Abstract:
:Amphiphysin-IgG was identified in 71 patients among 120,000 evaluated serologically for paraneoplastic autoantibodies. Clinical information was available for 63 patients. Cancer was detected in 50 (mostly limited), proven histologically in 46, and was imaged intrathoracically in 4 patients (lung, small-cell [27] and non-small cell [1]), breast [16] and melanoma [2]). Neurological accompaniments included (decreasing frequency): neuropathy, encephalopathy, myelopathy, stiff-man phenomena, and cerebellar syndrome. In a case examined neuropathologically, parenchymal T-lymphocyte infiltration (predominantly CD8(+)) was prominent in lower brainstem, spinal cord, and dorsal root ganglion. Coexisting paraneoplastic autoantibodies, identified in 74% of patients, predicted a common neoplasm and indicated other neuronal autoantigen targets that plausibly explained several neurological manifestations; for example, P/Q-type Ca(2+)-channel antibody with Lambert-Eaton syndrome (n = 5), anti-neuronal nuclear antibody type 1 with sensory neuronopathy (n = 7), K(+)-channel antibody with limbic encephalitis (n = 1) or neuromyotonia (n = 1), and collapsin response-mediator protein-5-IgG with optic neuritis (n = 3). Patients with isolated amphiphysin-IgG (n = 19) were more likely to be women (with breast cancer, p < 0.05) and to have myelopathy or stiff-man phenomena (p < 0.01). Overall, a minority of women (39%) and men (12%) had stiff-man phenomena. Only 10% of women (some with lung carcinoma) and 4% of men fulfilled diagnostic criteria for stiff-man syndrome.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Pittock SJ,Lucchinetti CF,Parisi JE,Benarroch EE,Mokri B,Stephan CL,Kim KK,Kilimann MW,Lennon VAdoi
10.1002/ana.20529keywords:
subject
Has Abstractpub_date
2005-07-01 00:00:00pages
96-107issue
1eissn
0364-5134issn
1531-8249journal_volume
58pub_type
杂志文章abstract::We have conducted a trial to randomly assess the efficacy and tolerance of intravenous immunoglobulin (i.v.Ig) or plasma exchange (PE) in myasthenia gravis (MG) exacerbation and to compare two doses of i.v.Ig. Eighty-seven patients with MG exacerbation were randomized to receive either three PE (n = 41), or i.v.Ig (n ...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ana.410410615
更新日期:1997-06-01 00:00:00
abstract::Nowadays, the "flipped classroom" approach is taking the center stage within medical education. However, very few reports on the implementation of the flipped classroom in neurology have been published to date, and this educational model still represents a challenge for students and educators alike. In this article, n...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25609
更新日期:2020-01-01 00:00:00
abstract::In this study, we preincubated the sera of 3 patients with neuropathies associated with elevated titers of IgM anti-GM1 antibodies, with increasing concentrations of intravenous Ig (IVIg) and assayed the inhibitory effect of this mixture on antibody binding to immobilized GM1 by an enzyme-linked immunosorbent assay. P...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410390121
更新日期:1996-01-01 00:00:00
abstract:OBJECTIVE:This study addresses an important problem in neurology, distinguishing tremor and ataxia using quantitative methods. Specifically, we aimed to quantitatively separate dysmetria, a cardinal sign of ataxia, from tremor in essential tremor (ET). METHODS:In Experiment 1, we compared 19 participants diagnosed wit...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25781
更新日期:2020-08-01 00:00:00
abstract::The nosological status of multifocal motor neuropathy remains controversial. The clinical and electrodiagnostic hallmarks suggest selective motor fiber involvement. In this study, we asked to what extent sensory nerves might be involved pathologically in multifocal motor neuropathy. Examination of sensory nerve biopsy...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410390308
更新日期:1996-03-01 00:00:00
abstract::The causes of amyotrophic lateral sclerosis, Parkinson disease, and Alzheimer disease are unknown. Furthermore, treatment for two of these conditions is almost totally lacking. The thesis is presented that each of these disorders is due to lack of a disorder-specific neurotrophic hormone. The hormone would be elaborat...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410100602
更新日期:1981-12-01 00:00:00
abstract::The clinical and neuropathological findings in 28 cases of paramedian thalamic and midbrain infarcts are reported. The 4 instances of unilateral paramedian thalamic infarct were characterized by mood and behavioral changes, limitation of the infarct to the center of the anatomical paramedian territory, and symmetrical...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410100204
更新日期:1981-08-01 00:00:00
abstract::A prospective clinical and biochemical study on the effects of treatment with haloperidol has been performed in seven patients with Tourette syndrome. Pretreatment cerebrospinal fluid levels of homovanillic acid (CSF HVA) were significantly reduce in all patients, whereas 5-hydroxyindoleacetic acid was reduced in only...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410120408
更新日期:1982-10-01 00:00:00
abstract::We studied the in vitro electrical activity of rat neostriatal neurons following chronic neuroleptic treatment. In haloperidol-treated rats, unlike naive animals, activation of neostriatal D2 dopamine receptors induced a potent presynaptic inhibition of glutamate-mediated excitatory synaptic potentials. Haloperidol tr...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410310404
更新日期:1992-04-01 00:00:00
abstract:OBJECTIVE:To investigate in older adults without dementia the relationships between socioeconomic status (SES) in childhood and magnetic resonance imaging (MRI)-derived brain volume measures typical of brain aging and Alzheimer's disease (AD). METHODS:Using a cross-sectional and longitudinal observation approach, we i...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22631
更新日期:2012-05-01 00:00:00
abstract::Powerful new methods for imaging both brain anatomy and brain function are appearing at an increasing rate. The modern era of minimally invasive, highly informative, neurological diagnostic imaging methods began with the introduction of x-ray computed tomography in the 1970s. More recently, positron emission tomograph...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.410320316
更新日期:1992-09-01 00:00:00
abstract::In multiple sclerosis (MS), the influx of activated T lymphocytes into the brain parenchyma leads to the subsequent damage of oligodendrocytes, the cells that produce central nervous system (CNS) myelin. We report here that interferon beta-1b (IFNbeta-1b), a drug shown to be efficacious in the treatment of patients wi...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410400607
更新日期:1996-12-01 00:00:00
abstract:OBJECTIVE:To examine age-related changes in the neural systems for reading in nonimpaired and dyslexic children and adolescents. METHODS:Functional magnetic resonance imaging was used to study age-related changes in the neural systems for reading in a cross-sectional sample of 232 right-handed children 7 to 18 years o...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21093
更新日期:2007-04-01 00:00:00
abstract::A 37-year-old homosexual man with the acquired immune deficiency syndrome (AIDS) developed progressive, ultimately fatal, neurological deficits 12 weeks after a course of cutaneous zoster. Premortem radiological procedures and cerebrospinal fluid analyses were nondiagnostic. At postmortem examination, several opportun...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410190212
更新日期:1986-02-01 00:00:00
abstract:OBJECTIVE:Amyotrophic lateral sclerosis (ALS) is a motor neuron disease whose pathophysiological deficits, causing impairment in motor function, are largely unknown. Here we propose that hydrogen sulfide (H2 S), as a glial-released inflammatory factor, contributes to ALS-mediated motor neuron death. METHODS:H2 S conce...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24372
更新日期:2015-04-01 00:00:00
abstract::Huntington's disease is a genetic disorder that causes motor dysfunction, personality changes, dementia, and premature death. There is currently no effective therapy. Several transgenic models of Huntington's disease are available, the most widely used of which is the R6/2 mouse, because of its rapid disease progressi...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10094
更新日期:2002-02-01 00:00:00
abstract::We report increased modification of proteins by 4-hydroxynonenal (HNE), a product of membrane lipid peroxidation, in the lumbar spinal cord of sporadic amyotrophic lateral sclerosis (ALS) patients versus that of neurologically normal controls. By immunohistochemistry, HNE-protein modification was detected in ventral h...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410440518
更新日期:1998-11-01 00:00:00
abstract::We describe a young child with tuberous sclerosis and cardiac rhabdomyoma who developed reversible heart block after being placed on carbamazepine treatment for seizures. Patients of any age with known or suspected intrinsic cardiac disease should be monitored for conduction disturbance if they are treated with carbam...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340417
更新日期:1993-10-01 00:00:00
abstract::The genetic architecture of common epilepsies is largely unknown. HCNs are excellent epilepsy candidate genes because of their fundamental neurophysiological roles. Screening in subjects with febrile seizures and genetic epilepsy with febrile seizures plus revealed that 2.4% carried a common triple proline deletion (d...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21909
更新日期:2010-04-01 00:00:00
abstract::A non-right-handed patient developed alexia without agraphia from a right occipital lobe infarction. An intracarotid amobarbital test showed left hemispheric dominance for speech. The cause of alexia in this patient could not be explained simply by the accepted disconnection hypothesis, which proposes that speech, han...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410030617
更新日期:1978-06-01 00:00:00
abstract::Ten laminin alpha2-deficient patients were identified by both immunofluorescence and immunoblotting (30% of congenital muscular dystrophy patients tested). Three of the laminin alpha2-deficient patients were carrying a diagnosis of infantile polymyositis prior to immunostaining studies. The clinical features in the 10...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410400515
更新日期:1996-11-01 00:00:00
abstract::Ganaxolone (3alpha-hydroxy-3beta-methyl-5alpha-pregnan-20-one) is a novel neurosteroid which has anticonvulsant properties in a number of seizure models as well as the ability to enhance function of the gamma-aminobutyric acid-A (GABA(A)) receptor complex via a neurosteroid binding site. The object of these experiment...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410440417
更新日期:1998-10-01 00:00:00
abstract::Cylindrical spirals are unique membranous structures that were detected in skeletal muscle of a mother and one of her two children; all three have percussion myotonia but no evidence of weak or wasted skeletal muscles. Muscle cramps, stiffness, posteffort muscle tightness, myotonic lid lag, and the cylinders appear or...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410070608
更新日期:1980-06-01 00:00:00
abstract::The topography and magnitude of increase in peripheral type benzodiazepine binding sites (omega 3 sites) was investigated autoradiographically in the brains of patients with ischemic cerebrovascular disease, with multiple sclerosis, and with malignant glioma. 3H-PK 11195, a selective omega 3 site ligand, was employed....
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410240603
更新日期:1988-12-01 00:00:00
abstract::The monoclonal antibodies anti-Leu-19 and anti-NKH-1 recognize the CD56 differentiation antigen expressed on natural killer (NK) cells and on a T-cell subset. Because CD56 is an isoform of neural cell adhesion molecule (N-CAM), we examined its expression on human muscle using antibodies to Leu-19, NKH-1, and purified ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410310109
更新日期:1992-01-01 00:00:00
abstract:OBJECTIVE:Infantile spasms are seizures associated with a severe epileptic encephalopathy presenting in the first 2 years of life, and optimal treatment continues to be debated. This study evaluates early and sustained response to initial treatments and addresses both clinical remission and electrographic resolution of...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24594
更新日期:2016-03-01 00:00:00
abstract:OBJECTIVE:Low vitamin D status at birth may be associated with risk of adult onset multiple sclerosis, but this link has not been studied directly. We assessed the relation between neonatal vitamin D concentrations, measured in stored blood samples, and risk of multiple sclerosis. METHODS:This was a population-based c...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24210
更新日期:2014-09-01 00:00:00
abstract::Multiple sclerosis patients who become pregnant experience a significant decrease in relapses that may be mediated by a shift in immune responses from T helper 1 to T helper 2. Animal models of multiple sclerosis have shown that the pregnancy hormone, estriol, can ameliorate disease and can cause an immune shift. We t...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.10301
更新日期:2002-10-01 00:00:00
abstract::The etiology of neurodegenerative diseases remains enigmatic; however, evidence for defects in energy metabolism, excitotoxicity, and for oxidative damage is increasingly compelling. It is likely that there is a complex interplay between these mechanisms. A defect in energy metabolism may lead to neuronal depolarizati...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.410380304
更新日期:1995-09-01 00:00:00
abstract::Cognitive and extrapyramidal effects of cholinomimetic therapy were evaluated in 8 patients with progressive supranuclear palsy. Each was randomized to a 10-day double-blind crossover trial of physostigmine and placebo. Physostigmine treatment was associated with marginal and inconsistent changes in long-term memory, ...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ana.410260318
更新日期:1989-09-01 00:00:00