Long-latency event-related potentials in the evaluation of cognitive function in children.

abstract：OBJECTIVE:To identify the genetic and physiologic basis for recessive myasthenic congenital myopathy in 2 families, suggestive of a channelopathy involving the sodium channel gene, SCN4A. METHODS:A combination of whole exome sequencing and targeted mutation analysis, followed by voltage-clamp studies of mutant sodium ...

journal_title：Neurology

authors： Elia N,Palmio J,Castañeda MS,Shieh PB,Quinonez M,Suominen T,Hanna MG,Männikkö R,Udd B,Cannon SC

更新日期：2019-03-26 00:00:00

abstract：BACKGROUND:Patients with ALS commonly exhibit pseudobulbar affect. METHODS:The authors conducted a multicenter, randomized, double-blind, controlled, parallel, three-arm study to test a defined combination of dextromethorphan hydrobromide (DM) and quinidine sulfate (Q) (AVP-923) for the treatment of pseudobulbar affec...

journal_title：Neurology

authors： Brooks BR,Thisted RA,Appel SH,Bradley WG,Olney RK,Berg JE,Pope LE,Smith RA,AVP-923 ALS Study Group.

更新日期：2004-10-26 00:00:00

abstract：:Clinical presentation of carotid artery dissection (CAD) is variable, including acute or subacute headache, cervical pain, or focal neurologic deficit that occurs spontaneously, after a high-speed neck manipulation, or trauma. Isolated lower cranial nerve palsy in carotid dissection is uncommon. ...

journal_title：Neurology

authors： Pongmoragot J,Bharatha A,Saposnik G

更新日期：2013-03-12 00:00:00

abstract：:The authors describe two patients with clinical manifestations of the osmotic demyelination syndrome (ODS) and unusual MRI findings of gadolinium-enhancing peripheral cortical abnormalities. They propose that these represent extrapontine manifestations of ODS because neither patient had a notable hypoxic-ischemic insu...

journal_title：Neurology

authors： Calakos N,Fischbein N,Baringer JR,Jay C

更新日期：2000-10-10 00:00:00

abstract：:The incidence of MS was assessed in a nationwide cohort study of 31,990 employees of Danish utility companies between 1900 and 1993. Overall, 32 cases of MS were diagnosed, as compared with 23.7 expected from national incidence rates, to yield a standardized incidence ratio of 1.35 (95% confidence interval, 0.92 to 1....

journal_title：Neurology

authors： Johansen C,Koch-Henriksen N,Rasmussen S,Olsen JH

更新日期：1999-04-12 00:00:00

abstract：:Fatal familial insomnia (FFI), a condition characterized by inability to sleep, dysautonomia, motor disturbances, and selective thalamic atrophy is a prion disease linked to a GAC----AAC mutation at codon 178 of the prion gene. These data were obtained from one kindred. We now report a second kindred affected by FFI a...

journal_title：Neurology

authors： Medori R,Montagna P,Tritschler HJ,LeBlanc A,Cortelli P,Tinuper P,Lugaresi E,Gambetti P

更新日期：1992-03-01 00:00:00

abstract：:Hemorrhage complicating acute carbon monoxide poisoning is usually confined to the basal ganglia. The authors report three people with acute carbon monoxide poisoning manifesting selective or prominent white matter involvement complicated by hemorrhagic infarction on CT and MRI in one and by subcortical T2*-weighted M...

journal_title：Neurology

authors： Finelli PF,DiMario FJ Jr

更新日期：2004-09-28 00:00:00

abstract：OBJECTIVES:To establish the role of high-resolution CT imaging and tests of vestibulocollic reflexes in diagnosing and understanding the pathogenesis of the Tullio phenomenon. BACKGROUND:The Tullio phenomenon is a syndrome in which acoustic stimulation produces symptoms and signs of vestibular activation. It has previ...

journal_title：Neurology

authors： Watson SR,Halmagyi GM,Colebatch JG

更新日期：2000-02-08 00:00:00

abstract：:We administered 3 commonly employed tests of mental status (the Information-Memory-Concentration test [IMC], the Mini-Mental State Examination [MMSE], and the Dementia Rating Scale [DRS]) to 92 patients with probable dementia of the Alzheimer type. The 3 tests were readministered to 55 of the patients (2-year subgroup...

journal_title：Neurology

authors： Salmon DP,Thal LJ,Butters N,Heindel WC

更新日期：1990-08-01 00:00:00

abstract：OBJECTIVE:To define the clinical and radiologic features of brainstem gliomas (BSGs) in children with neurofibromatosis type 1 (NF1). METHODS:We performed a retrospective cross-sectional study of 133 children with NF1 and concurrent BSGs cared for at 4 NF1 referral centers. BSG was determined using radiographic criter...

journal_title：Neurology

authors： Mahdi J,Shah AC,Sato A,Morris SM,McKinstry RC,Listernick R,Packer RJ,Fisher MJ,Gutmann DH

更新日期：2017-04-18 00:00:00

abstract：:The administration of lecithin has failed to improve memory in aged or demented individuals. We studied the effect of lecithin on another cholinergic function, REM sleep latency. Intravenous doses of physostigmine shortened the latency to the first REM sleep period, but oral lecithin did not. In contrast to physostigm...

journal_title：Neurology

authors： Kapen S,Fleming PD,Drachman DA

更新日期：1986-08-01 00:00:00

abstract：OBJECTIVE:To evaluate the effectiveness of yoga as an adjuvant to conventional medical management on clinical outcomes in patients with migraine. METHODS:CONTAIN was a prospective, randomized, open-label superiority trial with blinded endpoint assessment carried out at a single tertiary care academic hospital in New D...

journal_title：Neurology

authors： Kumar A,Bhatia R,Sharma G,Dhanlika D,Vishnubhatla S,Singh RK,Dash D,Tripathi M,Srivastava MVP

更新日期：2020-05-26 00:00:00

abstract：OBJECTIVE:We describe a novel congenital motor neuron disease with early demise due to respiratory insufficiency with clinical overlap with spinal muscular atrophy with respiratory distress (SMARD) type 1 but lacking a mutation in the IGHMBP2 gene. METHODS:Exome sequencing was used to identify a de novo mutation in th...

journal_title：Neurology

authors： Butterfield RJ,Stevenson TJ,Xing L,Newcomb TM,Nelson B,Zeng W,Li X,Lu HM,Lu H,Farwell Gonzalez KD,Wei JP,Chao EC,Prior TW,Snyder PJ,Bonkowsky JL,Swoboda KJ

更新日期：2014-04-15 00:00:00

abstract：OBJECTIVE:To investigate whether the fornix and mamillary bodies, being part of the limbic system, are abnormal in patients with mesial temporal lobe epilepsy (MTLE). BACKGROUND:The limbic system comprises the hippocampal formation, fornix, mamillary bodies, thalamus, and other integrated structures. This system is im...

journal_title：Neurology

authors： Kuzniecky R,Bilir E,Gilliam F,Faught E,Martin R,Hugg J

更新日期：1999-08-11 00:00:00

abstract：BACKGROUND:We evaluated the clinicoradiologic characteristics of patients with branch occlusive disease (BOD)-type intracranial atherosclerotic stroke (ICAS) compared with those of patients with non-BOD-type ICAS or with small artery disease (SAD). METHODS:We analyzed 201 consecutive patients with acute infarcts withi...

journal_title：Neurology

authors： Ryoo S,Park JH,Kim SJ,Kim GM,Chung CS,Lee KH,Kim JS,Bang OY

更新日期：2012-03-20 00:00:00

abstract：OBJECTIVE:To study the potential effect of the 2008 Institute of Medicine (IOM) work duty hour (WDH) recommendations on neurology residency programs. METHODS:This study evaluated resident sleepiness, personal study hours, quality of life, and satisfaction and faculty satisfaction during a control month using the Accre...

journal_title：Neurology

authors： Schuh LA,Khan MA,Harle H,Southerland AM,Hicks WJ,Falchook A,Schultz L,Finney GR

更新日期：2011-08-30 00:00:00

abstract：BACKGROUND:Krabbe disease (globoid cell leukodystrophy [GLD]) is an autosomal recessive lysosomal disorder affecting the central and peripheral nervous system. The authors performed MRS to characterize metabolic alterations and their regional variation in brain tissue in GLD in vivo. METHODS:Abnormalities of cerebral ...

journal_title：Neurology

authors： Brockmann K,Dechent P,Wilken B,Rusch O,Frahm J,Hanefeld F

更新日期：2003-03-11 00:00:00

abstract：OBJECTIVE:To determine, in a cross-sectional evaluation of nondemented individuals, if age-related memory decline is influenced by apolipoprotein E (apoE) genotype. BACKGROUND:The apoE-4 allele is an important risk factor for AD. PET in cognitively normal apoE-4 carriers (mean age, 56 years) shows reduced cerebral met...

journal_title：Neurology

authors： Caselli RJ,Graff-Radford NR,Reiman EM,Weaver A,Osborne D,Lucas J,Uecker A,Thibodeau SN

更新日期：1999-07-13 00:00:00

abstract：:A prospective trial of plasmapheresis and azathioprine or azathioprine alone was carrier out on 20 patients with chronic progressive MS. Seven of 10 patients in each group continued therapy for 12 months. When the groups were compared by disability ratings, the combination of plasmapheresis and azathioprine was no bet...

journal_title：Neurology

authors： Tindall RS,Walker JE,Ehle AL,Near L,Rollins J,Becker D

更新日期：1982-07-01 00:00:00

abstract：OBJECTIVE:To describe the detection frequency and clinical associations of immunoglobulin G (IgG) targeting dipeptidyl-peptidase-like protein-6 (DPPX), a regulatory subunit of neuronal Kv4.2 potassium channels. METHODS:Specimens from 20 patients evaluated on a service basis by tissue-based immunofluorescence yielded a...

journal_title：Neurology

authors： Tobin WO,Lennon VA,Komorowski L,Probst C,Clardy SL,Aksamit AJ,Appendino JP,Lucchinetti CF,Matsumoto JY,Pittock SJ,Sandroni P,Tippmann-Peikert M,Wirrell EC,McKeon A

更新日期：2014-11-11 00:00:00

abstract：OBJECTIVE:To evaluate the integrity of the dorsal striatal dopaminergic innervation in rigid and choreic Huntington disease (HD). BACKGROUND:Some patients with HD have an akinetic-rigid phenotype. It has been suggested that nigrostriatal in addition to striatal pathology is present in this subgroup. The authors sought...

journal_title：Neurology

authors： Bohnen NI,Koeppe RA,Meyer P,Ficaro E,Wernette K,Kilbourn MR,Kuhl DE,Frey KA,Albin RL

更新日期：2000-05-09 00:00:00

abstract：:Although psychological distress and cognitive dysfunction are well documented in adults with multiple sclerosis (MS), they are poorly understood in children with the disease. Psychosocial difficulty experienced by children and adolescents with MS involves factors common to all chronic illnesses in children, as well as...

journal_title：Neurology

authors： MacAllister WS,Boyd JR,Holland NJ,Milazzo MC,Krupp LB,International Pediatric MS Study Group.

更新日期：2007-04-17 00:00:00

abstract：:Many advances in prevention, diagnosis, and treatment of neurologic disease have emerged in the last few decades, resulting in reduced mortality and decreased disability. However, these advances have not benefitted all populations equally. A growing body of evidence indicates that barriers to care fall along racial an...

journal_title：Neurology

authors： Marulanda-Londoño ET,Bell MW,Hope OA,Leacock RO,O'Carroll CB,Posas J 3rd,Stover NP,Young R,Hamilton R

更新日期：2019-02-05 00:00:00

abstract：OBJECTIVE:To investigate the added value of hippocampal atrophy rates over whole brain volume measurements on MRI in patients with Alzheimer disease (AD), patients with mild cognitive impairment (MCI), and controls. METHODS:We included 64 patients with AD (67 +/- 9 years; F/M 38/26), 44 patients with MCI (71 +/- 6 yea...

journal_title：Neurology

authors： Henneman WJ,Sluimer JD,Barnes J,van der Flier WM,Sluimer IC,Fox NC,Scheltens P,Vrenken H,Barkhof F

更新日期：2009-03-17 00:00:00

abstract：:Arm-diaphragm synkinesis may occur after injury to the proximal portion of the brachial plexus or cervical nerve roots. Regenerating axons of phrenic motor neurons are misdirected to supply limb muscles. Electrodiagnostic investigations of three patients with symptoms referable to upper brachial plexus or cervical roo...

journal_title：Neurology

authors： Swift TR,Leshner RT,Gross JA

更新日期：1980-04-01 00:00:00

abstract：BACKGROUND:In patients with trigeminal neuralgia, results of clinical examination of sensory function are normal. Reflex and evoked potential studies have already provided information on large-afferent (non-nociceptive) function. Using laser-evoked potentials (LEP), the authors sought information on small-afferent (noc...

journal_title：Neurology

authors： Cruccu G,Leandri M,Iannetti GD,Mascia A,Romaniello A,Truini A,Galeotti F,Manfredi M

更新日期：2001-06-26 00:00:00

abstract：:Idiopathic cervical dystonia (CD) is the most common of the adult-onset focal dystonias. It manifests as involuntary muscle contractions that cause twisting or turning of the neck. The resulting abnormal head and neck postures are heterogeneous in their presentation. Because of the variable presentation of the disease...

journal_title：Neurology

authors： Stacy M

更新日期：2000-01-01 00:00:00

abstract：:In patients with spatial neglect, contralesional reflexive saccades toward suddenly appearing targets show direction-specific deficits. We examined whether these deficits also occur during free exploration of space. Neglect patients' voluntary eye movements showed reduced amplitudes for saccades in all directions but ...

journal_title：Neurology

authors： Niemeier M,Karnath HO

更新日期：2000-01-25 00:00:00

abstract：:We detected large numbers of HLA-DR-positive reactive microglia (macrophages), along with Lewy bodies and free melanin, in the substantia nigra of all cases studied with Parkinson's disease (5) and parkinsonism with dementia (PD) (5). We found similar, but less extensive, pathology in the substantia nigra of six of ni...

journal_title：Neurology

authors： McGeer PL,Itagaki S,Boyes BE,McGeer EG

更新日期：1988-08-01 00:00:00

abstract：:We examined serum-mediated cytotoxicity on cultured rat oligodendrocytes, using serum from patients with acute or chronic progressive multiple sclerosis and normal controls. We found heat-labile serum factors in serum from MS and also in controls. The cytotoxic effects of MS and normal sera were not restored by adding...

journal_title：Neurology

authors： Hirayama M,Lisak RP,Silberberg DH

更新日期：1986-02-01 00:00:00