Muscle acid protease activity in amyotrophic lateral sclerosis: correlation with clinical and pathologic features.

Abstract:

:Acid protease activity was increased in skeletal muscle of patients with ALS. The highest levels of activity were found in individuals with the clinically and histologically most affected muscle. High levels of proteolytic activity correlated with the extent of muscle atrophy, the presence of target fibers and the overall severity of disease.

journal_name

Neurology

journal_title

Neurology

authors

Antel JP,Chelmicka-Schorr E,Sportiello M,Stefansson K,Wollmann RL,Arnason BG

doi

10.1212/wnl.32.8.901

subject

Has Abstract

pub_date

1982-08-01 00:00:00

pages

901-3

issue

8

eissn

0028-3878

issn

1526-632X

journal_volume

32

pub_type

杂志文章
  • Functional paralysis of horizontal gaze.

    abstract::A 52-year-old steelworker displayed a consistent defect in leftward voluntary gaze across the midline. Both fast (saccadic) and slow (pursuit) eye movements were affected. Neuro-ophthalmologic evaluation revealed no additional neurologic or ophthalmologic deficit. Pupilary miosis occurred during leftward gaze attempt,...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.29.1.82

    authors: Troost BT,Troost EG

    更新日期:1979-01-01 00:00:00

  • Medial temporal lobe atrophy on MRI scans and the diagnosis of Alzheimer disease.

    abstract:BACKGROUND:Despite convenience, accessibility, and strong correlation to severity of Alzheimer disease (AD) pathology, medial temporal lobe atrophy (MTA) has not been used as a criterion in the diagnosis of prodromal and probable AD. METHODS:Using a newly validated visual rating system, mean MTA scores of three bilate...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000336925.79704.9f

    authors: Duara R,Loewenstein DA,Potter E,Appel J,Greig MT,Urs R,Shen Q,Raj A,Small B,Barker W,Schofield E,Wu Y,Potter H

    更新日期:2008-12-09 00:00:00

  • Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome.

    abstract:BACKGROUND:Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare multisystem disorder associated with plasma cell dyscrasia. There is increasing evidence that high-dose chemotherapy with autologous peripheral blood stem cell transplantation (Auto-PBSCT) is an efficacious t...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000323811.42080.a4

    authors: Kuwabara S,Misawa S,Kanai K,Suzuki Y,Kikkawa Y,Sawai S,Hattori T,Nishimura M,Nakaseko C

    更新日期:2008-11-18 00:00:00

  • Environmental risk factors for REM sleep behavior disorder: a multicenter case-control study.

    abstract:OBJECTIVE:Idiopathic REM sleep behavior disorder is a parasomnia characterized by dream enactment and is commonly a prediagnostic sign of parkinsonism and dementia. Since risk factors have not been defined, we initiated a multicenter case-control study to assess environmental and lifestyle risk factors for REM sleep be...

    journal_title:Neurology

    pub_type: 杂志文章,多中心研究

    doi:10.1212/WNL.0b013e31825dd383

    authors: Postuma RB,Montplaisir JY,Pelletier A,Dauvilliers Y,Oertel W,Iranzo A,Ferini-Strambi L,Arnulf I,Hogl B,Manni R,Miyamoto T,Mayer G,Stiasny-Kolster K,Puligheddu M,Ju Y,Jennum P,Sonka K,Santamaria J,Fantini ML,Zucconi

    更新日期:2012-07-31 00:00:00

  • The medial brachial fascial compartment syndrome following axillary arteriography.

    abstract:OBJECTIVE:To review clinical and electrodiagnostic features of the medial brachial fascial compartment syndrome, a complication of percutaneous axillary vessel puncture. METHODS:The authors reviewed electrodiagnostic examinations over a 20-year period. RESULTS:This syndrome presents with weakness, pain, and numbness ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000089488.35632.08

    authors: Tsao BE,Wilbourn AJ

    更新日期:2003-10-28 00:00:00

  • Revised Airlie House consensus guidelines for design and implementation of ALS clinical trials.

    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

    pub_type: 共识发展会议,杂志文章

    doi:10.1212/WNL.0000000000007242

    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

  • Neurosarcoidosis: cerebrospinal fluid lymphocyte subpopulations.

    abstract::CSF lymphocyte subpopulations of eight patients with neurosarcoidosis were examined. CSF or CT was abnormal in all. The CSF T4/T8 (helper/suppressor) ratio was elevated at 6.8 and 7.6 in two patients; in one, there were only CSF T4 cells. The ratio was normal in five patients. ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.37.5.878

    authors: Stern BJ,Griffin DE,Luke RA,Krumholz A,Johns CJ

    更新日期:1987-05-01 00:00:00

  • Effects of bromocriptine on periodic limb movements in human narcolepsy.

    abstract::We studied the effects of bromocriptine on periodic limb movements in sleep (PLMS) of narcoleptic patients. Bromocriptine significantly reduced the number and index of PLMS without improving the nocturnal sleep disruption in that disorder. These results support the hypothesis that dopaminergic mechanisms are involved ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.43.10.2134

    authors: Boivin DB,Lorrain D,Montplaisir J

    更新日期:1993-10-01 00:00:00

  • Up-regulation of hippocampal serotonin metabolism in mild cognitive impairment.

    abstract:OBJECTIVE:Recent studies have suggested modifications of serotonin cerebral metabolism and of 5-HT(1A) receptors density in Alzheimer disease (AD). This study aims at exploring hippocampus 5-HT(1A) receptor density in patients at the amnesic mild cognitive impairment (aMCI) and mild AD dementia stages. METHODS:With us...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000271377.52421.4a

    authors: Truchot L,Costes SN,Zimmer L,Laurent B,Le Bars D,Thomas-Antérion C,Croisile B,Mercier B,Hermier M,Vighetto A,Krolak-Salmon P

    更新日期:2007-09-04 00:00:00

  • Plasma multianalyte profiling in mild cognitive impairment and Alzheimer disease.

    abstract:OBJECTIVES:While plasma biomarkers have been proposed to aid in the clinical diagnosis of Alzheimer disease (AD), few biomarkers have been validated in independent patient cohorts. Here we aim to determine plasma biomarkers associated with AD in 2 independent cohorts and validate the findings in the multicenter Alzheim...

    journal_title:Neurology

    pub_type: 杂志文章,多中心研究

    doi:10.1212/WNL.0b013e318266fa70

    authors: Hu WT,Holtzman DM,Fagan AM,Shaw LM,Perrin R,Arnold SE,Grossman M,Xiong C,Craig-Schapiro R,Clark CM,Pickering E,Kuhn M,Chen Y,Van Deerlin VM,McCluskey L,Elman L,Karlawish J,Chen-Plotkin A,Hurtig HI,Siderowf A,Swens

    更新日期:2012-08-28 00:00:00

  • Respiratory chain and mitochondrial DNA in muscle and brain in Parkinson's disease patients.

    abstract::There are several reports of a defect of complex I in the substantia nigra (SN) of Parkinson's disease (PD) patients. To evaluate whether this is specific to dopaminergic neurons or the phenotypically relevant consequence of a widespread failure of the mitochondrial oxidative phosphorylation (OXPHOS) system, we measur...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.43.11.2262

    authors: DiDonato S,Zeviani M,Giovannini P,Savarese N,Rimoldi M,Mariotti C,Girotti F,Caraceni T

    更新日期:1993-11-01 00:00:00

  • Loss of independence in early Parkinson disease: A 5-year population-based incident cohort study.

    abstract:OBJECTIVE:To determine the risk, predictors, and prognosis of independence loss and institutionalization in patients with early Parkinson disease (PD). METHODS:We conducted a prospective population-based 5-year longitudinal study following 189 patients with incident PD and 174 controls matched for age, sex, and comorb...

    journal_title:Neurology

    pub_type: 杂志文章,多中心研究

    doi:10.1212/WNL.0000000000003213

    authors: Bjornestad A,Tysnes OB,Larsen JP,Alves G

    更新日期:2016-10-11 00:00:00

  • "Dark Victory" (prognosis negative): The beginnings of neurology on screen.

    abstract::In "Dark Victory," released in theaters in 1939, the diagnosis and management of a progressive brain tumor was a central part of the screenplay, and this film marked the beginnings of the depiction of neurologic disease in cinema. Bette Davis' cinematic portrayal of a young woman dying from a brain tumor is close to t...

    journal_title:Neurology

    pub_type: 历史文章,杂志文章

    doi:10.1212/WNL.0000000000002571

    authors: Wijdicks EFM

    更新日期:2016-04-12 00:00:00

  • Neurologic complications of autologous and allogeneic bone marrow transplantation in patients with leukemia: a comparative study.

    abstract::We retrospectively evaluated the neurologic complications in 425 patients who underwent bone marrow transplant (BMT) (310 allogeneic, 115 autologous) for leukemia. Forty-six patients (11%) developed 47 central and three peripheral neurologic complications. The most common complications were cerebral hemorrhage (3.8%),...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.46.4.1004

    authors: Graus F,Saiz A,Sierra J,Arbaiza D,Rovira M,Carreras E,Tolosa E,Rozman C

    更新日期:1996-04-01 00:00:00

  • Visual stimulation leads to activation of the nociceptive trigeminal nucleus in chronic migraine.

    abstract:OBJECTIVE:The visual system has often been described to be sensitized in migraineurs, with light being perceived as aversive or even painful. One possible explanation for this altered perception is crosslinks between the visual and the trigeminonociceptive system. Visual stimulation in chronic migraineurs on the level ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000005622

    authors: Schulte LH,Allers A,May A

    更新日期:2018-05-29 00:00:00

  • Bevacizumab for recurrent ependymoma.

    abstract:BACKGROUND:Ependymoma is a rare type of glioma, representing 5% of all CNS malignancies. Radiotherapy (RT) is commonly administered, but there is no standard chemotherapy. At recurrence, ependymoma is notoriously refractory to therapy and the prognosis is poor. In recurrent glioblastoma, encouraging responses with beva...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e3181c1df34

    authors: Green RM,Cloughesy TF,Stupp R,DeAngelis LM,Woyshner EA,Ney DE,Lassman AB

    更新日期:2009-11-17 00:00:00

  • Factors influencing sweat gland innervation in diabetes.

    abstract:BACKGROUND:Using a stereologic approach, the density of nerve fibers innervating sweat gland (SG) fragments in patients with diabetes mellitus (DM) and healthy controls using protein gene product (PGP), tyrosine hydroxylase (TH), and vasoactive intestinal peptide (VIP) was measured to determine which marker best detect...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000001488

    authors: Liu Y,Billiet J,Ebenezer GJ,Pan B,Hauer P,Wei J,Polydefkis M

    更新日期:2015-04-21 00:00:00

  • Competitive inhibition of brain hexokinase by metrizamide.

    abstract::We compared the effect of metrizamide and its parent compound glucosamine on the kinetics of dog brain hexokinase. The Michaelis constant (Km) for glucose rose from 0.065 to 0.15 to 0.28 mM in the presence of 0, 16, and 32 mM metrizamide, respectively. For 0, 1.5, and 3.7 mM glucosamine, the Km values were 0.065, 0.4,...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.32.3.320

    authors: Bertoni JM,Steinman CG

    更新日期:1982-03-01 00:00:00

  • Chronic polyradiculoneuropathy of infancy. A report of three cases with familial incidence.

    abstract::Two siblings and a third child exhibited a syndrome of progressive muscular weakness and wasting, closely resembling Werdnig-Hoffmann's disease. Autopsy of one of the siblings and the third child showed nearly total absence of myelin sheaths in the cranial and spinal nerve roots, relative preservation of axons, and no...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.26.6.565

    authors: Kasman M,Bernstein L,Schulman S

    更新日期:1976-06-01 00:00:00

  • Primary prevention trials in Alzheimer disease.

    abstract::Many new treatments under development for Alzheimer disease (AD) will be disease-modifying rather than symptomatic. Clinical evaluation of these treatments will require primary and secondary prevention trials. We describe some of the methodologic challenges in designing primary prevention trials for AD and illustrate ...

    journal_title:Neurology

    pub_type: 杂志文章,评审

    doi:10.1212/wnl.67.9_suppl_3.s2

    authors: Green RC,DeKosky ST

    更新日期:2006-11-14 00:00:00

  • Oral fluoroquinolone use and risk of peripheral neuropathy: a pharmacoepidemiologic study.

    abstract:OBJECTIVE:To quantify the risk of peripheral neuropathy (PN) with oral fluoroquinolone (FQ) use. METHODS:We conducted a case-control study within a cohort of men aged 45 to 80 years in the United States followed from 2001 to 2011. Cases were defined as those with the first physician visit diagnosis of PN, polyneuropat...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000000846

    authors: Etminan M,Brophy JM,Samii A

    更新日期:2014-09-30 00:00:00

  • Visuoperceptual function in visual agnosia.

    abstract::Two patients with visual agnosia underwent visual recognition and neuropsychological tests to characterize their perceptual functioning. Both had an initial "apperceptive profile" and evolved from cortical blindness. One had carbon monoxide intoxication and incidental agenesis of the splenium of the corpus callosum; t...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.38.11.1754

    authors: Mendez MF

    更新日期:1988-11-01 00:00:00

  • Delayed emergence of a parkinsonian disorder in 38% of 29 older men initially diagnosed with idiopathic rapid eye movement sleep behaviour disorder.

    abstract::We report longitudinal data on a group of 29 male patients 50 years of age or older who were initially diagnosed as having idiopathic REM sleep behavior disorder (RBD) after extensive polysomnographic and neurologic evaluations. Thirty-eight percent (11/29) were eventually diagnosed as having a parkinsonian disorder (...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.46.2.388

    authors: Schenck CH,Bundlie SR,Mahowald MW

    更新日期:1996-02-01 00:00:00

  • Attention deficit in Alzheimer's disease is not simulated by an anticholinergic/antihistaminergic drug and is distinct from deficits in healthy aging.

    abstract:OBJECTIVE:To evaluate attention deficit in Alzheimer's disease (AD) and its relationship to attention deficits associated with aging and with medications altering alertness. METHODS:Ten patients with probable AD, 10 healthy old controls, and 15 young controls performed a covert orienting of spatial attention task. You...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.44.4.657

    authors: Oken BS,Kishiyama SS,Kaye JA,Howieson DB

    更新日期:1994-04-01 00:00:00

  • Carbamazepine- induced hypertension.

    abstract::A 33-year-old man with complex partial seizures developed de novo hypertension after the initiation of carbamazepine, which resolved on discontinuation. Although rare, a variety of cardiovascular effects including hypertension have been reported with carbamazepine. Numerous mechanisms may be responsible; however, shar...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.59.2.275

    authors: Jette N,Veregin T,Guberman A

    更新日期:2002-07-23 00:00:00

  • Time trends in outcome of subarachnoid hemorrhage: Population-based study and systematic review.

    abstract:BACKGROUND:Treatment of aneurysmal subarachnoid hemorrhage (SAH) has changed substantially over the last 25 years but there is a lack of reliable population-based data on whether case-fatality or functional outcomes have improved. METHODS:We determined changes in the standardized incidence and outcome of SAH in the sa...

    journal_title:Neurology

    pub_type: 杂志文章,meta分析,评审

    doi:10.1212/WNL.0b013e3181dd42b3

    authors: Lovelock CE,Rinkel GJ,Rothwell PM

    更新日期:2010-05-11 00:00:00

  • Simple MRI score aids prediction of dementia in cerebral small vessel disease.

    abstract:OBJECTIVE:To determine whether a simple small vessel disease (SVD) score, which uses information available on rapid visual assessment of clinical MRI scans, predicts risk of cognitive decline and dementia, above that provided by simple clinical measures. METHODS:Three prospective longitudinal cohort studies (SCANS [St...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000009141

    authors: Amin Al Olama A,Wason JMS,Tuladhar AM,van Leijsen EMC,Koini M,Hofer E,Morris RG,Schmidt R,de Leeuw FE,Markus HS

    更新日期:2020-03-24 00:00:00

  • Treating melancholia at home: theoretical wisdom and grim reality in the career of E.C. Seguin.

    abstract::E.C. Seguin was one of the early, influential 19th-century neurologists who participated in the development of neurology as a specialty in the United States. Born in France, but raised from early childhood in the United States, Seguin published widely, developed a high-profile New York City practice, and was named Cli...

    journal_title:Neurology

    pub_type: 传,历史文章,杂志文章

    doi:10.1212/WNL.0b013e3182904f55

    authors: Goetz CG,Harter DH

    更新日期:2013-04-30 00:00:00

  • Methylphenidate, cognition, and epilepsy: A double-blind, placebo-controlled, single-dose study.

    abstract:OBJECTIVE:To evaluate the potential efficacy of immediate-release methylphenidate (MPH) for treating cognitive deficits in epilepsy. METHODS:This was a double-blind, randomized, single-dose, 3-period crossover study in patients with epilepsy and chronic cognitive complaints comparing the effects of placebo and MPH 10 ...

    journal_title:Neurology

    pub_type: 杂志文章,随机对照试验

    doi:10.1212/WNL.0000000000003564

    authors: Adams J,Alipio-Jocson V,Inoyama K,Bartlett V,Sandhu S,Oso J,Barry JJ,Loring DW,Meador K

    更新日期:2017-01-31 00:00:00

  • The use of "clinic room" presentation as an educational tool in the ambulatory care setting.

    abstract:OBJECTIVE:To evaluate the utility of "clinic room" case presentation in the ambulatory care setting. BACKGROUND:Neurology is increasingly an outpatient specialty. The transition from ward to clinic presents challenges for student and resident education. Interaction between attending physician and trainee is limited by...

    journal_title:Neurology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:

    authors: Smith AG,Bromberg MB,Singleton JR,Forshew DA

    更新日期:1999-01-15 00:00:00