MRI in juvenile ALS: a patient report.


:We describe the MR images of a patient with juvenile ALS. MRI of the brain showed bilateral hyperintensities along the corticospinal tracts extending from the corona radiata to the brainstem on T2-weighted images. These findings should be differentiated from the slight hyperintensities seen in the posterior limbs of the internal capsules in normal subjects.






Midani H,Truwit CL,Parry GJ




Has Abstract


1998-06-01 00:00:00












  • Carbamazepine toxicity resulting from generic substitution.

    abstract::Generic substitution is practiced widely in both hospital and community settings. There have been several reports of reduced serum concentrations and seizure exacerbation following generic substitution of Tegretol. We describe the first 2 cases of carbamazepine toxicity resulting from the substitution of Tegretol with...


    pub_type: 杂志文章


    authors: Gilman JT,Alvarez LA,Duchowny M

    更新日期:1993-12-01 00:00:00

  • A rating scale for Gilles de la Tourette's syndrome: description, reliability, and validity data.

    abstract::We developed a rating scale for tic disorders that uses only objective criteria and accommodates the variety of tic manifestations. Using short videotaped recordings with the examiner out of the taping room, we measured five tic variables: number of body areas affected, frequency of motor tics and vocalizations, and s...


    pub_type: 杂志文章


    authors: Goetz CG,Tanner CM,Wilson RS,Shannon KM

    更新日期:1987-09-01 00:00:00

  • Exploratory saccades show no direction-specific deficit in neglect.

    abstract::In patients with spatial neglect, contralesional reflexive saccades toward suddenly appearing targets show direction-specific deficits. We examined whether these deficits also occur during free exploration of space. Neglect patients' voluntary eye movements showed reduced amplitudes for saccades in all directions but ...


    pub_type: 杂志文章


    authors: Niemeier M,Karnath HO

    更新日期:2000-01-25 00:00:00

  • Effect of L-carnitine treatment for valproate-induced hepatotoxicity.

    abstract::The authors analyzed the association of L-carnitine treatment with hepatic survival in 92 patients with severe, symptomatic, valproate-induced hepatotoxicity. Forty-eight percent of the 42 patients treated with L-carnitine survived, but only 10% of the 50 patients treated solely with aggressive supportive care survive...


    pub_type: 杂志文章


    authors: Bohan TP,Helton E,McDonald I,König S,Gazitt S,Sugimoto T,Scheffner D,Cusmano L,Li S,Koch G

    更新日期:2001-05-22 00:00:00

  • Experimental spinal cord injury: effects of trauma or ischemia on TRH and muscarinic receptors.

    abstract::Traumatic spinal cord injury in rats and ischemic spinal cord injury in rabbits were associated with time-dependent, localized decreases in thyrotropin-releasing hormone (TRH) and muscarinic receptor binding. Changes were not evident in the first 24 to 48 hours, consistent with the hypothesis that secondary alteration...


    pub_type: 杂志文章


    authors: Faden AI,Pilotte NS,Burt DR

    更新日期:1986-05-01 00:00:00

  • Body mass index and the risk of Parkinson disease.

    abstract:OBJECTIVE:To examine the association between body mass index (BMI) and the risk of Parkinson disease (PD). METHODS:Study cohorts included 22,367 Finnish men and 23,439 women 25 to 59 years of age without a history of PD at baseline. Hazards ratios (HRs) of incident PD were estimated for different levels of BMI. RESUL...


    pub_type: 杂志文章


    authors: Hu G,Jousilahti P,Nissinen A,Antikainen R,Kivipelto M,Tuomilehto J

    更新日期:2006-12-12 00:00:00

  • The effects of myasthenic immunoglobulins on neuromuscular transmission and thymus histology in mice.

    abstract::An autoimmune reaction to the endplate acetylcholine receptor (AChR) is thought to be responsible for the muscular weakness of myasthenia gravis (MG). However, the significance of antithymic antibodies and the thymic AChR-like protein is still uncertain. We transferred immunoglobulins (Igs) from patients with MG to mi...


    pub_type: 杂志文章


    authors: Dalkara T,Onur R,Ruacan SA,Topaktaş S

    更新日期:1983-10-01 00:00:00

  • Relative effect of APOE ε4 on neuroimaging biomarker changes across the lifespan.

    abstract:OBJECTIVE:To provide a comprehensive understanding of APOE ε4 effects across the lifespan on the 3 main neuroimaging biomarkers. METHODS:Two hundred seven community-dwelling, cognitively normal APOE ε4 carriers and noncarriers aged 20-87 years were involved in this study. They underwent structural MRI, fluorodeoxygluc...


    pub_type: 临床试验,杂志文章


    authors: Gonneaud J,Arenaza-Urquijo EM,Fouquet M,Perrotin A,Fradin S,de La Sayette V,Eustache F,Chételat G

    更新日期:2016-10-18 00:00:00

  • Predictors of relapse rate in MS clinical trials.

    abstract:BACKGROUND:The annual relapse rate has been commonly used as a primary efficacy endpoint in phase III multiple sclerosis (MS) clinical trials. The aim of this study was to determine the relative contribution of different possible prognostic factors available at baseline to the on-study relapse rate in MS. METHODS:A to...


    pub_type: 杂志文章


    authors: Held U,Heigenhauser L,Shang C,Kappos L,Polman C,Sylvia Lawry Centre for MS Research.

    更新日期:2005-12-13 00:00:00

  • Regional cortical thinning in preclinical Huntington disease and its relationship to cognition.

    abstract::The authors studied presymptomatic individuals with the Huntington disease (HD) mutation to determine whether cortical thinning was present. They found thinning that was regionally selective, semi-independent of striatal volume loss, and correlated with cognitive performance. Early, extensive cortical involvement occu...


    pub_type: 杂志文章


    authors: Rosas HD,Hevelone ND,Zaleta AK,Greve DN,Salat DH,Fischl B

    更新日期:2005-09-13 00:00:00

  • Multiple sclerosis among utility workers.

    abstract::The incidence of MS was assessed in a nationwide cohort study of 31,990 employees of Danish utility companies between 1900 and 1993. Overall, 32 cases of MS were diagnosed, as compared with 23.7 expected from national incidence rates, to yield a standardized incidence ratio of 1.35 (95% confidence interval, 0.92 to 1....


    pub_type: 杂志文章


    authors: Johansen C,Koch-Henriksen N,Rasmussen S,Olsen JH

    更新日期:1999-04-12 00:00:00

  • Late-onset depressive symptoms increase the risk of dementia in small vessel disease.

    abstract:OBJECTIVE:We prospectively investigated the role of depressive symptoms (DS) on all-cause dementia in a population with small vessel disease (SVD), considering onset age of DS and cognitive performance. METHODS:The RUN DMC study (Radboud University Nijmegen Diffusion Tensor and Magnetic Resonance Cohort) is a prospect...


    pub_type: 杂志文章


    authors: van Uden IW,van der Holst HM,van Leijsen EM,Tuladhar AM,van Norden AG,de Laat KF,Claassen JA,van Dijk EJ,Kessels RP,Richard E,Tendolkar I,de Leeuw FE

    更新日期:2016-09-13 00:00:00

  • Patterns of inheritance in familial ALS.

    abstract::We investigated 185 families with ALS for evidence of anticipation and mitochondrial inheritance. Although initial analysis demonstrated significant anticipation of age at death between generations in patients with familial ALS, further analysis demonstrated features of regression to the mean, suggesting that the perc...


    pub_type: 杂志文章


    authors: Bradley M,Bradley L,de Belleroche J,Orrell RW

    更新日期:2005-05-10 00:00:00

  • Gamma hydroxybutyrate. Correlation of serum and cerebrospinal fluid levels with electroencephalographic and behavioral effects.

    abstract::Gamma hydroxybutyrate was administered to adult cats by intravenous infusion at varying dosages while an electroencephalogram (EEG) was recorded from electrodes placed stereotactically in the right and left hippocampus and thalamic intralaminar nucleus and from cortical electrodes. Blood cerebrospinal fluid samples we...


    pub_type: 杂志文章


    authors: Snead OC 3rd,Yu RK,Huttenlocher PR

    更新日期:1976-01-01 00:00:00

  • Presenting features and value of diagnostic procedures in leptomeningeal metastases.

    abstract:OBJECTIVE:To study the presenting features and value of routine diagnostic procedures in patients with leptomeningeal metastases (LMM) related to the primary malignancy to improve diagnostic assessment. METHODS:The authors studied the presenting features and value of routine diagnostic procedures in relation to the hi...


    pub_type: 杂志文章


    authors: van Oostenbrugge RJ,Twijnstra A

    更新日期:1999-07-22 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 ...


    pub_type: 杂志文章


    authors: Schulte LH,Allers A,May A

    更新日期:2018-05-29 00:00:00

  • Prolonged partial complex status epilepticus: EEG and behavioral observations.

    abstract::Four prolonged episodes of partial complex status epilepticus were observed in a teenaged girl. During each there was an "ictal" phase, in which her mental state frequently fluctuated between mildly clouded consciousness and almost total unresponsiveness; once, she was also blind during this phase of impaired consciou...


    pub_type: 杂志文章


    authors: Engel J Jr,Ludwig BI,Fetell M

    更新日期:1978-09-01 00:00:00

  • Serum leptin changes in epileptic patients who gain weight after therapy with valproic acid.

    abstract::Weight gain has been recognized as an adverse effect of valproic acid therapy, but there are are no data about serum leptin levels in patients receiving this drug. To evaluate if valproic acid treatment in epileptic patients in whom obesity develops modifies serum levels of insulin and leptin, 40 female patients with ...


    pub_type: 杂志文章


    authors: Verrotti A,Basciani F,Morresi S,de Martino M,Morgese G,Chiarelli F

    更新日期:1999-07-13 00:00:00

  • Familial X-linked myalgia and cramps: a nonprogressive myopathy associated with a deletion in the dystrophin gene.

    abstract::We report a family with an X-linked recessive disorder characterized by muscle cramps and myalgia. Nine affected male family members had high resting serum levels of creatine kinase, and well-developed musculature with calf hypertrophy but no evidence of muscular weakness. Symptoms began in childhood and did not progr...


    pub_type: 杂志文章


    authors: Gospe SM Jr,Lazaro RP,Lava NS,Grootscholten PM,Scott MO,Fischbeck KH

    更新日期:1989-10-01 00:00:00

  • Use of statins and incidence of dementia and cognitive impairment without dementia in a cohort study.

    abstract:OBJECTIVE:Evidence of a relation between use of lipid lowering drugs and cognitive outcomes is mixed. This study aimed to test the association between use of statins and incidence of dementia and cognitive impairment without dementia (CIND) over 5 years of follow-up. METHODS:Data were from a population-based cohort st...


    pub_type: 杂志文章


    authors: Cramer C,Haan MN,Galea S,Langa KM,Kalbfleisch JD

    更新日期:2008-07-29 00:00:00

  • Myasthenia gravis activities of daily living profile.

    abstract::The authors have developed an MG activities of daily living (ADL) profile (MG-ADL)-a simple eight-question survey of MG symptoms. In 254 consecutive encounters with established MG patients, the authors compared scores from the MG-ADL to the quantitative MG score (QMG)-a standardized, reliable scale used in clinical tr...


    pub_type: 杂志文章


    authors: Wolfe GI,Herbelin L,Nations SP,Foster B,Bryan WW,Barohn RJ

    更新日期:1999-04-22 00:00:00

  • Lumbosacral plexus neuritis.

    abstract::We studied four patients with neurologic disorders of the lumbosacral plexus. Except for location of symptoms, the disorder confirmed to criteria established for the clinical diagnosis of brachial plexus neuritis. Acute onset of pain in one or both legs was followed by weakness, loss of stretch reflexes, and sometimes...


    pub_type: 杂志文章


    authors: Sander JE,Sharp FR

    更新日期:1981-04-01 00:00:00

  • Defining the Lowest Threshold for Amyloid-PET to Predict Future Cognitive Decline and Amyloid Accumulation.

    abstract:INTRODUCTION:As clinical trials move toward earlier intervention, we sought to redefine the β-amyloid (Aβ)-PET threshold based on the lowest point in a baseline distribution that robustly predicts future Aβ accumulation and cognitive decline in 3 independent samples of clinically normal individuals. METHODS:Sequential...


    pub_type: 杂志文章


    authors: Farrell ME,Jiang S,Schultz AP,Properzi MJ,Price JC,Becker JA,Jacobs HIL,Hanseeuw BJ,Rentz DM,Villemagne VL,Papp KV,Mormino EC,Betensky RA,Johnson KA,Sperling RA,Buckley RF,Alzheimer's Disease Neuroimaging Initiative and t

    更新日期:2021-01-26 00:00:00

  • Ethylene oxide polyneuropathy.

    abstract::Sensorimotor polyneuropathy developed in two workers who had been exposed to ethylene oxide gas repeatedly for several months. Sural nerve biopsies revealed axonal degeneration with mild changes of the myelin sheath. Unmyelinated fibers were also involved. Muscle biopsies showed typical denervation atrophy. Symptoms i...


    pub_type: 杂志文章


    authors: Kuzuhara S,Kanazawa I,Nakanishi T,Egashira T

    更新日期:1983-03-01 00:00:00

  • Improvement of multifocal motor neuropathy during long-term weekly treatment with human immunoglobulin.

    abstract::In multifocal motor neuropathy (MMN), little is known of the long-term effect of human immunoglobulin (HIG) infusions as a maintenance therapy. We report a patient who improved after an initial HIG infusion of 2 g/kg and continued to improve both clinically and electrophysiologically during maintenance HIG treatment c...


    pub_type: 杂志文章


    authors: Van den Berg LH,Franssen H,Wokke JH

    更新日期:1995-05-01 00:00:00

  • Restless legs syndrome: a predictor of lower physical function.

    abstract::Restless legs syndrome (RLS) is characterized by an urge to move the legs. The symptoms are more intense in the evening and during periods of inactivity. RLS symptoms are relieved by movement. It is classified as a sleep disorder, but in many ways it is actually a disorder of wakefulness that seems to intrude upon the...


    pub_type: 评论,社论


    authors: Auerbach S,Walters AS

    更新日期:2014-04-08 00:00:00

  • Long-term antithrombotic treatment in intracranial hemorrhage survivors with atrial fibrillation.

    abstract:OBJECTIVE:To perform a systematic review and meta-analysis of studies reporting recurrent intracranial hemorrhage (ICH) and ischemic stroke (IS) in ICH survivors with atrial fibrillation (AF) during long-term follow-up. METHODS:A comprehensive literature search including MEDLINE, EMBASE, Cochrane library, clinical tri...


    pub_type: 杂志文章,meta分析,评审


    authors: Korompoki E,Filippidis FT,Nielsen PB,Del Giudice A,Lip GYH,Kuramatsu JB,Huttner HB,Fang J,Schulman S,Martí-Fàbregas J,Gathier CS,Viswanathan A,Biffi A,Poli D,Weimar C,Malzahn U,Heuschmann P,Veltkamp R

    更新日期:2017-08-15 00:00:00

  • Hemorrhagic infarction in white matter following acute carbon monoxide poisoning.

    abstract::Hemorrhage complicating acute carbon monoxide poisoning is usually confined to the basal ganglia. The authors report three people with acute carbon monoxide poisoning manifesting selective or prominent white matter involvement complicated by hemorrhagic infarction on CT and MRI in one and by subcortical T2*-weighted M...


    pub_type: 杂志文章


    authors: Finelli PF,DiMario FJ Jr

    更新日期:2004-09-28 00:00:00

  • Computed tomographic angiography for diagnosis of brain death.

    abstract::The authors report two patients with suspected brain death who required confirmatory tests other than clinical examination because of prolonged barbiturate administration for intracranial hypertension. Absence of intracranial blood flow was documented on CT angiography and confirmed by CT perfusion images. Cerebral an...


    pub_type: 杂志文章


    authors: Qureshi AI,Kirmani JF,Xavier AR,Siddiqui AM

    更新日期:2004-02-24 00:00:00

  • Effects of weight loss on the course of idiopathic intracranial hypertension in women.

    abstract:OBJECTIVE:To determine the role of weight loss in the treatment of idiopathic intracranial hypertension (IIH) in obese women. METHODS:Chart review of 250 patients with suspected IIH revealed 58 women who met our criteria, did not undergo early surgical intervention, and had adequate documentation of visual status, pap...


    pub_type: 杂志文章


    authors: Kupersmith MJ,Gamell L,Turbin R,Peck V,Spiegel P,Wall M

    更新日期:1998-04-01 00:00:00