Comparison of Phenotypic Characteristics and Prognosis Between Black and White Patients in a Tertiary ALS Clinic.

abstract：:Lubag (X-linked dystonia-parkinsonism) has been considered a sex-linked recessive trait and has been mapped to the pericentromeric region of the X chromosome. We studied a 54-year-old man with lubag and two of his female first cousins. Genetic typing was carried out using X chromosome markers. Fluorodopa PET was perfo...

journal_title：Neurology

authors： Waters CH,Takahashi H,Wilhelmsen KC,Shubin R,Snow BJ,Nygaard TG,Moskowitz CB,Fahn S,Calne DB

更新日期：1993-08-01 00:00:00

abstract：BACKGROUND:Glycine encephalopathy, also known as nonketotic hyperglycinemia (NKH), is an autosomal recessive disorder caused by a defect in the glycine cleavage system. NKH is classically associated with neonatal apnea, lethargy, hypotonia, and seizures, followed by severe psychomotor retardation in those who survive. ...

journal_title：Neurology

authors： Hoover-Fong JE,Shah S,Van Hove JL,Applegarth D,Toone J,Hamosh A

更新日期：2004-11-23 00:00:00

abstract：BACKGROUND:The histopathology of AIDS-associated myelopathy (AM) closely resembles that of myelopathies due to cobalamin or folate deficiency, with white matter vacuolization in the spinal cord. The pathogenesis of AM appears unrelated to direct HIV infection of the spinal cord. There is abnormal trans-methylation meta...

journal_title：Neurology

authors： Di Rocco A,Werner P,Bottiglieri T,Godbold J,Liu M,Tagliati M,Scarano A,Simpson D

更新日期：2004-10-12 00:00:00

abstract：:We administered the Mini-Mental State Exam (MMSE) to 194 healthy men and women, ages 40 to 89 years. Total score was significantly associated with age (p less than 0.0001), but not vocabulary, education, Beck's Depression Inventory Score, or sex. The lowest quartile cutoff scores for the MMSE by decade were 40s - 29; ...

journal_title：Neurology

authors： Bleecker ML,Bolla-Wilson K,Kawas C,Agnew J

更新日期：1988-10-01 00:00:00

abstract：:Inclusion-body myositis (IBM) is an inflammatory muscle disease that has proven resistant to treatment. Tumor necrosis factor molecules have been detected in muscle biopsies from patients with IBM. Etanercept is a TNFalpha receptor fusion protein that binds and inactivates tumor necrosis factor. Nine patients were tre...

journal_title：Neurology

authors： Barohn RJ,Herbelin L,Kissel JT,King W,McVey AL,Saperstein DS,Mendell JR

更新日期：2006-01-24 00:00:00

abstract：OBJECTIVE:To review the disciplinary process by which the American Academy of Neurology (AAN) enforces its formalized standards of professional conduct. METHODS:We reviewed the AAN's Disciplinary Action Policy. We tracked the elapsed time from receipt to final decision of all allegations ("complaints") of improper con...

journal_title：Neurology

authors： Hutchins JC,Sagsveen MG,Larriviere D

更新日期：2010-12-14 00:00:00

abstract：:Thyrotropin-releasing hormone (TRH) exerts many effects upon spinal cord function in animals, and may also play a role in human spinal cord function. We have used the technique of quantitative autoradiography to anatomically localize specific receptors for TRH within human spinal cord. Highest concentrations of TRH re...

journal_title：Neurology

authors： Manaker S,Winokur A,Rhodes CH,Rainbow TC

更新日期：1985-03-01 00:00:00

abstract：:This cross-sectional mail survey evaluated the prevalence and predictors of unconventional therapy (UT) utilization among 569 community-based people with MS. One-third reported having visited a UT practitioner in the last 6 months. Utilizers tended to report at least one medical comorbidity, earn at least $50,000 a ye...

journal_title：Neurology

authors： Schwartz CE,Laitin E,Brotman S,LaRocca N

更新日期：1999-02-01 00:00:00

abstract：OBJECTIVE:To analyze the effect of age-related DNA methylation changes in multiple cytosine-phosphate-guanine (CpG) sites (biological age [b-age]) on patient outcomes at 3 months after an ischemic stroke. METHODS:We included 511 patients with first-ever acute ischemic stroke assessed at Hospital del Mar (Barcelona, Sp...

journal_title：Neurology

authors： Soriano-Tárraga C,Mola-Caminal M,Giralt-Steinhauer E,Ois A,Rodríguez-Campello A,Cuadrado-Godia E,Gómez-González A,Vivanco-Hidalgo RM,Fernández-Cadenas I,Cullell N,Roquer J,Jiménez-Conde J

更新日期：2017-08-22 00:00:00

abstract：OBJECTIVE:To validate, examine the internal validity, and adapt to children the electronic version of the composite cerebellar functional severity (CCFS) score. METHODS:In this multicenter study, we compared the validated manual device with the new electronic version (n = 46) and analyzed its kinetics in 146 patients ...

journal_title：Neurology

authors： Filipovic Pierucci A,Mariotti C,Panzeri M,Giunti P,Boesch S,Schulz JB,Pandolfo M,Durr A,Tezenas du Montcel S,EFACTS Study Group.

更新日期：2015-03-24 00:00:00

abstract：BACKGROUND:The head impulse test (HIT) is a useful bedside test to identify peripheral vestibular deficits. However, such a deficit of the vestibulo-ocular reflex (VOR) may not be diagnosed because corrective saccades cannot always be detected by simple observation. The scleral search coil technique is the gold standar...

journal_title：Neurology

authors： MacDougall HG,Weber KP,McGarvie LA,Halmagyi GM,Curthoys IS

更新日期：2009-10-06 00:00:00

abstract：:The progressive myoclonus epilepsies (PMEs) are a devastating group of rare disorders(1) that manifest with increasing action myoclonus, which is also present at rest but activates with stimuli such as noise, light, or touch. Ultimately, patients become wheelchair-bound and experience early death. Neurologic signs tha...

journal_title：Neurology

authors： Knupp K,Wirrell E

更新日期：2014-02-04 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：OBJECTIVE:In this study, we compared a cognition-specific computer-based cognitive training program with a motion-controlled computer sports game that is not cognition-specific for their ability to enhance cognitive performance in various cognitive domains in patients with Parkinson disease (PD). METHODS:Patients with...

journal_title：Neurology

authors： Zimmermann R,Gschwandtner U,Benz N,Hatz F,Schindler C,Taub E,Fuhr P

更新日期：2014-04-08 00:00:00

abstract：:Hypohidrosis predisposes to hyperthermia and may indicate generalized thermoregulatory failure. To assess the sweating capacity in human poikilothermia, we performed a quantitative analysis of the central and peripheral sudomotor pathways in four women with acquired poikilothermia (aged 29 to 38 years) and nine contro...

journal_title：Neurology

authors： MacKenzie MA,Schönbaum E,Hermus AR,Wollersheim HC,Thien T,Smals AG,Kloppenborg PW

更新日期：1995-08-01 00:00:00

abstract：:The "modified host protein" model of scrapie proposes that the transmissible agent is composed of the degradation-resistant protein, Sp33-37, and that clinical and pathologic signs result from neurotoxic accumulations of this protein. Sp33-37 is an abnormal, amyloidogenic isoform of the normally occurring cellular pro...

journal_title：Neurology

authors： Bendheim PE,Brown HR,Rudelli RD,Scala LJ,Goller NL,Wen GY,Kascsak RJ,Cashman NR,Bolton DC

更新日期：1992-01-01 00:00:00

abstract：OBJECTIVE:To determine the incidence and clinical effects of antibodies that develop during treatment with natalizumab. METHODS:In two randomized, double-blind, placebo-controlled studies (natalizumab safety and efficacy in relapsing remitting multiple sclerosis [MS, AFFIRM] and safety and efficacy of natalizumab in c...

journal_title：Neurology

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

更新日期：2007-10-02 00:00:00

abstract：OBJECTIVE:To quantify the isoprostane 8,12-iso-iPF2alpha-VI in brain tissues obtained from patients with AD, patients with frontotemporal dementia (FTD), and controls. BACKGROUND:Enhanced brain oxidative stress with secondary damage to cellular macromolecules may play a role in the pathogenesis of AD and FTD. The isop...

journal_title：Neurology

authors： Yao Y,Zhukareva V,Sung S,Clark CM,Rokach J,Lee VM,Trojanowski JQ,Praticò D

更新日期：2003-08-26 00:00:00

abstract：:A specific form of deep cerebral hemisphere infarction was identified in 11 of 1,600 stroke register patients. Despite the CT finding of a deep striatocapsular lesion, these patients showed evidence of both cortical and capsular abnormalities. Angiography showed significant proximal carotid artery lesions as well as e...

journal_title：Neurology

authors： Bladin PF,Berkovic SF

更新日期：1984-11-01 00:00:00

abstract：:The authors studied the association of an exon 4 (E4*epsilon2/3/4) and three promoter polymorphisms of APOE with disease course and severity stratified by gender in 221 patients with multiple sclerosis from two overlapping population-based prevalence cohorts. Women carriers of the E4*epsilon2 allele took longer to att...

journal_title：Neurology

authors： Kantarci OH,Hebrink DD,Achenbach SJ,Pittock SJ,Altintas A,Schaefer-Klein JL,Atkinson EJ,De Andrade M,McMurray CT,Rodriguez M,Weinshenker BG

更新日期：2004-03-09 00:00:00

abstract：:We surveyed 297 patients with Parkinson disease (PD) with systematic screens and rigorous definitional criteria. Pathologic hypersexuality lifetime prevalence was 2.4%. Compulsive shopping was 0.7%. Combined with our pathologic gambling data, the lifetime prevalence of these behaviors was 6.1% and increases to 13.7% i...

journal_title：Neurology

authors： Voon V,Hassan K,Zurowski M,de Souza M,Thomsen T,Fox S,Lang AE,Miyasaki J

更新日期：2006-10-10 00:00:00

abstract：OBJECTIVE:To determine the current best practice for treatment of infantile spasms in children. METHODS:Database searches of MEDLINE from 1966 and EMBASE from 1980 and searches of reference lists of retrieved articles were performed. Inclusion criteria were the documented presence of infantile spasms and hypsarrhythmi...

journal_title：Neurology

authors： Mackay MT,Weiss SK,Adams-Webber T,Ashwal S,Stephens D,Ballaban-Gill K,Baram TZ,Duchowny M,Hirtz D,Pellock JM,Shields WD,Shinnar S,Wyllie E,Snead OC 3rd,American Academy of Neurology.,Child Neurology Society.

更新日期：2004-05-25 00:00:00

abstract：:We administered a battery of cognitive tests to 41 recently diagnosed Parkinson patients and 41 controls to assess the early neuropsychological changes associated with Parkinson's disease (PD). Parkinson subjects did as well as controls on tasks assessing attention and select language and visuospatial measures. Howeve...

journal_title：Neurology

authors： Levin BE,Llabre MM,Weiner WJ

更新日期：1989-04-01 00:00:00

abstract：:The goal of pharmacologic therapy for patients with epilepsy is to suppress seizures without side effects. There is growing interest in developing antiepileptogenic drugs capable of preventing the onset of seizures in patients at high risk and inducing a permanent remission of seizures in patients with epilepsy, i.e.,...

journal_title：Neurology

authors： Schachter SC

更新日期：2002-11-12 00:00:00

abstract：:Three family members intoxicated with methyl bromide presented with a variety of neuropsychiatric manifestations including coma, severe status epilepticus, hyporeflexia, and acute psychosis. The simulation of Reye syndrome in the child emphasizes the need for careful toxicologic screening of all children presenting wi...

journal_title：Neurology

authors： Shield LK,Coleman TL,Markesbery WR

更新日期：1977-10-01 00:00:00

abstract：:We describe a 43-year-old neurologically intact patient who reported episodes of diplopia and oscillopsia associated with a right-beating nystagmus and a skew deviation. These symptoms and signs were related to a left posterior epileptic EEG discharge. We suggest that these ocular motor signs derived from an ictal act...

journal_title：Neurology

authors： Galimberti CA,Versino M,Sartori I,Manni R,Martelli A,Tartara A

更新日期：1998-05-01 00:00:00

abstract：OBJECTIVES:To assess the association of primary retinal inflammation, namely retinal periphlebitis (RP) and microcystic macular edema, with clinical, brain, and retinal imaging biomarkers of multiple sclerosis (MS) severity. METHODS:One hundred patients with MS underwent a neurologic and ophthalmic examination, MRI, a...

journal_title：Neurology

authors： Ortiz-Pérez S,Martínez-Lapiscina EH,Gabilondo I,Fraga-Pumar E,Martínez-Heras E,Saiz A,Sanchez-Dalmau B,Villoslada P

更新日期：2013-09-03 00:00:00

abstract：OBJECTIVE:To assess the clinical, urodynamic, and neurophysiologic features of patients with persisting bladder, bowel, and sexual dysfunction after transverse myelitis in myelin oligodendrocyte glycoprotein antibody (MOG-Ab) disease. METHODS:Patients with a history of MOG-Ab disease-related transverse myelitis seen p...

journal_title：Neurology

authors： Li V,Malladi P,Simeoni S,Pakzad M,Everett R,Satukijchai C,Leite MI,Palace J,Panicker JN

更新日期：2020-11-24 00:00:00

abstract：OBJECTIVE:To evaluate the usefulness of a mismatch between the severity of acute clinical manifestations and the diffusion-weighted imaging (DWI) lesion in predicting early stroke outcome and infarct volume. METHODS:One hundred sixty-six patients with a hemispheric ischemic stroke of <12 hours' duration were studied. ...

journal_title：Neurology

authors： Dávalos A,Blanco M,Pedraza S,Leira R,Castellanos M,Pumar JM,Silva Y,Serena J,Castillo J

更新日期：2004-06-22 00:00:00

abstract：OBJECTIVE:To determine the potential of detection in CSF of specific Borrelia burgdorferi antigen, OspA, as a marker of infection in neurologic Lyme disease and compare this with the detection of antibody. DESIGN:CSF from 83 neurologic patients in an area highly endemic for Lyme disease was examined prospectively for ...

journal_title：Neurology

authors： Coyle PK,Schutzer SE,Deng Z,Krupp LB,Belman AL,Benach JL,Luft BJ

更新日期：1995-11-01 00:00:00