The role of calcium-binding proteins in selective motoneuron vulnerability in amyotrophic lateral sclerosis.

abstract：:The reported inverse association between the S18Y variant of the ubiquitin carboxy-terminal hydrolase L1 (UCHL1) gene and Parkinson's disease (PD) has strong biological plausibility. If confirmed, genetic association of this variant with PD may support molecular targeting of the UCHL1 gene and its product as a therape...

journal_title：Annals of neurology

authors： Maraganore DM,Lesnick TG,Elbaz A,Chartier-Harlin MC,Gasser T,Krüger R,Hattori N,Mellick GD,Quattrone A,Satoh J,Toda T,Wang J,Ioannidis JP,de Andrade M,Rocca WA,UCHL1 Global Genetics Consortium.

更新日期：2004-04-01 00:00:00

abstract：:We report early-onset parkinsonism and dementia of 18 years' duration in a 52-year-old man whose grandfather and father had suffered from a similar neurological disease. In this patient, we found neuronal loss in various brain regions including the substantia nigra and cerebral cortex, Lewy bodies, cotton wool plaques...

journal_title：Annals of neurology

authors： Ishikawa A,Piao YS,Miyashita A,Kuwano R,Onodera O,Ohtake H,Suzuki M,Nishizawa M,Takahashi H

更新日期：2005-03-01 00:00:00

abstract：:Hypoxic-ischemic brain injury in survivors of perinatal asphyxia is a frequently encountered clinical problem for which there is currently no effective therapy. Neurotrophins, such as brain-derived neurotrophic factor (BDNF), can protect responsive neurons against cell death in some injury paradigms. While the role of...

journal_title：Annals of neurology

authors： Cheng Y,Gidday JM,Yan Q,Shah AR,Holtzman DM

更新日期：1997-04-01 00:00:00

abstract：OBJECTIVE:Autosomal recessive primary microcephaly (MCPH) is a rare condition characterized by a reduced cerebral cortex accompanied with intellectual disability. Mutations in 17 genes have been shown to cause this phenotype. Recently, mutations in CIT, encoding CRIK (citron rho-interacting kinase)-a component of the c...

journal_title：Annals of neurology

authors： Moawia A,Shaheen R,Rasool S,Waseem SS,Ewida N,Budde B,Kawalia A,Motameny S,Khan K,Fatima A,Jameel M,Ullah F,Akram T,Ali Z,Abdullah U,Irshad S,Höhne W,Noegel AA,Al-Owain M,Hörtnagel K,Stöbe P,Baig SM,Nürnberg P

更新日期：2017-10-01 00:00:00

abstract：:Noradrenergic fibers were identified in rat cervical lymph nodes by fluorescence histochemistry and confirmed by radioenzymatic determination of norepinephrine. After superior cervical ganglionectomy, lymph node norepinephrine and noradrenergic fluorescence were strikingly decreased. The alpha-adrenergic radioligand [...

journal_title：Annals of neurology

authors： Giron LT Jr,Crutcher KA,Davis JN

更新日期：1980-11-01 00:00:00

abstract：:Human T-lymphotropic virus type I (HTLV-I) has been associated with adult T-cell leukemia/lymphoma (ATL), a malignancy of mature CD4-positive lymphocytes, and with tropical spastic paraparesis (TSP), a demyelinating neurological syndrome. This article describes the clinical and pathological features of ATL and reviews...

journal_title：Annals of neurology

authors： Murphy EL,Blattner WA

更新日期：1988-01-01 00:00:00

abstract：:Intranuclear inclusions are one of the ultrastructural hallmarks of oculopharyngeal muscular dystrophy (OPMD), a disorder caused by small polyalanine (GCG) expansions in the gene that codes for a ubiquitous nuclear protein called poly(A) binding protein 2 (PABP2). We studied OPMD skeletal muscle and found that 1.0 to ...

journal_title：Annals of neurology

authors： Becher MW,Kotzuk JA,Davis LE,Bear DG

更新日期：2000-11-01 00:00:00

abstract：:Amphiphysin-IgG was identified in 71 patients among 120,000 evaluated serologically for paraneoplastic autoantibodies. Clinical information was available for 63 patients. Cancer was detected in 50 (mostly limited), proven histologically in 46, and was imaged intrathoracically in 4 patients (lung, small-cell [27] and n...

journal_title：Annals of neurology

authors： Pittock SJ,Lucchinetti CF,Parisi JE,Benarroch EE,Mokri B,Stephan CL,Kim KK,Kilimann MW,Lennon VA

更新日期：2005-07-01 00:00:00

abstract：:We assessed nigrostriatal dopaminergic function in Parkinson's disease (PD) patients undergoing a double-blind, placebo-controlled surgical trial of embryonic dopamine cell implantation. Forty PD patients underwent positron emission tomography (PET) imaging with [18F]fluorodopa (FDOPA) prior to randomization to transp...

journal_title：Annals of neurology

authors： Nakamura T,Dhawan V,Chaly T,Fukuda M,Ma Y,Breeze R,Greene P,Fahn S,Freed C,Eidelberg D

更新日期：2001-08-01 00:00:00

abstract：:Axonal degeneration is a major cause of permanent neurological deficit in multiple sclerosis (MS), but no current therapies for the disease are known to be effective at axonal protection. Here, we examine the ability of a sodium channel-blocking agent, flecainide, to reduce axonal degeneration in an experimental model...

journal_title：Annals of neurology

authors： Bechtold DA,Kapoor R,Smith KJ

更新日期：2004-05-01 00:00:00

abstract：:Thalamic degeneration was present in 5 autopsied cases of X-chromosome-linked copper malabsorption (X-cLCM), Menkes' kinky hair disease. Among the thalamic nuclei, those in the formatio paraventricularis, intralamellaris, and extralamellaris were spared. The nuclei projecting to the granular cortices had severe neuron...

journal_title：Annals of neurology

authors： Iwata M,Hirano A,French JH

更新日期：1979-04-01 00:00:00

abstract：OBJECTIVE:To examine changes in the response properties of meningeal nociceptors that might lead to migraine pain and examine endogenous processes that could play a role in mediating them using a clinically relevant model of migraine triggering, namely infusion of the nitric oxide (NO) donor nitroglycerin (NTG). METHO...

journal_title：Annals of neurology

authors： Zhang X,Kainz V,Zhao J,Strassman AM,Levy D

更新日期：2013-06-01 00:00:00

abstract：:A 45-year-old woman was diagnosed as having the unclassified form of botulism. Her intestines may have been predisposed to colonization with Clostridium botulinum because of a jejunoileal bypass procedure that had been done several years earlier. One other similar case has been reported. ...

journal_title：Annals of neurology

authors： Freedman M,Armstrong RM,Killian JM,Boland D

更新日期：1986-11-01 00:00:00

abstract：OBJECTIVE:To develop and validate a questionnaire for assessing cutaneous allodynia (CA), and to estimate the prevalence and severity of CA in the migraine population. METHODS:Migraineurs (n = 11,388) completed the Allodynia Symptom Checklist, assessing the frequency of allodynia symptoms during headache. Response opt...

journal_title：Annals of neurology

authors： Lipton RB,Bigal ME,Ashina S,Burstein R,Silberstein S,Reed ML,Serrano D,Stewart WF,American Migraine Prevalence Prevention Advisory Group.

更新日期：2008-02-01 00:00:00

abstract：:Huntington's disease is an inherited disorder caused by expansion of a CAG trinucleotide repeat in the IT15 gene, which leads to expansion of a polyglutamine tract within the protein called huntingtin. Despite the characterization of the IT15 gene and the mutation involved in the disease, the normal function of huntin...

journal_title：Annals of neurology

authors： Gourfinkel-An I,Cancel G,Trottier Y,Devys D,Tora L,Lutz Y,Imbert G,Saudou F,Stevanin G,Agid Y,Brice A,Mandel JL,Hirsch EC

更新日期：1997-11-01 00:00:00

abstract：:We recorded horizontal smooth-pursuit responses to sinusoidal and step-ramp stimuli in 7 patients with unilateral frontal lobe lesions. Five patients had directional smooth-pursuit deficits, all with impairment toward the side of cerebral damage. Ipsidirectional pursuit defects involved pursuit maintenance to sinusoid...

journal_title：Annals of neurology

authors： Morrow MJ,Sharpe JA

更新日期：1995-04-01 00:00:00

abstract：:A recent observation has shown that a common polymorphism in the alpha1-antichymotrypsin (ACT) gene modifies the apolipoprotein E (ApoE) epsilon4-associated Alzheimer's disease (AD) risk identifying the combination of the ACT/AA and ApoE epsilon4/epsilon4 genotypes as a potential susceptibility marker for AD. We analy...

journal_title：Annals of neurology

authors： Nacmias B,Tedde A,Latorraca S,Piacentini S,Bracco L,Amaducci L,Guarnieri BM,Petruzzi C,Ortenzi L,Sorbi S

更新日期：1996-10-01 00:00:00

abstract：:Dichloroacetate (DCA) stimulates pyruvate dehydrogenase complex (PDHC) activity and lowers cerebral lactate concentrations. In the R6/2 and N171-82Q transgenic mouse models of Huntington's disease (HD), DCA significantly increased survival, improved motor function, delayed loss of body weight, attenuated the developme...

journal_title：Annals of neurology

authors： Andreassen OA,Ferrante RJ,Huang HM,Dedeoglu A,Park L,Ferrante KL,Kwon J,Borchelt DR,Ross CA,Gibson GE,Beal MF

更新日期：2001-07-01 00:00:00

abstract：:We report the clinical features of 12 patients with drop attacks associated with Meniere's syndrome. Each described a sensation of being pushed, thrown, or knocked to the ground or a sudden illusion of movement of the environment that led to a fall. These episodes were not accompanied by symptoms of their typical atta...

journal_title：Annals of neurology

authors： Baloh RW,Jacobson K,Winder T

更新日期：1990-09-01 00:00:00

abstract：OBJECTIVE:Newer magnetic resonance imaging (MRI) techniques have shown promise in capturing early Parkinson disease (PD)-related changes in the substantia nigra pars compacta (SNc), the key pathological loci. Their translational value, however, is hindered by technical complexity and inconsistent results. METHODS:A no...

journal_title：Annals of neurology

authors： Du G,Lewis MM,Sica C,Kong L,Huang X

更新日期：2019-01-01 00:00:00

abstract：:Six polypeptides resolved by two-dimensional electrophoresis of homogenates from human skeletal muscle have been identified as tropomyosin by electrophoretic and immunochemical methods. The 6 proteins are consistently present in approximately the same abundance in normal biceps, deltoid, gastrocnemius, and quadriceps ...

journal_title：Annals of neurology

authors： Giometti CS,Danon MJ

更新日期：1985-08-01 00:00:00

abstract：:The monoclonal antibodies anti-Leu-19 and anti-NKH-1 recognize the CD56 differentiation antigen expressed on natural killer (NK) cells and on a T-cell subset. Because CD56 is an isoform of neural cell adhesion molecule (N-CAM), we examined its expression on human muscle using antibodies to Leu-19, NKH-1, and purified ...

journal_title：Annals of neurology

authors： Illa I,Leon-Monzon M,Dalakas MC

更新日期：1992-01-01 00:00:00

abstract：:The seemingly unpredictable response to levodopa in patients with Parkinson's disease can be understood as an interaction between several distinct pharmacological effects of levodopa. The most important are a short-duration response with a half-life of minutes to hours and a long-duration response with a half-life of ...

journal_title：Annals of neurology

authors： Nutt JG,Holford NH

更新日期：1996-05-01 00:00:00

abstract：OBJECTIVE:To understand the mechanisms of skeletal muscle destruction and resistance to enzyme replacement therapy in Pompe disease, a deficiency of lysosomal acid alpha-glucosidase (GAA), in which glycogen accumulates in lysosomes primarily in cardiac and skeletal muscles. METHODS:We have analyzed compartments of the...

journal_title：Annals of neurology

authors： Fukuda T,Ewan L,Bauer M,Mattaliano RJ,Zaal K,Ralston E,Plotz PH,Raben N

更新日期：2006-04-01 00:00:00

abstract：OBJECTIVE:Visual Evoked Potentials (VEPs) following optic neuritis (ON) remain chronically prolonged, although standard visual tests indicate full recovery. We hypothesized that dynamic visual processes, such as motion perception, may be more vulnerable to slowed conduction in the optic nerve, and consequently be bette...

journal_title：Annals of neurology

authors： Raz N,Dotan S,Chokron S,Ben-Hur T,Levin N

更新日期：2012-04-01 00:00:00

abstract：:A 51-year-old diabetic woman developed bilateral rhinoorbitocerebral phycomycosis. Successful treatment was accomplished by correction of the ketoacidosis, surgical debridement, and amphotericin B therapy. Rhinoorbitocerebral phycomycosis is a fulminant and frequently fatal disease most often seen in debilitated hosts...

journal_title：Annals of neurology

authors： Kasper LH,Bernat JL,Nordgren RE,Reeves AG

更新日期：1979-08-01 00:00:00

abstract：:Using the technique of transcranial magnetic stimulation over the motor areas of cortex and recording electromyographic (EMG) responses from the first dorsal interosseous muscle, we measured the excitability of corticocortical inhibitory circuits at rest using a double pulse paradigm, in 11 patients with Parkinson's d...

journal_title：Annals of neurology

authors： Ridding MC,Inzelberg R,Rothwell JC

更新日期：1995-02-01 00:00:00

abstract：:A 40-year-old man with relapsing-remitting multiple sclerosis (MS) developed primary central nervous system lymphoma (PCNSL) after having received 21 doses of natalizumab monotherapy. PCNSL is a disease of the elderly, with the majority of patients being diagnosed in the 7th to 8th decade of life. Immunodeficiency, ia...

journal_title：Annals of neurology

authors： Schweikert A,Kremer M,Ringel F,Liebig T,Duyster J,Stüve O,Hemmer B,Berthele A

更新日期：2009-09-01 00:00:00

abstract：:Alzheimer's disease (AD) and Parkinson's disease (PD) are generally considered to be separate and distinct disease entities. However, a considerable amount of evidence demonstrates that these disorders share common clinical and neuropathologic features and that overlap between the two conditions is extensive. For exam...

journal_title：Annals of neurology

authors： Perl DP,Olanow CW,Calne D

更新日期：1998-09-01 00:00:00

abstract：:A sensitive enzyme-linked immunosorbent assay method was employed to measure interleukin-2 (IL-2) levels in cerebrospinal fluid (CSF) and sera from 30 patients with multiple sclerosis (MS) and 8 patients with other neurological diseases. Detectable levels of IL-2 were found in 6 sera and 9 CSF samples of 21 patients w...

journal_title：Annals of neurology

authors： Gallo P,Piccinno M,Pagni S,Tavolato B

更新日期：1988-12-01 00:00:00