Familial progressive bulbar and spinal muscular atrophy. Juvenile onset and late morbidity with ragged-red fibers.

abstract：:We studied 20 patients with an acute parietal stroke with hemisensory disturbances but no visual field deficit and no or only slight motor weakness, without thalamic involvement on CT or MRI and found three main sensory syndromes. (1) The pseudothalamic sensory syndrome consists of a faciobrachiocrural impairment of e...

journal_title：Neurology

authors： Bassetti C,Bogousslavsky J,Regli F

更新日期：1993-10-01 00:00:00

abstract：OBJECTIVE:Aiming to detect associations between neuroradiologic and EEG evaluations and long-term clinical outcome in order to detect possible prognostic factors, a detailed clinical and neuroimaging characterization of 67 cases of Aicardi syndrome (AIC), collected through a multicenter collaboration, was performed. M...

journal_title：Neurology

authors： Masnada S,Pichiecchio A,Formica M,Arrigoni F,Borrelli P,Accorsi P,Bonanni P,Borgatti R,Bernardina BD,Danieli A,Darra F,Deconinck N,De Giorgis V,Dulac O,Gataullina S,Giordano L,Guerrini R,La Briola F,Mastrangelo M,Mo

更新日期：2020-12-04 00:00:00

abstract：OBJECTIVE:To study the clinical and radiologic spectrum and genotype-phenotype correlation of 4H (hypomyelination, hypodontia, hypogonadotropic hypogonadism) leukodystrophy caused by mutations in POLR3A or POLR3B. METHODS:We performed a multinational cross-sectional observational study of the clinical, radiologic, and...

journal_title：Neurology

authors： Wolf NI,Vanderver A,van Spaendonk RM,Schiffmann R,Brais B,Bugiani M,Sistermans E,Catsman-Berrevoets C,Kros JM,Pinto PS,Pohl D,Tirupathi S,Strømme P,de Grauw T,Fribourg S,Demos M,Pizzino A,Naidu S,Guerrero K,van der

更新日期：2014-11-18 00:00:00

abstract：OBJECTIVE:Autonomic symptoms may occur frequently in diabetic and other neuropathies. There is a need to develop a simple instrument to measure autonomic symptoms in subjects with neuropathy and to test the validity of the instrument. METHODS:The Survey of Autonomic Symptoms (SAS) consists of 11 items in women and 12 ...

journal_title：Neurology

authors： Zilliox L,Peltier AC,Wren PA,Anderson A,Smith AG,Singleton JR,Feldman EL,Alexander NB,Russell JW

更新日期：2011-03-22 00:00:00

abstract：OBJECTIVE:To define a clinical syndrome associated with active drug abuse in HIV-infected individuals. METHODS:We performed a retrospective review to identify individuals treated at the Johns Hopkins Hospital from 1993 to 2008 who were HIV-infected and were actively abusing drugs and had bilateral basal ganglia lesion...

journal_title：Neurology

authors： Newsome SD,Johnson E,Pardo C,McArthur JC,Nath A

更新日期：2011-03-01 00:00:00

abstract：:Intramedullary cysticercosis of the spinal cord is rare; there have been only 26 previous cases. We describe a patient with low back pain, paraparesis, lumbosacral sensory loss, and signs of meningeal irritation. The clinical diagnosis was spinal cord tumor, but at surgery there was a cysticercus cyst within the lumbo...

journal_title：Neurology

authors： Akiguchi I,Fujiwara T,Matsuyama H,Muranaka H,Kameyama M

更新日期：1979-11-01 00:00:00

abstract：:We recorded interictal spikes with closely spaced scalp electrodes and sphenoidal electrodes in four patients with temporal lobe epilepsy. We used multiple dipole modeling to study the number, three-dimensional intracerebral location, time activity, and functional relationship of the neuronal sources underlying the ep...

journal_title：Neurology

authors： Baumgartner C,Lindinger G,Ebner A,Aull S,Serles W,Olbrich A,Lurger S,Czech T,Burgess R,Lüders H

更新日期：1995-01-01 00:00:00

abstract：OBJECTIVE:The objective of this study was to evaluate the association between spinal cord injury (SCI) and type 2 diabetes in a large representative sample and to determine whether an association exists irrespective of known risk factors for type 2 diabetes. METHODS:Data were obtained on 60,678 respondents to the Stat...

journal_title：Neurology

authors： Cragg JJ,Noonan VK,Dvorak M,Krassioukov A,Mancini GB,Borisoff JF

更新日期：2013-11-19 00:00:00

abstract：:We used riboflavin to treat a patient with lipid myopathy, reduced exercise capacity, intolerance to fasting, and reduced concentrations of carnitine in muscle and serum. Although carnitine concentrations did not change, exercise capacity doubled, and response to fasting improved. Muscle enzyme assay showed that palmi...

journal_title：Neurology

authors： Carroll JE,Shumate JB,Brooke MH,Hagberg JM

更新日期：1981-12-01 00:00:00

abstract：:Weight gain has been recognized as an adverse effect of valproic acid therapy, but there are are no data about serum leptin levels in patients receiving this drug. To evaluate if valproic acid treatment in epileptic patients in whom obesity develops modifies serum levels of insulin and leptin, 40 female patients with ...

journal_title：Neurology

authors： Verrotti A,Basciani F,Morresi S,de Martino M,Morgese G,Chiarelli F

更新日期：1999-07-13 00:00:00

abstract：:Spasticity may result in part from segmental spinal disinhibition. We determined the content and specific activity of glycine (the putative neurotransmitter thought to mediate spinal postsynaptic inhibition) and serine (the probable precursor of glycine) in feline spastic spinal cord following the intra-aortic adminis...

journal_title：Neurology

authors： Hall PV,Smith JE,Lane J,Mote T,Campbell R

更新日期：1979-02-01 00:00:00

abstract：:Juvenile-onset dystonia that improves after levodopa may occur in both dopa-responsive dystonia (DRD) and juvenile parkinsonism (JP), clinically similar conditions with different prognoses and management goals. The authors show normal striatal uptake of the dopamine transporter ligand FP-CIT with SPECT in a clinically...

journal_title：Neurology

authors： O'Sullivan JD,Costa DC,Gacinovic S,Lees AJ

更新日期：2001-01-23 00:00:00

abstract：:All Olmsted County, Minnesota, residents who experienced brain injury from 1935 through 1979 were identified and their medical records reviewed for survival and neurologic outcome. Minimum inclusion criteria included loss of consciousness or post-traumatic amnesia or neurologic evidence of brain injury or skull fractu...

journal_title：Neurology

authors： Wiederholt WC,Melton LJ 3rd,Annegers JF,Grabow JD,Laws ER Jr,Ilstrup DM

更新日期：1989-01-01 00:00:00

abstract：OBJECTIVE:Rebleeding of an aneurysm is a leading cause of morbidity and mortality after subarachnoid hemorrhage (SAH). Whereas numerous studies have demonstrated the risk factors associated with rebleeding, few data on complications of rebleeding, including its effect on the development of delayed cerebral ischemia (DC...

journal_title：Neurology

authors： Lord AS,Fernandez L,Schmidt JM,Mayer SA,Claassen J,Lee K,Connolly ES,Badjatia N

更新日期：2012-01-03 00:00:00

abstract：OBJECTIVE:To study the attributes of quantitative strength testing using hand-held dynamometry (HHD) as an efficacy measure in 2 large phase 3 amyotrophic lateral sclerosis (ALS) trials. METHODS:In the phase 3 trials of ceftriaxone and dexpramipexole, 513 and 943 patients, respectively, were enrolled in double-blind, ...

journal_title：Neurology

authors： Shefner JM,Liu D,Leitner ML,Schoenfeld D,Johns DR,Ferguson T,Cudkowicz M

更新日期：2016-08-09 00:00:00

abstract：BACKGROUND:Structural abnormalities were detected in bilateral primary sensorimotor areas in writer's cramp. Evidence in other primary dystonia, including blepharospasm and cervical dystonia, suggest that structural abnormalities may be observed in other brain areas such as the cerebellum in writer's cramp. OBJECTIVE:...

journal_title：Neurology

authors： Delmaire C,Vidailhet M,Elbaz A,Bourdain F,Bleton JP,Sangla S,Meunier S,Terrier A,Lehéricy S

更新日期：2007-07-24 00:00:00

abstract：OBJECTIVE:The aim of this prospective study was to survey our patients about their experience with our clinic's telemedicine program to better understand telemedicine's utility for families, and to improve patient satisfaction and ultimately patient care. METHODS:This was a prospective survey study of patients and the...

journal_title：Neurology

authors： Qubty W,Patniyot I,Gelfand A

更新日期：2018-05-08 00:00:00

abstract：:Although the long-term effects of acute anoxia have been studied, the effects of chronic hypoxia on the developing human brain have received little attention. We studied children with a cyanotic congenital heart defect to assess the impact of chronic hypoxia by eight measures: neurologic examination, visual evoked res...

journal_title：Neurology

authors： O'Dougherty M,Wright FS,Loewenson RB,Torres F

更新日期：1985-01-01 00:00:00

abstract：:A 53-year-old man presented with a painful, demyelinating sensorimotor peripheral neuropathy with lymphomatous infiltration on sural nerve biopsy, but no evidence of systemic lymphoma. The neuropathy responded to cytotoxic therapy. Seven years later he developed generalized lymphadenopathy due to B cell lymphoplasmacy...

journal_title：Neurology

authors： Ince PG,Shaw PJ,Fawcett PR,Bates D

更新日期：1987-07-01 00:00:00

abstract：:Article abstract-The authors analyzed whether the pituitary gland enlarges in intracranial hypotension syndrome by studying 11 consecutive patients. Initial MRI scans showed pituitary gland enlargement with a convex superior margin. Follow-up MRI studies revealed that the size of the gland invariably diminished in all...

journal_title：Neurology

authors： Alvarez-Linera J,Escribano J,Benito-León J,Porta-Etessam J,Rovira A

更新日期：2000-12-26 00:00:00

abstract：:Clinical, electrodiagnostic, and pathologic studies indicate that the Guillain-Barre syndromes (GBSs) include both primary demyelinating and primary axonal forms. The axonal forms are usually thought to have a poorer prognosis, with less chance for rapid or complete recovery. In northern China, epidemics of one axonal...

journal_title：Neurology

authors： Ho TW,Li CY,Cornblath DR,Gao CY,Asbury AK,Griffin JW,McKhann GM

更新日期：1997-03-01 00:00:00

abstract：:We evaluated the effects of isoniazid on tremor in 13 patients with MS. Patients were evaluated before treatment, after 1 month of therapy (1,000 mg daily), and 1 month after the last dose. Ten patients improved on at least one of three methods of evaluation. Transient side effects were common, possibly because there ...

journal_title：Neurology

authors： Duquette P,Pleines J,du Souich P

更新日期：1985-12-01 00:00:00

abstract：:Postprandial hypotension occurs commonly in patients with autonomic failure and may be due to attenuation of the normal sympathetic nervous system activation in response to meal ingestion. In a randomized, double-blind, placebo-controlled study, we investigated the therapeutic effect of the norepinephrine precursor 3,...

journal_title：Neurology

authors： Freeman R,Young J,Landsberg L,Lipsitz L

更新日期：1996-12-01 00:00:00

abstract：OBJECTIVE:To explore the incidence of depression and anxiety and to measure quality of life in women with idiopathic intracranial hypertension (IIH), a matched group cross-sectional study was conducted. Women with IIH (n = 28) were compared with control groups of weight- and age-matched women not diagnosed with IIH (n ...

journal_title：Neurology

authors： Kleinschmidt JJ,Digre KB,Hanover R

更新日期：2000-01-25 00:00:00

abstract：BACKGROUND:Clinical trials involving patients with glioblastoma (GBM) distinguish cohorts who are treated with enzyme-inducing anticonvulsants (EIAC). Such anticonvulsants induce hepatic P450 microsomal enzymes, which accelerate the metabolism of certain chemotherapy and molecular targeted agents. However, the resultan...

journal_title：Neurology

authors： Jaeckle KA,Ballman K,Furth A,Buckner JC

更新日期：2009-10-13 00:00:00

abstract：OBJECTIVE:To examine the genetic relationships among epilepsies with different seizure types--myoclonic, absence, and generalized tonic-clonic--within the idiopathic generalized epilepsies (IGEs). BACKGROUND:Careful phenotype definition in the epilepsies may allow division into groups that share susceptibility genes. ...

journal_title：Neurology

authors： Winawer MR,Marini C,Grinton BE,Rabinowitz D,Berkovic SF,Scheffer IE,Ottman R

更新日期：2005-08-23 00:00:00

abstract：:The majority of patients with traumatic brain injury (TBI) are classified as having a mild TBI. Despite being categorized as mild, these individuals report ongoing and complex symptoms, which negatively affect their ability to complete activities of daily living and overall quality of life. Some of the major symptoms ...

journal_title：Neurology

authors： Elenberger J,Kim B,de Castro-Abeger A,Rex TS

更新日期：2020-11-03 00:00:00

abstract：BACKGROUND:Migraine and headache in general have been associated with subsequent risk of stroke, primarily in retrospective case-control studies. Prospective data evaluating the association between specific headache forms and stroke are sparse. METHODS:A prospective cohort study was conducted among 39,754 US health pr...

journal_title：Neurology

authors： Kurth T,Slomke MA,Kase CS,Cook NR,Lee IM,Gaziano JM,Diener HC,Buring JE

更新日期：2005-03-22 00:00:00

abstract：:We compared the effect of metrizamide and its parent compound glucosamine on the kinetics of dog brain hexokinase. The Michaelis constant (Km) for glucose rose from 0.065 to 0.15 to 0.28 mM in the presence of 0, 16, and 32 mM metrizamide, respectively. For 0, 1.5, and 3.7 mM glucosamine, the Km values were 0.065, 0.4,...

journal_title：Neurology

authors： Bertoni JM,Steinman CG

更新日期：1982-03-01 00:00:00

abstract：OBJECTIVE:To estimate the lifetime cumulative incidence of dementia and its subtypes from a community-dwelling elderly population in Japan. METHODS:A total of 1,193 community-dwelling Japanese individuals without dementia, aged 60 years or older, were followed up prospectively for 17 years. The cumulative incidence of...

journal_title：Neurology

authors： Yoshida D,Ohara T,Hata J,Shibata M,Hirakawa Y,Honda T,Furuta Y,Oishi E,Sakata S,Kanba S,Kitazono T,Ninomiya T

更新日期：2020-08-04 00:00:00