Proteolytic stress: a unifying concept for the etiopathogenesis of Parkinson's disease.

abstract：:Ocular flutter is a rare horizontal eye movement disorder characterized by rapid saccadic oscillations. It has been hypothesized that it is caused by loss of "pause" neuronal inhibition of "burst" neuron function in the paramedian pontine reticular formation (PPRF); however, there have been no imaging studies confirmi...

journal_title：Annals of neurology

authors： Schon F,Hodgson TL,Mort D,Kennard C

更新日期：2001-09-01 00:00:00

abstract：:We report a neurological disease among Cree Indian children in a northern Quebec village. The disease manifests as severe mental retardation, cerebral atrophy with white matter changes and calcifications, and systemic immunological abnormalities. Eleven cases are known in five families. The familial incidence of cases...

journal_title：Annals of neurology

authors： Black DN,Watters GV,Andermann E,Dumont C,Kabay ME,Kaplan P,Meagher-Villemure K,Michaud J,O'Gorman G,Reece E

更新日期：1988-10-01 00:00:00

abstract：:A 71-year-old woman presented with a short history of episodes of severe left-sided orbital and temporal pain in paroxysms lasting 60 to 90 seconds, and accompanied by ipsilateral lacrimation of the eye, rhinorrhea, and conjunctival injection. Results of clinical examination and structural imaging were normal and a cl...

journal_title：Annals of neurology

authors： May A,Bahra A,Büchel C,Turner R,Goadsby PJ

更新日期：1999-11-01 00:00:00

abstract：:Using three adjustments, we have revised a 1976 prevalence count for multiple sclerosis in the United States. The adjustments were made to data from a US national survey; they used 1990 population projections from the US Bureau of the Census, and results of investigations conducted in Weld and Larimer Countries, Color...

journal_title：Annals of neurology

authors： Anderson DW,Ellenberg JH,Leventhal CM,Reingold SC,Rodriguez M,Silberberg DH

更新日期：1992-03-01 00:00:00

abstract：:The role of mitochondrial DNA (mtDNA) mutations in the pathogenesis of Leber's hereditary optic neuropathy (LHON) has yet to be characterized. Several clinical features of the disease imply that nuclear genes might also be involved in its expression. We have confirmed the presence of a severe NADH:coenzyme Q1 reductas...

journal_title：Annals of neurology

authors： Cock HR,Tabrizi SJ,Cooper JM,Schapira AH

更新日期：1998-08-01 00:00:00

abstract：:Because of the association of D-penicillamine (DP) therapy with myasthenia gravis, we have studied long-term DP treatment in five inbred strains of mice with doses comparable to those used in patients with rheumatoid arthritis. No clinical weakness or anti-acetylcholine receptor (AChR) antibody developed with up to 6 ...

journal_title：Annals of neurology

authors： Bever CT Jr,Dretchen KL,Blake GJ,Chang HW,Penn AS,Asofsky R

更新日期：1984-07-01 00:00:00

abstract：:Patterns of cerebral metabolic intercorrelations were compared in the resting state in 15 healthy young men (ages 20 to 32 years) and 15 healthy elderly men (ages 64 to 83 years). Controlling for whole-brain glucose metabolism, partial correlation coefficients were determined between pairs of regional cerebral metabol...

journal_title：Annals of neurology

authors： Horwitz B,Duara R,Rapoport SI

更新日期：1986-01-01 00:00:00

abstract：OBJECTIVE:Multiple sclerosis (MS) is characterized by demyelination centered around cerebral veins. Recent studies suggested this topographic pattern may be caused by venous congestion, a condition termed chronic cerebrospinal venous insufficiency (CCSVI). Published sonographic criteria of CCSVI include reflux in the d...

journal_title：Annals of neurology

authors： Doepp F,Paul F,Valdueza JM,Schmierer K,Schreiber SJ

更新日期：2010-08-01 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：:The North American study of plasmapheresis in Guillain-Barré syndrome (GBS) included early, standardized electrodiagnostic testing in 210 of the 245 patients. To determine the types of abnormalities and the relation to outcome, we analyzed the prospectively collected motor conduction data obtained during the first 30 ...

journal_title：Annals of neurology

authors： Cornblath DR,Mellits ED,Griffin JW,McKhann GM,Albers JW,Miller RG,Feasby TE,Quaskey SA

更新日期：1988-04-01 00:00:00

abstract：:Fourteen patients with cauda equina syndrome secondary to long-standing ankylosing spondylitis are described. The roughly symmetrical neurological deficits were very slowly progressive and began long after the onset of the spondylitis, usually well after the rheumatological symptoms had stopped. Eventually every patie...

journal_title：Annals of neurology

authors： Bartleson JD,Cohen MD,Harrington TM,Goldstein NP,Ginsburg WW

更新日期：1983-12-01 00:00:00

abstract：:The clinical and neuropathological findings in 28 cases of paramedian thalamic and midbrain infarcts are reported. The 4 instances of unilateral paramedian thalamic infarct were characterized by mood and behavioral changes, limitation of the infarct to the center of the anatomical paramedian territory, and symmetrical...

journal_title：Annals of neurology

authors： Castaigne P,Lhermitte F,Buge A,Escourolle R,Hauw JJ,Lyon-Caen O

更新日期：1981-08-01 00:00:00

abstract：:Mutations in DJ-1 (PARK7) have been reported in two consanguineous families with young-onset Parkinson's disease (YOPD). This study aims to confirm the presence of pathogenic DJ-1 mutations and determine their contribution in young-onset and more typical later onset Parkinson's disease (PD). The entire open reading fr...

journal_title：Annals of neurology

authors： Abou-Sleiman PM,Healy DG,Quinn N,Lees AJ,Wood NW

更新日期：2003-09-01 00:00:00

abstract：OBJECTIVE:To assess the ability for visual search and recognition of roadside targets and safety errors during a landmark and traffic sign identification task in drivers with Parkinson's disease (PD). METHODS:Seventy-nine drivers with PD and 151 neurologically normal older adults underwent a battery of visual, cogniti...

journal_title：Annals of neurology

authors： Uc EY,Rizzo M,Anderson SW,Sparks J,Rodnitzky RL,Dawson JD

更新日期：2006-10-01 00:00:00

abstract：:Striopallidodentate calcinosis (Fahr's disease) is characterized clinically by seizures, rigidity, and dementia and pathologically by mineral deposition in the basal ganglia, dentate nucleus, and cerebral cortex. Disorders of iron and calcium-phosphate metabolism are thought to play a role in its pathogenesis. We pres...

journal_title：Annals of neurology

authors： Beall SS,Patten BM,Mallette L,Jankovic J

更新日期：1989-10-01 00:00:00

abstract：OBJECTIVE:Autosomal-dominant lateral temporal epilepsy (ADLTE) is a genetic focal epilepsy characterized by auditory symptoms. Two genes, LGI1 and RELN, encoding secreted proteins, are implicated in the etiology of ADLTE, but half of the affected families remain genetically unsolved, and the underlying molecular mechan...

journal_title：Annals of neurology

authors： Dazzo E,Rehberg K,Michelucci R,Passarelli D,Boniver C,Vianello Dri V,Striano P,Striano S,Pasterkamp RJ,Nobile C

更新日期：2018-03-01 00:00:00

abstract：:Neuromuscular disorders reported in association with human immunodeficiency virus (HIV) infection include several forms of peripheral neuropathy and polymyositis. We report 11 patients with HIV-associated myopathy. Five patients with acquired immunodeficiency syndrome (AIDS), 2 with AIDS-related complex, and 4 otherwi...

journal_title：Annals of neurology

authors： Simpson DM,Bender AN

更新日期：1988-07-01 00:00:00

abstract：:Two brothers with profound neonatal hypotonia and hyporeflexia and electrodiagnostic testing consistent with lower motor neuron pathology were found to have a leukodystrophy. Using single-strand conformational polymorphism analysis and direct sequencing, a mutation within exon 3 of the gene encoding proteolipid protei...

journal_title：Annals of neurology

authors： Kaye EM,Doll RF,Natowicz MR,Smith FI

更新日期：1994-12-01 00:00:00

abstract：:Posterior choroidal artery (PChA) territory infarcts remain the least well-known type of thalamic infarcts. Our study of 10 personal cases, selected from 2,925 stroke patients admitted consecutively to a community-based primary care center, and 10 published cases of unilateral PChA territory infarct suggests that they...

journal_title：Annals of neurology

authors： Neau JP,Bogousslavsky J

更新日期：1996-06-01 00:00:00

abstract：:We have previously shown bilateral activation of motor pathways and the recruitment of additional motor areas in studies of groups of patients with recovery from motor stroke. We have now developed a new positron emission tomographic technique to measure the changes in regional cerebral blood flow elicited during a mo...

journal_title：Annals of neurology

authors： Weiller C,Ramsay SC,Wise RJ,Friston KJ,Frackowiak RS

更新日期：1993-02-01 00:00:00

abstract：:Two principal features of Alzheimer's disease (AD) are (1) the occurrence of neurofibrillary tangles (NFTs) and senile plaques, and (2) the loss of cortical cholinergic activity because of dysfunction of neurons in the basal forebrain cholinergic system. The relationship of these two abnormalities is an unresolved iss...

journal_title：Annals of neurology

authors： Rasool CG,Svendsen CN,Selkoe DJ

更新日期：1986-10-01 00:00:00

abstract：:We examined the expression of Fas antigen and Bcl-2 protein in thymic tissue surgically resected from 10 patients with myasthenia gravis, using immunocytochemical techniques. Histologically, thymic tissues from 7 myasthenia gravis patients showed hyperplasia, while 3 other patients had thymomas. In hyperplastic thymic...

journal_title：Annals of neurology

authors： Onodera J,Nakamura S,Nagano I,tobita M,Yoshioka M,Takeda A,Oouchi M,Itoyama Y

更新日期：1996-04-01 00:00:00

abstract：:Recently, variant mRNA transcripts for the astroglial glutamate transporter EAAT2 have been detected in brain tissues of 60% of patients with sporadic amyotrophic lateral sclerosis (SALS). We have tested the hypothesis that the gene for EAAT2 may be defective in some ALS cases. In 16 familial ALS (FALS) pedigrees with...

journal_title：Annals of neurology

authors： Aoki M,Lin CL,Rothstein JD,Geller BA,Hosler BA,Munsat TL,Horvitz HR,Brown RH Jr

更新日期：1998-05-01 00:00:00

abstract：:Polymorphonuclear neutral protease activity (PMN-NPA) was examined in 87 patients with definite multiple sclerosis (MS) (48 active, 39 inactive), 49 patients with other neurological diseases (OND), 24 patients with immune-mediated non-neurological diseases (INND), and 32 normal subjects. PMN-NPA was found to be signif...

journal_title：Annals of neurology

authors： Guarnieri B,Lolli F,Amaducci L

更新日期：1985-11-01 00:00:00

abstract：:We report on a new allele at the arylsulfatase A (ARSA) locus causing late-onset metachromatic leukodystrophy (MLD). In that allele arginine84, a residue that is highly conserved in the arylsulfatase gene family, is replaced by glutamine. In contrast to alleles that cause early-onset MLD, the arginine84 to glutamine s...

journal_title：Annals of neurology

authors： Kappler J,von Figura K,Gieselmann V

更新日期：1992-03-01 00:00:00

abstract：:Although Alzheimer's and Parkinson's diseases predominately affect elderly adults, the proteins that play a role in the pathogenesis of these diseases are expressed throughout life. In fact, many of the proteins hypothesized to be important in the progression of neurodegeneration play direct or indirect roles in the d...

journal_title：Annals of neurology

authors： Rogers D,Schor NF

更新日期：2010-02-01 00:00:00

abstract：OBJECTIVE:Nonketotic hyperglycinemia is a neurometabolic disorder characterized by intellectual disability, seizures, and spasticity. Patients with attenuated nonketotic hyperglycinemia make variable developmental progress. Predictive factors have not been systematically assessed. METHODS:We reviewed 124 patients stra...

journal_title：Annals of neurology

authors： Swanson MA,Coughlin CR Jr,Scharer GH,Szerlong HJ,Bjoraker KJ,Spector EB,Creadon-Swindell G,Mahieu V,Matthijs G,Hennermann JB,Applegarth DA,Toone JR,Tong S,Williams K,Van Hove JL

更新日期：2015-10-01 00:00:00

abstract：OBJECTIVE:A number of hypotheses have been put forward as to why humans respond to fever by seizing. The current leading hypotheses are that respiratory alkalosis produces an as yet unidentified change in neural excitability or that inflammatory mediators potentiate excitatory synaptic transmission. However, it is well...

journal_title：Annals of neurology

authors： Thomas EA,Hawkins RJ,Richards KL,Xu R,Gazina EV,Petrou S

更新日期：2009-08-01 00:00:00

abstract：:Endoneurial fluid pressure (EFP) was recorded by an active, servo-null pressure system after a glass micropipette was inserted into rat sciatic nerve undergoing wallerian degeneration. The lesions were produced by crushing the left sciatic nerve of the anesthetized animal at its point of entry into the thigh. Eighty-f...

journal_title：Annals of neurology

authors： Powell HC,Myers RR,Costello ML,Lampert PW

更新日期：1979-06-01 00:00:00

abstract：:Five monkeys trained to perform with the extremity contralateral to a stimulus had unilateral neglect induced by frontal and reticular formation lesions. Postoperatively the performance of the animals was abnormal only on ipsilateral stimulation, which suggests that the mechanism underlying neglect in these subjects i...

journal_title：Annals of neurology

authors： Watson RT,Miller BD,Heilman KM

更新日期：1978-06-01 00:00:00