu H,Ikari H,Yamamoto T,Kosaka K

    更新日期:2003-12-15 00:00:00

  • Anti-beta 2-glycoprotein I antibodies bind to central nervous system.

    abstract::Anti-beta 2-GPI antibodies (a beta 2-GPI) were found in serum from patients with anti-phospholipid syndrome (APS) and/or systemic lupus erythematosus (SLE). Since a beta 2-GPI are often found in patients with anti-cardiolipin antibodies (aCL), their role in thrombosis as well as other central nervous system (CNS) mani...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(98)00027-6

    authors: Caronti B,Pittoni V,Palladini G,Valesini G

    更新日期:1998-04-01 00:00:00

  • Abnormalities of visual function in hereditary motor and sensory neuropathy.

    abstract::Visual function was studied in a group of 15 patients with hereditary motor and sensory neuropathy (HMSN). Psychophysical measures of luminance and chromatic threshold and temporal contrast sensitivity were undertaken, together with visual evoked potentials (VEPs), visual fields and clinical neuro-ophthalmological exa...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(93)90296-b

    authors: Honan WP,Heron JR,Foster DH,Edgar GK,Scase MO

    更新日期:1993-02-01 00:00:00

  • Properties of muscles from chickens with inherited muscular dystrophy.

    abstract::Inherited muscular dystrophy of the chicken is an abnormality affecting the normal development and function of fast-twitch skeletal muscles. Several different strains of dystrophic chickens have been developed by selection for high lipid content in the pectoralis muscle and early onset of the disorder or by outcrossin...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(80)90073-8

    authors: Randall WR,Wilson BW

    更新日期:1980-05-01 00:00:00

  • Correlation between regional brain volume and olfactory function in very mild amnestic patients.

    abstract:BACKGROUND & AIMS:We aimed to determine neural correlates of olfactory detection and identification and analyze associations between cognitive function and olfactory identification or detection in very mild amnestic patients. METHODS:We recruited 70 patients with chief complaints of memory impairment diagnosed as amne...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2020.116686

    authors: Kashibayashi T,Takahashi R,Fujita J,Kamimura N,Okutani F,Kazui H

    更新日期:2020-04-15 00:00:00

  • The psychological impact of MND on patients and carers.

    abstract::Nineteen patients with Motor Neurone Disease (MND) who had been living with their partners for at least two years prior to the onset of their illness, together with their partners, completed self-report questionnaires to investigate the impact of MND on both patients and carers. Physical disability and impact of the i...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(98)00209-3

    authors: Goldstein LH,Adamson M,Jeffrey L,Down K,Barby T,Wilson C,Leigh PN

    更新日期:1998-10-01 00:00:00

  • The characteristics of camptocormia in patients with Parkinson's disease: A large cross-sectional multicenter study in Japan.

    abstract:PURPOSE:The goal of the present study was to clarify the clinical characteristics and laboratory results of parkinsonian symptoms among patients with and without camptocormia. METHODS:Seventy-eight Parkinson's disease (PD) patients with camptocormia and 78 PD patients without camptocormia underwent a neurological exam...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章,多中心研究

    doi:10.1016/j.jns.2015.09.015

    authors: Nakane S,Yoshioka M,Oda N,Tani T,Chida K,Suzuki M,Funakawa I,Inukai A,Hasegawa K,Kuroda K,Mizoguchi K,Shioya K,Sonoda Y,Matsuo H

    更新日期:2015-11-15 00:00:00

  • Class II antigen expression in peripheral neuropathies.

    abstract::The expression of class II antigen was studied in sural nerve biopsies from patients with peripheral neuropathies. These included patients with chronic demyelinating polyradiculoneuropathy (CIDP), non-immune mediated neuropathies of diverse etiologies and controls without evidence of neuropathy. The major finding in C...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(91)90065-f

    authors: Mitchell GW,Williams GS,Bosch EP,Hart MN

    更新日期:1991-04-01 00:00:00

  • A case of 3243A>G mutation in mtDNA presenting as apparently idiopathic hyperCKemia.

    abstract::The 3243A>G mutation of mtDNA usually is associated with MELAS syndrome. Here we report a patient with the 3243A>G mutation presenting only recurrent muscle fatigue and elevated levels of serum creatine kinase (CK). The mother of the proband was referred to us for type 2 diabetes mellitus, muscle pain and sensorineura...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2014.01.010

    authors: Rubegni A,Cardaioli E,Chini E,Da Pozzo P,Battisti C,Malandrini A,Federico A

    更新日期:2014-03-15 00:00:00

  • Stroke due to septic embolism resulting from Aspergillus aortitis in an immunocompetent patient.

    abstract:INTRODUCTION:Cerebral infarction secondary to Aspergillus arteritis or septic embolism is an exceptional finding. We present a case of multiple systemic embolism and cerebral infarction resulting from Aspergillus aortitis in an immunocompetent patient. PATIENT:A 65-year-old male with hypertension, hyperglycaemia and m...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2009.04.037

    authors: Abenza-Abildua MJ,Fuentes-Gimeno B,Morales-Bastos C,Aguilar-Amat MJ,Martinez-Sanchez P,Diez-Tejedor E

    更新日期:2009-09-15 00:00:00

  • Transient global amnesia: Only in already disrupted neuronal integrity of memory network?

    abstract::Transient global amnesia is a well-described clinical syndrome; however, the pathophysiology is perplexing. Structural imaging indicates that punctuate hippocampal lesions are the representative pathophysiology, although functional neuroimaging studies have reported that the various regions comprising the episodic mem...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2016.05.035

    authors: Moon Y,Oh J,Kwon KJ,Han SH

    更新日期:2016-09-15 00:00:00

  • Th2 shift in mononeuritis multiplex and increase of Th2 cells in chronic inflammatory demyelinating polyneuropathy: an intracellular cytokine analysis.

    abstract::To elucidate the T helper 1 (Th1)/T helper 2 (Th2) balance in various inflammatory neuropathies, we measured the ratio of intracellular interferon-gamma (IFN-gamma)-positive to IL-4-positive cells (intracellular IFN-gamma/IL-4 ratio) by flow cytometry in peripheral blood CD4(+) T cells of 14 patients with mononeuritis...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(01)00603-7

    authors: Horiuchi I,Ochi H,Murai H,Osoegawa M,Minohara M,Furuya H,Kira J

    更新日期:2001-12-15 00:00:00

  • An epidemic of optic neuropathy in Tanzania: characterization of the visual disorder and associated peripheral neuropathy.

    abstract::An epidemic of subacute bilateral visual failure has affected large numbers of teenagers and young adult Africans in coastal Tanzania since 1988. Previous work had indicated that many patients had sensory symptoms, but the nature of the neurological involvement was uncertain. The primary objective of this study was to...

    journal_title:Journal of the neurological sciences

    pub_type: 临床试验,杂志文章

    doi:10.1016/s0022-510x(96)00162-1

    authors: Plant GT,Mtanda AT,Arden GB,Johnson GJ

    更新日期:1997-02-12 00:00:00

  • Muscular dystrophy in the mdx mouse: histopathology of the soleus and extensor digitorum longus muscles.

    abstract::We have used light microscopic histomorphometry to quantify the developmental histopathological changes induced by muscular dystrophy in the soleus and extensor digitorum longus (EDL) muscles of the mdx mouse. We find that this X-linked disease exhibits early fibre necrosis with foci of invasive cells, clustering of a...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(87)90219-x

    authors: Carnwath JW,Shotton DM

    更新日期:1987-08-01 00:00:00

  • [Ultrastructural study of a chordoma].

    abstract::An ultrastructural study of a case of sacral chordoma is reported. The cell type was monomorphic. The epithelial nature and the secretory function of the tumour cells was obvious in view of their content of desmosomes and the presence of mucopolysaccharide in the ergastoplasm and in the extracellular space. Because of...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(75)90157-4

    authors: Tripler MF,Hassoun J,Toga M

    更新日期:1975-07-01 00:00:00

  • Basal ganglia germinoma: diagnostic value of MR spectroscopy and (11)C-methionine positron emission tomography.

    abstract::We herein report a 12-year-old girl with a basal ganglia germinoma who presented with right-sided hemiparesis after a minor head trauma. Magnetic resonance (MR) imaging revealed a minimally enhanced lesion involving the left putamen, thalamus, and corona radiata. The lesion showed low-signal intensity on T1-, and high...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2008.02.006

    authors: Fujii Y,Saito Y,Ogawa T,Fujii S,Kamitani H,Kondo S,Horie Y,Togawa M,Senda M,Maegaki Y,Ohno K

    更新日期:2008-07-15 00:00:00

  • Somatosensory evoked potentials in progressive supranuclear palsy.

    abstract::We report median and digital nerve somatosensory evoked potentials (SEPs) in 14 patients with probable progressive supranuclear palsy (PSP) along with transcortical long-loop reflexes, motor evoked potentials, and auditory startle responses. Enlarged cortical responses were found in 14 median and 13 digital nerve SEP ...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(00)00383-x

    authors: Kofler M,Müller J,Reggiani L,Wenning GK

    更新日期:2000-10-01 00:00:00