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


OBJECTIVE:To compare characteristics between Black and White patients with amyotrophic lateral sclerosis (ALS) in order to identify disparities and phenotypic variability. METHODS:We performed database review for patients seen between 1997 and 2020 at the Emory ALS Center in Atlanta, Georgia. Patients with ALS were included for analyses if race was self-reported as Black or White and symptom onset was prior to January 1, 2017. Variables examined include race, age at onset, diagnostic delay, site of onset, median income, C9orf72 mutation status, feeding tube and tracheostomy status, vital capacity, Amyotrophic Lateral Sclerosis Functional Rating Scale-revised(ALSFRS-R) score, and survival time. RESULTS:A total of 2,363 patient records were queried, and 1,298 were included in analysis; 203 self-identified as Black and 1,095 as White. Black patients had younger age at symptom onset, lower frequency of C9orf72 mutations, lower median income, longer diagnostic delay, and lower baseline ALSFRS-R and vital capacity compared to White patients. Black patients had a longer median survival than White patients; however, race was not an independent predictor of survival time when controlling for age at symptom onset, bulbar onset, and C9orf72 positivity. CONCLUSIONS:Black patients with ALS had longer median survival compared to White patients, but race was not independently associated with survival after controlling for age, site of onset, and C9orf72 status, factors known to predict prognosis. Black patients with ALS had longer diagnostic delay and lower baseline ventilatory and functional status at first clinic visit compared to White patients, which could be suggestive of barriers to tertiary care. Further studies are needed to identify the underlying causes of ALS racial differences.






Brand D,Polak M,Glass JD,Fournier CN




Has Abstract


2020-12-28 00:00:00








  • Phenotypic expression of X-linked dystonia-parkinsonism (lubag) in two women.

    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...


    pub_type: 杂志文章


    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

  • Natural history of nonketotic hyperglycinemia in 65 patients.

    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. ...


    pub_type: 杂志文章


    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

  • Treatment of AIDS-associated myelopathy with L-methionine: a placebo-controlled study.

    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...


    pub_type: 临床试验,杂志文章,随机对照试验


    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

  • Age-specific norms for the Mini-Mental State Exam.

    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; ...


    pub_type: 杂志文章


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

    更新日期:1988-10-01 00:00:00

  • Pilot trial of etanercept in the treatment of inclusion-body myositis.

    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...


    pub_type: 临床试验,杂志文章


    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

  • Upholding professionalism: the disciplinary process of the American Academy of Neurology.

    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...


    pub_type: 杂志文章


    authors: Hutchins JC,Sagsveen MG,Larriviere D

    更新日期:2010-12-14 00:00:00

  • Autoradiographic localization of thyrotropin-releasing hormone (TRH) receptors in human spinal cord.

    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...


    pub_type: 杂志文章


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

    更新日期:1985-03-01 00:00:00

  • Utilization of unconventional treatments by persons with MS: is it alternative or complementary?

    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...


    pub_type: 杂志文章


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

    更新日期:1999-02-01 00:00:00

  • Biological age is better than chronological as predictor of 3-month outcome in ischemic stroke.

    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...


    pub_type: 杂志文章


    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

  • Quantifiable evaluation of cerebellar signs in children.

    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 ...


    pub_type: 杂志文章,多中心研究


    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

  • The video head impulse test: diagnostic accuracy in peripheral vestibulopathy.

    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...


    pub_type: 杂志文章


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

    更新日期:2009-10-06 00:00:00

  • Progressive myoclonic epilepsies: it takes a village to make a diagnosis.

    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...


    pub_type: 评论,杂志文章


    authors: Knupp K,Wirrell E

    更新日期:2014-02-04 00:00:00

  • Role of cortisol in mood and memory in patients with intractable temporal lobe epilepsy.

    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...


    pub_type: 杂志文章


    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

  • Cognitive training in Parkinson disease: cognition-specific vs nonspecific computer training.

    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...


    pub_type: 杂志文章


    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

  • Sudomotor function in human poikilothermia.

    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...


    pub_type: 杂志文章


    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

  • Nearly ubiquitous tissue distribution of the scrapie agent precursor protein.

    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...


    pub_type: 杂志文章


    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

  • The incidence and significance of anti-natalizumab antibodies: results from AFFIRM and SENTINEL.

    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...


    pub_type: 杂志文章,随机对照试验


    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

  • Enhanced brain levels of 8,12-iso-iPF2alpha-VI differentiate AD from frontotemporal dementia.

    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...


    pub_type: 杂志文章


    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

  • Striatocapsular infarction: large infarcts in the lenticulostriate arterial territory.

    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...


    pub_type: 杂志文章


    authors: Bladin PF,Berkovic SF

    更新日期:1984-11-01 00:00:00

  • Association of APOE polymorphisms with disease severity in MS is limited to women.

    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...


    pub_type: 杂志文章


    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

  • Prevalence of repetitive and reward-seeking behaviors in Parkinson disease.

    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...


    pub_type: 杂志文章


    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

  • Practice parameter: medical treatment of infantile spasms: report of the American Academy of Neurology and the Child Neurology Society.

    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...


    pub_type: 杂志文章,评审


    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

  • Cognitive impairments associated with early Parkinson's disease.

    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...


    pub_type: 杂志文章


    authors: Levin BE,Llabre MM,Weiner WJ

    更新日期:1989-04-01 00:00:00

  • Drug-mediated antiepileptogenesis in humans.

    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.,...


    pub_type: 杂志文章,评审


    authors: Schachter SC

    更新日期:2002-11-12 00:00:00

  • Methyl bromide intoxication: neurologic features, including simulation of Reye syndrome.

    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...


    pub_type: 杂志文章


    authors: Shield LK,Coleman TL,Markesbery WR

    更新日期:1977-10-01 00:00:00

  • Epileptic skew deviation.

    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...


    pub_type: 杂志文章


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

    更新日期:1998-05-01 00:00:00

  • Retinal periphlebitis is associated with multiple sclerosis severity.

    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...


    pub_type: 杂志文章


    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

  • A clinico-neurophysiological study of urogenital dysfunction in MOG-antibody transverse myelitis.

    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...


    pub_type: 杂志文章


    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

  • The clinical-DWI mismatch: a new diagnostic approach to the brain tissue at risk of infarction.

    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. ...


    pub_type: 杂志文章


    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

  • Detection of Borrelia burgdorferi-specific antigen in antibody-negative cerebrospinal fluid in neurologic Lyme disease.

    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 ...


    pub_type: 杂志文章


    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