Cerebellar hemorrhage complicating isovaleric acidemia: a case report.

abstract：:Wolfram syndrome is an autosomal recessive disorder beginning in childhood that consists of four cardinal features: optic atrophy, diabetes mellitus, diabetes insipidus, and neurosensory hearing loss. Aside from these features, the clinical picture is highly variable and may include other neurologic abnormalities such...

journal_title：Neurology

authors： Rando TA,Horton JC,Layzer RB

更新日期：1992-06-01 00:00:00

abstract：OBJECTIVE:We sought to determine the association of dietary factors and risk of cognitive decline in a population at high risk of cardiovascular disease. METHODS:Baseline dietary intake and measures of the Mini-Mental State Examination were recorded in 27,860 men and women who were enrolled in 2 international parallel...

journal_title：Neurology

authors： Smyth A,Dehghan M,O'Donnell M,Anderson C,Teo K,Gao P,Sleight P,Dagenais G,Probstfield JL,Mente A,Yusuf S,ONTARGET and TRANSCEND Investigators.

更新日期：2015-06-02 00:00:00

abstract：:The American Academy of Neurology has published a comprehensive review and guidelines for the use of disease-modifying therapies (DMTs) in multiple sclerosis (MS) for the first time since 2002. These guidelines represent the work of MS experts, patients, and guideline experts and are based on their review of randomize...

journal_title：Neurology

authors： Corboy JR,Weinshenker BG,Wingerchuk DM

更新日期：2018-06-12 00:00:00

abstract：OBJECTIVE:To determine serial MRI and CT abnormalities around calcified cysts due to cysticercosis in previously treated patients during periods of seizure activity. BACKGROUND:Some patients with calcified lesions due to cysticercosis have seizures. How and why seizures occur in this setting are unknown. METHODS:Thre...

journal_title：Neurology

authors： Nash TE,Patronas NJ

更新日期：1999-09-11 00:00:00

abstract：:The ability to deliver enzymatically active human hexosaminidase A across the blood-brain barrier and into brain cells of the normal rat was examined. Following osmotic blood-brain barrier modification in the rat, intraarterially administered human hexosaminidase A and B were shown to cross the barrier and enter brain...

journal_title：Neurology

authors： Neuwelt EA,Barranger JA,Pagel MA,Quirk JM,Brady RO,Frenkel EP

更新日期：1984-08-01 00:00:00

abstract：:We compared the number of new gadolinium-enhancing and T2-weighted lesions on six monthly MRI scans in eight patients with relapsing multiple sclerosis before and during treatment with weekly intramuscular interferon beta-1a (Avonex; 30 microg). MRI activity was modestly reduced during treatment (p = 0.016). The treat...

journal_title：Neurology

authors： Waubant E,Goodkin DE,Sloan R,Andersson PB

更新日期：1999-09-11 00:00:00

abstract：:The authors report an unusual case of venous infarction of the spinal cord associated with endoscopic sclerotherapy for esophageal varices. MR imaging findings included signal abnormalities and abnormal enhancement of the affected spinal cord and T-8 vertebral body. A review of the hemodynamic changes associated with ...

journal_title：Neurology

authors： Heller SL,Meyer JR,Russell EJ

更新日期：1996-10-01 00:00:00

abstract：OBJECTIVE:To perform a single-center pilot investigation of early hematoma removal in patients with intracerebral hemorrhage (ICH). BACKGROUND:Considerable debate remains regarding the utility of surgical clot evacuation for ICH. METHODS:This was a prospective trial of open craniotomy within 12 hours of ICH symptom o...

journal_title：Neurology

authors： Morgenstern LB,Frankowski RF,Shedden P,Pasteur W,Grotta JC

更新日期：1998-11-01 00:00:00

abstract：:Many aging-associated neurologic disorders, including primary malignant brain tumors (MBT), share a common biphasic age-specific mortality rate pattern: initially increasing exponentially with age, and then declining. A modeling study using MBT mortality was conducted to determine if the observed biphasic pattern of M...

journal_title：Neurology

authors： Riggs JE

更新日期：2000-09-26 00:00:00

abstract：OBJECTIVE:To determine the patterns of diffusivity associated with cognitive domain functions in older adults without dementia. METHODS:We studied older adults without dementia (n = 220) who underwent neuropsychometric testing and a diffusion tensor imaging (DTI) examination at 3 T in a cross-sectional study. Memory, ...

journal_title：Neurology

authors： Kantarci K,Senjem ML,Avula R,Zhang B,Samikoglu AR,Weigand SD,Przybelski SA,Edmonson HA,Vemuri P,Knopman DS,Boeve BF,Ivnik RJ,Smith GE,Petersen RC,Jack CR Jr

更新日期：2011-07-05 00:00:00

abstract：:The authors report two cases of patients with word deafness. The word deafness occurred after a head injury for the first patient and after an arterio venous malformation embolization for the second patient. MRI demonstrated bilateral lesions of the inferior colliculi but brainstem auditory-evoked potentials (BAEP) we...

journal_title：Neurology

authors： Vitte E,Tankéré F,Bernat I,Zouaoui A,Lamas G,Soudant J

更新日期：2002-03-26 00:00:00

abstract：:We investigated blood-brain barrier (BBB) function in relation to Alzheimer's disease (AD) and vascular dementia (VAD) in the very elderly. Sixty-five 85-year-old persons from a population-based sample were followed for 3 years; 29 were demented at age 85 (13 with AD, 14 with VAD, and 2 with other dementias), 7 develo...

journal_title：Neurology

authors： Skoog I,Wallin A,Fredman P,Hesse C,Aevarsson O,Karlsson I,Gottfries CG,Blennow K

更新日期：1998-04-01 00:00:00

abstract：:We studied five patients with nonhemorrhagic thalamic infarction with neuropsychological tests, CT, and somatosensory evoked responses (SERs). The three patients with left thalamic lesions had abnormalities of language, memory, visuospatial processing, intellect, and personality-changes compatible with dementia. The t...

journal_title：Neurology

authors： Graff-Radford NR,Eslinger PJ,Damasio AR,Yamada T

更新日期：1984-01-01 00:00:00

abstract：:Acute myopathy and myoglobinuria with markedly elevated creatine kinase (CK) activity developed in an 18-year-old boy on two occasions after gasoline sniffing. No signs of CNS involvement were seen, and the symptoms receded in a few days. The amount of lead in blood and urine exceeded the reference value for subjects ...

journal_title：Neurology

authors： Kovanen J,Somer H,Schroeder P

更新日期：1983-05-01 00:00:00

abstract：OBJECTIVE:Given the limited information on cognitive function before Parkinson disease (PD) clinical onset in the general population, we sought to assess prodromal PD (pPD) probability and relate it to detailed cognitive performance in a community cohort. METHODS:In a population-based cohort of 1,629 dementia-free and...

journal_title：Neurology

authors： Bougea A,Maraki MI,Yannakoulia M,Stamelou M,Xiromerisiou G,Kosmidis MH,Ntanasi E,Dardiotis E,Hadjigeorgiou GM,Sakka P,Anastasiou CA,Stefanis L,Scarmeas N

更新日期：2019-05-07 00:00:00

abstract：:Four toluene abusers had evidence of severe multifocal central nervous system damage. Impairment of cognitive, cerebellar, brainstem, auditory, and pyramidal tract function, as well as CT evidence of cerebral cortical, cerebellar, and brainstem atrophy, have been noted. In addition, we found opsoclonus, ocular flutter...

journal_title：Neurology

authors： Lazar RB,Ho SU,Melen O,Daghestani AN

更新日期：1983-10-01 00:00:00

abstract：OBJECTIVE:To examine the relationship between carotid artery stenosis, other risk factors, and lacunar stroke. BACKGROUND:Carotid artery stenosis in patients presenting with lacune stroke may be coincidental or causal. The distinction by risk factor profile is uncertain. The risk and cause of subsequent stroke, and be...

journal_title：Neurology

authors： Inzitari D,Eliasziw M,Sharpe BL,Fox AJ,Barnett HJ

更新日期：2000-02-08 00:00:00

abstract：OBJECTIVE:Compare the cost and effectiveness of nonbenzodiazepine antiepileptic drugs (non-BZD AEDs) for treatment of BZD-resistant convulsive status epilepticus (SE). METHODS:Decision analysis model populated with effectiveness data from a systematic review and meta-analysis of the literature, and cost data from publ...

journal_title：Neurology

authors： Sánchez Fernández I,Gaínza-Lein M,Lamb N,Loddenkemper T

更新日期：2019-05-14 00:00:00

abstract：OBJECTIVE:To validate a neuropsychological algorithm for dementia diagnosis. METHODS:We developed a neuropsychological algorithm in a sample of 1,023 elderly residents of Cache County, UT. We compared algorithmic and clinical dementia diagnoses both based on DSM-III-R criteria. The algorithm diagnosed dementia when th...

journal_title：Neurology

authors： Tschanz JT,Welsh-Bohmer KA,Skoog I,West N,Norton MC,Wyse BW,Nickles R,Breitner JC

更新日期：2000-03-28 00:00:00

abstract：:In multifocal motor neuropathy (MMN), little is known of the long-term effect of human immunoglobulin (HIG) infusions as a maintenance therapy. We report a patient who improved after an initial HIG infusion of 2 g/kg and continued to improve both clinically and electrophysiologically during maintenance HIG treatment c...

journal_title：Neurology

authors： Van den Berg LH,Franssen H,Wokke JH

更新日期：1995-05-01 00:00:00

abstract：:This article presents the findings relating to the European subgroup of 91 patients in an international survey of the current diagnosis and treatment of patients with amyotrophic lateral sclerosis (ALS). The mean time between first symptoms and first consultation with a physician was 4.9 months, and mean delay in seei...

journal_title：Neurology

authors： Dengler R

更新日期：1999-01-01 00:00:00

abstract：BACKGROUND:TCH346 exerts antiapoptotic effects by binding to glyceraldehyde 3-phosphate dehydrogenase (GAPDH) and blocking the apoptotic pathway in which GAPDH is involved. Apoptosis is considered to be a key pathogenic mechanism in neurodegenerative diseases including ALS. METHODS:Patients were randomly assigned in a...

journal_title：Neurology

authors： Miller R,Bradley W,Cudkowicz M,Hubble J,Meininger V,Mitsumoto H,Moore D,Pohlmann H,Sauer D,Silani V,Strong M,Swash M,Vernotica E,TCH346 Study Group.

更新日期：2007-08-21 00:00:00

abstract：OBJECTIVE:To report the assessment of a patient exhibiting gustatory agnosia. METHODS:Preoperative and postoperative neuropsychological, neuroimaging, and chemosensory evaluations were performed in a 39-year-old woman undergoing surgical treatment for intractable epilepsy. RESULTS:Preoperative MRIs showed bilateral (...

journal_title：Neurology

authors： Small DM,Bernasconi N,Bernasconi A,Sziklas V,Jones-Gotman M

更新日期：2005-01-25 00:00:00

abstract：OBJECTIVE:Precise genetic analyses were conducted with ring finger protein 213 (RNF213) in relation to a particular clinical phenotype in Chinese patients with moyamoya disease (MMD) to determine whether heterozygosity is responsible for the early-onset and severe form of this disease. METHODS:A case-control study for...

journal_title：Neurology

authors： Wang Y,Zhang Z,Wei L,Zhang Q,Zou Z,Yang L,Li D,Shang M,Han C,Mambiya M,Bao X,Li Q,Hao F,Zhang K,Wang H,Liu S,Liu M,Zeng F,Nie F,Wang K,Liu W,Duan L

更新日期：2020-02-18 00:00:00

abstract：OBJECTIVE:To report pathologic findings in 124 Australian and North American cases of primary nemaline myopathy. METHODS:Results of 164 muscle biopsies from 124 Australian and North American patients with primary nemaline myopathy were reviewed, including biopsies from 19 patients with nemaline myopathy due to alpha-a...

journal_title：Neurology

authors： Ryan MM,Ilkovski B,Strickland CD,Schnell C,Sanoudou D,Midgett C,Houston R,Muirhead D,Dennett X,Shield LK,De Girolami U,Iannaccone ST,Laing NG,North KN,Beggs AH

更新日期：2003-02-25 00:00:00

abstract：BACKGROUND:The prognostic value of the biochemical changes seen with proton MR spectroscopy (1H MRS) in ischemic stroke was examined. Acute diffusion-weighted imaging (DWI) was used to identify regions of ischemia for 1H MRS voxel localization. METHODS:Nineteen patients had 36 1H MRS studies, 13 patients acutely (mean...

journal_title：Neurology

authors： Parsons MW,Li T,Barber PA,Yang Q,Darby DG,Desmond PM,Gerraty RP,Tress BM,Davis SM

更新日期：2000-08-22 00:00:00

abstract：BACKGROUND:Ullrich congenital muscular dystrophy (UCMD) is a form of merosin-positive congenital muscular dystrophy characterized by proximal contractures, distal laxity, rigidity of the spine, and respiratory complications. Recently, a deficiency of collagen VI on muscle and skin biopsy together with recessive mutatio...

journal_title：Neurology

authors： Mercuri E,Yuva Y,Brown SC,Brockington M,Kinali M,Jungbluth H,Feng L,Sewry CA,Muntoni F

更新日期：2002-05-14 00:00:00

abstract：:We studied 10 patients with transient global amnesia (TGA). Routine electroencephalograms (EEGs) were normal in six; two showed mild temporal lobe abnormalities, one had bitemporal delta activity with rare sharp waves, and one showed bioccipital rhythmic theta activity. Nasopharyngeal (NP) recordings were obtained in ...

journal_title：Neurology

authors： Rowan AJ,Protass LM

更新日期：1979-06-01 00:00:00

abstract：OBJECTIVE:To investigate the temporal relationship between inflammation and cerebral atrophy in a longitudinal study of 19 patients with relapsing-remitting multiple sclerosis (RRMS) using serial monthly contrast enhanced MRI examinations and monthly measurements of brain fractional volume (BFV) for an average of 4 (ra...

journal_title：Neurology

authors： Richert ND,Howard T,Frank JA,Stone R,Ostuni J,Ohayon J,Bash C,McFarland HF

更新日期：2006-02-28 00:00:00

abstract：OBJECTIVE:To study the presenting features and value of routine diagnostic procedures in patients with leptomeningeal metastases (LMM) related to the primary malignancy to improve diagnostic assessment. METHODS:The authors studied the presenting features and value of routine diagnostic procedures in relation to the hi...

journal_title：Neurology

authors： van Oostenbrugge RJ,Twijnstra A

更新日期：1999-07-22 00:00:00