Late-onset metachromatic leukodystrophy: molecular pathology in two siblings.

abstract：:Direct DNA tests for mutations in neurogenetic disorders are establishing a new era in diagnostic neurology. These highly accurate and specific tests will greatly improve diagnosis and genetic counseling, lead to more accurate assessment of prognosis, and reduce the cost of diagnostic evaluations. However, the use and...

journal_title：Annals of neurology

authors： Bird TD,Bennett RL

更新日期：1995-08-01 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：:Patients with atrial fibrillation are at risk for cerebral embolism; however, the roles of chronic anticoagulation or antiplatelet therapy for stroke prevention in patients with nonvalvular atrial fibrillation have been controversial. Recently, the results of three large prospective randomized trials that examined the...

journal_title：Annals of neurology

authors： Albers GW,Sherman DG,Gress DR,Paulseth JE,Petersen P

更新日期：1991-10-01 00:00:00

abstract：:Transcranial magnetic stimulation was performed in 2 patients with focal motor seizures in the right hand caused by a circumscribed tumor process affecting the left precentral gyrus. In both cases, paired-pulse transcranial magnetic stimulation showed a loss of intracortical inhibition for interstimulus intervals of 2...

journal_title：Annals of neurology

authors： Irlbacher K,Brandt SA,Meyer BU

更新日期：2002-07-01 00:00:00

abstract：:Nine patients who had suffered strokes were examined between 10 and 34 days after onset using positron emission tomography. DMO labeled with carbon 11 was used to evaluate brain acid-base balance, and the oxygen-15 inhalation technique was used to measure regional cerebral blood flow, the oxygen extraction fraction, a...

journal_title：Annals of neurology

authors： Syrota A,Castaing M,Rougemont D,Berridge M,Baron JC,Bousser MG,Pocidalo JJ

更新日期：1983-10-01 00:00:00

abstract：:Reductions in cerebral metabolism sufficient to impair cognition in normal individuals also occur in Alzheimer's disease (AD). The degree of clinical disability in AD correlates closely to the magnitude of the reduction in brain metabolism. Therefore, we tested whether impairments in tricarboxylic acid (TCA) cycle enz...

journal_title：Annals of neurology

authors： Bubber P,Haroutunian V,Fisch G,Blass JP,Gibson GE

更新日期：2005-05-01 00:00:00

abstract：:This review discusses the etiology and pathogenesis of Parkinson's disease (PD). Mitochondrial respiratory failure and oxidative stress appear to be two major contributors to nigral neuronal death in PD. Complex I deficiency has been reported by several groups and appears to be one of the basic abnormalities responsib...

journal_title：Annals of neurology

authors： Mizuno Y,Yoshino H,Ikebe S,Hattori N,Kobayashi T,Shimoda-Matsubayashi S,Matsumine H,Kondo T

更新日期：1998-09-01 00:00:00

abstract：OBJECTIVE:Intracellular amyloid β-protein (Aβ) contributes to neurodegeneration in Alzheimer disease (AD). Apomorphine (APO) is a dopamine receptor agonist for Parkinson disease and also protects against oxidative stress. Efficacy of APO for an AD mouse model and effects of APO on cell cultures are studied. METHODS:Th...

journal_title：Annals of neurology

authors： Himeno E,Ohyagi Y,Ma L,Nakamura N,Miyoshi K,Sakae N,Motomura K,Soejima N,Yamasaki R,Hashimoto T,Tabira T,LaFerla FM,Kira J

更新日期：2011-02-01 00:00:00

abstract：OBJECTIVE:This study addresses an important problem in neurology, distinguishing tremor and ataxia using quantitative methods. Specifically, we aimed to quantitatively separate dysmetria, a cardinal sign of ataxia, from tremor in essential tremor (ET). METHODS:In Experiment 1, we compared 19 participants diagnosed wit...

journal_title：Annals of neurology

authors： Casamento-Moran A,Yacoubi B,Wilkes BJ,Hess CW,Foote KD,Okun MS,Wagle Shukla A,Vaillancourt DE,Christou EA

更新日期：2020-08-01 00:00:00

abstract：:Human prion diseases can occur as an idiopathic disorder (sporadic Creutzfeldt-Jakob disease) or can be acquired, as is the case for variant Creutzfeldt-Jakob disease. These disorders are characterized by the accumulation of a protease-resistant form of the host-encoded prion protein termed PrP(Sc) in the brains of af...

journal_title：Annals of neurology

authors： Head MW,Bunn TJ,Bishop MT,McLoughlin V,Lowrie S,McKimmie CS,Williams MC,McCardle L,MacKenzie J,Knight R,Will RG,Ironside JW

更新日期：2004-06-01 00:00:00

abstract：:Pallidopyramidal disease is a rare disease of young patients in which they manifest a parkinsonian syndrome and pyramidal signs. Pallidopyramidal disease has been attributed to a degeneration of the pallidum and the pyramidal tract, although only 1 patient has been studied postmortem. In the present report, [18F]fluor...

journal_title：Annals of neurology

authors： Remy P,Hosseini H,Degos JD,Samson Y,Agid Y,Warter JM,Vidailhet M

更新日期：1995-12-01 00:00:00

abstract：:We studied neuronal activity and its relation to the accumulation of neurofibrillary tangles in Alzheimer's disease (AD) neurons by in situ hybridization to cytochrome oxidase subunit III messenger RNA, a marker of mitochondrial energy metabolism. In AD midtemporal cortex, levels of cytochrome oxidase subunit III mess...

journal_title：Annals of neurology

authors： Hatanpää K,Brady DR,Stoll J,Rapoport SI,Chandrasekaran K

更新日期：1996-09-01 00:00:00

abstract：:We report a prospective clinical and electroencephalographic study of 19 patients with juvenile myoclonic epilepsy and absence seizures. Absences began 1 to 9 (4.5 +/- 2.5) years before myoclonic jerks and generalized tonic-clonic seizures. Clinical manifestations during the absence ictus showed great variation, rangi...

journal_title：Annals of neurology

authors： Panayiotopoulos CP,Obeid T,Waheed G

更新日期：1989-04-01 00:00:00

abstract：:Despite many years of intensive research, multiple sclerosis (MS) defies understanding and treatment remains suboptimal. The prevailing hypothesis is that MS is immune mediated and that experimental allergic encephalomyelitis (EAE) is a suitable model to elucidate pathogenesis and devise therapy. This review examines ...

journal_title：Annals of neurology

authors： Sriram S,Steiner I

更新日期：2005-12-01 00:00:00

abstract：:Positron emission tomography was used to study the effect of supratentorial infarction on cerebellar metabolic rate for oxygen and cerebellar blood flow. In a control group of patients, the mean cerebellar metabolic rate for oxygen was 2.97 +/- 0.11 (standard error of the mean [SEM] ) ml-1 . min-1 . hg-1 and mean cere...

journal_title：Annals of neurology

authors： Martin WR,Raichle ME

更新日期：1983-08-01 00:00:00

abstract：:Patients with multiple sclerosis (MS) frequently have selective depletion of the CD45R+CD4+ T-cell subset during active phases of disease. To study the relationship between changes in this subset and the onset of objective clinical exacerbations of disease, a longitudinal study was undertaken. Two CD4+ T-cell subsets ...

journal_title：Annals of neurology

authors： Rose LM,Ginsberg AH,Rothstein TL,Ledbetter JA,Clark EA

更新日期：1988-08-01 00:00:00

abstract：OBJECTIVE:Guillain-Barré syndrome (GBS) is an acute postinfectious immune-mediated polyneuropathy. Although preceding respiratory tract infections with Mycoplasma pneumoniae have been reported in some cases, the role of M. pneumoniae in the pathogenesis of GBS remains unclear. We here cultured, for the first time, M. p...

journal_title：Annals of neurology

authors： Meyer Sauteur PM,Huizinga R,Tio-Gillen AP,Roodbol J,Hoogenboezem T,Jacobs E,van Rijn M,van der Eijk AA,Vink C,de Wit MC,van Rossum AM,Jacobs BC

更新日期：2016-10-01 00:00:00

abstract：OBJECTIVE:Transient high-frequency oscillations (HFOs; 150-600Hz) in local field potentials generated by human hippocampal and parahippocampal areas have been related to both physiological and pathological processes. The cellular basis and effects of normal and abnormal forms of HFOs have been controversial. This lack ...

journal_title：Annals of neurology

authors： Alvarado-Rojas C,Huberfeld G,Baulac M,Clemenceau S,Charpier S,Miles R,de la Prida LM,Le Van Quyen M

更新日期：2015-02-01 00:00:00

abstract：:We demonstrate that the 3-hydroxy-3-methyl-glutaryl-coenzyme A (HMG-CoA) reductase inhibitors atorvastatin and simvastatin enhance functional outcome and induce brain plasticity when administered after stroke to rats. With atorvastatin treatment initiated 1 day after stroke, animals exhibited significant increases in ...

journal_title：Annals of neurology

authors： Chen J,Zhang ZG,Li Y,Wang Y,Wang L,Jiang H,Zhang C,Lu M,Katakowski M,Feldkamp CS,Chopp M

更新日期：2003-06-01 00:00:00

abstract：OBJECTIVE:Delayed cerebral ischemia (DCI) is a common, disabling complication of subarachnoid hemorrhage (SAH). Preventing DCI is a key focus of neurocritical care, but interventions carry risk and cannot be applied indiscriminately. Although retrospective studies have identified continuous electroencephalographic (cEE...

journal_title：Annals of neurology

authors： Rosenthal ES,Biswal S,Zafar SF,O'Connor KL,Bechek S,Shenoy AV,Boyle EJ,Shafi MM,Gilmore EJ,Foreman BP,Gaspard N,Leslie-Mazwi TM,Rosand J,Hoch DB,Ayata C,Cash SS,Cole AJ,Patel AB,Westover MB

更新日期：2018-05-01 00:00:00

abstract：:Microelectrode recording methods for stereotactic localization of the subthalamic nucleus (STN) and surrounding structures are described. These methods accurately define targets for chronic deep brain stimulation in the treatment of Parkinson's disease. Mean firing rates and a burst index were determined for all recor...

journal_title：Annals of neurology

authors： Hutchison WD,Allan RJ,Opitz H,Levy R,Dostrovsky JO,Lang AE,Lozano AM

更新日期：1998-10-01 00:00:00

abstract：:The titer of the scrapie agent was determined by measurements of time intervals from inoculation to onset of illness and from inoculation to death. Both intervals were found to be inversely proportional to the size of the dose injected intracerebrally into random-bred weanling Syrian hamsters. The logarithms of the ti...

journal_title：Annals of neurology

authors： Prusiner SB,Cochran SP,Groth DF,Downey DE,Bowman KA,Martinez HM

更新日期：1982-04-01 00:00:00

abstract：OBJECTIVE:Whether intracerebral hemorrhage (ICH) associated with non-vitamin K antagonist oral anticoagulants (NOAC-ICH) has a better outcome compared to ICH associated with vitamin K antagonists (VKA-ICH) is uncertain. METHODS:We performed a systematic review and individual patient data meta-analysis of cohort studie...

journal_title：Annals of neurology

authors： Tsivgoulis G,Wilson D,Katsanos AH,Sargento-Freitas J,Marques-Matos C,Azevedo E,Adachi T,von der Brelie C,Aizawa Y,Abe H,Tomita H,Okumura K,Hagii J,Seiffge DJ,Lioutas VA,Traenka C,Varelas P,Basir G,Krogias C,Purrucke

更新日期：2018-11-01 00:00:00

abstract：OBJECTIVE:The study was undertaken to determine whether normobaric hypoxia causes elevated brain volume and intracranial pressure in individuals with symptoms consistent with acute mountain sickness (AMS). METHODS:Thirteen males age = (26 (sd 6)) years were exposed to normobaric hypoxia (12% O2 ) and normoxia (21% O2 ...

journal_title：Annals of neurology

authors： Lawley JS,Alperin N,Bagci AM,Lee SH,Mullins PG,Oliver SJ,Macdonald JH

更新日期：2014-06-01 00:00:00

abstract：:Regular, evidence-based assignment of patients to etiologic stroke categories is essential to enable valid comparison among studies. We designed an algorithm (SSS-TOAST) that incorporated recent advances in stroke imaging and epidemiology to identify the most probable TOAST category in the presence of evidence for mul...

journal_title：Annals of neurology

authors： Ay H,Furie KL,Singhal A,Smith WS,Sorensen AG,Koroshetz WJ

更新日期：2005-11-01 00:00:00

abstract：:The nosological status of multifocal motor neuropathy remains controversial. The clinical and electrodiagnostic hallmarks suggest selective motor fiber involvement. In this study, we asked to what extent sensory nerves might be involved pathologically in multifocal motor neuropathy. Examination of sensory nerve biopsy...

journal_title：Annals of neurology

authors： Corse AM,Chaudhry V,Crawford TO,Cornblath DR,Kuncl RW,Griffin JW

更新日期：1996-03-01 00:00:00

abstract：:The site of autonomic deficit was investigated in 4 patients with loss of thermoregulatory flushing and sweating on one side of the face but no associated miosis (harlequin syndrome). In 2 patients the sudomotor deficit included the hand. Signs of postganglionic sympathetic deficit included pathological lacrimal sweat...

journal_title：Annals of neurology

authors： Drummond PD,Lance JW

更新日期：1993-12-01 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：:We report the clinical, radiological, and neuropathological features of selective brainstem injury in an asphyxiated term infant. Disproportionate injury to thalamus, basal ganglia, and brainstem with relative sparing of cortex and subcortical white matter is observed occasionally after acute total asphyxia. Although ...

journal_title：Annals of neurology

authors： Roland EH,Hill A,Norman MG,Flodmark O,MacNab AJ

更新日期：1988-01-01 00:00:00

abstract：:Striatal 18F-6-fluorodopa (FD) uptake constants were measured by positron emission tomography in (1) normal cynomolgus monkeys and (2) a series of cynomolgus and rhesus monkeys that had received intracarotid infusions of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). After the animals were killed, the number and...

journal_title：Annals of neurology

authors： Pate BD,Kawamata T,Yamada T,McGeer EG,Hewitt KA,Snow BJ,Ruth TJ,Calne DB

更新日期：1993-09-01 00:00:00