Abstract:
:Aquaporin-4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorder (NMOSD) is a rare but often severe autoimmune disease with median onset around 40 years of age. We report characteristics of three very-late-onset NMOSD (including complete NMO) patients >75 years of age, in whom this diagnosis initially seemed unlikely because of their age and age-associated concomitant diseases, and briefly review the literature. All three patients, aged 79, 82 and 88 years, presented with a spinal cord syndrome as the first clinical manifestation of AQP4-Ab-positive NMOSD. They all had severe relapses unless immunosuppressive therapy was initiated, and one untreated patient died of a fatal NMOSD course. Two patients developed side effects of immunosuppression. We conclude that a first manifestation of NMOSD should be considered even in patients beyond the age of 75 years with a compatible syndrome, especially longitudinally extensive myelitis. Early diagnosis and treatment are feasible and highly relevant. Special attention is warranted in the elderly to recognize adverse effects of immunosuppressive therapies as early as possible.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Krumbholz M,Hofstadt-van Oy U,Angstwurm K,Kleiter I,Jarius S,Paul F,Aktas O,Buchholz G,Kern P,Straube A,Kümpfel Tdoi
10.1007/s00415-015-7766-8subject
Has Abstractpub_date
2015-05-01 00:00:00pages
1379-84issue
5eissn
0340-5354issn
1432-1459journal_volume
262pub_type
杂志文章abstract::Few regional and seasonal Guillain-Barré syndrome (GBS) clusters have been reported so far. It is unknown whether patients suffering from sporadic GBS differ from GBS clusters with respect to clinical and paraclinical parameters, HLA association and antibody response to glycosphingolipids and Campylobacter jejuni (Cj)...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8237-6
更新日期:2016-10-01 00:00:00
abstract::Atrial fibrillation (AF) is considered a predictor for severe stroke and poor outcome. The aim was to evaluate whether AF is associated with poor outcome in acute ischemic stroke (IS) patients treated with intravenous thrombolysis (IVT). In a retrospective study, 157 consecutive IS patients (98 males, mean age 67.3 +/...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5452-4
更新日期:2010-06-01 00:00:00
abstract::Clinical and neuropathological data of a 50-year-old woman with an unusual multisystem degeneration are presented. Clinically the illness was characterized by progressive ataxia with ophthalmoplegia and multiple cranial nerve palsies. Neuropathological investigation showed a severe and selective degeneration of the de...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313709
更新日期:1985-01-01 00:00:00
abstract::A woman affected by chronic progressive external ophthalmoplegia and muscle mitochondrial DNA deletion was studied by phosphorus magnetic resonance spectroscopy (31P-MRS) prior to and after 1 and 7 months of treatment with oral lipoic acid. Before treatment a decreased phosphocreatine (PCr) content was found in the oc...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00873552
更新日期:1995-07-01 00:00:00
abstract::We evaluated cyclosporine A (CsA) treatment in 9 patients (6 female and 3 male), 16-63 years old, with severe myasthenia gravis (MG) for a mean period of 2 years (range 16-36 months). All of the patients had been previously treated either with corticosteroids or by combined immunotherapy, and 5 needed periodic plasma ...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s004150050141
更新日期:1997-09-01 00:00:00
abstract::This study of frontotemporal dementia (FTD) was carried out to determine whether MR spectroscopy can provide an in vivo marker for the neuronal loss and gliosis that occur in this condition. We compared spectra in frontal and temporal regions known to be affected early in the course of the disease with spectra in the ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0045-y
更新日期:2006-07-01 00:00:00
abstract::Amyloid positron emission tomography ([18F] florbetaben (FBB) PET) can be used to determine concomitant Alzheimer's disease (AD) in idiopathic normal pressure hydrocephalus (iNPH) patients. FBB PET scans and the tap test were performed in 31 patients with clinically suspected iNPH, and amyloid positive (iNPH/FBB+) and...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8650-5
更新日期:2018-01-01 00:00:00
abstract::An IgM antibody cytotoxic to normal human lymphocytes was found in 46.2% (male: 61.5%; female: 30.8%) of sera from chronic schizophrenics by means of a complement-dependent cytotoxicity test. The relationship between this antibody and the immunological manifestations of schizophrenia is discussed. ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314296
更新日期:1987-06-01 00:00:00
abstract::Six patients with congenital myotonia and 4 patients with myotonic dystrophy have been examined clinically before and after the administration of N-propyl-ajmalin, an alkaloid frequently used as a cardiac antiarrhythmic drug. All patients but one reported a good to moderate improvement of their myotonic muscle stiffne...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00316381
更新日期:1975-09-01 00:00:00
abstract::Various parameters of histamine metabolism were studied in patients with migraine, cluster headache and chronic paroxysmal hemicrania. These included urinary excretion of radioactivity and of 14C histamine and its metabolites, exhaled 14CO2 and fecal radioactivity after oral as well as subcutaneous administration of r...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00312944
更新日期:1977-09-12 00:00:00
abstract:OBJECTIVE:To report the clinical features and long-term outcome of 22 newly diagnosed paraneoplastic patients with GABAB receptor antibodies (GABABR-Abs). METHODS:Retrospective clinical study of CSF-confirmed cases of GABABR-Abs encephalitis. RESULTS:We identified 22 patients (4 female) with GABABR-Abs, with a median...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9132-0
更新日期:2019-01-01 00:00:00
abstract:BACKGROUND:Brain tumors cause significant morbidity and mortality due to rapid progression and high recurrence risks. Reliable biomarkers to improve diagnosis thereof are desirable. OBJECTIVE:This work aimed to identify panels of biomarkers for diagnostic purposes using cerebrospinal fluid (CSF)-based metabolomics. M...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09665-7
更新日期:2020-04-01 00:00:00
abstract::Fatigue is a frequent and often severe symptom in multiple sclerosis. Pathogenic mechanisms proposed for fatigue include the release of proinflammatory cytokines, which is thought to have an important effect on changes in the blood-brain barrier (BBB). To investigate whether fatigue is related to BBB disruption we stu...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s004150050382
更新日期:1999-06-01 00:00:00
abstract:OBJECTIVE:Near occlusion (NO) of the internal carotid artery (ICA) with full collapse (NOFC) is a rare condition, with a prevalence of around 1%. Guidelines on carotid stenosis recommend a conservative treatment in patients with a single-event ipsilateral to a NOFC, but the optimal treatment for patients with recurrent...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8939-z
更新日期:2018-08-01 00:00:00
abstract:OBJECTIVE:To characterize the relation between brain functional connectivity and disability in patients with multiple sclerosis; to investigate the existence of critical values of both disability and functional connectivity corresponding to exhaustion of functional adaptive mechanisms. METHODS:Hundred-and-nineteen pat...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9075-5
更新日期:2018-12-01 00:00:00
abstract::The computed tomography, magnetic resonance imaging and angiographic findings are described in a patient with Klippel-Trenaunay syndrome, who also had a cerebral haemorrhage from an arteriovenous malformation. The resulting aphasia disappeared completely after resorption of the haemorrhage. In this syndrome, the occur...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313992
更新日期:1986-02-01 00:00:00
abstract::Posterior leukoencephalopathy typically denotes neurotoxicity from immunosuppressive agents, malignant hypertension or eclampsia. It has not been documented in central nervous system angiitis. We present three cases associated with isolated cerebral angiitis after review of all cases of isolated CNS angiitis from 1998...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-003-1021-4
更新日期:2003-04-01 00:00:00
abstract::We have studied the relationship between the apolipoprotein E gene (APOE) and the development of myoclonus, tremors, rigidity and seizures in 168 patients with probable early-onset Alzheimer's disease (AD). There was a statistically significantly lower risk of tremor for carriers of the epsilon 4 allele of APOE. This ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00900501
更新日期:1996-06-01 00:00:00
abstract::The clinicopathological findings of a 50-year-old man, who developed cervicothoracic syringomyelia at the age of 25 are presented. He was given radiation therapy at the age of 33. At the age of 57 he developed a foramen jugulare syndrome on the left, caused by a low grade leiomyosarcoma. Etiologically, the most attrac...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313150
更新日期:1980-01-01 00:00:00
abstract::We examined the influence of alleles at the HLA loci, previously found to be associated with multiple sclerosis (MS) in Sardinia, on the clinical course of the disease in 835 relapsing (R) and 100 primary progressive (PP) patients. Multivariate analysis was carried out on predisposing 0301 or non-associated DPB1 allel...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0957-y
更新日期:2006-02-01 00:00:00
abstract::Myotonic dystrophy (DM) is an autosomal dominant neuromuscular disease with an estimated incidence of 1 in 8000 and is the most common form of muscular dystrophy affecting adults. An unstable, untranslated part of the myotonic dystrophy protein kinase gene on the long arm of chromosome 19, composed of CTG repeats, is ...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1007/s004150050220
更新日期:1998-05-01 00:00:00
abstract::The seasonal occurrence of relapses was analysed retrospectively in a group of 53 patients with treated dermatomyositis (DM) or polymyositis (PM). In DM, the incidence of both myositic and cutaneous relapses was highest in summer whereas in the PM group relapses was more evenly distributed throughout the seasons but l...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150200036
更新日期:2002-04-01 00:00:00
abstract::Peripheral neuropathies have various causes, both infectious and non-infectious. When we think of "epidemics", we often refer to an infectious or even post-infectious origin. Nevertheless, the history of mankind is marked by episodes of epidemics of peripheral neuropathies of non-infectious nature, either of nutrition...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10216-8
更新日期:2020-09-11 00:00:00
abstract::Although central facial paresis (CFP) is a major symptom of stroke, there is a lack of studies on the motor and non-motor disabilities in stroke patients. A prospective cohort study was performed at admission for inpatient rehabilitation and discharge of post-stroke phase of 112 patients (44% female, median age: 64 ye...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9099-x
更新日期:2019-01-01 00:00:00
abstract::The diagnosis of Hallervorden-Spatz disease (HSD) has usually been made post mortem, although the recent description of characteristic abnormalities in the globus pallidus has suggested the possibility of an in vivo diagnosis. We present the clinical histories, neurological features and MRI findings of 11 patients, di...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00856805
更新日期:1992-10-01 00:00:00
abstract::5-Hydroxytryptamine (5-HT; serotonin) has long been implicated in the aetiology of migraine but the evidence remains circumstantial and certainly not definitive. Numerous papers have reviewed the background which is briefly outlined here. Although the continued belief in the primary involvement of 5-HT in the genesis ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF01642905
更新日期:1991-01-01 00:00:00
abstract::Muscle samples obtained in patients with different neuromuscular diseases were stored at 0 degree C for periods of 6-12h and were subsequently frozen for histochemical processing. Compared to reference samples which had been immediately frozen, the individual test sections displayed different degrees of swelling of th...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313934
更新日期:1984-01-01 00:00:00
abstract::We report two adult Japanese sisters with L-2-hydroxy-glutaric aciduria (acidemia), both of whom were much older (aged 57, 47 years old) than previously reported patients (from neonate to 44 years old), and who presented with differing severity. Magnetic resonance imaging revealed typical subcortical white matter lesi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050367
更新日期:1999-05-01 00:00:00
abstract::There has been a resurgence in the use of functional neurosurgery for Parkinson's disease. An important factor that has played a role in this development is the recent understanding of the functional anatomy of the basal ganglia including a knowledge of the changes in the activities of neurons in the internal segment ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF03161079
更新日期:1999-09-01 00:00:00
abstract::Using a novel trial design, we prospectively examined the effect of intravenous immunoglobulin in seven patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a double-blind, placebo-controlled cross-over study. We suggest that the commonly used manual muscle testing and Rankin scale are not...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1007/BF00868527
更新日期:1996-03-01 00:00:00