Abstract:
:Using proton magnetic resonance spectroscopic imaging, we studied the cerebellum of 9 patients with cerebellar degeneration and of 9 age-matched normal control subjects. This technique permits the simultaneous measurement of N-acetylaspartate, choline-containing compounds, creatine/phosphocreatine, and lactate signal intensities from four 15-mm slices divided into 0.84-ml single-volume elements. Because patients with cerebellar degeneration often show substantial atrophy on magnetic resonance imaging (MRI), we specifically chose to analyze the spectroscopic signals only from tissue that did not have an atrophic appearance on the MRI. The spectroscopic findings showed a significant reduction of N-acetylaspartate in all parts of the cerebellum, a significant correlation with MRI scores of cerebellar atrophy, and a significant correlation with clinical rating scores of cerebellar disturbance. Our method of analysis suggests the presence of a neurodegenerative process in cerebellar areas that do not appear to be atrophic on the MRI. Some limitations of proton magnetic resonance spectroscopic imaging in the present study were related to the partial field inhomogeneity characteristics of the posterior fossa, the anatomical location of the cerebellum, and the particularly severe cerebellar atrophy in some of the patients.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Tedeschi G,Bertolino A,Massaquoi SG,Campbell G,Patronas NJ,Bonavita S,Barnett AS,Alger JR,Hallett Mdoi
10.1002/ana.410390111subject
Has Abstractpub_date
1996-01-01 00:00:00pages
71-8issue
1eissn
0364-5134issn
1531-8249journal_volume
39pub_type
杂志文章abstract::We describe the clinical and oculographic findings in 4 families with episodic ataxia and interictal nystagmus (EA-2) linked to chromosome 19p. Episodes varied from pure ataxia to combinations of symptoms suggesting involvement of the cerebellum, brainstem, and cortex. Some affected individuals exhibited a progressive...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410410105
更新日期:1997-01-01 00:00:00
abstract:OBJECTIVE:Recanalization of arterial obstruction is associated with improved clinical outcomes. There are no controlled data demonstrating whether arterial obstruction status predicts the treatment effect of intravenous (IV) tissue plasminogen activator (tPA). We aimed to determine if the presence of arterial obstructi...
journal_title:Annals of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ana.22444
更新日期:2011-10-01 00:00:00
abstract::We present the clinicopathological features of two siblings from a consanguineous marriage who presented with respiratory hypoventilation and died 10 days and 4 years later, respectively. This disorder showed extensive tau neuropathology, and both had a novel homozygous S352L tau gene mutation. This is the first descr...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10747
更新日期:2003-11-01 00:00:00
abstract::Cytogenetic analysis has become an important part of the diagnostic evaluation of most hematological neoplasms. However, there is limited information on the value of cytogenetic analysis in most solid tumors, including cerebral astrocytomas. This report summarizes a prospective cytogenetic study of 99 human cerebral a...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410310512
更新日期:1992-05-01 00:00:00
abstract:OBJECTIVE:To assess risk factors associated with care for stroke symptoms. METHODS:Using data from the population-based national cohort study (REasons for Geographic And Racial Differences in Stroke) conducted January 25, 2003-February 28, 2007 (N = 23,664), we assessed care-seeking behavior among 3,668 participants w...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21357
更新日期:2008-04-01 00:00:00
abstract:OBJECTIVE:We examined the sarcolemma of skeletal muscle from patients with facioscapulohumeral muscular dystrophy (FSHD1A) to learn if, as in other murine and human muscular dystrophies, its organization and relationship to nearby contractile structures are altered. METHODS:Unfixed biopsies of control and FSHD deltoid...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20750
更新日期:2006-02-01 00:00:00
abstract::The site of autonomic deficit was investigated in 4 patients with loss of thermoregulatory flushing and sweating on one side of the face but no associated miosis (harlequin syndrome). In 2 patients the sudomotor deficit included the hand. Signs of postganglionic sympathetic deficit included pathological lacrimal sweat...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340610
更新日期:1993-12-01 00:00:00
abstract::Alexander's law refers to the phenomenon in which the spontaneous nystagmus of a patient with a vestibular lesion is more intense when the patient looks in the quick-phase than in the slow-phase direction. Alexander's law was investigated in normal subjects as well as patients with vestibular lesions. During sinusoida...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410160614
更新日期:1984-12-01 00:00:00
abstract::Malignant tumors are known to have a "remote" or nonmetastatic effect on the central and peripheral nervous systems. Eight patients were seen with proximal muscle weakness in association with bronchogenic carcinoma (5), carcinoma of breast (2), and leukemia (1). Electromyography demonstrated small polyphasic motor uni...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410040314
更新日期:1978-09-01 00:00:00
abstract::We recently reported that systemic administration of a proteasome inhibitor induced a progressive levodopa-responsive, bradykinetic syndrome in rats with imaging, pathological, and biochemical features that strikingly resemble what is found in PD. This model has the potential to be a useful tool for studying the mecha...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20936
更新日期:2006-08-01 00:00:00
abstract::We examined the expression of Fas antigen and Bcl-2 protein in thymic tissue surgically resected from 10 patients with myasthenia gravis, using immunocytochemical techniques. Histologically, thymic tissues from 7 myasthenia gravis patients showed hyperplasia, while 3 other patients had thymomas. In hyperplastic thymic...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410390414
更新日期:1996-04-01 00:00:00
abstract::In a prospective phase I/II clinical study, we treated eight patients suffering from recurrent glioblastoma multiform with stereotactically guided intratumoral convection-enhanced delivery of an HSV-1-tk gene-bearing liposomal vector and systemic ganciclovir. Noninvasive identification of target tissue together with a...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.10688
更新日期:2003-10-01 00:00:00
abstract:OBJECTIVE:Alterations of the gut microbiome in Parkinson disease (PD) have been repeatedly demonstrated. However, little is known about whether such alterations precede disease onset and how they relate to risk and prodromal markers of PD. We investigated associations of these features with gut microbiome composition. ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25788
更新日期:2020-08-01 00:00:00
abstract::Although the static encephalopathies of childhood are common, little is known of the underlying alterations in the neurochemical anatomy of the brain. Using quantitative morphological techniques, we examined the effect of perinatal hypoxia-ischemia on the number and distribution of cholinergic (ACh) neurons in a unila...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410270113
更新日期:1990-01-01 00:00:00
abstract::Lead intoxication in rats reliably produces segmental demyelination. Following a single intravenous injection of radioactive lead, localization of tracer was observed sequentially by quantitative electron microscopical autoradiography. The animals injected had been on a lead-containing diet for 70 days; as a result, t...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410180206
更新日期:1985-08-01 00:00:00
abstract::Intellectual disability is common and is associated with significant morbidity. Until the latter half of the 20th century, there were no efficacious treatments. Following initial breakthroughs associated with newborn screening and metabolic corrections, little progress was made until recently. With improved understand...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.24002
更新日期:2013-09-01 00:00:00
abstract::Passive joint position sense was tested in 10 subjects after unilateral total hip replacement surgery (which included capsulectomy). Varied initial limb position, amplitude, and velocity of movement were used. The unoperated side was used as a control. Statistically barely significant errors (p less than 0.025) on the...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410130612
更新日期:1983-06-01 00:00:00
abstract::In view of the favorable claims of Cook and others, an open trial of chronic dorsal column stimulation was undertaken in 9 patients who had moderately advanced multiple sclerosis (MS) but were still ambulatory. No objective improvement was noted by either author during the postoperative observation period of 6 to 37 m...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410060115
更新日期:1979-07-01 00:00:00
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 n...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20529
更新日期:2005-07-01 00:00:00
abstract::Fourteen patients with cauda equina syndrome secondary to long-standing ankylosing spondylitis are described. The roughly symmetrical neurological deficits were very slowly progressive and began long after the onset of the spondylitis, usually well after the rheumatological symptoms had stopped. Eventually every patie...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410140610
更新日期:1983-12-01 00:00:00
abstract::A young woman with Kearns-Sayre syndrome and progressive central nervous system deterioration over 15 years had decreased plasma and cerebrospinal fluid folate levels while receiving phenytoin for a seizure disorder. A muscle biopsy showed a "ragged red fiber" myopathy with reduced muscle carnitine and mitochondrial e...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410130620
更新日期:1983-06-01 00:00:00
abstract::A 51-year-old man with non-HLA-DR2 histocompatibility developed classic signs and symptoms of the narcoleptic tetrad soon after recovering from an episode of cardiopulmonary insufficiency, which occurred during induction of surgical anesthesia. Symptoms included excessive daytime sleepiness, hypnagogic hallucinations,...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410190516
更新日期:1986-05-01 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:OBJECTIVE:Neuronal loss, a key substrate of irreversible disability in multiple sclerosis (MS), is a recognized feature of MS cortical pathology of which the cause remains unknown. Fibrin(ogen) deposition is neurotoxic in animal models of MS, but has not been evaluated in human progressive MS cortex. The aim of this st...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24997
更新日期:2017-08-01 00:00:00
abstract::Oxidative damage to DNA may play a role in both normal aging and in neurodegenerative diseases. We examined whether Alzheimer's disease (AD) is associated with increased oxidative damage to nDNA and mtDNA in postmortem brain tissue. We measured the oxidized nucleoside, 8-hydroxy-2'-deoxyguanosine (OH8dG), in DNA isola...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410360510
更新日期:1994-11-01 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:Acquired epilepsy is a devastating long-term risk of various brain insults, including trauma, stroke, infections, and status epilepticus (SE). There is no preventive treatment for patients at risk. Attributable to the complex alterations involved in epileptogenesis, it is likely that multitargeted approaches ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24804
更新日期:2016-12-01 00:00:00
abstract::Monoclonal antibodies that target CD20 expressing B cells represent an important new treatment option for patients with multiple sclerosis (MS). B-cell-depleting therapy is highly effective against relapsing forms of the disease and is also the first treatment approach proven to protect against disability worsening in...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.25119
更新日期:2018-01-01 00:00:00
abstract::Based on prior reports of free light chains of immunoglobulin G (IgG) in the cerebrospinal fluid (CSF) of patients with multiple sclerosis (MS), we quantitated free kappa and lambda chains and whole IgG concentrations using sensitive and specific radioimmunoassays (RIAs). The RIA for free kappa chains had a sensitivit...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410200111
更新日期:1986-07-01 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