Abnormal ventilation during exercise in McArdle's syndrome: modulation by substrate availability.

Abstract:

:We evaluated ventilation during cycle exercise in four men lacking myophosphorylase. In submaximal exercise of similar relative intensity, ventilation was higher relative to oxygen uptake in McArdle patients than in normal men. The exercise ventilatory response returned to normal after glucose infusion, by fasting to increase free fatty acid availability or by combining fasting and submaximal exercise. Excessive ventilation in exercise was potentiated by inhibiting lipolysis with nicotinic acid. The excessive ventilatory effort and resultant respiratory alkalosis may contribute to exercise intolerance.

journal_name

Neurology

journal_title

Neurology

authors

Haller RG,Lewis SF

doi

10.1212/wnl.36.5.716

subject

Has Abstract

pub_date

1986-05-01 00:00:00

pages

716-9

issue

5

eissn

0028-3878

issn

1526-632X

journal_volume

36

pub_type

杂志文章
  • HLA-DR,DQ and APOE genotypes and gender influence in Sardinian primary progressive MS.

    abstract::The authors examined the influence of APOE and human leukocyte antigen-DRB1-DQB1 polymorphisms on the course of multiple sclerosis in 871 patients, 773 with relapsing and 98 with primary progressive disease, and 348 control subjects. The risk of the primary progressive course was increased (odds ratio = 6.81, p = 0.00...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.WNL.0000150579.54878.00

    authors: Cocco E,Sotgiu A,Costa G,Murru MR,Mancosu C,Murru R,Lai M,Contu P,Marrosu MG

    更新日期:2005-02-08 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

  • PET studies of cerebral glucose metabolism in idiopathic torticollis.

    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

    pub_type: 杂志文章

    doi:10.1212/wnl.36.5.653

    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

  • Declining incidence of multiple sclerosis in the Orkney Islands.

    abstract::The incidence of MS in the Orkney Islands has been updated from 1941 to September 21, 1983. Since 1965, MS incidence rates have fallen significantly when compared with those for 1941 to 1964. Alterations in age-specific prevalence, mean duration of illness, and mean age of the MS population are consistent with the dec...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.35.4.545

    authors: Cook SD,Cromarty JI,Tapp W,Poskanzer D,Walker JD,Dowling PC

    更新日期:1985-04-01 00:00:00

  • Horner's syndrome after coronary artery bypass surgery.

    abstract::We established the frequency of Horner's syndrome (HS) in 248 elective patients after coronary artery bypass surgery. Patients were evaluated neurologically pre- and post-operatively and 6 months after surgery. Nineteen patients (7.7%) developed unilateral HS postoperatively, 12 involving the left eye. The finding per...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.46.1.181

    authors: Barbut D,Gold JP,Heinemann MH,Hinton RB,Trifiletti RR

    更新日期:1996-01-01 00:00:00

  • The effect of nerve growth factor, ciliary neurotrophic factor, and ACTH analogs on cisplatin neurotoxicity in vitro.

    abstract::Cisplatin, used to treat ovarian, bladder, and testicular cancers, causes a sensory dose-limiting neuropathy. Preliminary observations in humans and animals suggest that nerve damage may be prevented by ACTH analogs, particularly those belonging to the melanocortin class, and by nerve growth factor (NGF). We establish...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.44.3_part_1.488

    authors: Windebank AJ,Smith AG,Russell JW

    更新日期:1994-03-01 00:00:00

  • Diagnosis and misdiagnosis of adult neuronal ceroid lipofuscinosis (Kufs disease).

    abstract:OBJECTIVE:To critically re-evaluate cases diagnosed as adult neuronal ceroid lipofuscinosis (ANCL) in order to aid clinicopathologic diagnosis as a route to further gene discovery. METHODS:Through establishment of an international consortium we pooled 47 unsolved cases regarded by referring centers as ANCL. Clinical a...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000002943

    authors: Berkovic SF,Staropoli JF,Carpenter S,Oliver KL,Kmoch S,Anderson GW,Damiano JA,Hildebrand MS,Sims KB,Cotman SL,Bahlo M,Smith KR,Cadieux-Dion M,Cossette P,Jedličková I,Přistoupilová A,Mole SE,ANCL Gene Discovery Consortiu

    更新日期:2016-08-09 00:00:00

  • From benign fasciculations and cramps to motor neuron disease.

    abstract::Fasciculations and cramps may occur in motor neuron disease or as part of a more benign syndrome. A man with apparently benign fasciculations and cramps for 4 years developed progressive muscle weakness and wasting. Such a previously undocumented evolution of benign fasciculations and cramps to motor neuron disease ma...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.36.7.997

    authors: Fleet WS,Watson RT

    更新日期:1986-07-01 00:00:00

  • Effects of statins on human cerebral cholesterol metabolism and secretion of Alzheimer amyloid peptide.

    abstract::Cerebral cholesterol metabolism has been linked with production of amyloid peptide (Abeta) crucial in AD. The association between use of cholesterol-lowering drugs (statins) and AD disease is currently being intensely discussed. In this case-control study on elderly nondemented subjects, the authors provide the first ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.59.8.1257

    authors: Fassbender K,Stroick M,Bertsch T,Ragoschke A,Kuehl S,Walter S,Walter J,Brechtel K,Muehlhauser F,Von Bergmann K,Lütjohann D

    更新日期:2002-10-22 00:00:00

  • Catechol-O-methyltransferase inhibition increases striatal L-dopa and dopamine: an in vivo study in rats.

    abstract::We administered Ro 40-7592, an inhibitor of the enzyme catechol-O-methyltransferase (COMT) that crosses the blood-brain barrier, to rats and monitored extracellular catecholamine levels in the corpus striatum before and after the intraperitoneal administration of a bolus of l-dopa. Acute administration of Ro 40-7592 i...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.42.3.683

    authors: Brannan T,Martínez-Tica J,Yahr MD

    更新日期:1992-03-01 00:00:00

  • Relation between MR abnormalities and patterns of cognitive impairment in multiple sclerosis.

    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

    pub_type: 杂志文章

    doi:10.1212/wnl.50.6.1601

    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

  • Singing seizures.

    abstract::Automatisms are commonly seen in epilepsy, either ictally or postictally. However, most automatisms are simple, with hand movements, mouth smacking, nose-rubbing, repetition of a single word, or coughing, grunting, or screeching. Complex automatisms are less common and striking. The authors report two cases of seizure...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000031430.40085.93

    authors: Doherty MJ,Wilensky AJ,Holmes MD,Lewis DH,Rae J,Cohn GH

    更新日期:2002-11-12 00:00:00

  • Combined IV-intra-arterial thrombolysis: a color-coded duplex pilot study.

    abstract::The authors compared the transcranial color-coded duplex pattern of the middle cerebral arteries (MCAs) before and after IV and combined IV-intra-arterial (IV-IA) thrombolysis in consecutive first-ever stroke patients. Patients receiving combined IV-IA thrombolysis showed greater improvement in flow signal and higher ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000224884.05596.53

    authors: Perren F,Loulidi J,Graves R,Yilmaz H,Rüfenacht D,Landis T,Sztajzel R

    更新日期:2006-07-25 00:00:00

  • Cerebral air embolism complicating cardiac ablation procedures.

    abstract::Two patients with similar courses of neurologic impairment and subsequent recovery after cerebral air embolism complicating cardiac ablation procedures are described. Hyperbaric oxygen therapy, combined with aggressive resuscitative efforts, appears to have contributed to each patient's recovery. ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.56.6.792

    authors: Hinkle DA,Raizen DM,McGarvey ML,Liu GT

    更新日期:2001-03-27 00:00:00

  • Brachial plexus neuropathy as the initial presentation of systemic lupus erythematosus.

    abstract::A patient presented with bilateral brachial plexus neuropathy which 2 weeks later evolved into a polyneuropathy involving all four extremities. On further investigation, the patient was found to have systemic lupus erythematosus. Treatment with steroids was accompanied by complete resolution of the neurologic findings...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.29.12.1633-a

    authors: Bloch SL,Jarrett MP,Swerdlow M,Grayzel AI

    更新日期:1979-12-01 00:00:00

  • Dystrophin expression and somatic reversion in prednisone-treated and untreated Duchenne dystrophy. CIDD Study Group.

    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

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1212/wnl.41.5.661

    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

  • Practice guideline update summary: Pharmacologic treatment for pediatric migraine prevention: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Headache Society.

    abstract:OBJECTIVE:To provide updated evidence-based recommendations for migraine prevention using pharmacologic treatment with or without cognitive behavioral therapy in the pediatric population. METHODS:The authors systematically reviewed literature from January 2003 to August 2017 and developed practice recommendations usin...

    journal_title:Neurology

    pub_type: 杂志文章,评审

    doi:10.1212/WNL.0000000000008105

    authors: Oskoui M,Pringsheim T,Billinghurst L,Potrebic S,Gersz EM,Gloss D,Holler-Managan Y,Leininger E,Licking N,Mack K,Powers SW,Sowell M,Victorio MC,Yonker M,Zanitsch H,Hershey AD

    更新日期:2019-09-10 00:00:00

  • Phase II study of monthly pasireotide LAR (SOM230C) for recurrent or progressive meningioma.

    abstract:OBJECTIVE:A subset of meningiomas recur after surgery and radiation therapy, but no medical therapy for recurrent meningioma has proven effective. METHODS:Pasireotide LAR is a long-acting somatostatin analog that may inhibit meningioma growth. This was a phase II trial in patients with histologically confirmed recurre...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000001153

    authors: Norden AD,Ligon KL,Hammond SN,Muzikansky A,Reardon DA,Kaley TJ,Batchelor TT,Plotkin SR,Raizer JJ,Wong ET,Drappatz J,Lesser GJ,Haidar S,Beroukhim R,Lee EQ,Doherty L,Lafrankie D,Gaffey SC,Gerard M,Smith KH,McCluskey

    更新日期:2015-01-20 00:00:00

  • Reversible alexia, mitochondrial myopathy, and lactic acidemia.

    abstract::A 11-year-old boy of short stature had recurrent right temporal pounding headaches of 7 months' duration, and progressive visual loss for 3 days. There was a left hemianopia, alexia without agraphia, and diffuse muscle weakness. Investigation established the presence of a mitochondrial myopathy with pyruvate and lacti...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.29.5.717

    authors: Skoglund RR

    更新日期:1979-05-01 00:00:00

  • Journal club: pretreatment EEG in childhood absence epilepsy.

    abstract::Childhood absence epilepsy (CAE) is defined by absence seizures in a normally developing child, with onset between 4 and 10 years of age.(1) Typical absence seizures consist of behavioral arrest with or without automatisms, usually last 30-60 seconds, and demonstrate a characteristic 3-Hz generalized spike-wave (GSW) ...

    journal_title:Neurology

    pub_type: 杂志文章,随机对照试验

    doi:10.1212/WNL.0000000000000380

    authors: Bernson-Leung ME,Mazumdar M

    更新日期:2014-05-06 00:00:00

  • Facial asymmetry, hippocampal pathology, and remote symptomatic seizures: a temporal lobe epileptic syndrome.

    abstract::We performed a prospective study of neuroimaging studies and temporal lobe pathology in 13 patients with facial asymmetry and intractable partial epilepsy of temporal lobe origin. The 13 patients, derived from 50 consecutive patients with nonlesional medically refractory partial epilepsy, were deemed appropriate candi...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.43.4.725

    authors: Cascino GD,Luckstein RR,Sharbrough FW,Jack CR Jr

    更新日期:1993-04-01 00:00:00

  • Plasmapheresis in acute episodes of fulminant CNS inflammatory demyelination.

    abstract::We present six patients with acute fulminant episodes of CNS inflammatory demyelination who responded to therapeutic plasmapheresis after failing a course of high-dose methylprednisolone administered IV. Neurologic improvement occurred in three of the patients following the second plasmapheresis. Dramatic improvement ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.43.6.1100

    authors: Rodriguez M,Karnes WE,Bartleson JD,Pineda AA

    更新日期:1993-06-01 00:00:00

  • Methionine-enkephalin, substance P, and homovanillic acid in the CSF of parkinsonian patients.

    abstract::Methionine-enkephalin, substance P, and homovanillic acid concentrations were measured in the CSF of subjects not affected by neurologic disorders (group 1), and in parkinsonian patients who had a slight or moderate (group 2) or severe (group 3) disability. Homovanillic acid and substance P concentrations in the CSF o...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.34.4.516

    authors: Pezzoli G,Panerai AE,Di Giulio A,Longo A,Passerini D,Carenzi A

    更新日期:1984-04-01 00:00:00

  • Local amygdala structural differences with 3T MRI in patients with Alzheimer disease.

    abstract:OBJECTIVE:Histologic studies show that the amygdala is affected by Alzheimer disease (AD) pathology, and its medial aspect is the most involved. We aimed to assess in vivo local structural differences in the amygdala of patients with AD using high-field MRI. METHODS:A total of 19 patients with AD (mean age 76, SD 6 ye...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e31820d62d9

    authors: Cavedo E,Boccardi M,Ganzola R,Canu E,Beltramello A,Caltagirone C,Thompson PM,Frisoni GB

    更新日期:2011-02-22 00:00:00

  • Perfusion MRI detects rCBF abnormalities in early stages of HIV-cognitive motor complex.

    abstract:OBJECTIVE:To evaluate patients with early HIV-cognitive motor complex (HIV-CMC) for possible regional cerebral blood flow (rCBF) abnormalities on perfusion MRI (pMRI). BACKGROUND:Nuclear medicine techniques have demonstrated global and focal cerebral perfusion abnormalities in patients with HIV dementia. Ultrafast pMR...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.54.2.389

    authors: Chang L,Ernst T,Leonido-Yee M,Speck O

    更新日期:2000-01-25 00:00:00

  • Neuronal antinuclear antibody in sensory neuronopathy from lung cancer.

    abstract::We found an antinuclear antibody highly restricted to nuclei of neurons in two patients with subacute sensory neuronopathy complicating oat cell carcinoma of the lung. Serum was tested by indirect immunofluorescence and immunoperoxidase staining. At low concentrations of antibody, only the nuclei of the neurons were s...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.35.4.538

    authors: Graus F,Cordon-Cardo C,Posner JB

    更新日期:1985-04-01 00:00:00

  • Phase I/II multicenter ketogenic diet study for adult superrefractory status epilepticus.

    abstract:OBJECTIVE:To investigate the feasibility, safety, and efficacy of a ketogenic diet (KD) for superrefractory status epilepticus (SRSE) in adults. METHODS:We performed a prospective multicenter study of patients 18 to 80 years of age with SRSE treated with a KD treatment algorithm. The primary outcome measure was signif...

    journal_title:Neurology

    pub_type: 杂志文章,多中心研究

    doi:10.1212/WNL.0000000000003690

    authors: Cervenka MC,Hocker S,Koenig M,Bar B,Henry-Barron B,Kossoff EH,Hartman AL,Probasco JC,Benavides DR,Venkatesan A,Hagen EC,Dittrich D,Stern T,Radzik B,Depew M,Caserta FM,Nyquist P,Kaplan PW,Geocadin RG

    更新日期:2017-03-07 00:00:00

  • Structural and functional brain connectivity in presymptomatic familial frontotemporal dementia.

    abstract:OBJECTIVE:We aimed to investigate whether cognitive deficits and structural and functional connectivity changes can be detected before symptom onset in a large cohort of carriers of microtubule-associated protein tau and progranulin mutations. METHODS:In this case-control study, 75 healthy individuals (aged 20-70 year...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e31828407bc

    authors: Dopper EG,Rombouts SA,Jiskoot LC,Heijer Td,de Graaf JR,Koning Id,Hammerschlag AR,Seelaar H,Seeley WW,Veer IM,van Buchem MA,Rizzu P,van Swieten JC

    更新日期:2013-02-26 00:00:00

  • A novel sodium channel mutation in a family with hypokalemic periodic paralysis.

    abstract:OBJECTIVE:To identify the cause of hypokalemic periodic paralysis (HOKPP) in a family whose disease is not caused by a mutation in the dihydropyridine-sensitive (DHP) receptor alpha1-subunit gene (CACNA1S). BACKGROUND:Hypokalemic periodic paralysis is primarily caused by mutations within CACNA1S. Genetic heterogeneity...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.53.9.1932

    authors: Bulman DE,Scoggan KA,van Oene MD,Nicolle MW,Hahn AF,Tollar LL,Ebers GC

    更新日期:1999-12-10 00:00:00

  • False localizing sign of cervico-thoracic CSF leak in spontaneous intracranial hypotension.

    abstract:OBJECTIVE:Spontaneous spinal CSF leaks are an important cause of new-onset headaches. Such leaks are reported to be particularly common at the cervico-thoracic junction. The authors undertook a study to determine the significance of these cervico-thoracic CSF leaks. METHODS:The patient population consisted of a consec...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000001697

    authors: Schievink WI,Maya MM,Chu RM,Moser FG

    更新日期:2015-06-16 00:00:00