Abstract：
:There is still uncertainty regarding risk factors for cancer occurrence in patients with myasthenia gravis (MG). The objective of this study is to determine the prevalence of extrathymic neoplasms in patients with MG and the factors associated with tumor occurrence. The archives of four tertiary MG centers were consulted and patients were interviewed on the main clinical features of the disease, the presence and type(s) of extrathymic neoplasms and other autoimmune disorders, and their symptomatic and immunosuppressant treatments (with detailed schedules). A retrospective cohort survey was undertaken comparing the demographic and clinical variables of patients with extrathymic neoplasms to those of the remaining MG population. 2,479 patients were traced and interviewed personally or through informants. The sample included 1,490 women and 989 men (mean age 54.7 years). Other autoimmune disorders were present in 216 cases (8.7%). Thymectomy was performed in 1,549 cases (62.5%), thymic hyperplasia and thymoma being the most common findings. Acetylcholinesterase-inhibitors were the most common treatment (93.5%), followed by steroids (64.3%), azathioprine (35.0%), plasma exchange (13.2%), immunoglobulins (7.5%), cyclosporine (5.3%), and cyclophosphamide (5.0%). 221 patients (8.9%) had one or more extrathymic tumors, 168 of which occurred after disease onset. Patients with and without extrathymic neoplasms were followed for 14.8 and 13.9 years, respectively. Variables shown by multivariate analysis to be associated with increased neoplastic risk included older age, thymoma and immunoglobulin use. Extrathymic tumors are a common finding in patients with MG and tend to be associated with age, thymoma, and immunoglobulin use.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Citterio A,Beghi E,Millul A,Evoli A,Mantegazza R,Antozzi C,Baggi F,Cornelio F,Durelli L,Clerico M,Piccolo G,Cosi Vdoi
10.1007/s00415-009-5091-9subject
Has Abstractpub_date
2009-08-01 00:00:00pages
1221-7issue
8eissn
0340-5354issn
1432-1459journal_volume
256pub_type
杂志文章abstract：:Disability in multiple sclerosis (MS) patients is associated with white matter (WM) and gray matter (GM) pathology, and both processes contribute differently over the disease course. Total and regional GM volume loss can be imaged via voxel-based morphometry (VBM). Here, we retrospectively analyzed a group of 213 MS p...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-016-8114-3
更新日期：2016-06-01 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：:Horizontal eye-movement responses of four patients with Alzheimer-type dementia were recorded using the infrared oculographic technique. Abnormally frequent saccadic intrusions occurred during the fixation and slow, smooth pursuit tasks in all four patients. Saccadic intrusions have previously been reported in the lit...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313742
更新日期：1983-01-01 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：:We examined 199 consecutive patients who underwent 220 liver transplantations, to define the type, frequency and aetiology of posttransplant neurological complications and their prognostic value. We found neurological complications in 63 patients (32%), mostly involving the central nervous system. The most frequent co...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF02444004
更新日期：1996-02-01 00:00:00
abstract：OBJECTIVE:To characterize the progression of the cognitive involvement in patients affected by myotonic dystrophy type 1 (DM1) by a longitudinal neuropsychological follow-up study. METHODS:In a previous study we documented an ageing-related decline of frontal and temporal cognitive functions in juvenile/adult forms of...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-008-0017-5
更新日期：2008-11-01 00:00:00
abstract：:Parkinson's disease (PD) is a neurodegenerative disease caused by both genetic and environmental factors. Sirtuins are highly-conserved, NAD-dependent class III deacetylases that regulate a variety of cellular functions. Most of the known sirtuins have been involved in animal models of neurodegenerative disorders, suc...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-013-6970-7
更新日期：2013-09-01 00:00:00
abstract：:Whereas the effect of interferons (IFNs) on magnetic resonance imaging (MRI) outcome measures in patients with multiple sclerosis (MS) has been convincingly shown, little work has been done to define the between-patient heterogeneity of treatment response. Our aim was to assess the distribution of the effect of IFNbet...
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/s00415-005-0885-x
更新日期：2005-12-01 00:00:00
abstract：:Premature atherosclerosis and cardiac complications have been reported among the systemic manifestations of cerebrotendinous xanthomatosis (CTX), a rare bile acid disorder with predominantly neurological features. In some cases, myocardial infarction has been the cause of sudden death. We examined nine CTX patients to...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150050274
更新日期：1998-11-01 00:00:00
abstract：:Cerebellar ataxias (CAs) represent a heterogeneous group of sporadic or inherited disorders. The clinical spectrum of CAs is continuously expanding. Our understanding of the mechanisms leading to the clinical deficits has improved over these last decades, in particular thanks to progress in genetics, neuroimaging and ...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-020-09717-3
更新日期：2020-04-01 00:00:00
abstract：:A complex neurological syndrome, which rapidly appeared in a 54-year-old woman, created strong diagnostic difficulties. In fact, while the carotid-angiography was negative and CSF not significant, the scintigraphy suggested a multifocal metastatic or infarctual pathology. The CT scan easily allowed us to resolve the d...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313384
更新日期：1981-01-01 00:00:00
abstract：PURPOSE:Lamotrigine (LTG) and valproate (VPA) are widely used in the treatment of generalized epilepsies. Nevertheless seizure aggravation with LTG has been reported in juvenile myoclonic epilepsy and epilepsy with myoclonic absences. The aim of this study was to describe clinical features and EEG findings in patients ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-002-0857-3
更新日期：2002-10-01 00:00:00
abstract：:Multifocal motor neuropathy (MMN) can be differentiated from motor neuron disease by electrophysiological evidence of conduction block. To increase the probability of recording conduction block, we studied the whole nerve length including proximal segments in 84 patients with pure motor syndromes, using a special stim...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00873973
更新日期：1996-10-01 00:00:00
abstract：:The occurrence of spontaneous internal carotid or vertebral artery dissection after childbirth remains rare. To our knowledge, seven cases of arterial dissection in the postpartum period have been described in the literature as single case reports. We report four additional cases of internal carotid and vertebral arte...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s004150050437
更新日期：1999-08-01 00:00:00
abstract：BACKGROUND:Progression of parkinsonian motor impairment is usually rapid and relentless in multiple system atrophy (MSA). However, it may also be subject to considerable variation. Prospective natural history studies using validated rating scales are required to accurately determine the progression of parkinsonism in M...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-005-0617-2
更新日期：2005-01-01 00:00:00
abstract：:The aim of the study was to identify the main factors that impact mobility impairment in multiple sclerosis (MS) patients in Italy. Clinicians from a large number of Italian MS centers took part in a Delphi process aimed at obtaining consensus statements among the participants. Large consensus was obtained for stateme...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-013-7230-6
更新日期：2014-03-01 00:00:00
abstract：:Diminished activity of uroporphyrinogen I-synthetase in the liver and other tissues may be regarded to be the primary genetic deficiency of acute intermittent porphyria (AIP). Increased production and renal excretion of delta-aminolevulinic acid (ALA) und porphobilinogen (PBG) are secondary phenomena. The neuropsychia...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00316572
更新日期：1977-03-21 00:00:00
abstract：OBJECTIVE:To determine the response to treatment and the long-term outcome of patients with the antisynthetase syndrome associated with anti-Jo-1-antibodies. PATIENTS AND METHODS:A total of 12 patients with histologically proven myositis and anti-Jo-1-autoantibodies were evaluated over a mean follow-up period of 66.4 ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-004-0449-5
更新日期：2004-07-01 00:00:00
abstract：:Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) are the major immune neuropathies. Although a detailed understanding of the pathogenesis of these conditions is not yet available, the multiple effects of IVIg on the immune and inflamm...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-008-3003-z
更新日期：2008-07-01 00:00:00
abstract：BACKGROUND:Bilateral pallidal lesions induce a range of cognitive and motor disorders, principally a parkinsonian syndrome in which severe disturbances of gait and gait initiation are frequently reported. However, the precise clinical features of these disorders (and the role of the pallidum therein) remain to be estab...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-006-0066-6
更新日期：2006-05-01 00:00:00
abstract：:Parkinson's disease (PD) is characterised both clinically and pathologically by features that distinguish it from other parkinsonian disorders including, for instance, multiple system atrophy and progressive supranuclear palsy. The aetiologies of PD includes both genetic and environmental influences. Several single ge...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-011-6016-y
更新日期：2011-05-01 00:00:00
abstract：:Neurological complications following rubella are only rarely encountered. However, in many cases severe neurological impairment may occur, leading to permanent disability. In a recent epidemic of rubella in Israel during the years 1978-1979, 20 patients with severe neurological complications have been seen. We report ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313402
更新日期：1982-01-01 00:00:00
abstract：:Neurological manifestations in pandemics frequently cause short and long-term consequences which are frequently overlooked. Despite advances in the treatment of infectious diseases, nervous system involvement remains a challenge, with limited treatments often available. The under-recognition of neurological manifestat...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-020-10261-3
更新日期：2020-10-26 00:00:00
abstract：AIM:To assess: (1) the prevalence of convexity subarachnoid hemorrhage (cSAH) in infective endocarditis (IE); (2) its relationship with IE features; (3) the associated lesions; (4) whether cSAH is a predictor of future hemorrhage; (5) whether cSAH could cause cortical superficial siderosis (cSS). METHODS:We retrospect...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-09953-7
更新日期：2020-10-01 00:00:00
abstract：:Cognitive impairment belongs to the core symptoms in MS affecting quality of life, self-esteem, and social as well as occupational functioning. Due to this high impact on patients' well-being efficient treatment concepts are required. Imaging studies on cognition have shown that functional reorganisation takes place s...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-007-2013-6
更新日期：2007-05-01 00:00:00
abstract：:Two patients are described, in whom MRI detected unilateral signal abnormalities in the region of the inferior olivary nuclei, suggesting an early stage of olivary hypertrophy. MRI was performed 4 and 7 weeks respectively after traumatic brain-stem injury. Palatal myoclonus was concomitantly observed in one patient, w...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00867352
更新日期：1993-07-01 00:00:00
abstract：:Untreated sleep disorders may contribute to secondary causes of uncontrolled hypertension, cardiovascular disease (CVD), and stroke. Restless legs syndrome, or Willis-Ekbom Disease (RLS/WED), is a common sensorimotor disorder with a circadian rhythmicity defined by an uncontrollable urge to move the legs that worsens ...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-013-7065-1
更新日期：2014-06-01 00:00:00
abstract：:Three patients with postural and intention cerebellar tremor caused by a cerebellar infarction in the superior cerebellar artery distribution were studied; treatment with carbamazepine resulted in marked improvement. ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00328507
更新日期：1989-12-01 00:00:00
abstract：:In order to identify the precise location of the primary motor area for the diaphragm with respect to the classical motor homunculus, functional magnetic resonance imaging (fMRI) experiments were performed utilizing independent component-cross correlation- sequential epoch (ICS) analysis on a high-field (3.0 Tesla) sy...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-004-0413-4
更新日期：2004-06-01 00:00:00
abstract：:Spinocerebellar ataxia (SCA) type 7 is an autosomal dominant disorder characterized by neural loss, mainly in the cerebellum and regions of the brainstem and particularly the inferior olivary complex. This neurodegeneration disease is associated with expansion of unstable CAG repeats within the 5'-translated region of...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150070131
更新日期：2000-08-01 00:00:00
