Craniofacial and cutaneous findings expand the phenotype of hereditary neuralgic amyotrophy.

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：:We performed a retrospective study to characterize the type, frequency, and timing of neurologic complications in patients with B-cell chronic lymphocytic leukemia (B-CLL). We reviewed 962 total charts with a median follow-up time of 57.5 months. There were 109 cases (11.3%) of neurologic complications, including 69 c...

journal_title：Neurology

authors： Bower JH,Hammack JE,McDonnell SK,Tefferi A

更新日期：1997-02-01 00:00:00

abstract：:We studied the effect of a single oral intake of 30 to 40 grams of alcohol in 4 consecutive patients presenting with Ramsay Hunt syndrome, the most common type of degenerative progressive myoclonic epilepsy (PME) encountered in southern Europe. Clinical and polygraphic monitoring demonstrated abatement of myoclonus in...

journal_title：Neurology

authors： Genton P,Guerrini R

更新日期：1990-09-01 00:00:00

abstract：:Mutations in DJ-1 are a cause of autosomal recessive parkinsonism. Polymorphism of genes implicated in hereditary forms of parkinsonism may be a predisposing factor in sporadic Parkinson's disease (PD). The authors analyzed whether a polymorphism (g.168_185del) within exon 1 of DJ-1 contributes to the risk of sporadic...

journal_title：Neurology

authors： Eerola J,Hernandez D,Launes J,Hellström O,Hague S,Gulick C,Johnson J,Peuralinna T,Hardy J,Tienari PJ,Singleton AB

更新日期：2003-10-14 00:00:00

abstract：OBJECTIVE:To examine the effects of natalizumab on low-contrast letter acuity as a prespecified tertiary endpoint in two randomized clinical trials and to evaluate the usefulness of low-contrast letter acuity testing as a candidate test of visual function in multiple sclerosis (MS). METHODS:AFFIRM and SENTINEL were ra...

journal_title：Neurology

authors： Balcer LJ,Galetta SL,Calabresi PA,Confavreux C,Giovannoni G,Havrdova E,Hutchinson M,Kappos L,Lublin FD,Miller DH,O'Connor PW,Phillips JT,Polman CH,Radue EW,Rudick RA,Stuart WH,Wajgt A,Weinstock-Guttman B,Wynn DR,Lyn

更新日期：2007-04-17 00:00:00

abstract：:Cocaine, especially in its alkaloidal or "crack" form, has been increasingly associated with cerebrovascular disease. Before the crack epidemic, cocaine hydrochloride (HCl) was also implicated as a cause of stroke. However, less is known about the differences in stroke subtypes, age at stroke onset, or presence of und...

journal_title：Neurology

authors： Levine SR,Brust JC,Futrell N,Brass LM,Blake D,Fayad P,Schultz LR,Millikan CH,Ho KL,Welch KM

更新日期：1991-08-01 00:00:00

abstract：BACKGROUND:Oculopharyngodistal myopathy (OPDM) has been reported as a rare, adult-onset hereditary muscle disease with putative autosomal dominant and autosomal recessive inheritance. Patients with OPDM present with progressive ocular, pharyngeal, and distal limb muscle involvement. The genetic defect causing OPDM has ...

journal_title：Neurology

authors： Durmus H,Laval SH,Deymeer F,Parman Y,Kiyan E,Gokyigiti M,Ertekin C,Ercan I,Solakoglu S,Karcagi V,Straub V,Bushby K,Lochmüller H,Serdaroglu-Oflazer P

更新日期：2011-01-18 00:00:00

abstract：:To determine the frequency of subjective sleep problems in spinocerebellar ataxia type 6 (SCA6), the authors surveyed 25 patients and 25 age-matched controls with the Epworth Sleepiness Scale (ESS) and Pittsburgh Sleep Quality Index (PSQI). The ESS was higher in patients with SCA6 (9.12 +/- 5.80; CI: 2.28) than in con...

journal_title：Neurology

authors： Howell MJ,Mahowald MW,Gomez CM

更新日期：2006-05-09 00:00:00

abstract：BACKGROUND:Inherited and de novo mutations in sodium channel genes underlie a variety of channelopathies. Mutations in SCN2A, encoding the brain sodium channel Na(V)1.2, have previously been reported to be associated with benign familial neonatal infantile seizures, febrile seizures plus, and intractable epilepsy of in...

journal_title：Neurology

authors： Liao Y,Anttonen AK,Liukkonen E,Gaily E,Maljevic S,Schubert S,Bellan-Koch A,Petrou S,Ahonen VE,Lerche H,Lehesjoki AE

更新日期：2010-10-19 00:00:00

abstract：:To evaluate when it is possible to discontinue anticonvulsant treatment in children with cryptogenic partial epilepsy, the authors studied 89 epileptic children divided into two groups: Group A, 45 children whose therapy was discontinued after 1 year from the last seizure; and Group B, 44 children whose therapy was st...

journal_title：Neurology

authors： Verrotti A,Morresi S,Basciani F,Cutarella R,Morgese G,Chiarelli F

更新日期：2000-11-14 00:00:00

abstract：:A national survey, based on a probability sample of patients admitted to short-term hospitals in the United States during 1973 to 1974 with a discharge diagnosis of an intracranial neoplasm, was conducted in 157 hospitals. The annual incidence was estimated at 17,000 for primary intracranial neoplasms and 17,400 for s...

journal_title：Neurology

authors： Walker AE,Robins M,Weinfeld FD

更新日期：1985-02-01 00:00:00

abstract：:Spontaneous arm levitation (SAL) is well-recognized in cortical-basal ganglionic degeneration, but not in other neurodegenerative diseases. We report six cases of progressive supranuclear palsy (PSP) with SAL. Pathologic examination in four revealed the classic features of PSP with additional areas of focal cortical d...

journal_title：Neurology

authors： Barclay CL,Bergeron C,Lang AE

更新日期：1999-03-10 00:00:00

abstract：OBJECTIVE:To characterize the clinical course of pathologically diagnosed hippocampal sclerosis dementia (HSD). BACKGROUND:Dementia associated with HSD is incompletely characterized. Previous studies suggest similarities to both Alzheimer disease (AD) and frontotemporal dementia (FTD). METHODS:Case-control analysis o...

journal_title：Neurology

authors： Blass DM,Hatanpaa KJ,Brandt J,Rao V,Steinberg M,Troncoso JC,Rabins PV

更新日期：2004-08-10 00:00:00

abstract：BACKGROUND:Semiology is very useful in the diagnosis and classification of seizures. Some clinical signs occur primarily with specific localization-related epilepsies. OBJECTIVE:To evaluate postictal nose-rubbing as a potential diagnostic sign and a potential lateralizing or localizing indicator. METHODS:We reviewed ...

journal_title：Neurology

authors： Geyer JD,Payne TA,Faught E,Drury I

更新日期：1999-03-10 00:00:00

abstract：:Recent evidence suggests that the vasopressin analogue desglycinamide-arginine8-vasopressin (DGAVP) might specifically benefit mild brain trauma patients. We investigated the effect of intranasal DGAVP treatment in 32 patients who had sustained a mild head injury for 3 months in a double-blind, placebo-controlled, mat...

journal_title：Neurology

authors： Bohnen NI,Twijnstra A,Jolles J

更新日期：1993-01-01 00:00:00

abstract：:Recent clinical neuropathologic reports are inconsistent with previous models of how pontine neural circuits generate saccadic eye movements. We present a hypothetical explanation of these and other clinical and experimental findings based on a new model that accurately predicts the eye signs in pontine lesions. ...

journal_title：Neurology

authors： Kaneko CR

更新日期：1989-07-01 00:00:00

abstract：:Concepts of pathogenesis in Parkinson's disease (PD) have been based on attempts to understand the mechanisms responsible for nigral dopaminergic cell death. Pathogenesis has been proposed to involve oxidative and nitrative stress, excitotoxicity, inflammation, mitochondrial dysfunction, and altered proteolysis. These...

journal_title：Neurology

authors： Jenner P,Olanow CW

更新日期：2006-05-23 00:00:00

abstract：BACKGROUND:Primary angiitis of the CNS (PACNS) is a diagnostically challenging disorder. In patients whose diagnosis is ascertained solely by cerebral angiography without histologic verification, a benign monophasic clinical course with favorable response to a brief course of immunosuppressive therapy is often reported...

journal_title：Neurology

authors： Woolfenden AR,Tong DC,Marks MP,Ali AO,Albers GW

更新日期：1998-07-01 00:00:00

abstract：:The motor effects of direct agonists which act selectively on certain dopamine receptors were studied in monkeys rendered hemiparkinsonian by unilateral intracarotid injection of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). The D-2 dopamine agonist, LY 171555, but not the D-1 agonist, SKF 38393, reduced parkin...

journal_title：Neurology

authors： Barone P,Bankiewicz KS,Corsini GU,Kopin IJ,Chase TN

更新日期：1987-10-01 00:00:00

abstract：:Patients, clinicians, and hospitals have undergone monumental changes during the coronavirus disease 2019 (COVID-19) pandemic. This crisis has forced us to consider the obligations that we neurologists have to our individual patients as well as the greater community. By returning to our fundamental understanding of th...

journal_title：Neurology

authors： Rubin MA,Bonnie RJ,Epstein L,Hemphill C,Kirschen M,Lewis A,Suarez JI,Ethics, Law, and Humanities Committee, a joint committee of the American Academy of Neurology, American Neurological Association, and Child Neurology Society; in collabor

更新日期：2020-07-28 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：:The incidence of contrast-induced nephropathy was examined in 1,075 patients receiving routine CT angiography and CT perfusion brain imaging at a single institution. Fifty-two patients had a creatinine rise of > or =0.5 mg/dL. In four patients (0.37%), the administration of IV contrast medium possibly contributed to r...

journal_title：Neurology

authors： Josephson SA,Dillon WP,Smith WS

更新日期：2005-05-24 00:00:00

abstract：OBJECTIVE:The aim of the study was to perform a third cognitive assessment in our pediatric-onset multiple sclerosis (MS) patient cohort and determine predictors of the individual cognitive outcome. METHODS:After 4.7 ± 0.7 years from baseline evaluation, 48 of 63 patients in the original cohort were reassessed on an e...

journal_title：Neurology

authors： Amato MP,Goretti B,Ghezzi A,Hakiki B,Niccolai C,Lori S,Moiola L,Falautano M,Viterbo RG,Patti F,Cilia S,Pozzilli C,Bianchi V,Roscio M,Martinelli V,Comi G,Portaccio E,Trojano M,MS Study Group of the Italian Neurological

更新日期：2014-10-14 00:00:00

abstract：:We assessed the ability to recognize epileptiform abnormalities on a video review of ambulatory cassette EEGs (A/EEG) by comparing two independent interpretations of an A/EEG to those derived from a routine recording and intensive monitoring by cable telemetry (C/EEG) of the same 40 patients. Both A/EEG readings concu...

journal_title：Neurology

authors： Ebersole JS,Leroy RF

更新日期：1983-07-01 00:00:00

abstract：BACKGROUND:Neutralizing antibodies (NAB) to interferon beta (IFNbeta) occur in about one-third of MS patients treated with IFNbeta-1b and there is an association with a loss of clinical and MRI efficacy. However, there are no data regarding the bioavailability of IFNbeta-1b in patients with and without NAB. METHODS:Th...

journal_title：Neurology

authors： Deisenhammer F,Reindl M,Harvey J,Gasse T,Dilitz E,Berger T

更新日期：1999-04-12 00:00:00

abstract：:We describe a patient who presented with unilateral abdominal herniation due to weakness of abdominal musculature, associated with extensive syringomyelia. Electromyographic evidence of focal denervation and reinnervation was present in the weak muscles consistent with anterior horn cell loss. We compare these unusual...

journal_title：Neurology

authors： Coleman RJ,Ingram DA

更新日期：1991-10-01 00:00:00

abstract：:Four patients with disabling action tremor in the setting of MS were treated with isoniazid (800 to 1200 mg per day). All patients showed significant improvement of the tremor, allowing more functional use of their extremities. To the best of our knowledge, this is the only medical treatment for this type of tremor. ...

journal_title：Neurology

authors： Sabra AF,Hallett M,Sudarsky L,Mullally W

更新日期：1982-08-01 00:00:00

abstract：OBJECTIVE:We sought to assess the prevalence, correlates, and consequences of periodic limb movements of sleep (PLMS) in persons with obstructive sleep apnea (OSA) and the effect (worsening or improvement) of continuous positive airway pressure (CPAP) therapy on PLMS in a large prospective multicenter randomized contro...

journal_title：Neurology

authors： Budhiraja R,Javaheri S,Pavlova MK,Epstein LJ,Omobomi O,Quan SF

更新日期：2020-04-28 00:00:00

abstract：:Co-morbid conditions frequently occur in childhood epilepsy and may significantly affect epilepsy and its treatment. Similarly, epilepsy and antiepileptic drugs (AEDs) may affect these associated conditions. Co-morbidities that have a significant association with childhood epilepsy include attention-deficit/hyperactiv...

journal_title：Neurology

authors： Pellock JM

更新日期：2004-03-09 00:00:00

abstract：OBJECTIVE:To compare the clinical features of patients showing a classical phenotype of facioscapulohumeral muscular dystrophy (FSHD) with genetic and epigenetic characteristics of the FSHD1 and FSHD2 loci D4Z4 and SMCHD1. METHODS:This is a national multicenter cohort study. We measured motor strength, motor function,...

journal_title：Neurology

authors： Sacconi S,Briand-Suleau A,Gros M,Baudoin C,Lemmers RJLF,Rondeau S,Lagha N,Nigumann P,Cambieri C,Puma A,Chapon F,Stojkovic T,Vial C,Bouhour F,Cao M,Pegoraro E,Petiot P,Behin A,Marc B,Eymard B,Echaniz-Laguna A,Laf

更新日期：2019-05-07 00:00:00