Wolfram syndrome: evidence of a diffuse neurodegenerative disease by magnetic resonance imaging.

abstract：OBJECTIVE:To characterize humoral response to cerebellum in patients with gluten ataxia. BACKGROUND:Gluten ataxia is a common neurologic manifestation of gluten sensitivity. METHODS:The authors assessed the reactivity of sera from patients with gluten ataxia (13), newly diagnosed patients with celiac disease without ...

journal_title：Neurology

authors： Hadjivassiliou M,Boscolo S,Davies-Jones GA,Grünewald RA,Not T,Sanders DS,Simpson JE,Tongiorgi E,Williamson CA,Woodroofe NM

更新日期：2002-04-23 00:00:00

abstract：:Pathology and imaging studies have shown that patients with Parkinson disease (PD) have a prolonged period of uncertain duration when vulnerable neuronal populations are degenerating, but typical motor symptoms have not yet developed. This provides both an opportunity-it may be best to test new medications and, ultima...

journal_title：Neurology

authors： Marek K,Jennings D

更新日期：2009-02-17 00:00:00

abstract：:The effects of a single oral dose of levodopa administered in a randomized, double-blind, placebo-controlled cross-over design on formation of a motor memory were studied by a training protocol in patients with chronic stroke. Levodopa enhanced the ability of motor training to encode an elementary motor memory relativ...

journal_title：Neurology

authors： Floel A,Hummel F,Breitenstein C,Knecht S,Cohen LG

更新日期：2005-08-09 00:00:00

abstract：OBJECTIVE:To characterize the present state of brain death (BD) determination in actual practice relative to contemporary American Academy of Neurology (AAN) guidelines. METHODS:We reviewed the charts of all adult (16 years and older) BD organ donors during 2011 from 68 heterogeneous hospitals in the Midwest United St...

journal_title：Neurology

authors： Shappell CN,Frank JI,Husari K,Sanchez M,Goldenberg F,Ardelt A

更新日期：2013-12-03 00:00:00

abstract：OBJECTIVE:This observational multimodality brain imaging study investigates emergence of endophenotypes of late-onset Alzheimer disease (AD) risk during endocrine transition states in a cohort of clinically and cognitively normal women and age-matched men. METHODS:Forty-two 40- to 60-year-old cognitively normal women ...

journal_title：Neurology

authors： Mosconi L,Berti V,Quinn C,McHugh P,Petrongolo G,Varsavsky I,Osorio RS,Pupi A,Vallabhajosula S,Isaacson RS,de Leon MJ,Brinton RD

更新日期：2017-09-26 00:00:00

abstract：:Capecitabine is used to treat advanced breast and gastrointestinal malignancies. A single case of encephalopathy and three cases of peripheral neuropathy are the only neurotoxicities reported. The authors report five additional cases of capecitabine-induced multifocal leukoencephalopathy. ...

journal_title：Neurology

authors： Videnovic A,Semenov I,Chua-Adajar R,Baddi L,Blumenthal DT,Beck AC,Simuni T,Futterer S,Gradishar W,Tellez C,Raizer JJ

更新日期：2005-12-13 00:00:00

abstract：:We treated 64 emergency room patients with a primary vascular headache with dihydroergotamine (DHE), meperidine, or butorphanol. Post-treatment pain scores were lowest in the DHE group (p less than 0.01). Eight of 21 patients receiving DHE had greater than 90% reduction in pain compared with three of 19 patients recei...

journal_title：Neurology

authors： Belgrade MJ,Ling LJ,Schleevogt MB,Ettinger MG,Ruiz E

更新日期：1989-04-01 00:00:00

abstract：BACKGROUND:Among patients with a patent foramen ovale (PFO) and a prior cryptogenic ischemic stroke or TIA, the absolute and relative risk of recurrent events is unclear. METHODS:We conducted a systematic review and meta-analysis of clinical studies in any language published up to February 2008. We included studies re...

journal_title：Neurology

authors： Almekhlafi MA,Wilton SB,Rabi DM,Ghali WA,Lorenzetti DL,Hill MD

更新日期：2009-07-14 00:00:00

abstract：:Thirty-eight survivors from among 117 patients hospitalized after out-of-hospital cardiac arrest were evaluated approximately 3 1/2 years later. Twenty patients were living; 18 had died. Fifty-three percent had resumed independent social activities, but only 32% had returned to work. Eight of 14 patients tested were n...

journal_title：Neurology

authors： Earnest MP,Yarnell PR,Merrill SL,Knapp GL

更新日期：1980-12-01 00:00:00

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

authors： Skoglund RR

更新日期：1979-05-01 00:00:00

abstract：:Twenty-seven patients with static or worsening chronic inflammatory-demyelinating polyradiculoneuropathy were randomly assigned to alternate day decremental prednisone therapy alone or with azathioprine (2 mg/kg) for 9 months. No statistically significant alterations were demonstrated between these treatment schedules...

journal_title：Neurology

authors： Dyck PJ,O'Brien P,Swanson C,Low P,Daube J

更新日期：1985-08-01 00:00:00

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...

journal_title：Neurology

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

abstract：OBJECTIVES:Intracerebral hemorrhage (ICH) is a highly lethal disease of the elderly. Use of statins is increasingly widespread among the elderly, and therefore common in patients who develop ICH. Accumulating data suggests that statins have neuroprotective effects, but their association with ICH outcome has been incons...

journal_title：Neurology

authors： Biffi A,Devan WJ,Anderson CD,Ayres AM,Schwab K,Cortellini L,Viswanathan A,Rost NS,Smith EE,Goldstein JN,Greenberg SM,Rosand J

更新日期：2011-05-03 00:00:00

abstract：:A veterinarian contracted rabies in the course of laboratory work with homogenates of rabid goat brain. Epidemiologic study determined a respiratory mode of transmission. After a fulminant encephalitic illness, formed rabies virions were identified in the synaptic zones of the olfactory glomeruli. Identification, isol...

journal_title：Neurology

authors： Conomy JP,Leibovitz A,McCombs W,Stinson J

更新日期：1977-01-01 00:00:00

abstract：:We performed proton magnetic resonance spectroscopic imaging (1H-MRSI) in patients with motor neuron disease (MND) to evaluate the distribution and extent of cortical neuron damage or loss as reflected by decreased N-acetyl (NA) to creatine (Cr) resonance intensity ratios. We examined premotor (superior frontal gyrus)...

journal_title：Neurology

authors： Pioro EP,Antel JP,Cashman NR,Arnold DL

更新日期：1994-10-01 00:00:00

abstract：:We investigated the possible role of potassium deficiency, a recognized cause of myopathy, in the pathogenesis of experimental acute alcoholic myopathy (EAM) in the rat. Alcohol-treated animals receiving the same dietary potassium as controls developed mild hypokalemia; however, muscle potassium was preserved or eleva...

journal_title：Neurology

authors： Haller RG,Carter NW,Ferguson E,Knochel JP

更新日期：1984-04-01 00:00:00

abstract：OBJECTIVE:To evaluate the production rate of CSF in patients with differing disease states. METHODS:The authors measured the production rate of CSF in three groups of patients: five patients with PD below age 60 (aged 51 +/- 4 years, mean +/- SD), nine with PD over age 60 (aged 69 +/- 6 years, mean +/- SD), and seven ...

journal_title：Neurology

authors： Silverberg GD,Heit G,Huhn S,Jaffe RA,Chang SD,Bronte-Stewart H,Rubenstein E,Possin K,Saul TA

更新日期：2001-11-27 00:00:00

abstract：:Migraine is associated with stimulus hypersensitivity, increased evoked cortical responses, and abnormal 5-HT levels in peripheral blood. We studied cortical auditory evoked potentials (AEPs) between attacks in 35 patients suffering from migraine without aura (MO, n = 25) or with aura (MA, n = 10) and in 25 healthy vo...

journal_title：Neurology

authors： Wang W,Timsit-Berthier M,Schoenen J

更新日期：1996-05-01 00:00:00

abstract：:The rate of spontaneous resolution of a recently diagnosed solitary cerebral cysticercus granuloma as visualized on CT scan was prospectively studied in 210 patients presenting with seizures. The proportion of granulomas that had resolved completely at different time intervals after the index scan was 18.8% at 3 month...

journal_title：Neurology

authors： Rajshekhar V

更新日期：2001-12-26 00:00:00

abstract：OBJECTIVE:To assess the impact of intracranial arterial stenosis on the progression from mild cognitive impairment (MCI) to Alzheimer disease (AD). METHODS:A total of 423 participants with MCI were included and evaluated with clinical and neuropsychological examinations annually for 4 years. The incidence of dementia ...

journal_title：Neurology

authors： Zhu J,Wang Y,Li J,Deng J,Zhou H

更新日期：2014-03-11 00:00:00

abstract：:Many studies have demonstrated that dipyridamole is not purely an antiplatelet agent but also exerts its antithrombotic properties through the vessel wall. Using a laboratory model in which bovine endothelial cells are seeded to form a subendothelial matrix (SEM), investigators have shown that dipyridamole enhances th...

journal_title：Neurology

authors： Eisert WG

更新日期：2001-01-01 00:00:00

abstract：OBJECTIVE:To improve clinical recognition and provide research diagnostic criteria for three clinical syndromes associated with frontotemporal lobar degeneration. METHODS:Consensus criteria for the three prototypic syndromes-frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia-were developed b...

journal_title：Neurology

authors： Neary D,Snowden JS,Gustafson L,Passant U,Stuss D,Black S,Freedman M,Kertesz A,Robert PH,Albert M,Boone K,Miller BL,Cummings J,Benson DF

更新日期：1998-12-01 00:00:00

abstract：OBJECTIVE:To clarify the genetic, clinicopathologic, and neuroimaging characteristics of patients with hereditary diffuse leukoencephalopathy with spheroids (HDLS) with the colony stimulating factor 1 receptor (CSF-1R) mutation. METHODS:We performed molecular genetic analysis of CSF-1R in patients with HDLS. Detailed ...

journal_title：Neurology

authors： Konno T,Tada M,Tada M,Koyama A,Nozaki H,Harigaya Y,Nishimiya J,Matsunaga A,Yoshikura N,Ishihara K,Arakawa M,Isami A,Okazaki K,Yokoo H,Itoh K,Yoneda M,Kawamura M,Inuzuka T,Takahashi H,Nishizawa M,Onodera O,Kakita

更新日期：2014-01-14 00:00:00

abstract：OBJECTIVE:Dravet syndrome is a rare neurodevelopmental disorder characterized by seizures and other neurologic problems. SCN1A mutations account for ∼80% of cases. Animal studies have implicated mutation-related dysregulated cortical inhibitory networks in its pathophysiology. We investigated such networks in people wi...

journal_title：Neurology

authors： Stern WM,Sander JW,Rothwell JC,Sisodiya SM

更新日期：2017-04-25 00:00:00

abstract：OBJECTIVE:To relate signs and symptoms to morphologic changes and presence of multiple mitochondrial DNA (mtDNA) deletions in a patient with autosomal dominant progressive external ophthalmoplegia (adPEO) and mitochondrial myopathy. BACKGROUND:An etiologic association between the somatic multiple mtDNA deletions in ad...

journal_title：Neurology

authors： Moslemi AR,Melberg A,Holme E,Oldfors A

更新日期：1999-07-13 00:00:00

abstract：OBJECTIVE:To assess the pathophysiologic changes in patients with spontaneous intracranial hypotension (SIH) based on measures of CSF dynamics, and on the duration of symptoms, in a retrospective case-controlled study. METHODS:We included consecutive patients investigated for SIH at our department from January 2012 to...

journal_title：Neurology

authors： Häni L,Fung C,Jesse CM,Ulrich CT,Miesbach T,Cipriani DR,Dobrocky T,Z'Graggen WJ,Raabe A,Piechowiak EI,Beck J

更新日期：2020-07-21 00:00:00

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

authors： Etminan M,Brophy JM,Samii A

更新日期：2014-09-30 00:00:00

abstract：OBJECTIVES:To evaluate basal ganglia changes along the amyotrophic lateral sclerosis (ALS)-ALS-frontotemporal dementia (FTD) continuum using multiple, complementary imaging techniques. METHODS:Sixty-seven C9orf72-negative patients with ALS and 39 healthy controls were included in a cross-sectional quantitative MRI stu...

journal_title：Neurology

authors： Machts J,Loewe K,Kaufmann J,Jakubiczka S,Abdulla S,Petri S,Dengler R,Heinze HJ,Vielhaber S,Schoenfeld MA,Bede P

更新日期：2015-10-13 00:00:00

abstract：OBJECTIVE:To determine whether there are distinct thalamic regions statistically associated with either contraversive or ipsiversive disturbance of verticality perception measured by subjective visual vertical (SVV). METHODS:We used modern statistical lesion behavior mapping on a sample of 37 stroke patients with isol...

journal_title：Neurology

authors： Baier B,Conrad J,Stephan T,Kirsch V,Vogt T,Wilting J,Müller-Forell W,Dieterich M

更新日期：2016-01-12 00:00:00

abstract：:Epilepsy is common in people with intellectual and developmental disabilities (IDD). In adulthood, patients with IDD and epilepsy (IDD-E) have neurologic, psychiatric, medical, and social challenges compounded by fragmented and limited care. With increasing neurologic disability, there is a higher frequency of epileps...

journal_title：Neurology

authors： Devinsky O,Asato M,Camfield P,Geller E,Kanner AM,Keller S,Kerr M,Kossoff EH,Lau H,Kothare S,Singh BK,Wirrell E

更新日期：2015-10-27 00:00:00