Abstract:
BACKGROUND:The best treatment for polyneuropathy associated with IgM monoclonal gammopathy (MGUS) is unknown. Oral cyclophosphamide combined with prednisone showed limited efficacy in a previous open label pilot study. We therefore performed a double-blind, randomized, placebo-controlled study of combined oral cyclophosphamide and prednisone in IgM MGUS polyneuropathy. METHODS:Thirty-five patients with progressive IgM MGUS polyneuropathy were included. After stratification for anti-MAG antibodies patients were randomized to oral cyclophosphamide 500 mg once daily for 4 days combined with oral prednisone 60 mg once daily for 5 days (treatment) (n = 16), or placebo (n = 19), repeated every 28 days for six times. Primary outcome was improvement of the Rivermead Mobility Index (RMI). Secondary outcomes were improvement of the modified Rankin scale, Medical Research Council and sensory sum scores, levels of M protein, EMG, and Short Form-36 scale after treatment. Patients were examined at 0, 6, 12, 18, and 24 months. RESULTS:After 6 months of treatment and at later follow-up, no difference in change of the RMI between the two groups was observed. Change of the Rankin scale was similar in both groups. Other outcome parameters showed more improvement in the treatment group: the MRC sum score improved more from 6 to 24 months after treatment; the sensory sum score improved more at 6 months; the SF 36 mean health change score and physical role score improved more; and the median nerve distal conduction (abductor pollicis brevis muscle) improved more in the treatment group. The most common adverse event was nausea. CONCLUSIONS:Compared with placebo treatment, this first double-blind randomized trial with cyclophosphamide and prednisone in IgM MGUS polyneuropathy showed no beneficial effect on the functional scales, but a beneficial effect on muscle strength and sensation was observed.
journal_name
Neurologyjournal_title
Neurologyauthors
Niermeijer JM,Eurelings M,van der Linden MW,Lokhorst HM,Franssen H,Fischer K,Teunissen LL,van den Berg LH,Schobben F,Wokke JH,Notermans NCdoi
10.1212/01.wnl.0000265055.28218.ccsubject
Has Abstractpub_date
2007-07-03 00:00:00pages
50-9issue
1eissn
0028-3878issn
1526-632Xpii
69/1/50journal_volume
69pub_type
杂志文章,随机对照试验相关文献
NEUROLOGY文献大全abstract:OBJECTIVE:To investigate whether vitamin D, smoking, and anti-Epstein-Barr virus (EBV) antibody concentrations predict long-term cognitive status and neuroaxonal injury in multiple sclerosis (MS). METHODS:This study was conducted among 278 patients with clinically isolated syndrome who participated in the clinical tri...
journal_title:Neurology
pub_type: 杂志文章,多中心研究
doi:10.1212/WNL.0000000000009371
更新日期:2020-05-05 00:00:00
abstract:INTRODUCTION:As clinical trials move toward earlier intervention, we sought to redefine the β-amyloid (Aβ)-PET threshold based on the lowest point in a baseline distribution that robustly predicts future Aβ accumulation and cognitive decline in 3 independent samples of clinically normal individuals. METHODS:Sequential...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000011214
更新日期:2021-01-26 00:00:00
abstract::Recent evidence suggests that the vasopressin analogue desglycinamide-arginine8-vasopressin (DGAVP) might specifically benefit mild brain trauma patients. We investigated the effect of intranasal DGAVP treatment in 32 patients who had sustained a mild head injury for 3 months in a double-blind, placebo-controlled, mat...
journal_title:Neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1212/wnl.43.1_part_1.103
更新日期:1993-01-01 00:00:00
abstract::We report a Japanese Becker muscular dystrophy (BMD) patient with occasional myalgia and cramps during normal activity that developed at the age of 28 months. His family history was negative for neuromuscular diseases. Muscle biopsy analyses, including dystrophin immunostaining, disclosed no clinically relevant findin...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.46.5.1347
更新日期:1996-05-01 00:00:00
abstract:OBJECTIVE:Pigmented orthochromatic leukodystrophy (POLD) and hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) are rare neurodegenerative disorders characterized by cerebral white matter abnormalities, myelin loss, and axonal swellings. The striking overlap of clinical and pathologic features of these...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e31828726a7
更新日期:2013-03-12 00:00:00
abstract::We assessed the severity and temporal profile of distant neuromuscular effects from a single dose (280 units) of botulinum neurotoxin injected into neck muscles for torticollis. We performed single-fiber EMG studies on the biceps brachii of six patients to measure jitter (20 pairs) and fiber density on the initial tre...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.38.11.1780
更新日期:1988-11-01 00:00:00
abstract::Although not approved by the US Food and Drug Administration for the treatment of status epilepticus (SE), valproic acid (VPA) is an emerging option for this purpose. The authors reviewed 63 patients (30 men) with SE treated with IV VPA (average dose, 31.5 mg/kg). Analysis of demographic, clinical, and treatment infor...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/01.WNL.0000149527.47600.5A
更新日期:2005-01-25 00:00:00
abstract:OBJECTIVE:Prospective memory (PM) describes the ability to fulfill previously planned intentions and is highly dependent on executive functions. Previous studies have shown deficits in executive functions in patients with juvenile myoclonic epilepsy (JME) and in their unaffected siblings. JME has a strong genetic predi...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e318202010a
更新日期:2010-12-14 00:00:00
abstract::Parenchymatous intracerebral hemorrhage (ICH) is a serious, infrequent complication of thrombolytic therapy for acute myocardial infarction. We studied the clinical and radiologic features, manner of presentation, associated factors, and temporal course in 23 patients with ICH associated with 150 mg or 100 mg recombin...
journal_title:Neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1212/wnl.45.4.649
更新日期:1995-04-01 00:00:00
abstract:OBJECTIVE:We sought cognitive event-related potential (ERP) biomarkers of disease progression and subsequent conversion to dementia in mild cognitive impairment (MCI). BACKGROUND:Two ERP components, the P600 and N400, are sensitive to abnormal episodic/declarative memory and semantic processing. When congruous categor...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000281689.28759.ab
更新日期:2008-05-06 00:00:00
abstract:OBJECTIVE:To clarify the genetic, clinicopathologic, and neuroimaging characteristics of patients with hereditary diffuse leukoencephalopathy with spheroids (HDLS) with the colony stimulating factor 1 receptor (CSF-1R) mutation. METHODS:We performed molecular genetic analysis of CSF-1R in patients with HDLS. Detailed ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000000046
更新日期:2014-01-14 00:00:00
abstract::The levels of plasma free carnitine and ketone bodies have been found to fluctuate inversely in fasting individuals without muscle disease. Circulating short-chain acyl-carnitines paralleled beta-hydroxybutyrate levels. A patient with lipid storage myopathy and muscle carnitine deficiency, and his two daughters, devel...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.29.6.780
更新日期:1979-06-01 00:00:00
abstract::We describe three patients with acute chiasmal visual field loss caused by hemorrhage within an intrachiasmal arteriovenous malformation. One patient had five episodes; vision improved three times without treatment and twice after surgical decompression. Surgical intervention restored vision in two of the three patien...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.34.8.1007
更新日期:1984-08-01 00:00:00
abstract::The authors recruited 19 nonambulant patients with Guillain-Barré syndrome into a pilot, double-blind, randomized, placebo-controlled safety trial of interferon beta 1a (IFN[beta]-1a) (Rebif). Participants received IFN[beta]-1a or placebo subcutaneously three times weekly, 22 microg for the first week and then 44 micr...
journal_title:Neurology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1212/01.wnl.0000092019.53628.88
更新日期:2003-11-11 00:00:00
abstract:OBJECTIVE:To use a combined neurogenetic-neuroimaging approach to examine the functional consequences of preclinical dopaminergic nigrostriatal dysfunction in the human motor system. Specifically, we examined how a single heterozygous mutation in different genes associated with recessively inherited Parkinson disease a...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000338699.56379.11
更新日期:2009-03-24 00:00:00
abstract::The authors evaluated education attainment and neuropsychological deficits in children with sickle cell disease (SCD) and silent cerebral infarcts. Children with silent infarcts had twice the rate of school difficulties as children without infarcts. Eighty percent of silent infarct cases had clinically significant cog...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.56.8.1109
更新日期:2001-04-24 00:00:00
abstract:OBJECTIVE:To assess longitudinal follow-up of the incidence of multiple sclerosis (MS) through five decades and estimate the prevalence rate in Hordaland County, Norway, on January 1, 2003. METHODS:All patients with MS diagnosed from 1953 to 2003 were identified in the patient records of the Department of Neurology, H...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000195549.95448.b9
更新日期:2006-01-24 00:00:00
abstract::The relationship between multiple sclerosis (MS) and primary Sjögren's syndrome (PSS) is ambiguous; it was suggested that some patients diagnosed with MS may instead have PSS. In a recent epidemiologic study, the prevalence of PSS was 2.7% in southern Sweden. We randomly selected 30 patients with definite MS from our ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.42.4.845
更新日期:1992-04-01 00:00:00
abstract:BACKGROUND/OBJECTIVE:Apoptosis, or programmed cell death, is an evolutionary conserved mechanism essential for morphogenesis and tissue homeostasis, but it plays an important role also in pathologic conditions, including neurologic disorders. Its execution pathway is critically regulated at the mitochondrial level. Evi...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.56.8.1070
更新日期:2001-04-24 00:00:00
abstract::Supporting young people with pediatric multiple sclerosis can be challenging for families and health care providers. Adolescents may be more resilient than adults in reaction to the diagnosis but can have more difficulty planning for their futures. Appropriate, sensitive, and focused health provision should include co...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000002879
更新日期:2016-08-30 00:00:00
abstract::Rats with unilateral lesions of the substantia nigra were treated with L-dopa 25 mg/kg subcutaneously 30 minutes after treatment with carbidopa 25 mg/kg intraperitoneally. In the ipsilateral striatum, dopamine levels achieved a peak of 1.57 +/- 0.63 micrograms/g (control 0.19 +/- 0.05 micrograms/g) at 20 minutes and d...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.34.8.1105
更新日期:1984-08-01 00:00:00
abstract::The effect of imipramine on the absorption of a single dose of levodopa was studied in male volunteers. By delaying gastric emptying and retarding delivery to intestinal absorptive sites, imipramine interfered with the absorption of levodopa. This action caused by the anticholinergic effect of imipramine. The retardat...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.25.11.1029
更新日期:1975-11-01 00:00:00
abstract::We measured the concentrations of the monoamines, their precursors, and their metabolites, and the activity of choline acetyltransferase (ChAT) in basal ganglia and cortical regions of postmortem brains from cases with histologically verified pure Alzheimer's disease (AD), AD with diffusely distributed Lewy bodies (Le...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.43.10.1927
更新日期:1993-10-01 00:00:00
abstract::One hundred and eight multiple sclerosis (MS) patients and 108 matched controls were studied for antibody levels and cellular immune responses to several viruses. There were significant increases in the mean titers of complement fixation (CF) or hemagglutination inhibition (HI), and complement-mediated cytotoxicity (C...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.28.6.613
更新日期:1978-06-01 00:00:00
abstract:OBJECTIVE:To evaluate the utility of MRI hippocampal and entorhinal cortex atrophy in predicting conversion from mild cognitive impairment (MCI) to Alzheimer disease (AD). METHODS:Baseline brain MRI was performed in 139 patients with MCI, broadly defined, and 63 healthy controls followed for an average of 5 years (ran...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000256697.20968.d7
更新日期:2007-03-13 00:00:00
abstract::We gave three adult rhesus monkeys seven IV injections of manganese chloride at approximately 1-week intervals. We evaluated neurologic status by serial clinical examinations and performed a levodopa test if the animal developed features of basal ganglia dysfunction. After the animals were killed, we performed neuropa...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.46.2.492
更新日期:1996-02-01 00:00:00
abstract::Cisplatin, used to treat ovarian, bladder, and testicular cancers, causes a sensory dose-limiting neuropathy. Preliminary observations in humans and animals suggest that nerve damage may be prevented by ACTH analogs, particularly those belonging to the melanocortin class, and by nerve growth factor (NGF). We establish...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.44.3_part_1.488
更新日期:1994-03-01 00:00:00
abstract::The authors describe the outcome of five patients with a cardiac thrombus selected among 183 patients with stroke (2.7 %) who were given IV tissue plasminogen activator (tPA). No early systemic or cerebral embolism occurred. Two patients made a complete recovery at 3 months. Two patients had a moderate outcome. One pa...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.57.11.2122
更新日期:2001-12-11 00:00:00
abstract::We describe a 66-year-old woman with long-standing HTLV-I-associated myelopathy who developed multiple parenchymal hemorrhages and whose angiogram suggested cerebral vasculitis. After cyclophosphamide and glucocorticoid therapy, both her acute deficits and chronic paraparesis improved. HTLV-I may be an etiologic agent...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.43.2.412
更新日期:1993-02-01 00:00:00
abstract::The authors report a double-blind, placebo-controlled, crossover study of talampanel in 49 patients with refractory partial seizures. Three doses of talampanel were investigated based on differences in patients' concomitant antiepileptic drug usage. Talampanel showed efficacy in reducing seizure frequency (p = 0.001) ...
journal_title:Neurology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1212/wnl.58.11.1680
更新日期:2002-06-11 00:00:00