The T9176G mtDNA mutation severely affects ATP production and results in Leigh syndrome.

abstract：OBJECTIVE:To test the hypothesis that higher-challenge gait and balance tasks are more sensitive than traditional metrics to subtle patient-reported gait dysfunction and future fall risk in early multiple sclerosis (MS). METHODS:Persons with early MS (n = 185; ≤5 years diagnosed) reported gait function (MS Walking Sca...

journal_title：Neurology

authors： Brandstadter R,Ayeni O,Krieger SC,Harel NY,Escalon MX,Katz Sand I,Leavitt VM,Fabian MT,Buyukturkoglu K,Klineova S,Riley CS,Lublin FD,Miller AE,Sumowski JF

更新日期：2020-03-31 00:00:00

abstract：:Mitochondrial and cytoplasmic malic isoenzymes were assayed fluorometrically in digitonin-fractionated fibroblasts from three patients with Friedreich's ataxia (FA). Normal activity was found, failing to verify an earlier report of reduced fibroblast mitochondrial malic enzyme activity in FA. The previously reported d...

journal_title：Neurology

authors： Fernandez RJ,Civantos F,Tress E,Maltese WA,De Vivo DC

更新日期：1986-06-01 00:00:00

abstract：OBJECTIVE:To assess the effects of levodopa on resting-state brain metabolism in PD. BACKGROUND:In previous studies the authors used [18F] fluorodeoxyglucose (FDG) and PET to quantify regional metabolic abnormalities in PD. They found that this disease is characterized reproducibly by a specific abnormal PD-related pa...

journal_title：Neurology

authors： Feigin A,Fukuda M,Dhawan V,Przedborski S,Jackson-Lewis V,Mentis MJ,Moeller JR,Eidelberg D

更新日期：2001-12-11 00:00:00

abstract：BACKGROUND:The antivirally active MxA protein is induced by interferon (IFN) alpha/beta and inhibits the replication of single-stranded RNA viruses including measles virus (MV). The authors investigated whether the MxA gene contributed to the development of subacute sclerosing panencephalitis (SSPE) in Japanese individ...

journal_title：Neurology

authors： Torisu H,Kusuhara K,Kira R,Bassuny WM,Sakai Y,Sanefuji M,Takemoto M,Hara T

更新日期：2004-02-10 00:00:00

abstract：OBJECTIVES:Despite many neuropsychological studies to differentiate subcortical vascular dementia (SVaD) from Alzheimer disease (AD), previous studies did not eliminate confounding effects of mixed Alzheimer and vascular pathology. We aimed to investigate neuropsychological differences between patients with Pittsburgh ...

journal_title：Neurology

authors： Yoon CW,Shin JS,Kim HJ,Cho H,Noh Y,Kim GH,Chin JH,Oh SJ,Kim JS,Choe YS,Lee KH,Lee JH,Seo SW,Na DL

更新日期：2013-02-05 00:00:00

abstract：OBJECTIVE:To evaluate the efficacy and safety of eptinezumab, a humanized anti-calcitonin gene-related peptide monoclonal antibody, in the preventive treatment of chronic migraine (CM). METHODS:The Prevention of Migraine via Intravenous ALD403 Safety and Efficacy-2 (PROMISE-2) study was a phase 3, multicenter, randomi...

journal_title：Neurology

authors： Lipton RB,Goadsby PJ,Smith J,Schaeffler BA,Biondi DM,Hirman J,Pederson S,Allan B,Cady R

更新日期：2020-03-31 00:00:00

abstract：OBJECTIVE:To explore the incidence of depression and anxiety and to measure quality of life in women with idiopathic intracranial hypertension (IIH), a matched group cross-sectional study was conducted. Women with IIH (n = 28) were compared with control groups of weight- and age-matched women not diagnosed with IIH (n ...

journal_title：Neurology

authors： Kleinschmidt JJ,Digre KB,Hanover R

更新日期：2000-01-25 00:00:00

abstract：:Progressive supranuclear palsy (PSP) is a relatively common neurodegenerative tauopathy clinically characterized by parkinsonism, axial rigidity, and supranuclear gaze palsy. Pathologic findings of PSP are neuronal loss, gliosis, and neurofibrillary tangles in basal ganglia, diencephalon, and brainstem; there is incre...

journal_title：Neurology

authors： Fujioka S,Algom AA,Murray ME,Strongosky A,Soto-Ortolaza AI,Rademakers R,Ross OA,Wszolek ZK,Dickson DW

更新日期：2013-05-28 00:00:00

abstract：:We studied a family in which the father and 7 of 10 children had episodes of Bell's palsy. Five of the eight affected family members also had ocular motor palsies. Facial nerve and blink reflex studies in four affected siblings demonstrated asymmetrically reduced amplitude of evoked responses without delayed conductio...

journal_title：Neurology

authors： Aldrich MS,Beck RW,Albers JW

更新日期：1987-08-01 00:00:00

abstract：:Free amino acid determinations in the cerebrospinal fluid of patients with Parkinson's disease or other extrapyramidal disorders showed that in both groups most amino acids were increased. Significantly increased amino acids were neutral and basic amino acids. Amino acid clusters were present and corresponded with the...

journal_title：Neurology

authors： Lakke JP,Teelken AW

更新日期：1976-05-01 00:00:00

abstract：OBJECTIVE:To investigate the relationship between the presence of a circadian body temperature rhythm and behaviorally assessed consciousness levels in patients with disorders of consciousness (DOC; i.e., vegetative state/unresponsive wakefulness syndrome or minimally conscious state). METHODS:In a cross-sectional stu...

journal_title：Neurology

authors： Blume C,Lechinger J,Santhi N,del Giudice R,Gnjezda MT,Pichler G,Scarpatetti M,Donis J,Michitsch G,Schabus M

更新日期：2017-05-16 00:00:00

abstract：OBJECTIVE:We endeavored to develop an unruptured intracranial aneurysm (UIA) treatment score (UIATS) model that includes and quantifies key factors involved in clinical decision-making in the management of UIAs and to assess agreement for this model among specialists in UIA management and research. METHODS:An internat...

journal_title：Neurology

authors： Etminan N,Brown RD Jr,Beseoglu K,Juvela S,Raymond J,Morita A,Torner JC,Derdeyn CP,Raabe A,Mocco J,Korja M,Abdulazim A,Amin-Hanjani S,Al-Shahi Salman R,Barrow DL,Bederson J,Bonafe A,Dumont AS,Fiorella DJ,Gruber A,H

更新日期：2015-09-08 00:00:00

abstract：:In a series of 100 patients exhibiting clinical and molecular features of facioscapulohumeral muscular dystrophy (FSHMD), five patients had conduction defects or arrhythmia in the absence of cardiovascular risk factors--namely, intraventricular conduction delay and supraventricular arrhythmia induced by electrophysiol...

journal_title：Neurology

authors： Laforêt P,de Toma C,Eymard B,Becane HM,Jeanpierre M,Fardeau M,Duboc D

更新日期：1998-11-01 00:00:00

abstract：:We gave three adult rhesus monkeys seven IV injections of manganese chloride at approximately 1-week intervals. We evaluated neurologic status by serial clinical examinations and performed a levodopa test if the animal developed features of basal ganglia dysfunction. After the animals were killed, we performed neuropa...

journal_title：Neurology

authors： Olanow CW,Good PF,Shinotoh H,Hewitt KA,Vingerhoets F,Snow BJ,Beal MF,Calne DB,Perl DP

更新日期：1996-02-01 00:00:00

abstract：BACKGROUND:Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with complicated pathogenesis that poses challenges with respect to diagnosis and monitoring of disease progression. OBJECTIVES:To identify a biomarker panel that elucidates ALS disease pathogenesis, distinguishes patients with A...

journal_title：Neurology

authors： Mitchell RM,Freeman WM,Randazzo WT,Stephens HE,Beard JL,Simmons Z,Connor JR

更新日期：2009-01-06 00:00:00

abstract：OBJECTIVES:To investigate capacity to consent to medical treatment (competency) in cognitively impaired patients with PD. BACKGROUND:Although competency has been studied empirically in patients with cortical dementia (AD), no empirical studies have examined competency in patients with PD or other subcortical neurodege...

journal_title：Neurology

authors： Dymek MP,Atchison P,Harrell L,Marson DC

更新日期：2001-01-09 00:00:00

abstract：OBJECTIVE:To study and provide an update on the state of clinical research in neurology in the United States. METHODS:US American Academy of Neurology members and chairs of departments of neurology were surveyed regarding clinical research in 2016. NIH data on the neuroscience pipeline and extramural grant funding wer...

journal_title：Neurology

authors： Hall DA,Ramos AR,Gelfand JM,Videnovic A,Benatar M,Cahill C,Kluger BM,Goldman M

更新日期：2018-04-10 00:00:00

abstract：:Levodopa is the most effective drug treatment for Parkinson's disease (PD). However, its chronic use is associated with dyskinesia, motor fluctuations, and hallucinations. There are various approaches to the management of levodopa complications. Motor fluctuations can be treated with a long-acting levodopa preparation...

journal_title：Neurology

authors： Jankovic J

更新日期：2000-01-01 00:00:00

abstract：OBJECTIVE:To validate a neuropsychological algorithm for dementia diagnosis. METHODS:We developed a neuropsychological algorithm in a sample of 1,023 elderly residents of Cache County, UT. We compared algorithmic and clinical dementia diagnoses both based on DSM-III-R criteria. The algorithm diagnosed dementia when th...

journal_title：Neurology

authors： Tschanz JT,Welsh-Bohmer KA,Skoog I,West N,Norton MC,Wyse BW,Nickles R,Breitner JC

更新日期：2000-03-28 00:00:00

abstract：:We studied two patients with Meige syndrome who developed alpha-methyldopa-induced parkinsonism. Opposite responses of parkinsonian and dystonic symptoms to antiparkinson drugs in some cases suggest a functionally reciprocal relationship between these disorders. ...

journal_title：Neurology

authors： Sechi GP,Demontis G,Rosati G

更新日期：1985-11-01 00:00:00

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

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

abstract：:Since the emergence of the specialty, neurologists have worked with a rather restricted list of relatively inexpensive pharmacologic agents. This is rapidly changing with the development of new agents for the treatment of migraine, multiple sclerosis, Parkinson's disease, Alzheimer's disease, and epilepsy, accelerated...

journal_title：Neurology

authors： Gunderson CH

更新日期：1995-03-01 00:00:00

abstract：OBJECTIVE:To test the hypothesis that extratemporal neuronal networks are involved in dystonic posturing (DP) observed in mesial temporal epilepsy (MTLE). METHODS:The authors analyzed electroclinical findings in 36 patients with MTLE with or without DP. Three DP types were defined (types I, II, III) corresponding to a...

journal_title：Neurology

authors： Rusu V,Chassoux F,Landré E,Bouilleret V,Nataf F,Devaux BC,Turak B,Semah F

更新日期：2005-11-22 00:00:00

abstract：:To test the safety and efficacy of recombinant human insulin-like growth factor-I (rhIGF-I) in ALS, 183 patients from eight European centers were randomized to receive double-blind placebo (n = 59) or rhIGF-I 0.1 mg/kg/day (n = 124) subcutaneously for 9 months. At study completion, the primary efficacy outcome measure...

journal_title：Neurology

authors： Borasio GD,Robberecht W,Leigh PN,Emile J,Guiloff RJ,Jerusalem F,Silani V,Vos PE,Wokke JH,Dobbins T

更新日期：1998-08-01 00:00:00

abstract：BACKGROUND:To evaluate the time course of major vessel recanalization under IV thrombolysis in relation to functional outcome in acute ischemic stroke. METHODS:A total of 99 patients with an acute anterior circulation vessel occlusion who underwent IV thrombolysis were included. All patients had a standardized admissi...

journal_title：Neurology

authors： Wunderlich MT,Goertler M,Postert T,Schmitt E,Seidel G,Gahn G,Samii C,Stolz E,Duplex Sonography in Acute Stroke (DIAS) Study Group.,Competence Network Stroke.

更新日期：2007-04-24 00:00:00

abstract：:Spinal cord compression by extramedullary hemopoietic tissue is a rare complication of hemolytic anemias. This is the first report of this complication in hemolytic anemia caused by pyruvate-kinase deficiency. Indium scan, computed tomography (CT) scan, and myelography were helpful in diagnosis. Surgery and radiothera...

journal_title：Neurology

authors： Rutgers MJ,van der Lugt PJ,van Turnhout JM

更新日期：1979-04-01 00:00:00

abstract：:Three sisters with adult fucosidosis showed prominent psychomotor retardation, gargoyle features, and angiokeratoma corporis diffusum, meeting the criteria for type II fucosidosis. By histochemical and ultrastructural studies, biopsy of rectal mucosa revealed many abnormal macrophages that were filled with fucose-rich...

journal_title：Neurology

authors： Ikeda S,Kondo K,Oguchi K,Yanagisawa N,Horigome R,Murata F

更新日期：1984-04-01 00:00:00

abstract：OBJECTIVE:To determine whether cardiac magnetic resonance imaging (CMR) could be useful in identifying previously undiagnosed cardiomyopathies in a cohort of patients with ischemic stroke who underwent standard etiologic investigation and to describe the type and frequency of these cardiomyopathies. METHODS:We perform...

journal_title：Neurology

authors： Fonseca AC,Marto JP,Pimenta D,Guimarães T,Alves PN,Inácio N,Viana-Baptista M,Pinho E Melo T,Pinto FJ,Ferro JM,Almeida AG

更新日期：2020-01-07 00:00:00

abstract：:In 41 cases of verified ruptured saccular aneurysm, we prospectively predicted the presence or absence of delayed symptomatic cerebral vasospasm. CT criteria quantifying the extent and location of subarachnoid blood (developed in our previous retrospective study) were used in this prospective series of patients. Twent...

journal_title：Neurology

authors： Kistler JP,Crowell RM,Davis KR,Heros R,Ojemann RG,Zervas T,Fisher CM

更新日期：1983-04-01 00:00:00

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

authors： Bernson-Leung ME,Mazumdar M

更新日期：2014-05-06 00:00:00