18F-FDG-PET correlates of cognitive impairment in ALS.

abstract：:We studied 35 patients with nonseptic cardiogenic cerebral emboli. In 28 cases, there was sudden onset with maximal neurologic deficit within 1 hour; in 7 cases, there was progression in 24 hours. In six cases, neurologic abnormality worsened in the first week after an initial period of stabilization. CT showed eviden...

journal_title：Neurology

authors： Weisberg LA

更新日期：1985-06-01 00:00:00

abstract：:We report the cases of 5 patients from 2 sibships with the "adult" or chronic form of GM2 gangliosidosis and 2 patients from another sibship with the juvenile form. We demonstrated hexosaminidase A deficiency in all cases but in 1 sibship the enzymatic profile was identical to that in Tay-Sachs disease, whereas in the...

journal_title：Neurology

authors： Specola N,Vanier MT,Goutières F,Mikol J,Aicardi J

更新日期：1990-01-01 00:00:00

abstract：:We studied a family in which the father and 7 of 10 children had episodes of Bell's palsy. Five of the eight affected family members also had ocular motor palsies. Facial nerve and blink reflex studies in four affected siblings demonstrated asymmetrically reduced amplitude of evoked responses without delayed conductio...

journal_title：Neurology

authors： Aldrich MS,Beck RW,Albers JW

更新日期：1987-08-01 00:00:00

abstract：BACKGROUND:Co-occurrence of Parkinson disease (PD) and melanoma has been reported in numerous studies. If this was due to common genetic mechanisms, a positive family history of melanoma would be associated with an excessive PD risk, independent of environmental risk factors for PD. METHODS:We prospectively examined a...

journal_title：Neurology

authors： Gao X,Simon KC,Han J,Schwarzschild MA,Ascherio A

更新日期：2009-10-20 00:00:00

abstract：OBJECTIVE:To determine (1) if mortality among patients with idiopathic intracranial hypertension (IIH) enrolled in the Intracranial Hypertension Registry (IHR) is different from that of the general population of the United States and (2) what the leading underlying causes of death are among this cohort. METHODS:Mortal...

journal_title：Neurology

authors： Hermes SM,Miller NR,Waslo CS,Benes SC,Tanne E

更新日期：2020-08-18 00:00:00

abstract：:The presence of the APOE epsilon 4 allele encoding apolipoprotein E4 (apoE4) is the major genetic risk factor for late-onset Alzheimer's disease (AD). However, the molecular and cellular mechanisms by which APOE epsilon 4 renders AD risk are unclear. In this report, we present genetic evidence that an apoE receptor, L...

journal_title：Neurology

authors： Kang DE,Saitoh T,Chen X,Xia Y,Masliah E,Hansen LA,Thomas RG,Thal LJ,Katzman R

更新日期：1997-07-01 00:00:00

abstract：OBJECTIVE:To examine whether prior statin use affects outcome and intracranial hemorrhage (ICH) rates in stroke patients receiving IV thrombolysis (IVT). METHODS:In a pooled observational study of 11 IVT databases, we compared outcomes between statin users and nonusers. Outcome measures were excellent 3-month outcome ...

journal_title：Neurology

authors： Engelter ST,Soinne L,Ringleb P,Sarikaya H,Bordet R,Berrouschot J,Odier C,Arnold M,Ford GA,Pezzini A,Zini A,Rantanen K,Rocco A,Bonati LH,Kellert L,Strbian D,Stoll A,Meier N,Michel P,Baumgartner RW,Leys D,Tatlisum

更新日期：2011-08-30 00:00:00

abstract：OBJECTIVE:To perform a double-blind, randomized study comparing efficacy and safety of daily and weekend prednisone in boys with Duchenne muscular dystrophy (DMD). METHODS:A total of 64 boys with DMD who were between 4 and 10 years of age were randomized at 1 of 12 centers of the Cooperative International Neuromuscula...

journal_title：Neurology

authors： Escolar DM,Hache LP,Clemens PR,Cnaan A,McDonald CM,Viswanathan V,Kornberg AJ,Bertorini TE,Nevo Y,Lotze T,Pestronk A,Ryan MM,Monasterio E,Day JW,Zimmerman A,Arrieta A,Henricson E,Mayhew J,Florence J,Hu F,Connolly A

更新日期：2011-08-02 00:00:00

abstract：OBJECTIVE:To evaluate the associations of mild behavioral impairment (MBI) with cognitive deficits and patterns of gray matter changes in Parkinson disease (PD). METHODS:Sixty patients with PD without dementia and 29 healthy controls underwent a cognitive neuropsychological evaluation and structural MRI scan. MBI was ...

journal_title：Neurology

authors： Yoon EJ,Ismail Z,Hanganu A,Kibreab M,Hammer T,Cheetham J,Kathol I,Sarna JR,Martino D,Furtado S,Monchi O

更新日期：2019-08-20 00:00:00

abstract：:Long-term hypothalamic stimulation is effective in improving drug-resistant chronic cluster headache (CH). We assessed acute hypothalamic stimulation to resolve ongoing CH attacks in 16 patients implanted to prevent chronic CH, investigating 136 attacks. A pain intensity reduction of > or =50% occurred in 25 of 108 ev...

journal_title：Neurology

authors： Leone M,Franzini A,Broggi G,Mea E,Cecchini AP,Bussone G

更新日期：2006-11-28 00:00:00

abstract：BACKGROUND:Multiple system atrophy (MSA) is an atypical parkinsonian syndrome including cerebellar impairment and poor response to levodopa. We assessed right hand motor activation in patients with MSA before and after an acute levodopa challenge in comparison with patients with PD and healthy volunteers (HVs). METHOD...

journal_title：Neurology

authors： Payoux P,Brefel-Courbon C,Ory-Magne F,Regragui W,Thalamas C,Balduyck S,Durif F,Azulay JP,Tison F,Blin O,Esquerre JP,Rascol O

更新日期：2010-09-28 00:00:00

abstract：:The disease locus for the clinically heterogeneous childhood spinal muscular atrophies (SMA) maps to the chromosome 5 subregion, 5q11.2-13.3. The beta-subunit of beta-D-N-acetylhexosaminidase (hexosaminidase) (EC 3.2.1.52) (Hex B) maps to the same region, and the protein required for substrate recognition by this enzy...

journal_title：Neurology

authors： Kleyn PW,Brzustowicz LM,Wilhelmsen KC,Freimer NB,Miller JM,Munsat TL,Gilliam TC

更新日期：1991-09-01 00:00:00

abstract：:A 53-year-old man presented with multiple right cranial nerve palsies that included the tongue. When the tongue was protruded, it deviated to the right. However, when the tongue was not protruded, he could readily turn the tip of the tongue to the left, but not to the right. Protrusion of the tongue requires the actio...

journal_title：Neurology

authors： Riggs JE

更新日期：1984-10-01 00:00:00

abstract：BACKGROUND:After 1 to 4 years, seizure-free children with epilepsy are encouraged to stop daily antiepileptic drug (AED) treatment. Approximately 70% are successful. The authors examined how often intractable epilepsy follows discontinuation of AED treatment in a population-based cohort of children with epilepsy. METH...

journal_title：Neurology

authors： Camfield P,Camfield C

更新日期：2005-03-22 00:00:00

abstract：OBJECTIVES:SCN1A mutations were recently reported in several patients with severe myoclonic epilepsy in infancy (SMEI). The authors analyzed SCN1A mutations in 93 patients with SMEI and made genotype-phenotype correlation to clarify the role of this gene in the etiology of SMEI. METHODS:All patients fulfilled the crit...

journal_title：Neurology

authors： Nabbout R,Gennaro E,Dalla Bernardina B,Dulac O,Madia F,Bertini E,Capovilla G,Chiron C,Cristofori G,Elia M,Fontana E,Gaggero R,Granata T,Guerrini R,Loi M,La Selva L,Lispi ML,Matricardi A,Romeo A,Tzolas V,Valseriati

更新日期：2003-06-24 00:00:00

abstract：:Central nervous system (CNS) dysfunction in patients with systemic lupus erythematosus (SLE) is highly variable, although it is often described under a single heading of "neuropsychiatric" or "CNS" SLE. To clarify these CNS abnormalities, we studied 91 lupus patients, 63 of whom had CNS symptoms or signs, over 599 pat...

journal_title：Neurology

authors： Futrell N,Schultz LR,Millikan C

更新日期：1992-09-01 00:00:00

abstract：:Regional cerebral glucose metabolism was studied in 16 patients with idiopathic torticollis, using positron emission tomography. Analysis of subcortical regions revealed no consistent focal abnormality of cerebral metabolic rate for glucose, but there was a bilateral breakdown of the normal relationships between the t...

journal_title：Neurology

authors： Stoessl AJ,Martin WR,Clark C,Adam MJ,Ammann W,Beckman JH,Bergstrom M,Harrop R,Rogers JG,Ruth TJ

更新日期：1986-05-01 00:00:00

abstract：:In thymomas associated with myasthenia gravis (MG), the authors found that perivascular infiltrates of memory lymphocytes and mature dendritic cells (DCs) were more frequent in patients with early improvement after thymectomy than in patients without response to thymectomy. Although these findings may be limited to pa...

journal_title：Neurology

authors： Nagane Y,Utsugisawa K,Akutsu H,Sato Y,Terayama Y

更新日期：2005-09-13 00:00:00

abstract：OBJECTIVE:To investigate the genetic and physiologic basis of the neuromuscular symptoms of hypotonia-cystinuria syndrome (HCS) and isolated PREPL deficiency, and their response to therapy. METHODS:We performed molecular genetic, histochemical, immunoblot, and ultrastructural studies, investigated neuromuscular transm...

journal_title：Neurology

authors： Régal L,Shen XM,Selcen D,Verhille C,Meulemans S,Creemers JW,Engel AG

更新日期：2014-04-08 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：OBJECTIVE:In multiple sclerosis (MS), the aim of therapies is to prevent the accumulation of irreversible disability. This is difficult to assess given the short time course of clinical trials. MRI markers and relapses are often used as surrogate of disability in MS studies, but their validity remains controversial. We...

journal_title：Neurology

authors： Sormani MP,Li DK,Bruzzi P,Stubinski B,Cornelisse P,Rocak S,De Stefano N

更新日期：2011-11-01 00:00:00

abstract：:The mechanism by which prednisone improves muscle strength and function in Duchenne muscular dystrophy (DMD) is unknown. We addressed the possibility that clinical improvement was related to prednisone-induced alterations in skeletal muscle dystrophin. We performed muscle biopsies on patients at the conclusion of a ra...

journal_title：Neurology

authors： Burrow KL,Coovert DD,Klein CJ,Bulman DE,Kissel JT,Rammohan KW,Burghes AH,Mendell JR

更新日期：1991-05-01 00:00:00

abstract：OBJECTIVE:To compare the clinical features of patients with chronic daily headache (CDH) with idiopathic intracranial hypertension without papilledema (IIHWOP) to those with normal CSF pressure. METHODS:A case-control study was conducted at a tertiary headache center. Cases consisted of 25 consecutive patients (24 wom...

journal_title：Neurology

authors： Wang SJ,Silberstein SD,Patterson S,Young WB

更新日期：1998-07-01 00:00:00

abstract：:A 3-year 8-month-old girl died after 14 months of illness characterized by episodes of intermittent ataxia associated with oculomotor palsy, hypotonia, mental confusion, and disturbances of consciousness. In the last 4 months of life, there were signs of liver dysfunction. Pyruvate dehydrogenase and alpha-ketoglutarat...

journal_title：Neurology

authors： DiDonato S,Rimoldi M,Moise A,Bertagnoglio B,Uziel G

更新日期：1979-12-01 00:00:00

abstract：OBJECTIVE:To examine the lifetime prevalence and the concordance risk of cluster headache (CH) in a twin sample representative of the Swedish general population. METHODS:The authors assessed CH as defined by the second edition of the International Classification of Headache Disorders in 31,750 registered twins born fr...

journal_title：Neurology

authors： Ekbom K,Svensson DA,Pedersen NL,Waldenlind E

更新日期：2006-09-12 00:00:00

abstract：BACKGROUND:In children with tuberous sclerosis, the PET tracer alpha[11C]methyl-L-tryptophan (AMT) has been shown to be selectively taken up by epileptogenic tubers, thus allowing differentiation from nonepileptogenic tubers in the interictal state. OBJECTIVE:To determine whether cortical areas showing increased AMT u...

journal_title：Neurology

authors： Juhász C,Chugani DC,Muzik O,Shah A,Asano E,Mangner TJ,Chakraborty PK,Sood S,Chugani HT

更新日期：2003-03-25 00:00:00

abstract：:Isovaleric acidemia (IVA) is an inborn error of leucine metabolism, resulting in an accumulation of isovaleric acid in the body fluids. The neuropathologic findings in an 11-day-old infant with IVA consisted of diffuse cerebral edema, massive cerebellar hemorrhage, upward transtentorial herniation, and focal degenerat...

journal_title：Neurology

authors： Fischer AQ,Challa VR,Burton BK,McLean WT

更新日期：1981-06-01 00:00:00

abstract：:A patient with thymoma-associated neuromyotonia and voltage-gated potassium channel (Kv1.2 and Kv1.6) antibodies by immunoprecipitation and rat brain immunolabeling was treated successfully with immunoadsorption and cyclophosphamide. Curiously, glutamic acid decarboxylase antibodies, absent at onset, appeared later. S...

journal_title：Neurology

authors： Antozzi C,Frassoni C,Vincent A,Regondi MC,Andreetta F,Bernasconi P,Ciano C,Chang T,Cornelio F,Spreafico R,Mantegazza R

更新日期：2005-04-12 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：: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