Abstract：
OBJECTIVES:There is insufficient data on the efficacy of intravenous immunoglobulins (IVIg) as maintenance treatment in myasthenia gravis (MG). We therefore examined response to maintenance IVIg therapy in a cohort of MG patients. METHODS:We reviewed all MG patient files treated with IVIg in our neuro-immunology clinic from 1/1995 to 9/2012. Patients treated with maintenance IVIg for a minimum of one year were separately analyzed. Disease severity was evaluated according to the Myasthenia Gravis Foundation of America clinical classification. RESULTS:IVIg was considered for maintenance therapy in 52 MG patients who had not responded to pyridostigmine, prednisone, azathioprine or combinations of these drugs. Fifteen patients did not improve with initial IVIg while thirty seven patients had a beneficial response and were treated with maintenance IVIg for an average of 5.9 years (range 1-17 years). Twenty two (59%) patients were female with an average age onset of disease 44.8 years. Thirty three were seropositive for acetylcholine receptor antibody and 13 had previous thymectomy. Twenty three and 14 patients achieved mild or moderate improvement respectively in disease activity while on IVIg therapy but none achieved full remission. Beneficial response was associated with older age, bulbar presentation, seropositivity and a higher antibody titer and less with ocular presentation. IVIg enabled reduction of other treatments including pyridostigmine, prednisone and azathioprine. CONCLUSION:In this retrospective study on a relative small cohort of MG patients maintenance IVIg therapy was successful in reducing symptoms of MG but seems to be ineffective in inducing full remission or reducing disease activity. IVIg should be regarded only as symptomatic therapy in MG.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Hellmann MA,Mosberg-Galili R,Lotan I,Steiner Idoi
10.1016/j.jns.2013.10.043subject
Has Abstractpub_date
2014-03-15 00:00:00pages
39-42issue
1-2eissn
0022-510Xissn
1878-5883pii
S0022-510X(13)03005-0journal_volume
338pub_type
杂志文章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
更新日期：2001-12-15 00:00:00
abstract：BACKGROUND AND PURPOSE:Patent foramen ovale (PFO) can be diagnosed by contrast-enhanced transcranial Doppler (cTCD), but no definitive criteria exist to correlate the results obtained from cTCD with diagnoses of PFO obtained by contrast-enhanced transesophageal echocardiography (cTEE). The aim of the present study was ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.02.003
更新日期：2010-06-15 00:00:00
abstract：:Ophthalmological examination and visual evoked responses (VERs) were repeated at 6-120 (mean 46) months after the first attack of acute optic neuritis in 80 patients who had abnormal VERs in 98 symptomatic eyes at the initial examination. The wide field VER returned to within the normal range in 19/98 (19%) symptomati...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(86)90075-4
更新日期：1986-10-01 00:00:00
abstract：:We measured the levels of some biological metals: copper (Cu), iron (Fe), magnesium (Mg), manganese (Mn), and zinc (Zn) in the cerebrospinal fluid (CSF) in patients with neurodegenerative diseases (52 patients with amyotrophic lateral sclerosis (ALS)), 21 patients with Alzheimer's disease (AD), and 20 patients with Pa...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.01.003
更新日期：2011-04-15 00:00:00
abstract：BACKGROUND:Myasthenia gravis (MG) is the autoimmune disorder in which the thymus is considered the pathogenic organ. Thymectomy (TE) is a therapeutic option for MG and often ameliorates clinical symptoms. METHODS:We evaluated clinical features and outcomes after TE in patients without thymoma and the influence of TE w...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.08.029
更新日期：2015-11-15 00:00:00
abstract：PURPOSE:To investigate the direct and indirect associations of sleep hygiene with daytime sleepiness, depressive symptoms, and quality of life (QoL), in newly diagnosed, untreated patients with mild obstructive sleep apnea (OSA). METHODS:Data were collected from adults with mild OSA. The Sleep Hygiene Index (SHI), Sle...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.10.017
更新日期：2015-12-15 00:00:00
abstract：:Contraction tension and kinetics of the peroneus longus muscle were studied in dogs with the Duchenne homologue, golden retriever muscular dystrophy (GRMD), in advance of evaluating localized therapies such as myoblast transplantation. Absolute and both muscle- and body-weight-corrected twitch tension in GRMD dogs wer...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(94)90210-0
更新日期：1994-05-01 00:00:00
abstract：:Cranial dystonia is normally considered as a pure movement disorder. Sensory symptoms have not received much attention, but we found ill-defined pain, discomfort, distortion of sensory modalities, 'phantom' kinetic or postural sensations in the orofacial areas subsequently involved by the dyskinesia in all of 11 conse...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(93)90318-s
更新日期：1993-06-01 00:00:00
abstract：OBJECTIVE:Despite the strong evidence for the efficacy of surgery to treat temporal lobe epilepsy, the number of such surgeries has plateaued in western countries. This study examined trends in the number of epilepsy cases diagnosed, number of surgeries performed, and certain characteristics of surgery patients in west...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2019.06.026
更新日期：2019-08-15 00:00:00
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
更新日期：2015-01-15 00:00:00
abstract：:The occurrence of clusters of Marchi-positive granules and of Marchi-positive myelinoid bodies at different postnatal developmental stages was examined lightmicroscopically in Vibratome sections from the cervical lateral funiculus of the cat, after perfusion-fixation with glutaraldehyde. The findings show that cluster...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(77)90093-4
更新日期：1977-10-01 00:00:00
abstract：:The predictive value of serum cortisol level on the prognosis in acute brain infarction of the carotid circulation territory was studied in 101 patients younger than 70 years. The levels of 7 a.m. and 7 p.m. serum cortisol were measured initially and at 1 week. All patients underwent a computed cerebral tomography (CT...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(93)90083-b
更新日期：1993-05-01 00:00:00
abstract：:As an inhibitor of the immune system and a longevity drug, rapamycin has been suggested as a treatment for Alzheimer's disease, although the underlying mechanisms remain to be clarified. To elucidate the mechanisms, we performed a high-throughput quantitative proteomics analysis and bioinformatics analysis of the chan...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2019.02.022
更新日期：2019-04-15 00:00:00
abstract：:Parkinson's disease (PD) is a common disorder of the central nervous system in the elderly. The pathogenesis of PD is a complex process, with genetics as an important contributing factor. This factor may stem from mitochondrial gene variations and mutations as well as from nuclear gene variations and mutations. More r...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2014.07.067
更新日期：2014-11-15 00:00:00
abstract：:A broad spectrum of immunologic parameters was investigated in 13 cases of myotonic muscular dystrophy (MyD), including those for cellular immunity which has attracted little attention in the past. One was a 46-year-old woman having both MyD and a thymoma. This association between MyD and thymoma is probably coinciden...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(80)90007-6
更新日期：1980-08-01 00:00:00
abstract：:Wernicke's encephalopathy is often undiagnosed, particularly in non-alcoholics. There are very few reports of non-alcoholic patients diagnosed with Korsakoff syndrome in the absence of a prior diagnosis of Wernicke's encephalopathy and no studies of diffusion tensor imaging in non-alcoholic Korsakoff syndrome. We repo...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.09.025
更新日期：2016-11-15 00:00:00
abstract：:This study indicates the impact of nicotine and pesticides (organochlorine and organophosphate insecticides used in agriculture) on neuronal α7-nicotinic acetylcholine receptor expression in brainstem regions receiving cholinergic projections in human perinatal life. An in-depth anatomopathological examination of the ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.11.014
更新日期：2015-01-15 00:00:00
abstract：:The enzyme-linked immunosorbent assay (ELISA) was used to determine levels of specific IgG antibodies against measles, rubella, vaccinia, corona (OC43) and mumps viruses in cerebrospinal fluid (CSF) and serum of 18 patients with clinically definite multiple sclerosis (MS), 8 patients with optic neuritis (ON), 27 patie...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(82)90031-4
更新日期：1982-12-01 00:00:00
abstract：OBJECTIVE:We carried out a meta-analysis focusing on the relationships between rs1137070 C>T and rs1799836 A>G polymorphisms in the MAO gene as a modifier of Parkinson disease (PD) susceptibility. METHOD:A literature search of the Cochrane Library Database, Web of Science, PubMed, CINAHL, EMBASE, and the Chinese Biome...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,meta分析
doi：10.1016/j.jns.2014.07.016
更新日期：2014-10-15 00:00:00
abstract：OBJECTIVE:Neurodegenerative diseases are often characterized by the presence of intracellular or extracellular protein aggregates in the central nervous system. Mutations of TARDBP gene have been shown to cause Amyotrophic Lateral Sclerosis and have been reported to present with clinical heterogeneity including parkins...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2018.04.043
更新日期：2018-07-15 00:00:00
abstract：BACKGROUND:Neurofibromatosis 1 (NF1) belongs to the autosomal dominant neurocutaneous disorders' group, which mainly includes NF1 and NF2, tuberous sclerosis, von Hippel-Lindau disease and Cerebral Cavernous Malformations (CCMs). NF1 has a major impact on the nervous system, eye, skin, bone or cardiovascular system. Ce...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.02.023
更新日期：2015-03-15 00:00:00
abstract：:Corticosteroids (Cs) are widely used for treatment of multiple sclerosis (MS) acute relapses because of the potent immunosuppressive and anti-inflammatory properties. As for patients with relapsing-remitting (RR) MS, short-term administrations of Cs markedly less severity of symptoms and promote faster recovery of cli...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2004.04.019
更新日期：2004-08-15 00:00:00
abstract：BACKGROUND:Natalizumab therapy reduces inflammation and degeneration of the CNS in relapsing-remitting multiple sclerosis (RRMS). In cerebrospinal fluid (CSF) the concentration of 24S-hydroxycholesterol (24OHC) reflect neurodegeneration, whereas 27-hydroxycholesterol (27OHC) is dependent on the integrity of the blood-b...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.08.1537
更新日期：2015-11-15 00:00:00
abstract：:The scalp distribution of the response to stimulation of the tibial nerve at the medial malleolus was systematically analysed. The somatosensory evoked potential (SEP) was recorded with electrodes placed in a transversal line over the ipsilateral and contralateral postcentral gyri and in a sagittal line over the longi...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(83)90038-2
更新日期：1983-05-01 00:00:00
abstract：:A 53-year old woman developed slowly progressive dysarthria, mild enlargement of the tongue and dysphagia since 1 year ago. All neurological differential diagnoses that could have explained the bulbar symptoms were excluded. The swallowing sequence was pathologic and immunoglobulins were markedly reduced. A bone marro...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(96)05326-9
更新日期：1997-04-15 00:00:00
abstract：:Hemichorea associated with carotid artery occlusive disease is extremely rare. It has been recently suggested that carotid artery stenosis should be considered in the differential diagnosis of chorea, even in the absence of a preceding stroke or transient ischemic attack. Although the pathophysiology of this condition...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.08.068
更新日期：2011-01-15 00:00:00
abstract：:An accumulation of SCA6 cases has been observed in the Chugoku area of Western Japan. In the Tottori prefecture, located in the northeastern part of the Chugoku district, we observed a cluster of SCA6 families within the eastern area, suggesting that there may be a founder in the Japanese SCA6 population. Genotyping w...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(01)00453-1
更新日期：2001-03-15 00:00:00
abstract：OBJECTIVE:To describe the neurological and neuroradiological features of acquired hemophagocytic lymphohistiocytosis (HLH) in adulthood by reporting a series of cases. METHODS:Ten consecutive patients who were diagnosed with HLH at Medstar Georgetown University Hospital and Walter Reed National Military Medical Center...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.07.017
更新日期：2015-10-15 00:00:00
abstract：:In Creutzfeldt-Jakob disease (CJD), there are prominent ultrastructural alterations of the plasma membrane, which contains many glycolipids and glycoproteins. Glycosidases can degrade glycolipids and glycoproteins. Gangliosides, a subset of glycolipids, are decreased in amount at the terminal stages of CJD, and CJD in...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(88)90178-5
更新日期：1988-03-01 00:00:00
abstract：:The localization of the olivo-cerebellar fibers in the inferior cerebellar peduncle was examined in a patient who showed severe degeneration of the left olivary nucleus due to an old hemorrhagic infarct in the contralateral cerebellum. Other precerebellar nuclei which send their cerebellipetal axons to the inferior ce...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(78)90139-9
更新日期：1978-10-01 00:00:00
