Abstract:
BACKGROUND:Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare multisystem disorder associated with plasma cell dyscrasia. There is increasing evidence that high-dose chemotherapy with autologous peripheral blood stem cell transplantation (Auto-PBSCT) is an efficacious treatment. OBJECTIVE:To elucidate the extent and time course of neurologic improvement after Auto-PBSCT in patients with POEMS syndrome. METHODS:Clinical and electrophysiologic findings in nine patients were reviewed. The median follow-up period was 20 months (range, 8 to 49 months). Serum levels of vascular endothelial growth factor (VEGF) were measured by ELISA. RESULTS:Serum VEGF levels rapidly decreased a month after Auto-PBSCT. Within 3 months, neurologic improvement began, and all the patients showed substantial neurologic recovery during the next 3 months. Particularly, three initially chairbound patients regained ability to walk at 6 months. Nerve conduction studies showed significant increases in conduction velocities and amplitudes within 6 months of treatment. At the end of follow-up periods, neuropathy was still improving, and no patients had recurrence of symptoms. CONCLUSION:Autologous peripheral blood stem cell transplantation results in obvious neurologic improvement within 6 months, presumably by extensive axonal regeneration and remyelination. This therapy could be considered as a first line treatment for patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome with younger onset even if they are tetraplegic.
journal_name
Neurologyjournal_title
Neurologyauthors
Kuwabara S,Misawa S,Kanai K,Suzuki Y,Kikkawa Y,Sawai S,Hattori T,Nishimura M,Nakaseko Cdoi
10.1212/01.wnl.0000323811.42080.a4subject
Has Abstractpub_date
2008-11-18 00:00:00pages
1691-5issue
21eissn
0028-3878issn
1526-632Xpii
01.wnl.0000323811.42080.a4journal_volume
71pub_type
杂志文章相关文献
NEUROLOGY文献大全abstract::A right-handed woman with independent left- and right-sided temporal lobe discharges was studied by continuous EEG and videotape monitoring for 3 weeks. Changes in affect were noted in the immediate and extended postictal periods and varied with the side of discharge. After a left-sided discharge, she became globally ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.35.9.1335
更新日期:1985-09-01 00:00:00
abstract:BACKGROUND:Sleep-disordered breathing (SDB) and respiratory failure (RF) are complications of acid maltase deficiency (AMD), a rare hereditary myopathy. OBJECTIVE:To define the relationship between lung and respiratory muscle function, to establish incidence and patterns of SDB, and to determine daytime predictors of ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.57.7.1290
更新日期:2001-10-09 00:00:00
abstract::CSF lymphocyte subpopulations of eight patients with neurosarcoidosis were examined. CSF or CT was abnormal in all. The CSF T4/T8 (helper/suppressor) ratio was elevated at 6.8 and 7.6 in two patients; in one, there were only CSF T4 cells. The ratio was normal in five patients. ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.37.5.878
更新日期:1987-05-01 00:00:00
abstract:OBJECTIVE:To evaluate trends in atrial fibrillation (AF) prevalence in acute ischemic stroke (AIS) and TIA in the United States. METHODS:We used the Nationwide Inpatient Sample to retrospectively compute weighted prevalence of AF in AIS (n = 4,355,140) and TIA (n = 1,816,459) patients admitted to US hospitals from 200...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000003321
更新日期:2016-11-08 00:00:00
abstract::We evaluated the accuracy and interobserver variability of selected ictal and postictal behavioral changes. Three observers, blinded to clinical history, EEG, and side of surgical resection, analyzed videotapes of 166 seizures in 38 patients, looking for lateralizing signs. Twenty-seven patients with temporal lobe res...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.43.12.2519
更新日期:1993-12-01 00:00:00
abstract::We performed a case-control study to investigate the role of recent infection as stroke risk factor and to identify pathogenetic pathways linking infection and stroke. We examined 166 consecutive patients with acute cerebrovascular ischemia and 166 patients hospitalized for nonvascular and noninflammatory neurologic d...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.50.1.196
更新日期:1998-01-01 00:00:00
abstract:OBJECTIVE:To study the impact of negative expectation related to receiving a placebo (the "lessebo effect") on efficacy outcome measures of symptomatic treatments in Parkinson disease (PD). METHODS:We conducted meta-analyses of double-blind randomized controlled trials (RCTs) of dopamine agonists in PD and compared th...
journal_title:Neurology
pub_type: 杂志文章,meta分析
doi:10.1212/WNL.0000000000000340
更新日期:2014-04-22 00:00:00
abstract::The authors retrospectively reviewed the efficacy of mycophenolate mofetil (MMF; mean dose, 2 g/day given for an average of 14 months) in 21 patients with immune demyelinating polyneuropathy. There were no significant differences in the median strength, sensory, or Rankin scores before and after treatment. In patients...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/01.wnl.0000134676.05850.c0
更新日期:2004-08-24 00:00:00
abstract:OBJECTIVES:To assess the association of established multiple sclerosis (MS) risk variants in 3,254 African Americans (1,162 cases and 2,092 controls). METHODS:Human leukocyte antigen (HLA)-DRB1, HLA-DQB1, and HLA-A alleles were typed by molecular techniques. Single nucleotide polymorphism (SNP) genotyping was conducte...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e31829bfe2f
更新日期:2013-07-16 00:00:00
abstract::Mitoxantrone is the first drug approved for the treatment of secondary progressive multiple sclerosis (SPMS) in the United States. This assessment considers use of mitoxantrone in the treatment of MS. Mitoxantrone probably reduces the clinical attack rate and reduces attack-related MRI outcomes in patients with relaps...
journal_title:Neurology
pub_type: 指南,杂志文章,实务指引,评审
doi:10.1212/01.wnl.0000095425.84407.39
更新日期:2003-11-25 00:00:00
abstract::Methionine-enkephalin, substance P, and homovanillic acid concentrations were measured in the CSF of subjects not affected by neurologic disorders (group 1), and in parkinsonian patients who had a slight or moderate (group 2) or severe (group 3) disability. Homovanillic acid and substance P concentrations in the CSF o...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.34.4.516
更新日期:1984-04-01 00:00:00
abstract::A 25-year-old man with a long history of temporal lobe epilepsy developed right occipital lobe seizures and a progressive right homonymous hemianopia. MRI showed diffuse enhancement of the left temporoparieto-occipital white matter and cortical thickening of the left medial temporal lobe. The resected temporal lobe re...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.52.3.654
更新日期:1999-02-01 00:00:00
abstract::Effective diagnosis and treatment of cytomegalovirus infection of the nervous system in AIDS patients has been limited by a lack of sensitive diagnostic measures. Retrospective series suggest a poor prognosis for cytomegalovirus encephalitis with rapid mortality. Polymerase chain reaction amplification of cytomegalovi...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.46.2.444
更新日期:1996-02-01 00:00:00
abstract::We examined measures of lead dose that reflect intensity of exposure and cumulative exposure for differential association with neuropsychological functional domains and with neuropsychological performance by age. Eighty active lead smelter workers assessed for verbal memory and visuomotoric skills had a mean (range) a...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.48.3.639
更新日期:1997-03-01 00:00:00
abstract:OBJECTIVE:To report the CNS syndromes of patients ≥60 years of age with antibodies against neuronal surface antigens but no evidence of brain MRI and CSF inflammatory changes. METHODS:This was a retrospective clinical analysis of patients with antibodies against neuronal surface antigens who fulfilled 3 criteria: age ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000004541
更新日期:2017-10-03 00:00:00
abstract::An emerging issue in stroke prevention and stroke treatment is that therapies shown to be effective in clinical trials are not being used or are being used suboptimally. One of many explanations relates to cost, ranging from concerns about inappropriate use of societal resources to more immediate concerns of organizat...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/wnl.51.3_suppl_3.s31
更新日期:1998-09-01 00:00:00
abstract:BACKGROUND:Fragile X-associated tremor/ataxia syndrome (FXTAS) is a recently described, underrecognized neurodegenerative disorder of aging fragile X mental retardation 1 (FMR1) premutation carriers, particularly men. Core motor features are action tremor, gait ataxia, and parkinsonism. Carriers have expanded CGG repea...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000281692.98200.f5
更新日期:2008-04-15 00:00:00
abstract::The electronic medical record (EMR) is changing the landscape of medical practice in the modern age. Increasing emphasis on quality metric reporting, data-driven documentation, and timely coding and billing are pressuring institutions across the country to adopt the latest EMR technology. The impact of these systems o...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/WNL.0000000000000819
更新日期:2014-09-23 00:00:00
abstract::We used riboflavin to treat a patient with lipid myopathy, reduced exercise capacity, intolerance to fasting, and reduced concentrations of carnitine in muscle and serum. Although carnitine concentrations did not change, exercise capacity doubled, and response to fasting improved. Muscle enzyme assay showed that palmi...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.31.12.1557
更新日期:1981-12-01 00:00:00
abstract:OBJECTIVE:Guillain-Barré syndrome (GBS) is characterized by nerve infiltration of leukocytes and autoantibodies of the immunoglobulin G (IgG) isotype directed against nerve constituents. Leukocyte receptors for IgG (FcgammaR) constitute an important link between the humoral and cellular parts of the immune system and c...
journal_title:Neurology
pub_type: 临床试验,杂志文章
doi:10.1212/wnl.54.8.1661
更新日期:2000-04-25 00:00:00
abstract::Study of diagnostic practice is necessary to optimize neurologists' clinical performance and ensure patient safety. To our knowledge, this report is the first set of systematic observations of diagnostic practices of community neurologists in their clinics. The study consisted of six 2-week periods of in situ observat...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3182a840c7
更新日期:2013-10-15 00:00:00
abstract:BACKGROUND:The major histocompatibility complex (MHC) has been consistently associated with susceptibility to MS and the course of several other human autoimmune diseases. A putative association between the course and severity of MS and the MHC remains controversial. METHODS:DR and DQ genotyping by either restriction ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.51.3.742
更新日期:1998-09-01 00:00:00
abstract:OBJECTIVES:Assuming the involvement of homocysteine in a generalized small-vessel disease, we investigated the association of homocysteine levels with progression of white matter lesions, lacunar infarcts, and kidney disease. METHODS:Within the SMART-MR (Second Manifestations of ARTerial disease-Magnetic Resonance) St...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000000168
更新日期:2014-03-04 00:00:00
abstract:OBJECTIVE:To validate, examine the internal validity, and adapt to children the electronic version of the composite cerebellar functional severity (CCFS) score. METHODS:In this multicenter study, we compared the validated manual device with the new electronic version (n = 46) and analyzed its kinetics in 146 patients ...
journal_title:Neurology
pub_type: 杂志文章,多中心研究
doi:10.1212/WNL.0000000000001403
更新日期:2015-03-24 00:00:00
abstract:OBJECTIVE:To verify the previously reported association between long-term use of β2-adrenoreceptor (β2AR) agonist and antagonist with reduced and increased risk of Parkinson disease (PD), respectively. METHODS:We obtained odds ratios (ORs) associating time of β2AR agonist and antagonist use with PD risk in nationwide ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000007694
更新日期:2019-07-09 00:00:00
abstract::In 10 patients with chronic autonomic failure, the sympathetic perivascular nerve plexuses from quadriceps muscle biopsies were studied by catecholamine fluorescence and electronmicroscopy. There was almost complete absence of catecholamine fluorescence and fewer than normal numbers of small granular (noradrenergic) v...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.31.12.1501
更新日期:1981-12-01 00:00:00
abstract::Several drugs that inhibit the effects of serotonin may reduce or prevent experimental CNS ischemic damage, but these drugs are not approved for human use in the United States. Administration of cyproheptadine (which is available for clinical use) 15 minutes before or 5 minutes after the onset increased the duration o...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.35.4.584
更新日期:1985-04-01 00:00:00
abstract:BACKGROUND:Although the APOE epsilon 4 allele increases the risk of developing AD, the effects of the epsilon 4 allele on brain atrophy in clinical AD patients are controversial. OBJECTIVE:To investigate a possible relationship between the genetic variants of APOE and brain atrophy in patients with AD. METHODS:Using ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.57.8.1461
更新日期:2001-10-23 00:00:00
abstract:BACKGROUND:The topodiagnostic value and specificity of nystagmus in patients with mesencephalic lesions and its relation to tonic torsional deficits and vertical saccade deficits is controversial and anecdotal. METHODS:The authors examined 11 patients with vascular MRI-identified mesencephalic lesions and clinical evi...
journal_title:Neurology
pub_type: 临床试验,杂志文章
doi:10.1212/01.wnl.0000038387.90128.8d
更新日期:2002-12-24 00:00:00
abstract::A 57-year-old chronically immunosuppressed woman with systemic lupus erythematosus developed fulminant meningoencephalitis due to Acanthamoeba castellanii. Amebic trophozoites were also found in the lungs, suggesting a primary pulmonary focus of infection. This case illustrates that Acanthamoeba can cause a fulminant,...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.31.2.174
更新日期:1981-02-01 00:00:00