Disease severity in children and adults with Pompe disease related to age and disease duration.

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：OBJECTIVE:To determine whether metformin is associated with a lower incidence of dementia than sulfonylureas. METHODS:This was a retrospective cohort study of US veterans ≥65 years of age with type 2 diabetes who were new users of metformin or a sulfonylurea and had no dementia. Follow-up began after 2 years of therap...

journal_title：Neurology

authors： Orkaby AR,Cho K,Cormack J,Gagnon DR,Driver JA

更新日期：2017-10-31 00:00:00

abstract：OBJECTIVE:This study prospectively examined the relationships among late night salivary cortisol (NSC) levels and depressive symptoms, memory performance, and hippocampal volumes in patients with medically intractable temporal lobe epilepsy (TLE) and the potential mediating effects of cortisol in the relationships betw...

journal_title：Neurology

authors： Busch RM,Frazier T,Chapin JS,Hamrahian AH,Diehl B,Alexopoulos A,Unnwongse K,Naugle RI,Kubu CS,Tesar GE,Najm IM

更新日期：2012-04-03 00:00:00

abstract：:The authors reviewed the incidence of cancer, repetitive nerve stimulation findings, and response to treatment in 73 patients with Lambert-Eaton myasthenic syndrome. Thirty-one patients (42%) had lung cancer, 29 small cell. Doubling of the compound motor action potential amplitude in three tested distal muscles was se...

journal_title：Neurology

authors： Tim RW,Massey JM,Sanders DB

更新日期：2000-06-13 00:00:00

abstract：:Trigeminal neuralgia is an uncommon but troublesome symptom of multiple sclerosis that can be refractory to conventional treatments. Misoprostol, a long-acting prostaglandin E1 analogue, relieved pain in six of seven patients who had failed to respond to conventional pharmacologic therapy. ...

journal_title：Neurology

authors： Reder AT,Arnason BG

更新日期：1995-06-01 00:00:00

abstract：OBJECTIVE:To determine the role of oxidative stress in mediating HIV dementia and to identify novel therapeutic compounds that may block this oxidative stress. METHODS:Brain tissue from patients with HIV encephalitis and macaques with simian immune deficiency virus encephalitis was immunostained for lipid peroxidation...

journal_title：Neurology

authors： Turchan J,Pocernich CB,Gairola C,Chauhan A,Schifitto G,Butterfield DA,Buch S,Narayan O,Sinai A,Geiger J,Berger JR,Elford H,Nath A

更新日期：2003-01-28 00:00:00

abstract：:In the first half of the 19th century, European physicians-including Marshall Hall, Moritz Romberg, and Bernardus Brach-described loss of postural control in darkness of patients with severely compromised proprioception. Romberg and Brach emphasized the relationship between this sign and tabes dorsalis. Later, other n...

journal_title：Neurology

authors： Lanska DJ,Goetz CG

更新日期：2000-10-24 00:00:00

abstract：:We present a case of uncontrolled temporal lobe epilepsy due to a focal left temporal neuronal heterotopia associated with the nevoid-basal-cell carcinoma syndrome. This is the first pathologically described lesion associated with temporal lobe epilepsy in this syndrome. Because the patient's seizures resolved after a...

journal_title：Neurology

authors： Hogan RE,Tress B,Gonzales MF,King JO,Cook MJ

更新日期：1996-02-01 00:00:00

abstract：:We tested the hypothesis that asymptomatic carriers of dopa-responsive dystonia (DRD) have increased dopamine D2 receptors in the striatum that protect them from the clinical manifestations of dopaminergic deficiency. We examined striatal D2-receptor binding in (1) symptomatic subjects (treated and untreated) and (2) ...

journal_title：Neurology

authors： Kishore A,Nygaard TG,de la Fuente-Fernandez R,Naini AB,Schulzer M,Mak E,Ruth TJ,Calne DB,Snow BJ,Stoessl AJ

更新日期：1998-04-01 00:00:00

abstract：:Acute in-stent thrombosis is a well-known complication of carotid angioplasty and stenting (CAS) and often due to resistance to or inadequate treatment with platelet-inhibiting agents. The authors report three cases of a delayed in-stent thrombosis after more than a week but less than 3 months after CAS. In all cases,...

journal_title：Neurology

authors： Buhk JH,Wellmer A,Knauth M

更新日期：2006-05-23 00:00:00

abstract：:We studied the effect of CSF drainage on cerebral blood flow (CBF) in normal pressure hydrocephalus (NPH) and non-NPH dementia using the 133Xenon inhalation technique. Dementia patients had lower CBF than matched elderly normals. Flow values for NPH and non-NPH patients did not differ before or after CSF drainage. CBF...

journal_title：Neurology

authors： Kushner M,Younkin D,Weinberger J,Hurtig H,Goldberg H,Reivich M

更新日期：1984-01-01 00:00:00

abstract：OBJECTIVE:This study correlated the extent of abnormalities detected by different magnetic resonance imaging (MRI) techniques [proton density (PD)-weighted, T1-weighted, and magnetization transfer imaging (MTI)] with the overall cognitive, frontal lobe, and memory impairments in patients with MS. PATIENTS:There were 3...

journal_title：Neurology

authors： Rovaris M,Filippi M,Falautano M,Minicucci L,Rocca MA,Martinelli V,Comi G

更新日期：1998-06-01 00:00:00

abstract：:The syndrome of increased intracranial pressure without hydrocephalus or mass lesion and with normal CSF composition, previously referred to as pseudotumor cerebri, is a diagnosis of exclusion now termed idiopathic intracranial hypertension (IIH). Diagnostic criteria of this disorder have not been updated since the Mo...

journal_title：Neurology

authors： Friedman DI,Jacobson DM

更新日期：2002-11-26 00:00:00

abstract：:Monocytes are recruited to active sites of multiple sclerosis (MS) demyelination and may promote local tissue injury by generating an inflammatory response, mediated in part by the production of toxic oxygen metabolites. Corticosteroids are frequently and effectively used to ameliorate MS exacerbations, despite inadeq...

journal_title：Neurology

authors： Fisher M,Levine PH,Doyle EM,Arpano MM,Bergeron DA,Cohen RA,Hoogasian JJ

更新日期：1991-02-01 00:00:00

abstract：:Myoclonus--dystonia is a genetically heterogeneous autosomal dominant disorder caused by a mutation in the D2 dopamine receptor on chromosome 11 and a locus on chromosome 7q21-q31. The authors tested linkage to the chromosome 7q candidate region in four families with either myoclonic dystonia (n = 3) or essential myoc...

journal_title：Neurology

authors： Vidailhet M,Tassin J,Durif F,Nivelon-Chevallier A,Agid Y,Brice A,Dürr A

更新日期：2001-05-08 00:00:00

abstract：OBJECTIVE:Paroxysmal kinesigenic dyskinesia (PKD) is a rare disorder characterized by recurrent attacks of hyperkinetic movements. PKD can be isolated or associated with benign infantile seizures as part of the infantile convulsions with choreoathetosis (ICCA) syndrome. Mutations in the PRRT2 gene were recently identif...

journal_title：Neurology

authors： Méneret A,Grabli D,Depienne C,Gaudebout C,Picard F,Dürr A,Lagroua I,Bouteiller D,Mignot C,Doummar D,Anheim M,Tranchant C,Burbaud P,Jedynak CP,Gras D,Steschenko D,Devos D,Billette de Villemeur T,Vidailhet M,Brice A,

更新日期：2012-07-10 00:00:00

abstract：OBJECTIVE:Evaluating the TBI incidence during the COVID-19 pandemic era compared with similar period in previous year. BACKGROUND:The emergence of SARS-CoV2 influenced the evolution of governmental and hospital policy worldwide, which might eventually impact many aspects. The incidence of many diseases in the Emergenc...

journal_title：Neurology

authors： Prawiroharjo P,Pangeran D,Supriawan H,Lastri D,Mayza A,Zairinal RA,Dewi AR,Asmaniar F,Ramli Y

更新日期：2020-11-17 00:00:00

abstract：OBJECTIVE:To determine the cumulative incidence of epilepsy in a population-based cohort of patients with multiple sclerosis (MS) and to investigate the association between epilepsy and clinical features of MS. METHODS:All available patients in the Swedish MS register (n = 14,545) and 3 age- and sex-matched controls p...

journal_title：Neurology

authors： Burman J,Zelano J

更新日期：2017-12-12 00:00:00

abstract：:The authors describe a patient with bilateral papilledema, visual field abnormalities, poorly reactive pupils, meningeal enhancement on cranial MRI, and diffuse brain parenchymal hypervascularity. The opening pressure at the time of lumbar puncture was normal, and results of other CSF studies were normal. All abnormal...

journal_title：Neurology

authors： O'Halloran HS,Berger JR,Baker RS,Lee WB,Pearson PA,Ryan S

更新日期：1999-12-10 00:00:00

abstract：OBJECTIVE:Subjects with diabetes are reported to have an increased risk of dementia and cognitive impairment. However, the underlying causes remain unknown. We investigated the longitudinal associations between midlife insulin secretion, glucose metabolism, and the subsequent development of Alzheimer disease (AD) and d...

journal_title：Neurology

authors： Rönnemaa E,Zethelius B,Sundelöf J,Sundström J,Degerman-Gunnarsson M,Berne C,Lannfelt L,Kilander L

更新日期：2008-09-30 00:00:00

abstract：:Tizanidine, an imidazoline that acts as an agonist at alpha 2-adrenergic receptors, has been shown to be effective in reducing spasticity caused by MS. This multicenter study (14 sites) assessed the efficacy and safety of oral tizanidine in patients who had spinal cord injury of > 12 months' duration. Of the 124 patie...

journal_title：Neurology

authors： Nance PW,Bugaresti J,Shellenberger K,Sheremata W,Martinez-Arizala A

更新日期：1994-11-01 00:00:00

abstract：OBJECTIVE:To investigate the relationship between cerebral amyloid angiopathy (CAA), dementia, and cognitive function in an autopsy sample of 211 Japanese-American men from the population-based Honolulu-Asia Aging Study. METHODS:Starting in 1991, participants were assessed with the Cognitive Abilities Screening Instru...

journal_title：Neurology

authors： Pfeifer LA,White LR,Ross GW,Petrovitch H,Launer LJ

更新日期：2002-06-11 00:00:00

abstract：BACKGROUND:Several studies indicate semantic fluency more sensitively discriminates patients with Alzheimer disease (AD) from normal elderly persons, with disproportionate impairment of semantic over phonemic fluency. OBJECTIVE:To determine the ability of abbreviated fluency measures in the clinic setting (1-minute le...

journal_title：Neurology

authors： Canning SJ,Leach L,Stuss D,Ngo L,Black SE

更新日期：2004-02-24 00:00:00

abstract：:A woman had a progressive neurologic syndrome beginning at age 3 and lasting for three decades. Clinical manifestations included severe mental deterioration, spastic paralysis, myoclonus, and tremors. A postmortem examination showed ubiquitous infiltration of neurons by lipofuscin and deposits of pigment in the globus...

journal_title：Neurology

authors： Jervis GA,Pullarkat RK

更新日期：1978-05-01 00:00:00

abstract：OBJECTIVE:To revise the 1999 Airlie House consensus guidelines for the design and implementation of preclinical therapeutic studies and clinical trials in amyotrophic lateral sclerosis (ALS). METHODS:A consensus committee comprising 140 key members of the international ALS community (ALS researchers, clinicians, patie...

journal_title：Neurology

authors： van den Berg LH,Sorenson E,Gronseth G,Macklin EA,Andrews J,Baloh RH,Benatar M,Berry JD,Chio A,Corcia P,Genge A,Gubitz AK,Lomen-Hoerth C,McDermott CJ,Pioro EP,Rosenfeld J,Silani V,Turner MR,Weber M,Brooks BR,Miller

更新日期：2019-04-02 00:00:00

abstract：BACKGROUND:Hematopoietic stem cell transplantation (HSCT) may slow the progression of Krabbe disease (KD) if performed early in the disease. The authors' studies indicate that more than 90% of patients with KD have severe abnormalities in peripheral nerve conduction. OBJECTIVE:To assess the effect of HSCT on nerve con...

journal_title：Neurology

authors： Siddiqi ZA,Sanders DB,Massey JM

更新日期：2006-07-25 00:00:00

abstract：:A model of latent infection by varicella-zoster virus (VZV) was obtained in the adult rat. Inoculation of VZV-infected cells in the skin led to infection of the peripheral nervous system. Latency was characterized by a long-lasting presence of the viral genome, of selected viral gene transcripts, and of at least one v...

journal_title：Neurology

authors： Sadzot-Delvaux C,Debrus S,Nikkels A,Piette J,Rentier B

更新日期：1995-12-01 00:00:00

abstract：:Central nervous system involvement in melioidosis is unusual. We describe a 34-year-old man who developed a Pseudomonas pseudomallei meningitis, manifested as bilateral third-nerve palsies, 13 years after having been in southeast Asia. Diagnosis was established by a fourfold rise in the serum antibody titer for the ba...

journal_title：Neurology

authors： Beck RW,Janssen RS,Smiley ML,Schatz NJ,Savino PJ,Rubin DH

更新日期：1984-01-01 00:00:00

abstract：OBJECTIVE:To determine whether whole-brain, event-related fMRI can distinguish healthy older adults with known Alzheimer disease (AD) risk factors (family history, APOE epsilon4) from controls using a semantic memory task involving discrimination of famous from unfamiliar names. METHODS:Sixty-nine cognitively asymptom...

journal_title：Neurology

authors： Seidenberg M,Guidotti L,Nielson KA,Woodard JL,Durgerian S,Antuono P,Zhang Q,Rao SM

更新日期：2009-08-25 00:00:00

abstract：OBJECTIVE:We developed the Recurrence and Recovery Questionnaire (RRQ) by converting the Pediatric Stroke Outcome Measure (PSOM) to a questionnaire for telephone interview and sought to validate the RRQ in a large cohort. METHOD:We analyzed parents' RRQ responses and same-day PSOM assessments for 232 children who had ...

journal_title：Neurology

authors： Lo WD,Ichord RN,Dowling MM,Rafay M,Templeton J,Halperin A,Smith SE,Licht DJ,Moharir M,Askalan R,Deveber G,International Pediatric Stroke Study (IPSS) Investigators.

更新日期：2012-08-28 00:00:00