Abstract:
:A rare form of plasma cell dyscrasia characterized by associated polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes has been termed the POEMS syndrome. The pathophysiology is unknown; plasma cell dyscrasia is essential; secondary manifestations are unexplained. We report a 67-year-old man with a 7-month history of progressive weakness and numbness of the legs. Clinical examination revealed sensorimotor polyneuropathy, predominantly affecting the lower extremities, hepatomegaly, and skin haemangiomas. Additional investigations disclosed IgG-lambda monoclonal serum protein, endocrine abnormalities, elevated cerebrospinal fluid protein level and an osteoblastic lesion of the lumbar vertebra. Biopsy of the osteosclerotic vertebra showed a marked lymphoplasmocytic infiltrate. MRI of the liver disclosed two haemangiomas; this association has not been reported previously.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Vidaković A,Simić P,Stojisavljević N,Elezović I,Trikić R,Apostolski Sdoi
10.1007/BF00839213keywords:
subject
Has Abstractpub_date
1992-01-01 00:00:00pages
49-52issue
1eissn
0340-5354issn
1432-1459journal_volume
239pub_type
杂志文章abstract::Stroke patients with "pusher syndrome" show severe misperception of their own upright body orientation although visual-vestibular processing is almost intact. This dissociation argues for a second graviceptive system in humans for the perception of body orientation. Recent studies revealed that the posterior thalamus ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0025-7
更新日期:2006-04-01 00:00:00
abstract:BACKGROUND:Studies provide inconclusive results on the question whether loss of mental wellbeing is actually associated with decline in physical function in amyotrophic lateral sclerosis (ALS). The purpose of this study was to determine predictors of mental wellbeing in ALS. METHODS:In total, n = 330 ALS patients were...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10027-x
更新日期:2020-12-01 00:00:00
abstract::We recently reported findings that loss of cortical acetylcholinesterase (AChE) activity is greater in parkinsonian dementia than in Alzheimer's disease (AD). In this study we determined cognitive correlates of in vivo cortical AChE activity in patients with parkinsonian dementia (PDem, n = 11), Parkinson's disease wi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0971-0
更新日期:2006-02-01 00:00:00
abstract::To evaluate the clinical and electrophysiological similarities and differences between two large groups of patients with Charcot-Marie-Tooth disease, i.e. CMT1A and CMT2, we performed a post hoc comparison of clinical and electrophysiological data. Most CMT1A and CMT2 patients had the classical CMT phenotype. Age of o...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0260-6
更新日期:2006-12-01 00:00:00
abstract:BACKGROUND:Dermic fibroblasts have been proposed as a potential genetic-ALS cellular model. This study aimed to explore whether dermic fibroblasts from patients with sporadic-ALS (sALS) recapitulate alterations typical of ALS motor neurons and exhibit abnormal DNA-damage response. METHODS:Dermic fibroblasts were obtai...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09704-8
更新日期:2020-05-01 00:00:00
abstract::A patient with chronic relapsing inflammatory demyelinating polyneuropathy was successfully treated with plasma exchanges and cyclosporin A (CsA). Dynamometric measurements of hand force during the time of CsA treatment showed a highly significant correlation between hand force and CsA blood levels. The largest influe...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314752
更新日期:1990-08-01 00:00:00
abstract::Natalizumab, an anti-alpha4 integrin antibody, significantly reduces the number of visibly enhancing multiple sclerosis (MS) lesions. In this substudy of a 2-year trial of natalizumab monotherapy versus placebo, contrast-enhanced imaging investigated for subtle blood brain barrier (BBB) leakage in relapsing remitting ...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1007/s00415-006-0356-z
更新日期:2007-03-01 00:00:00
abstract::Pontocerebellar hypoplasia type 2 (PCH2) is a very rare autosomal recessive disorder. We report two unrelated female patients born to consanguineous parents presenting with this condition. Patient 1 showed a classical clinical/neuroradiological phenotype of PCH2 with dyskinesia/dystonia. Patient 2 had a neonatal onset...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150200069
更新日期:2002-05-01 00:00:00
abstract::We investigated the efficacy of botulinum toxin A (BtxA) therapy in patients with atypical parkinsonian disorders (APD) exhibiting different types of disabling focal dystonia unresponsive to oral drug therapy. Eight patients with functionally disabling focal dystonia out of a series of 60 consecutive patients with APD...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
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更新日期:2002-03-01 00:00:00
abstract::The spinocerebellar ataxias (SCAs) with autosomal dominant inheritance are a clinically and genetically heterogeneous group of neurological disorders with overlapping as well as highly variable phenotypes primarily affecting the cerebellum. To date, 28 different loci have been identified. Nine SCAs are caused by repea...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-5209-0
更新日期:2009-11-01 00:00:00
abstract:BACKGROUND:The frontal assessment battery (FAB) test is a composite tool for assessing executive functions related to the frontal lobe. Neuropsychological and blood-flow studies indicate distinct patterns of deterioration of anterior and posterior cortical function in Alzheimer's disease (AD) and subcortical vascular d...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0251-7
更新日期:2006-11-01 00:00:00
abstract::Fabry disease (FD) is a rare inherited disorder of the metabolism, associated with renal, cardiac, and cerebrovascular complications. Ischemic and hemorrhagic stroke in FD present with a similar proportion to that observed in the general population, but usually at an early age. Ischemic stroke may result from cardiac ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-011-6278-4
更新日期:2012-06-01 00:00:00
abstract::The objective of the study was to identify differences in treatment strategies for anti-NMDA receptor encephalitis based on specialty of treating physicians, geographic location, and years in practice. We conducted an anonymous worldwide electronic survey through the Practice Current section of Neurology® Clinical Pra...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8407-1
更新日期:2017-04-01 00:00:00
abstract::Mutations in C19orf12 have been recently identified as the molecular genetic cause of a subtype of neurodegeneration with brain iron accumulation (NBIA). Given the mitochondrial localization of the gene product the new NBIA subtype was designated mitochondrial membrane protein-associated neurodegeneration. Frequent fe...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6521-7
更新日期:2012-11-01 00:00:00
abstract:BACKGROUND AND PURPOSE:The association between anticardiolipin antibodies (aCL) and thrombosis is well recognized, but its role as an independent risk factor for stroke is not. The study's aim was to investigate the presence of antiphospholipid antibodies (aPL) and ischemic vascular events by using both traditional mea...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-003-1045-9
更新日期:2003-05-01 00:00:00
abstract::We assessed the prevalence of vertebral artery (VA) stenosis or occlusion and its influence on outcome in patients with acute basilar artery occlusion (BAO). We studied 141 patients with acute BAO enrolled in the Basilar Artery International Cooperation Study (BASICS) registry of whom baseline CT angiography (CTA) of ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7583-5
更新日期:2015-02-01 00:00:00
abstract:BACKGROUND:Ultrafast brain MRI is required for uncooperative patients and time-critical diseases such as stroke because it reduces scan times and motion artifacts. This study investigated the clinical feasibility of a 1-min ultrafast brain MRI protocol for detecting intracranial abnormalities in restless and uncooperat...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9149-4
更新日期:2019-02-01 00:00:00
abstract::A case of CT-diagnosed brain-stem haematoma associated with signs of dorsal medullary involvement is reported. The patient recovered completely within 1 month. ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313834
更新日期:1985-01-01 00:00:00
abstract::Although multiple sclerosis (MS) is considered to be an inflammatory demyelinating disease, increasing evidence indicates that it is also an axonal pathology; indeed, studies of experimental allergic encephalitis showed that several neuronal proteins such as synapsins take part in the pathogenesis of the axonal dysfun...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0293-7
更新日期:2004-02-01 00:00:00
abstract:OBJECTIVE:To investigate whether intracranial arachnoid cysts (AC) compromise visual attention and if so, whether surgical cyst decompression leads to improvement in visual attention performance. METHODS:The experiments were carried out on 27 patients with temporal (n=21) or frontal (n=6) AC, and 27 healthy control su...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
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abstract::The MS-ID (Multiple Sclerosis Information Dividend) project was initiated by the European Multiple Sclerosis Platform (EMSP) in 2007 in order to identify and address major inequalities of MS treatment and care and thus eliminate disparities across the EU. One major approach to reach these goals in the longer term is t...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
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journal_title:Journal of neurology
pub_type: 杂志文章
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更新日期:1997-07-01 00:00:00
abstract::Even though trigeminovestibular connections are well established in animals, mastication-induced dizziness has been described only as a vascular steal phenomenon in humans. We determined induction or modulation of nystagmus in two index patients with mastication-induced vertigo, 12 normal controls, and 52 additional p...
journal_title:Journal of neurology
pub_type: 杂志文章
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更新日期:2014-03-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00858729
更新日期:1993-02-01 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:BACKGROUND:Late-onset Pompe disease (LOPD) is a rare, metabolic disease primarily affecting the musculoskeletal and respiratory systems. Forced vital capacity (FVC) is commonly used to measure pulmonary function; however, associations between FVC and other LOPD outcomes remain unclear. METHODS:A systematic literature ...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析
doi:10.1007/s00415-019-09401-1
更新日期:2019-09-01 00:00:00
abstract::We investigated the levels of transforming growth factor beta 1 (TGF-beta 1) in cerebrospinal fluid (CSF) in children with meningitis, with a view to prognostic relevance. CSF TGF-beta 1 levels on admission were measured by a sandwich enzyme immunoassay in children with bacterial meningitis (n = 16), aseptic meningiti...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s004150050159
更新日期:1997-10-01 00:00:00
abstract:BACKGROUND:Diagnostic delay of hereditary transthyretin amyloidosis (ATTRv, v for variant) prevents timely treatment and, therefore, concurs to the mortality of the disease. The aim of the present study was to explore with nerve ultrasound (US) possible red flags for early diagnosis in ATTRv patients with carpal tunnel...
journal_title:Journal of neurology
pub_type: 杂志文章
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更新日期:2021-01-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0312-8
更新日期:2004-03-01 00:00:00
abstract::The relationship between atherosclerosis and cognitive function is less well studied in Chinese populations. In addition, the results among middle-aged adults have been mixed. We aimed to investigate the association of atherosclerosis measured by carotid intima-media thickness (CIMT) and cognitive function in middle-a...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8234-9
更新日期:2016-10-01 00:00:00