Abnormal unloading reflex in a patient with infarction of the medial lemniscus.

abstract：OBJECTIVE:Clinical outcomes in multiple sclerosis (MS) are highly variable. We aim to determine the long-term clinical outcomes in MS, and to identify early prognostic features of these outcomes. METHODS:One hundred thirty-two people presenting with a clinically isolated syndrome were prospectively recruited between 1...

journal_title：Annals of neurology

authors： Chung KK,Altmann D,Barkhof F,Miszkiel K,Brex PA,O'Riordan J,Ebner M,Prados F,Cardoso MJ,Vercauteren T,Ourselin S,Thompson A,Ciccarelli O,Chard DT

更新日期：2020-01-01 00:00:00

abstract：:Fuel utilization in two adult patients with the myopathic form of very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency and five healthy subjects was investigated with stable isotopes during exercise at 50% of VO2max. The findings indicate that residual VLCAD activity in the patients is sufficient to maintain norm...

journal_title：Annals of neurology

authors： ØRngreen MC,Nørgaard MG,Sacchetti M,van Engelen BG,Vissing J

更新日期：2004-08-01 00:00:00

abstract：:Pathological evidence suggests that alterations of the blood-brain barrier (BBB) may occur in association with human immunodeficiency virus (HIV) dementia (HIVD). Increased BBB permeability could contribute to the development of dementia by facilitating the entry of activated and infected monocytes, as well as potenti...

journal_title：Annals of neurology

authors： Conant K,McArthur JC,Griffin DE,Sjulson L,Wahl LM,Irani DN

更新日期：1999-09-01 00:00:00

abstract：OBJECTIVE:Hemodynamic impairment in one hemisphere has been shown to trigger ipsilateral motor activation in the opposite hemisphere on functional imaging. We hypothesized that reversing the hypoperfusion would normalize the motor activation pattern. METHODS:We studied four patients with high-grade stenosis and impair...

journal_title：Annals of neurology

authors： Chmayssani M,Lazar RM,Hirsch J,Marshall RS

更新日期：2009-02-01 00:00:00

abstract：:Very long chain acyl-coenzyme A (acyl-CoA) dehydrogenase (VLCAD) deficiency is a severe disorder of mitochondrial beta-oxidation in infants. We report adult onset of attacks of painful rhabdomyolysis. Gas chromatography identified strongly elevated levels of tetradecenoic acid, 14:1(n-9), tetradecadienoic acid, 14:2(n...

journal_title：Annals of neurology

authors： Smelt AH,Poorthuis BJ,Onkenhout W,Scholte HR,Andresen BS,van Duinen SG,Gregersen N,Wintzen AR

更新日期：1998-04-01 00:00:00

abstract：OBJECTIVE:Accumulation of mitochondrial DNA (mtDNA) damage has been associated with aging and abnormal oxidative metabolism. We hypothesized that in human immunodeficiency virus-associated sensory neuropathy (HIV-SN), damaged mtDNA accumulates in distal nerve segments, and that a spatial pattern of mitochondrial dysfun...

journal_title：Annals of neurology

authors： Lehmann HC,Chen W,Borzan J,Mankowski JL,Höke A

更新日期：2011-01-01 00:00:00

abstract：OBJECTIVE:Deletions of CACNA1A, encoding the α1 subunit of CaV 2.1 channels, cause epilepsy with ataxia in humans. Whereas the deletion of Cacna1a in γ-aminobutyric acidergic (GABAergic) interneurons (INs) derived from the medial ganglionic eminence (MGE) impairs cortical inhibition and causes generalized seizures in N...

journal_title：Annals of neurology

authors： Jiang X,Lupien-Meilleur A,Tazerart S,Lachance M,Samarova E,Araya R,Lacaille JC,Rossignol E

更新日期：2018-09-01 00:00:00

abstract：OBJECTIVE:Metrics of diffusion tensor imaging (DTI) and magnetization transfer imaging (MTI) can detect diffuse axonal injury in traumatic brain injury (TBI). The relationship between the changes in these imaging measures and the underlying pathologies is still relatively unknown. This study investigated the radiologic...

journal_title：Annals of neurology

authors： Tu TW,Williams RA,Lescher JD,Jikaria N,Turtzo LC,Frank JA

更新日期：2016-06-01 00:00:00

abstract：OBJECTIVE:This study was untaken to investigate the association of micro brain infarcts (MBIs) with antemortem global cognitive function (CF), and whether brain weight (BW) and Alzheimer lesions (neurofibrillary tangles [NFTs] or neuritic plaques [NPs]) mediate the association. METHODS:Subjects were 436 well-character...

journal_title：Annals of neurology

authors： Launer LJ,Hughes TM,White LR

更新日期：2011-11-01 00:00:00

abstract：:Gene therapy methods have continued to develop rapidly, and many initial limitations that hampered clinical application have been overcome. Thus serious consideration of clinical application of gene therapy is warranted for selected disorders in which the pathogenesis is well defined. Parkinson's disease has been the ...

journal_title：Annals of neurology

authors： Kang UJ,Nakamura K

更新日期：2003-01-01 00:00:00

abstract：OBJECTIVE:Alzheimer disease (AD) is the most common form of dementia and is responsible for a huge and growing health care burden in the developed and developing world. The polygenic risk score (PRS) approach has shown 75 to 84% prediction accuracy of identifying individuals with AD risk. METHODS:In this study, we tes...

journal_title：Annals of neurology

authors： Leonenko G,Shoai M,Bellou E,Sims R,Williams J,Hardy J,Escott-Price V,Alzheimer's Disease Neuroimaging Initiative.

更新日期：2019-09-01 00:00:00

abstract：:Disorders of neuronal migration in cerebral cortex are associated with neurological impairments, including mental retardation and epilepsy. Their causes and pathophysiology remain largely unknown, however. In patients with Zellweger disease, a lethal panperoxisomal disorder, and in mice lacking the Pxr1 import recepto...

journal_title：Annals of neurology

authors： Gressens P,Baes M,Leroux P,Lombet A,Van Veldhoven P,Janssen A,Vamecq J,Marret S,Evrard P

更新日期：2000-09-01 00:00:00

abstract：:The development of a neuroprotective therapy that slows, stops, or reverses neurodegeneration in Parkinson's disease (PD) is the single most important unresolved issue in the management of this disorder. Current therapies provide effective control of symptoms, particularly in the early stages of the disease, but disea...

journal_title：Annals of neurology

authors： Olanow CW,Kieburtz K,Schapira AH

更新日期：2008-12-01 00:00:00

abstract：:Although the basal ganglia have been implicated in the development of movement disorders since the 1940s, the exact role played by these structures has remained elusive. The development of the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-monkey model of parkinsonism, and the recent resurgence of surgical therapy for t...

journal_title：Annals of neurology

authors： Vitek JL,Giroux M

更新日期：2000-04-01 00:00:00

abstract：:The term Charcot-Wilbrand syndrome (CWS) denotes dream loss following focal brain damage. We report the first case of CWS, in whom neuropsychological functions, extension of the underlying lesion, and sleep architecture changes were assessed. A 73-year-old woman reported a total dream loss after acute, bilateral occip...

journal_title：Annals of neurology

authors： Bischof M,Bassetti CL

更新日期：2004-10-01 00:00:00

abstract：:An infant presented with massive hyperchylomicronemia and a severe encephalopathy. MRI showed marked lipid deposition throughout the brain. Despite the normalization of the biochemistry, there was little clinical improvement, and at 18 months of age she has severe developmental delay, a strikingly abnormal MRI. Apolip...

journal_title：Annals of neurology

authors： Wilson CJ,Priore Oliva C,Maggi F,Catapano AL,Calandra S

更新日期：2003-06-01 00:00:00

abstract：:We demonstrated that an IgM M-protein from a patient with motor neuron syndrome had antibody activity against gangliosides GM1, GD1b, and asialo GM1. Studies with a sugar-binding lectin suggested that the epitope in the patient's M-IgM involved the Gal(beta 1-3) GalNAc moiety. Immunohistological techniques demonstrate...

journal_title：Annals of neurology

authors： Nardelli E,Steck AJ,Barkas T,Schluep M,Jerusalem F

更新日期：1988-05-01 00:00:00

abstract：OBJECTIVE:To test the hypothesis that variability in SNCA Rep1, a polymorphic dinucleotide microsatellite in the promoter region of the gene encoding α-synuclein, modifies the association between head injury and Parkinson's disease (PD) risk. METHODS:Participants in the Farming and Movement Evaluation (FAME) and the S...

journal_title：Annals of neurology

authors： Goldman SM,Kamel F,Ross GW,Jewell SA,Bhudhikanok GS,Umbach D,Marras C,Hauser RA,Jankovic J,Factor SA,Bressman S,Lyons KE,Meng C,Korell M,Roucoux DF,Hoppin JA,Sandler DP,Langston JW,Tanner CM

更新日期：2012-01-01 00:00:00

abstract：:Traumatic coma was produced in 45 monkeys by accelerating the head without impact in one of three directions. The duration of coma, degree of neurological impairment, and amount of diffuse axonal injury (DAI) in the brain were directly related to the amount of coronal head motion used. Coma of less than 15 minutes (co...

journal_title：Annals of neurology

authors： Gennarelli TA,Thibault LE,Adams JH,Graham DI,Thompson CJ,Marcincin RP

更新日期：1982-12-01 00:00:00

abstract：:The etiopathogenesis of Parkinson's disease (PD) has been elusive. Recently, several lines of evidence have converged to suggest that defects in the ubiquitin-proteasome system and proteolytic stress underlie nigral pathology in both familial and sporadic forms of the illness. In support of this concept, mutations in ...

journal_title：Annals of neurology

authors： McNaught KS,Olanow CW

更新日期：2003-01-01 00:00:00

abstract：OBJECTIVE:Episodic memory retrieval is reliant upon cognitive control systems, of which 2 have been identified with functional neuroimaging: a cingulo-opercular salience network (SN) and a frontoparietal executive network (EN). In Alzheimer's disease (AD), pathology is distributed throughout higher-order cortices. The ...

journal_title：Annals of neurology

authors： Dhanjal NS,Wise RJ

更新日期：2014-08-01 00:00:00

abstract：:Macrophages are prominent participants in inflammatory demyelinating neuropathies. To provide a different means of evaluating macrophage behavior, we used immunostaining of teased nerve fibers and endoneurial blood vessels. We assessed the frequency with which macrophages were seen in inflammatory demyelinating neurop...

journal_title：Annals of neurology

authors： Griffin JW,Stoll G,Li CY,Tyor W,Cornblath DR

更新日期：1990-01-01 00:00:00

abstract：:Systems for automatic assessment of cutaneous touch-pressure, vibratory, and thermal sensation have been developed. These systems use stimuli which are quantified and reproducible, a two-alternative forced-choice technique, and programmed steps to test, score, and report. If normal responses from series of healthy per...

journal_title：Annals of neurology

authors： Dyck PJ,Zimmerman IR,O'Brien PC,Ness A,Caskey PE,Karnes J,Bushek W

更新日期：1978-12-01 00:00:00

abstract：:In a patient with acquired immunodeficiency disease syndrome (AIDS) and muscle weakness, a muscle biopsy specimen disclosed degeneration of muscle fibers, regeneration, and focal endomysial mononuclear inflammation. A conspicuous feature was the presence of perivascular macrophages within the endomysium that showed po...

journal_title：Annals of neurology

authors： Chad DA,Smith TW,Blumenfeld A,Fairchild PG,DeGirolami U

更新日期：1990-10-01 00:00:00

abstract：:The developmental sequence of sleep cycles has been found to be a useful index of central nervous system maturation in premature infants. To determine the effects of severe reversible stress on the maturation of sleep cycles, 6- to 8-hour sleep studies were done on 10 premature infants with severe hyaline membrane dis...

journal_title：Annals of neurology

authors： Holmes GL,Logan WJ,Kirkpatrick BV,Meyer EC

更新日期：1979-12-01 00:00:00

abstract：:The Pilot Stroke Data Bank obtained information on 94 patients with intracerebral hemorrhage. These data were used to identify factors predictive of 30-day outcome from among 85 demographic, historical, clinical, and laboratory variables generally available to clinicians on the day of admission. The 9 univariate facto...

journal_title：Annals of neurology

authors： Tuhrim S,Dambrosia JM,Price TR,Mohr JP,Wolf PA,Heyman A,Kase CS

更新日期：1988-08-01 00:00:00

abstract：:To investigate the clinical features of transient cerebral hypoxia, syncope was induced in 56 of 59 healthy volunteers through a sequence of hyperventilation, orthostasis, and Valsalva maneuver. All events were monitored on video by two cameras. Complete syncope with falling and loss of consciousness was observed in 4...

journal_title：Annals of neurology

authors： Lempert T,Bauer M,Schmidt D

更新日期：1994-08-01 00:00:00

abstract：:The rate of local cerebral glucose utilization was determined for quantification of energy metabolism in macrostructures of the sensorimotor system during propagation of focal motor seizures in 24-month-old monkeys. The rate was measured in 4 control animals and in 4 monkeys each with seizures limited to the contralat...

journal_title：Annals of neurology

authors： Caveness WF,Kato M,Malamut BL,Hosokawa S,Wakisaka S,O'Neill RR

更新日期：1980-03-01 00:00:00

abstract：:The first electrophysiological study of the human lateral geniculate nucleus (LGN), optic radiation, striate, and extrastriate visual areas is presented in the context of presurgical evaluation of three epileptic patients (Patients 1, 2, and 3). Visual-evoked potentials to pattern reversal and face presentation were r...

journal_title：Annals of neurology

authors： Krolak-Salmon P,Hénaff MA,Tallon-Baudry C,Yvert B,Guénot M,Vighetto A,Mauguière F,Bertrand O

更新日期：2003-01-01 00:00:00

abstract：:Increased glutamatergic transmission in the basal ganglia is implicated in the pathophysiology of Parkinson's disease. However, the mechanisms by which activation of glutamate receptors produce parkinsonism are unknown. Therefore, we examined whether the glutamate agonists N-methyl-D-aspartate (NMDA), alpha-amino-3-hy...

journal_title：Annals of neurology

authors： Klockgether T,Turski L

更新日期：1993-10-01 00:00:00