Abstract:
:A woman had a progressive neurologic syndrome beginning at age 3 and lasting for three decades. Clinical manifestations included severe mental deterioration, spastic paralysis, myoclonus, and tremors. A postmortem examination showed ubiquitous infiltration of neurons by lipofuscin and deposits of pigment in the globus pallidus and substantia nigra, as well as senile changes of nerve cells. Biochemical investigation of brain lipids showed an alteration of fatty acid composition of serine phosphoglycerides.
journal_name
Neurologyjournal_title
Neurologyauthors
Jervis GA,Pullarkat RKdoi
10.1212/wnl.28.5.500subject
Has Abstractpub_date
1978-05-01 00:00:00pages
500-3issue
5eissn
0028-3878issn
1526-632Xjournal_volume
28pub_type
杂志文章相关文献
NEUROLOGY文献大全abstract::Systematic screening of many compounds in animal models led to identification of the established antiepileptic drugs (AEDs). By contrast, the newer AEDs were specifically designed to enhance an inhibitory process or to inhibit a specific excitatory pathway. However, it was later discovered that some of the designed dr...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:
更新日期:2000-01-01 00:00:00
abstract::Neurologic abnormalities were seen in 13 of 20 patients with a history of chronic solvent vapor (primarily toluene) abuse for 2 or more years. The patients were evaluated after an abstinence period of at least 4 weeks, to avoid neurologic effects of acute intoxication. Neurologic signs included cognitive (60%), pyrami...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.36.5.698
更新日期:1986-05-01 00:00:00
abstract::Manipulation of afferent inputs may temporarily modulate dystonic spasms. Ten patients with writer's cramp were enrolled in a double-blind, randomized, crossover study in which the effects of transcutaneous electrical stimulation (TENS) and placebo treatment were compared. Patients were evaluated using four measures o...
journal_title:Neurology
pub_type: 杂志文章,随机对照试验
doi:10.1212/01.WNL.0000163851.70927.7E
更新日期:2005-06-14 00:00:00
abstract:OBJECTIVE:To evaluate urinary neurotrophin receptor p75 extracellular domain (p75ECD) levels as disease progression and prognostic biomarkers in amyotrophic lateral sclerosis (ALS). METHODS:The population in this study comprised 45 healthy controls and 54 people with ALS, 31 of whom were sampled longitudinally. Urinar...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000003741
更新日期:2017-03-21 00:00:00
abstract:OBJECTIVE:To determine the association between traumatic brain injury (TBI) and any clinically diagnosed α-synucleinopathy including Parkinson disease (PD), dementia with Lewy bodies (DLB), PD dementia (PDD), and multiple system atrophy (MSA). METHODS:Using the medical records-linkage system of the Rochester Epidemiol...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000008995
更新日期:2020-02-25 00:00:00
abstract::This community-based study analyzed 54 patients with definite or probable tuberculous meningitis (TBM) in New Mexico from 1970 through 1990. Patients ranged in age from 4 months to 86 years. The highest age-specific incidence occurred in the elderly, but 22% of patients were less than 10 years old. Native American pat...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.43.9.1775
更新日期:1993-09-01 00:00:00
abstract::Previous anatomic and physiologic studies suggest brainstem dysfunction in cranial-cervical dystonia. To further explore this, we studied suprathreshold acoustic reflex waveforms in 15 such patients. A unique feature of this technique is its ability to reject movement artifacts before averaging the acoustic reflex wav...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.42.3.594
更新日期:1992-03-01 00:00:00
abstract:BACKGROUND:Expert knowledge may compensate for age-related declines in basic cognitive and sensory-motor abilities in some skill domains. We investigated the influence of age and aviation expertise (indexed by Federal Aviation Administration pilot ratings) on longitudinal flight simulator performance. METHODS:Over a 3...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000255943.10045.c0
更新日期:2007-02-27 00:00:00
abstract::Apraxia is the loss of the ability to perform learned skilled movements correctly. In right-handers, apraxia and aphasia are most frequently associated with left-hemisphere lesions. When they are dissociated, however, aphasia is more common in the absence of apraxia than vice versa. There are two hypotheses that can a...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.45.7.1379
更新日期:1995-07-01 00:00:00
abstract::A patient with a postoperative fistula of the left posterior semicircular canal is presented. Negative pressure in the external ear canal produced upbeat-torsional nystagmus, which was recorded in three dimensions using binocular scleral search coils. The nystagmus was conjugate, without skew deviation, and its trajec...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.54.10.2016
更新日期:2000-05-23 00:00:00
abstract:BACKGROUND AND OBJECTIVE:Posterior cortical atrophy (PCA) is a progressive neurodegenerative syndrome that presents with cortical visual dysfunction and relatively preserved memory. Although higher cortical visual syndromes are well known in PCA, visual field defects detected by computerized visual field (CVF) perimetr...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e31823e9f2a
更新日期:2011-12-13 00:00:00
abstract:BACKGROUND:After Pokémon viewing triggered an epidemic of seizures in Japan, many efforts have been made to design safety guidelines and systems to protect subjects with photosensitivity. The authors developed a new method based upon nonlinear diffusion techniques capable of filtering the epileptogenic content of a vid...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.WNL.0000152875.67527.61
更新日期:2005-03-08 00:00:00
abstract:OBJECTIVE:To review clinical and electrodiagnostic features of the medial brachial fascial compartment syndrome, a complication of percutaneous axillary vessel puncture. METHODS:The authors reviewed electrodiagnostic examinations over a 20-year period. RESULTS:This syndrome presents with weakness, pain, and numbness ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000089488.35632.08
更新日期:2003-10-28 00:00:00
abstract::To identify clinical and electrophysiologic features related to IV immunoglobulin (IVIg) responsiveness in chronic inflammatory demyelinating polyneuropathy (CIDP), the authors conducted a multicenter study on 312 patients with CIDP (199 responders and 113 nonresponders). Muscle atrophy and decreased compound muscle a...
journal_title:Neurology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1212/01.WNL.0000158680.89323.F8
更新日期:2005-04-26 00:00:00
abstract::We describe a 78-year-old man with right hemiballism as the presenting symptom of a metastatic occult adenocarcinoma of the lung. CT demonstrated two contrast-enhancing lesions: one in the left subthalamic region and the other in the left parietal region. Metastatic tumor was confirmed at autopsy. ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.34.2.204
更新日期:1984-02-01 00:00:00
abstract::In a previously unreported family with olivopontocerebellar atrophy, the kindred contained over 600 individuals in five generations. Of 83 offsping of affected individuals who over over 38.8 years of age (the mean age of the onset of disease in this family), 47 had ataxia; there was autosomal dominant transmission. Cl...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.30.1.12
更新日期:1980-01-01 00:00:00
abstract::This paper reviews the issue of hepatotoxicity with the use of the catechol-O-methly transferase (COMT) inhibitors tolcapone and entacapone. Neither drug caused hepatotoxicity in preclinical toxicity testing. However, in clinical trials of tolcapone, liver chemistry tests were elevated more than 3 times above the uppe...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:
更新日期:2000-01-01 00:00:00
abstract:OBJECTIVE:To perform an open-label, long-term efficacy and safety/tolerability study of vagus nerve stimulation (VNS) of 454 patients with refractory epilepsy. METHODS:Subjects were enrolled from five clinical trials of VNS between 1988 and 1995 after undergoing an implantation of a pulse generator in the chest and a ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.53.8.1731
更新日期:1999-11-10 00:00:00
abstract:OBJECTIVE:To investigate whether sex and race differences exist in dementia diagnosis risk associated with traumatic brain injury (TBI) among older veterans. METHODS:Using Fine-Gray regression models, we investigated incident dementia diagnosis risk with TBI exposure by sex and race. RESULTS:After the exclusion of ba...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000010617
更新日期:2020-09-29 00:00:00
abstract::Some epilepsy patients quickly develop resistance to antiepileptic drugs (AEDs). We report a case of a patient with refractory epilepsy with daily seizures who initially responded to levetiracetam daily therapy, but then returned to baseline seizure frequency. However, when levetiracetam was given once weekly, the pat...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000198500.84481.da
更新日期:2006-02-28 00:00:00
abstract::A 33-year-old man with untreated hypertension had sudden onset of signs and symptoms suggestive of a dorsal lateral medullary syndrome. He died after 27 days. Postmortem studies revealed intramedullary hemorrhage with extension into the fourth ventricle and hypertensive cardiovascular disease. ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.35.6.925
更新日期:1985-06-01 00:00:00
abstract:OBJECTIVE:To investigate the association between early depressive behavior after stroke onset and occurrence of poststroke depression (PSD) at 3- and 12-month follow-up evaluations. METHODS:The study prospectively included 273 patients with first-ever single uncomplicated ischemic stroke. In the stroke unit, nurses sc...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.WNL.0000150935.05940.2D
更新日期:2005-02-08 00:00:00
abstract::The efficacy and safety of donepezil as a treatment for patients with mild to moderate Alzheimer's disease (AD) was investigated in a multicenter, double-blind study. Patients were randomly assigned to treatment with placebo (n = 162), 5 mg/d donepezil (n = 154), or 10 mg/d donepezil (n = 157) for 24 weeks followed by...
journal_title:Neurology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1212/wnl.50.1.136
更新日期:1998-01-01 00:00:00
abstract:OBJECTIVE:The natural history of succinic semialdehyde dehydrogenase (SSADH) deficiency in adulthood is unknown; we elucidate the clinical manifestations of the disease later in life. METHODS:A 63-year-old man with long-standing intellectual disability was diagnosed with SSADH deficiency following hospitalization for ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000001906
更新日期:2015-09-08 00:00:00
abstract:OBJECTIVE:Pigmented orthochromatic leukodystrophy (POLD) and hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) are rare neurodegenerative disorders characterized by cerebral white matter abnormalities, myelin loss, and axonal swellings. The striking overlap of clinical and pathologic features of these...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e31828726a7
更新日期:2013-03-12 00:00:00
abstract::There was a report of spongiform encephalopathy transmitted to Syrian hamsters by intracerebral inoculation with the blood buffy coat of patients with Alzheimer's disease (AD) and their unaffected first-degree relatives. We attempted to verify that report, taking measures to reduce the risk of contaminating samples wi...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.44.6.1111
更新日期:1994-06-01 00:00:00
abstract::A 52-year-old woman had a fatal intracerebral hemorrhage after dental manipulation. Normotensive in the past, the initial blood pressure was high but rapidly returned to normal. Necropsy showed no vascular malformation or evidence of hypertensive vascular disease. Clinical and experimental data show that stimulation o...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.37.3.511
更新日期:1987-03-01 00:00:00
abstract:OBJECTIVE:To determine whether metformin is associated with a lower incidence of dementia than sulfonylureas. METHODS:This was a retrospective cohort study of US veterans ≥65 years of age with type 2 diabetes who were new users of metformin or a sulfonylurea and had no dementia. Follow-up began after 2 years of therap...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000004586
更新日期:2017-10-31 00:00:00
abstract:OBJECTIVE:To examine the role of mutations in GABRB3 encoding the β3 subunit of the GABAA receptor in individual patients with epilepsy with regard to causality, the spectrum of genetic variants, their pathophysiology, and associated phenotypes. METHODS:We performed massive parallel sequencing of GABRB3 in 416 patient...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000003565
更新日期:2017-01-31 00:00:00
abstract::We evaluated 85 patients with serologic evidence of Borrelia burgdorferi infection. Manifestations included encephalopathy (41), neuropathy (27), meningitis (2), multiple sclerosis (MS) (6), and psychiatric disorders (3). We performed lumbar punctures in 53, brain MRI in 33, and evoked potentials (EPs) in 33. Only pat...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.39.6.753
更新日期:1989-06-01 00:00:00