Abstract:
:Characteristic visual symptoms and signs in 12 patients with neoplasms or aneurysms involving the optic tract are summarized. Blurred vision was the most common initial manifestation. Optic atrophy became apparent in 7 of the 12 patients. Most patients had uniocular central scotomas with reduced visual acuity, and 2 had homonymous scotomas. Field defects were frequently incomplete and incongruous, combining central scotomas with elements of homonymous and bitemporal amblyopias. Seven patients had endocrine disturbances and memory deficits in addition to their visual symptoms.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Bender MB,Bodis-Wollner Idoi
10.1002/ana.410030302subject
Has Abstractpub_date
1978-03-01 00:00:00pages
187-93issue
3eissn
0364-5134issn
1531-8249journal_volume
3pub_type
杂志文章abstract:OBJECTIVE:Small-fiber sensory and autonomic symptoms are early presentations of familial amyloid polyneuropathy (FAP) with transthyretin (TTR) mutations. This study aimed to explore the potential of skin nerve pathologies as early and disease-progression biomarkers and their relationship with skin amyloid deposits. ME...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25433
更新日期:2019-04-01 00:00:00
abstract:OBJECTIVE:To assess the ability for visual search and recognition of roadside targets and safety errors during a landmark and traffic sign identification task in drivers with Parkinson's disease (PD). METHODS:Seventy-nine drivers with PD and 151 neurologically normal older adults underwent a battery of visual, cogniti...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20958
更新日期:2006-10-01 00:00:00
abstract:OBJECTIVE:Loss of function mutations in PINK1 typically lead to early onset Parkinson disease (PD). Zebrafish (Danio rerio) are emerging as a powerful new vertebrate model to study neurodegenerative diseases. We used a pink1 mutant (pink(-/-) ) zebrafish line with a premature stop mutation (Y431*) in the PINK1 kinase d...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.23999
更新日期:2013-12-01 00:00:00
abstract::Patients with senile dementia of the Alzheimer type frequently have difficulty performing visual tasks. These difficulties may be due, at least partially, to degenerative changes in both the primary visual pathway and the visual association areas. To determine whether retinal ganglion cell dysfunction contributes to v...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410260208
更新日期:1989-08-01 00:00:00
abstract::Hypoxic-ischemic brain injury in survivors of perinatal asphyxia is a frequently encountered clinical problem for which there is currently no effective therapy. Neurotrophins, such as brain-derived neurotrophic factor (BDNF), can protect responsive neurons against cell death in some injury paradigms. While the role of...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410410416
更新日期:1997-04-01 00:00:00
abstract::We have previously shown bilateral activation of motor pathways and the recruitment of additional motor areas in studies of groups of patients with recovery from motor stroke. We have now developed a new positron emission tomographic technique to measure the changes in regional cerebral blood flow elicited during a mo...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410330208
更新日期:1993-02-01 00:00:00
abstract::Imaging of cerebrovascular beta-amyloid (cerebral amyloid angiopathy) is complicated by the nearly universal overlap of this pathology with Alzheimer's pathology. We performed positron emission tomographic imaging with Pittsburgh Compound B on 42-year-old man with early manifestations of Iowa-type hereditary cerebral ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21528
更新日期:2008-11-01 00:00:00
abstract::Stereotaxic injection of kainic acid into rat striatum produces neuronal degeneration and neurochemical alterations resembling Huntington's disease (HD). Since correction of the deficiency in gamma-aminobutyric acid (GABA) in HD may be of therapeutic value, the efficacy of drugs that inhibit the GABA-degrading enzyme ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410020407
更新日期:1977-10-01 00:00:00
abstract::We report the clinical features of 12 patients with drop attacks associated with Meniere's syndrome. Each described a sensation of being pushed, thrown, or knocked to the ground or a sudden illusion of movement of the environment that led to a fall. These episodes were not accompanied by symptoms of their typical atta...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410280314
更新日期:1990-09-01 00:00:00
abstract::To examine the antiparkinsonian effects of blocking glycineB receptors, we designed a pilot study testing the potent and selective antagonist, PAMQX, in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-treated primates. PAMQX had no intrinsic effects but markedly potentiated the antiparkinsonian action of levodopa. In a d...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20279
更新日期:2004-11-01 00:00:00
abstract::Human T-cell lymphotropic virus type III (HTLV-III) has been isolated from neural tissues and cerebrospinal fluid (CSF) of patients with neurological syndromes associated with the acquired immune deficiency syndrome (AIDS) and the virus may be directly involved in the pathogenesis of the syndromes. To detect HTLV-III ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410200304
更新日期:1986-09-01 00:00:00
abstract:OBJECTIVE:GM2 gangliosidoses are lysosomal diseases due to biallelic mutations in the HEXA (Tay-Sachs disease [TS]) or HEXB (Sandhoff disease [SD]) genes, with subsequent low hexosaminidase(s) activity. Most patients have childhood onset, but some experience the first symptoms during adolescence/adulthood. This study a...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25689
更新日期:2020-04-01 00:00:00
abstract::Although most therapeutic efforts and experimental stroke models focus on the concept of complete occlusion of the middle cerebral artery as a result of embolism from the carotid artery or cardiac chamber, relatively little is known about the stroke mechanism of intrinsic middle cerebral artery stenosis. Differences i...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10250
更新日期:2002-07-01 00:00:00
abstract::We report an immunohistochemical study of the mitochondrial alpha-ketoglutarate dehydrogenase complex (KGDHC) in the substantia nigra in Parkinson's disease. The KGDHC, the three enzyme complex catalyzing the oxidation of alpha-ketoglutarate to succinate through succinic semialdehyde, is the rate-regulating enzyme of ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410350212
更新日期:1994-02-01 00:00:00
abstract::Two members of a family with a neuropathy resembling Charcot-Marie-Tooth disease were unable to relax their muscles after voluntary contraction. Muscle spasm often outlasted voluntary contraction by 30 seconds or more before subsiding into myokymia and fasciculations. The posture of the hand during muscle spasm resemb...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410050605
更新日期:1979-06-01 00:00:00
abstract::The duration of the antiparkinsonian action of levodopa was studied in 48 patients with various response patterns to the oral administration of the dopamine precursor. Deterioration in motor scores after abrupt cessation of a steady-state intravenous levodopa infusion occurred at two successive rates: an initial rapid...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410240303
更新日期:1988-09-01 00:00:00
abstract::Axonal degeneration is a major cause of permanent neurological deficit in multiple sclerosis (MS), but no current therapies for the disease are known to be effective at axonal protection. Here, we examine the ability of a sodium channel-blocking agent, flecainide, to reduce axonal degeneration in an experimental model...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20045
更新日期:2004-05-01 00:00:00
abstract::Serological evidence of either acute cytomegalovirus (CMV) or Epstein-Barr virus (EBV) infection was sought in a large series of patients with Guillain-Barré syndrome (GBS) and control subjects. Using an indirect immunofluorescent technique, IgM antibody directed against CMV was found in the serum of 33 of 220 GBS pat...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410090709
更新日期:1981-01-01 00:00:00
abstract::Infection by the human immunodeficiency virus type 1 (HIV-1) often results in neurological dysfunction including HIV dementia (HIVD). Alterations in cytokine and redox balance are thought to play important roles in the pathogenesis of HIVD, but the specific mechanisms underlying neuronal dysfunction and death are unkn...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10828
更新日期:2004-02-01 00:00:00
abstract::Previous studies using two seizure paradigms, electroconvulsive shock and kindling, suggested potential sites of endogenous thyrotropin-releasing hormone (TRH) action in specific epileptogenic areas. We studied TRH gene expression and TRH receptors in rat limbic areas using the kindling model of epilepsy. Immunoassaya...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410330112
更新日期:1993-01-01 00:00:00
abstract::Biallelic DNAJC12 mutations were described in children with hyperphenylalaninemia, neurodevelopmental delay, and dystonia. We identified DNAJC12 homozygous null variants (c.187A>T;p.K63* and c.79-2A>G;p.V27Wfs*14) in two kindreds with early-onset parkinsonism. Both probands had mild intellectual disability, mild nonpr...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25048
更新日期:2017-10-01 00:00:00
abstract::We performed magnetic resonance imaging and magnetic resonance spectroscopic imaging on 28 patients with multiple sclerosis stratified for disability and clinical course (relapsing with at least partial remissions or secondary progressive disease). Lesions were segmented on the conventional proton density and T2-weigh...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.410410314
更新日期:1997-03-01 00:00:00
abstract:OBJECTIVE:In multiple sclerosis (MS), cerebral gray matter (GM) atrophy correlates more strongly than white matter (WM) atrophy with disability. The corresponding relationships in the spinal cord (SC) are unknown due to technical limitations in assessing SC GM atrophy. Using phase-sensitive inversion recovery (PSIR) ma...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24241
更新日期:2014-10-01 00:00:00
abstract::Mutations in DJ-1 (PARK7) have been reported in two consanguineous families with young-onset Parkinson's disease (YOPD). This study aims to confirm the presence of pathogenic DJ-1 mutations and determine their contribution in young-onset and more typical later onset Parkinson's disease (PD). The entire open reading fr...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10675
更新日期:2003-09-01 00:00:00
abstract::We performed dynamic [18F]fluorodeoxyglucose ([18F]FDG) positron emission tomographic (PET) analyses in 8 patients. Rate constants of influx (K1*), efflux (k2*), phosphorylation (k3*), and dephosphorylation (k4*) were derived for the regions of interest (ROIs), which included (1) the hypometabolic epileptogenic region...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410430615
更新日期:1998-06-01 00:00:00
abstract::A 30-year-old male drug abuser developed ophthalmoplegia, bulbar paralysis, and limb weakness responsive to edrophonium. However, potentiation of a low-amplitude evoked muscle action potential was produced with repetitive nerve stimulation at 10 Hz, and the clinical and electrophysiological data suggested the diagnosi...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410160314
更新日期:1984-09-01 00:00:00
abstract::If patients with left-sided hemispatial neglect bisect lines incorrectly because hemianopia or sensory hemiinattention prevents them from seeing how far the line extends to the left, a strategy that ensures their seeing the left side of the line in their normal field should improve performance. If patients have hemisp...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410050210
更新日期:1979-02-01 00:00:00
abstract::An in vitro preparation has been developed in which epileptogenesis in mammalian central nervous system tissue may be studied. Addition of sodium penicillin to the medium bathing slices of guinea pig hippocampus induced epileptiform activity similar to that seen in hippocampal penicillin foci in vivo. Epileptiform eve...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410010510
更新日期:1977-05-01 00:00:00
abstract::We recorded horizontal smooth-pursuit responses to sinusoidal and step-ramp stimuli in 7 patients with unilateral frontal lobe lesions. Five patients had directional smooth-pursuit deficits, all with impairment toward the side of cerebral damage. Ipsidirectional pursuit defects involved pursuit maintenance to sinusoid...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410370406
更新日期:1995-04-01 00:00:00
abstract::We here report the second and third mutations in alphaB-crystallin causing myofibrillar myopathy. Two patients had adult-onset muscle weakness. Patient 1 had cervical, limb girdle, and respiratory muscle weakness and died of respiratory failure. Patient 2 had proximal and distal leg muscle weakness. Both had myopathic...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10767
更新日期:2003-12-01 00:00:00