Heat opens axon initial segment sodium channels: a febrile seizure mechanism?

abstract：:We analyzed the prion protein gene (PRNP) region in patients with transmissible spongiform encephalopathy associated with the PRNP D178N mutation. The results suggest that the D178N chromosomes had independent origins in each affected pedigree or apparently sporadic case. A de novo spontaneous PRNP mutation was observ...

journal_title：Annals of neurology

authors： Dagvadorj A,Petersen RB,Lee HS,Cervenakova L,Shatunov A,Budka H,Brown P,Gambetti P,Goldfarb LG

更新日期：2002-09-01 00:00:00

abstract：:Two families with a point mutation in mtDNA associated with myoclonic epilepsy and ragged-red fiber disease showed pronounced clinical heterogeneity. The mothers of the two families had adult-onset myopathy with ragged-red fibers, partial deficiency of cytochrome c oxidase, and sensory neuropathy. Members of the first...

journal_title：Annals of neurology

authors： Graf WD,Sumi SM,Copass MK,Ojemann LM,Longstreth WT Jr,Shanske S,Lombes A,DiMauro S

更新日期：1993-06-01 00:00:00

abstract：OBJECTIVE:To create a data-driven computational model that identifies brain regions most frequently influenced by successful deep brain stimulation (DBS) of the globus pallidus (GP) for advanced, medication-resistant, generalized dystonia. METHODS:We studied a retrospective cohort of 21 DYT1 primary dystonia patients ...

journal_title：Annals of neurology

authors： Cheung T,Noecker AM,Alterman RL,McIntyre CC,Tagliati M

更新日期：2014-07-01 00:00:00

abstract：:Clinical electrophysiological studies are important in the evaluation of patients with Guillain-Barré syndrome. Physiological evidence of demyelination occurs in almost all patients with Guillain-Barré syndrome, especially if serial studies are performed. Criteria for demyelination are proposed. Prognostic information...

journal_title：Annals of neurology

authors： Cornblath DR

更新日期：1990-01-01 00:00:00

abstract：OBJECTIVE:To clarify the histopathological alterations of microglia in the brains of patients with hereditary diffuse leukoencephalopathy with spheroids (HDLS) caused by mutations of the gene encoding the colony stimulating factor-1 receptor (CSF-1R). METHODS:We examined 5 autopsied brains and 1 biopsy specimen from a...

journal_title：Annals of neurology

authors： Tada M,Konno T,Tada M,Tezuka T,Miura T,Mezaki N,Okazaki K,Arakawa M,Itoh K,Yamamoto T,Yokoo H,Yoshikura N,Ishihara K,Horie M,Takebayashi H,Toyoshima Y,Naito M,Onodera O,Nishizawa M,Takahashi H,Ikeuchi T,Kakita A

更新日期：2016-10-01 00:00:00

abstract：OBJECTIVE:To investigate whether soluble growth stimulation expressed gene 2 (sST2), a prognostic marker in cardiovascular and inflammatory disorders, is associated with neurological injury after aneurysmal subarachnoid hemorrhage (SAH). METHODS:We studied SAH patients from 2 independent cohorts. Outcome assessments i...

journal_title：Annals of neurology

authors： Bevers MB,Wolcott Z,Bache S,Hansen C,Sastre C,Mylvaganam R,Koch MJ,Patel AB,Møller K,Kimberly WT

更新日期：2019-09-01 00:00:00

abstract：:Few studies evaluate neuropathological correlates of behavioral changes in Alzheimer disease (AD). We identified 31 autopsy patients with a diagnosis of definite AD. Behavioral changes were assessed with the Neuropsychiatric Inventory. Brain sections were collected from bilateral orbitofrontal and left anterior cingul...

journal_title：Annals of neurology

authors： Tekin S,Mega MS,Masterman DM,Chow T,Garakian J,Vinters HV,Cummings JL

更新日期：2001-03-01 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：: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：: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：:We describe a family of 9 affected individuals in three generations with nocturnal oro-facio-brachial partial seizures, secondarily generalized partial seizures, and centro-temporal epileptiform discharges, associated with oral and speech dyspraxia and cognitive impairment. The speech disorder was prominent, but diffe...

journal_title：Annals of neurology

authors： Scheffer IE,Jones L,Pozzebon M,Howell RA,Saling MM,Berkovic SF

更新日期：1995-10-01 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：:Regional cerebral blood flow (rCBF) was measured in human subjects during saccadic eye movements by a 254-channel dynamic gamma camera. Focal rCBF increases were repeatedly observed in an area within the middle precentral and premotor regions which corresponds to the frontal eye field in humans. Our findings suggest t...

journal_title：Annals of neurology

authors： Melamed E,Larsen B

更新日期：1979-01-01 00:00:00

abstract：:Cytological evaluation of cerebrospinal fluid (CSF) is an important means of following response to intracavitary chemotherapy for leptomeningeal malignancy. We studied the feasibility of quantitative cytological evaluation by retrospective analysis of serial CSF specimens from 7 patients receiving phase I intracavitar...

journal_title：Annals of neurology

authors： Russack V,Kim S,Chamberlain MC

更新日期：1993-07-01 00:00:00

abstract：:GM1b is a minor ganglioside in human peripheral nerves. Serum anti-GM1b antibodies frequently are present in patients with Guillain-Barré syndrome (GBS). In this collaborative study, we investigated the antecedent infections, clinical features, and response to treatment of GBS patients with anti-GM1b antibodies. Of 13...

journal_title：Annals of neurology

authors： Yuki N,Ang CW,Koga M,Jacobs BC,van Doorn PA,Hirata K,van der Meché FG

更新日期：2000-03-01 00:00:00

abstract：:A 42-year-old homosexual man without evidence of immune deficiency developed cerebral granulomatous angiitis in association with the isolation of human T-lymphotropic virus type III (HTLV-III) from brain tissue and cerebrospinal fluid. This syndrome may be an additional neurological sequela of HTLV-III infection. ...

journal_title：Annals of neurology

authors： Yankner BA,Skolnik PR,Shoukimas GM,Gabuzda DH,Sobel RA,Ho DD

更新日期：1986-09-01 00:00:00

abstract：:Magnetic resonance imaging (MRI) provides a powerful tool for assessing disease activity in multiple sclerosis (MS), and its role as a surrogate marker for monitoring treatment efficacy is now becoming established. The most commonly used MRI parameters in treatment trials are (1) monthly gadolinium-enhanced MRI, with ...

journal_title：Annals of neurology

authors： Molyneux PD,Filippi M,Barkhof F,Gasperini C,Yousry TA,Truyen L,Lai HM,Rocca MA,Moseley IF,Miller DH

更新日期：1998-03-01 00:00:00

abstract：OBJECTIVE:Neuronal channelopathies cause brain disorders, including epilepsy, migraine, and ataxia. Despite the development of mouse models, pathophysiological mechanisms for these disorders remain uncertain. One particularly devastating channelopathy is Dravet syndrome (DS), a severe childhood epilepsy typically cause...

journal_title：Annals of neurology

authors： Liu Y,Lopez-Santiago LF,Yuan Y,Jones JM,Zhang H,O'Malley HA,Patino GA,O'Brien JE,Rusconi R,Gupta A,Thompson RC,Natowicz MR,Meisler MH,Isom LL,Parent JM

更新日期：2013-07-01 00:00:00

abstract：:Twenty-six patients with late-stage Parkinson disease were given 0.4 to 15 mg of pergolide mesylate daily in addition to, or as replacement for, levodopa or bromocriptine therapy. Despite treatment with individually determined optimum doses of levodopa, bromocriptine, and anticholinergics, they had shown response fail...

journal_title：Annals of neurology

authors： Lang AE,Quinn N,Brincat S,Marsden CD,Parkes JD

更新日期：1982-09-01 00:00:00

abstract：:We compared epilepsy phenotypes with genotypes of Angelman syndrome (AS), including chromosome 15q11-13 deletions (class I), uniparental disomy (class II), methylation imprinting abnormalities (class III), and mutation in the UBE3A gene (class IV). Twenty patients were prospectively selected based on clinical cytogene...

journal_title：Annals of neurology

authors： Minassian BA,DeLorey TM,Olsen RW,Philippart M,Bronstein Y,Zhang Q,Guerrini R,Van Ness P,Livet MO,Delgado-Escueta AV

更新日期：1998-04-01 00:00:00

abstract：OBJECTIVE:Both animal and human data suggest that noradrenergic stimulation may enhance motor performance after brain damage. We conducted a placebo-controlled, double-blind and crossover design study to investigate the effects of noradrenergic stimulation on the cortical motor system in hemiparetic stroke patients. M...

journal_title：Annals of neurology

authors： Wang LE,Fink GR,Diekhoff S,Rehme AK,Eickhoff SB,Grefkes C

更新日期：2011-02-01 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：:Apolipoprotein E has been implicated in modifying neurological outcome after traumatic brain injury, although the mechanisms by which this occurs remain poorly defined. To investigate the role of endogenous apolipoprotein E following acute brain injury, noninvasive magnetic resonance imaging was performed on anestheti...

journal_title：Annals of neurology

authors： Lynch JR,Pineda JA,Morgan D,Zhang L,Warner DS,Benveniste H,Laskowitz DT

更新日期：2002-01-01 00:00:00

abstract：:Autopsy examination confirmed the diagnosis of subacute necrotizing encephalomyelopathy (SNE) in a 7-month-old male infant who underwent several metabolic studies before death. Intermittent lactic acidemia and fumaric aciduria, an extreme hyperglycemic response to an intravenous bolus of alanine, and an elevated total...

journal_title：Annals of neurology

authors： DeVivo DC,Haymond MW,Obert KA,Nelson JS,Pagliara AS

更新日期：1979-12-01 00:00:00

abstract：:Various C-terminally truncated amyloid beta peptides (Abeta) are linked to Alzheimer's disease (AD) pathogenesis. Cerebrospinal fluid (CSF) concentrations of Abeta38, Abeta40, and Abeta42 were measured by enzyme-linked immunosorbent assay in 30 patients with AD and 26 control subjects. CSF Abeta42 levels was decreased...

journal_title：Annals of neurology

authors： Schoonenboom NS,Mulder C,Van Kamp GJ,Mehta SP,Scheltens P,Blankenstein MA,Mehta PD

更新日期：2005-07-01 00:00:00

abstract：:Using placebo data from 3 randomized multiple sclerosis (MS) trials with uniform inclusion criteria, we investigated heterogeneity of Expanded Disability Status Scale (EDSS) progression by geographical areas. Our analysis revealed a significantly lower EDSS progression in Eastern European countries (10.8%) compared wi...

journal_title：Annals of neurology

authors： Bovis F,Signori A,Carmisciano L,Maietta I,Steinerman JR,Li T,Tansy AP,Sormani MP

更新日期：2018-10-01 00:00:00

abstract：:A prospective clinical and biochemical study on the effects of treatment with haloperidol has been performed in seven patients with Tourette syndrome. Pretreatment cerebrospinal fluid levels of homovanillic acid (CSF HVA) were significantly reduce in all patients, whereas 5-hydroxyindoleacetic acid was reduced in only...

journal_title：Annals of neurology

authors： Singer HS,Butler IJ,Tune LE,Seifert WE Jr,Coyle JT

更新日期：1982-10-01 00:00:00

abstract：:More than a decade ago myasthenic symptoms were observed in rabbits immunized with acetylcholine receptor (AChR) [119] and AChR deficiency was found at the neuromuscular junction in human myasthenia gravis (MG) [36]. By 1977 the autoimmune character of MG and the pathogenic role of AChR antibodies had been established...

journal_title：Annals of neurology

authors： Engel AG

更新日期：1984-11-01 00:00:00

abstract：:Seizures in temporal lobe epilepsy can be classified as hypersynchronous and low-voltage fast according to their onset patterns. Experimental evidence suggests that low-voltage fast-onset seizures mainly result from the synchronous activity of γ-aminobutyric acid-releasing cells. In this study, we tested this hypothes...

journal_title：Annals of neurology

authors： Shiri Z,Manseau F,Lévesque M,Williams S,Avoli M

更新日期：2015-03-01 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