Evaluation of sleep and daytime somnolence in spinocerebellar ataxia type 6 (SCA6).

abstract：BACKGROUND:Mutations in the early growth response 2 (EGR2) gene have recently been found in patients with congenital hypomyelinating neuropathy and Charcot-Marie-Tooth type 1 (CMT1) disease. OBJECTIVE:To determine the frequency of EGR2 mutations in patients with a diagnosis of CMT1, Dejerine-Sottas syndrome (DSS), or ...

journal_title：Neurology

authors： Timmerman V,De Jonghe P,Ceuterick C,De Vriendt E,Löfgren A,Nelis E,Warner LE,Lupski JR,Martin JJ,Van Broeckhoven C

更新日期：1999-06-10 00:00:00

abstract：:We examined the cassette-recorded 24-hour ambulatory EEG findings in children who had either clinically definite seizures or episodic behavioral disturbances not regarded as epileptic on clinical grounds. Among 40 epileptic patients, 22 had one or more attacks during the 24-hour recording session. In 15 of these patie...

journal_title：Neurology

authors： Aminoff MJ,Goodin DS,Berg BO,Compton MN

更新日期：1988-04-01 00:00:00

abstract：:We report an infant with molybdenum cofactor deficiency (MCD) and a unique clinical presentation of hemiplegia, hypotonia, dystonia, and bilateral basal ganglia changes. Biochemistry revealed absent serum homocysteine, low concentrations of plasma cystine, high levels of urinary S-sulfocysteine and sulfite, and high l...

journal_title：Neurology

authors： Graf WD,Oleinik OE,Jack RM,Weiss AH,Johnson JL

更新日期：1998-09-01 00:00:00

abstract：:Migration from an area where MS is common to an area where it is rare (and vice versa) affects the risk of MS, provided migration occurs in childhood. A childhood infection might explain this effect. Therefore, the age pattern of infectious diseases in different regions was examined. A higher proportion of children sh...

journal_title：Neurology

authors： Alter M,Zhang ZX,Davanipour Z,Sobel E,Zibulewski J,Schwartz G,Friday G

更新日期：1986-10-01 00:00:00

abstract：:Human cytomegalovirus and herpes simplex virus DNA probes were labeled in vitro and hybridized to the DNA extracted from brains of patients with multiple sclerosis (MS) to detect the presence of genetic information related to these viruses. We were unable to detect any virus-related genetic information complementary t...

journal_title：Neurology

authors： Aulakh GS,Albrecht P,Tourtellotte WW

更新日期：1980-05-01 00:00:00

abstract：OBJECTIVES:Homonymous hemianopia (HH) is the most frequent visual-field defect after a stroke. Some of these patients also have visual hallucinations, the origin and frequency of which remain largely unknown. The aims of this work were to determine the occurrence of visual hallucinations among poststroke hemianopic pat...

journal_title：Neurology

authors： Martinelli F,Perez C,Caetta F,Obadia M,Savatovsky J,Chokron S

更新日期：2020-05-05 00:00:00

abstract：:Migraine, especially migraine with aura (MA), is an established risk factor for ischemic lesions of the brain. Recent evidence has also linked migraine to a broader range of ischemic vascular disorders including angina, myocardial infarction, coronary revascularization, claudication, and cardiovascular mortality. The ...

journal_title：Neurology

authors： Bigal ME,Kurth T,Hu H,Santanello N,Lipton RB

更新日期：2009-05-26 00:00:00

abstract：:To evaluate possible progressive metabolic changes in Duchenne muscular dystrophy, we used 31P nuclear magnetic resonance spectroscopy to measure high-energy phosphate compounds and phosphorylated diesters (PDE) in resting gastrocnemius muscle of 14 Duchenne patients and 10 normal boys. The patients had higher inorgan...

journal_title：Neurology

authors： Younkin DP,Berman P,Sladky J,Chee C,Bank W,Chance B

更新日期：1987-01-01 00:00:00

abstract：:This study reports the results of a prospective trial of nonoperative management of the cubital tunnel syndrome in 128 patients. Forty-three of the patients had bilateral ulnar nerve compression. At study's end, information was available on 94% of patients and 164 managed extremities, at a mean of 58.6 months of follo...

journal_title：Neurology

authors： Dellon AL,Hament W,Gittelshon A

更新日期：1993-09-01 00:00:00

abstract：:Medical therapy is the mainstay for epilepsy, with most patients well controlled on a single antiepileptic drug (AED). In this non-refractory group, many patients have medication side effects and occasional seizures. Approximately 30% of patients with partial epilepsy and 25% of patients with generalized epilepsy are ...

journal_title：Neurology

authors： Nadkarni S,LaJoie J,Devinsky O

更新日期：2005-06-28 00:00:00

abstract：OBJECTIVE:Structural and functional lesion localization in patients with hemispatial neglect. DESIGN:Location and severity of brain damage on CT and SPECT correlated with neglect performance as assessed with a battery of drawings, line bisection, and line and shape cancellation subtests. PATIENTS:Participants include...

journal_title：Neurology

authors： Leibovitch FS,Black SE,Caldwell CB,Ebert PL,Ehrlich LE,Szalai JP

更新日期：1998-04-01 00:00:00

abstract：OBJECTIVE:To assess the association of early morning serum cortisol with cognitive performance and brain structural integrity in community-dwelling young and middle-aged adults without dementia. METHODS:We evaluated dementia-free Framingham Heart Study (generation 3) participants (mean age 48.5 years, 46.8% men) who u...

journal_title：Neurology

authors： Echouffo-Tcheugui JB,Conner SC,Himali JJ,Maillard P,DeCarli CS,Beiser AS,Vasan RS,Seshadri S

更新日期：2018-11-20 00:00:00

abstract：:Acquired aphasia with convulsive disorder is an unusual condition in childhood, characterized by loss of language function associated with a paroxysmal electroencephalogram. To determine the course and outcome of this disorder, we evaluated nine patients 10 to 28 years after the onset of aphasia. Four patients had rec...

journal_title：Neurology

authors： Mantovani JF,Landau WM

更新日期：1980-05-01 00:00:00

abstract：:We followed 419 patients with a first, unprovoked, primarily or secondarily generalized tonic-clonic seizure, randomized to immediate antiepileptic treatment or to treatment only in the event of seizure recurrence. The probability of achieving a 2-year remission was 72 vs 57% at 3 months, 84 to 79% at 3 years, and 85 ...

journal_title：Neurology

authors： Leone MA,Solari A,Beghi E,FIRST Group.

更新日期：2006-12-26 00:00:00

abstract：OBJECTIVE:To characterize a large family with X-linked Charcot-Marie-Tooth (CMT) neuropathy without mutations in the gap junction protein B1 (GJB1) gene, which has an unusual phenotype that is different in some aspects from classic CMTX1. METHODS:We tested CMT families consistent with X-linked inheritance for GJB1 mut...

journal_title：Neurology

authors： Huttner IG,Kennerson ML,Reddel SW,Radovanovic D,Nicholson GA

更新日期：2006-12-12 00:00:00

abstract：BACKGROUND:Among patients with a patent foramen ovale (PFO) and a prior cryptogenic ischemic stroke or TIA, the absolute and relative risk of recurrent events is unclear. METHODS:We conducted a systematic review and meta-analysis of clinical studies in any language published up to February 2008. We included studies re...

journal_title：Neurology

authors： Almekhlafi MA,Wilton SB,Rabi DM,Ghali WA,Lorenzetti DL,Hill MD

更新日期：2009-07-14 00:00:00

abstract：OBJECTIVE:To assess the pathophysiologic changes in patients with spontaneous intracranial hypotension (SIH) based on measures of CSF dynamics, and on the duration of symptoms, in a retrospective case-controlled study. METHODS:We included consecutive patients investigated for SIH at our department from January 2012 to...

journal_title：Neurology

authors： Häni L,Fung C,Jesse CM,Ulrich CT,Miesbach T,Cipriani DR,Dobrocky T,Z'Graggen WJ,Raabe A,Piechowiak EI,Beck J

更新日期：2020-07-21 00:00:00

abstract：BACKGROUND:The head impulse test (HIT) is a useful bedside test to identify peripheral vestibular deficits. However, such a deficit of the vestibulo-ocular reflex (VOR) may not be diagnosed because corrective saccades cannot always be detected by simple observation. The scleral search coil technique is the gold standar...

journal_title：Neurology

authors： MacDougall HG,Weber KP,McGarvie LA,Halmagyi GM,Curthoys IS

更新日期：2009-10-06 00:00:00

abstract：OBJECTIVE:To quantify the effects of cognitive training (CT) on cognitive and behavioral outcome measures in patients with Parkinson disease (PD). METHODS:We systematically searched 5 databases for randomized controlled trials (RCTs) of CT in patients with PD reporting cognitive or behavioral outcomes. Efficacy was me...

journal_title：Neurology

authors： Leung IH,Walton CC,Hallock H,Lewis SJ,Valenzuela M,Lampit A

更新日期：2015-11-24 00:00:00

abstract：OBJECTIVE:To examine the long-term cognitive trajectories of individuals with normal cognition at baseline and distinct amyloid/tau/neurodegeneration (ATN) profiles. METHODS:Pooling data across 4 cohort studies, 814 cognitively normal participants (mean baseline age = 59.6 years) were classified into 8 ATN groups usin...

journal_title：Neurology

authors： Soldan A,Pettigrew C,Fagan AM,Schindler SE,Moghekar A,Fowler C,Li QX,Collins SJ,Carlsson C,Asthana S,Masters CL,Johnson S,Morris JC,Albert M,Gross AL

更新日期：2019-04-02 00:00:00

abstract：:We performed a 3-month, double-blind, placebo-controlled trial of 300 mg of gangliosides (Cronassial) in 40 outpatients with amyotrophic lateral sclerosis (ALS). We evaluated drug effect through physical examinations and symptom scales. Though this dosage had no major toxic effect, Cronassial treatment did not signifi...

journal_title：Neurology

authors： Lacomblez L,Bouche P,Bensimon G,Meininger V

更新日期：1989-12-01 00:00:00

abstract：OBJECTIVE:To evaluate the efficacy and safety of eptinezumab, a humanized anti-calcitonin gene-related peptide monoclonal antibody, in the preventive treatment of chronic migraine (CM). METHODS:The Prevention of Migraine via Intravenous ALD403 Safety and Efficacy-2 (PROMISE-2) study was a phase 3, multicenter, randomi...

journal_title：Neurology

authors： Lipton RB,Goadsby PJ,Smith J,Schaeffler BA,Biondi DM,Hirman J,Pederson S,Allan B,Cady R

更新日期：2020-03-31 00:00:00

abstract：OBJECTIVE:The Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society develop practice parameters as strategies for patient management based on analysis of evidence. For this parameter, the authors reviewed available evidence on the evaluation of the...

journal_title：Neurology

authors： Lewis DW,Ashwal S,Dahl G,Dorbad D,Hirtz D,Prensky A,Jarjour I,Quality Standards Subcommittee of the American Academy of Neurology.,Practice Committee of the Child Neurology Society.

更新日期：2002-08-27 00:00:00

abstract：OBJECTIVE:To determine the prognosis of asymptomatic carotid artery occlusion. BACKGROUND:As opposed to symptomatic carotid occlusion, little information is available on the prognosis of asymptomatic carotid occlusion. METHOD:Thirty never-symptomatic and 81 symptomatic patients with carotid occlusion underwent baseli...

journal_title：Neurology

authors： Powers WJ,Derdeyn CP,Fritsch SM,Carpenter DA,Yundt KD,Videen TO,Grubb RL Jr

更新日期：2000-02-22 00:00:00

abstract：OBJECTIVE:To investigate the feasibility and clinical utility of using passive electrocorticography (ECoG) for online spatial-temporal functional mapping (STFM) of language cortex in patients being monitored for epilepsy surgery. METHODS:We developed and tested an online system that exploits ECoG's temporal resolution...

journal_title：Neurology

authors： Wang Y,Fifer MS,Flinker A,Korzeniewska A,Cervenka MC,Anderson WS,Boatman-Reich DF,Crone NE

更新日期：2016-03-29 00:00:00

abstract：:Epilepsy is common in people with intellectual and developmental disabilities (IDD). In adulthood, patients with IDD and epilepsy (IDD-E) have neurologic, psychiatric, medical, and social challenges compounded by fragmented and limited care. With increasing neurologic disability, there is a higher frequency of epileps...

journal_title：Neurology

authors： Devinsky O,Asato M,Camfield P,Geller E,Kanner AM,Keller S,Kerr M,Kossoff EH,Lau H,Kothare S,Singh BK,Wirrell E

更新日期：2015-10-27 00:00:00

abstract：OBJECTIVE:Clinical trials of fetal neural tissue transplantation for Huntington disease (HD) have been conducted with variable clinical results. However, no long-term analysis of graft survival and integration has been published. Here, we report the pathologic findings in two patients with HD who died 74 and 79 months ...

journal_title：Neurology

authors： Keene CD,Sonnen JA,Swanson PD,Kopyov O,Leverenz JB,Bird TD,Montine TJ

更新日期：2007-06-12 00:00:00

abstract：OBJECTIVE:To assess the medial plantar nerve action potential (NAP) and skin biopsy in the evaluation of suspected distal sensory neuropathies (SN) with normal routine nerve conduction studies (NCS). METHODS:A total of 110 consecutive patients with suspected distal SN and normal routine NCS underwent medial plantar NA...

journal_title：Neurology

authors： Herrmann DN,Ferguson ML,Pannoni V,Barbano RL,Stanton M,Logigian EL

更新日期：2004-09-14 00:00:00

abstract：:A young girl who initially presented with jaw hypomobility at age 4 years subsequently developed signs of a slowly progressive myopathy. Rods were found in all sampled muscles, and were associated with fibrous contractures in the temporomandibular joint muscles. Rods in fibers undergoing necrosis displayed a pattern o...

journal_title：Neurology

authors： Powers JM,Young GF,Bass EB Jr,Reed FE Jr

更新日期：1980-09-01 00:00:00

abstract：BACKGROUND:A large proportion of survivors of traumatic brain injury (TBI) have persistent cognitive impairments, the profile of which does not always correspond to the size and location of injuries. One possible explanation could be that TBI-induced damage extends beyond obvious lesion sites to affect remote brain net...

journal_title：Neurology

authors： Kasahara M,Menon DK,Salmond CH,Outtrim JG,Taylor Tavares JV,Carpenter TA,Pickard JD,Sahakian BJ,Stamatakis EA

更新日期：2010-07-13 00:00:00