Adult-onset leukoencephalopathy with vanishing white matter with a missense mutation in EIF2B5.

abstract：:The relevance of neutralizing antibodies (NAb) to interferon-beta (IFNbeta) for patients with MS treated with IFNbeta injections has been controversial. Published data from several double-blind, randomized clinical trials demonstrate that disease activity in IFNbeta-treated patients with MS is similar to placebo-treat...

journal_title：Neurology

authors： Rudick RA

更新日期：2003-11-11 00:00:00

abstract：:To identify clinical and electrophysiologic features related to IV immunoglobulin (IVIg) responsiveness in chronic inflammatory demyelinating polyneuropathy (CIDP), the authors conducted a multicenter study on 312 patients with CIDP (199 responders and 113 nonresponders). Muscle atrophy and decreased compound muscle a...

journal_title：Neurology

authors： Iijima M,Yamamoto M,Hirayama M,Tanaka F,Katsuno M,Mori K,Koike H,Hattori N,Arimura K,Nakagawa M,Yoshikawa H,Hayasaka K,Onodera O,Baba M,Yasuda H,Saito T,Nakazato M,Nakashima K,Kira J,Kaji R,Oka N,Sobue G

更新日期：2005-04-26 00:00:00

abstract：:We evaluated cognitive outcome in a group of 37 patients who had undergone surgery for rupture and repair of a single intracranial aneurysm at least 6 months previously. We compared outcome--assessed by tests of intelligence, attention, executive functions sensitive to frontal lobe lesions, memory, neglect, and mood, ...

journal_title：Neurology

authors： Tidswell P,Dias PS,Sagar HJ,Mayes AR,Battersby RD

更新日期：1995-05-01 00:00:00

abstract：:A randomized trial was completed to assess the feasibility of a 2-year education rehabilitation program for students with sickle cell disease and memory deficits. Eleven students were assigned to tutoring with or without memory training for 2 years. Eighty-two percent completed the program. Evidence of improvement in ...

journal_title：Neurology

authors： King AA,White DA,McKinstry RC,Noetzel M,Debaun MR

更新日期：2007-06-05 00:00:00

abstract：:The intensive study of seizures via serial 24-hour EEG-video monitoring has allowed enhanced observation of sleep patterns among epilepsy patients and has revealed that disturbed rest is common in this population. Previous work has shown that sleep deprivation of any type can exacerbate seizure activity, leading to sp...

journal_title：Neurology

authors： Sammaritano M,Sherwin A

更新日期：2000-01-01 00:00:00

abstract：:We enrolled 116 patients in a multicenter, randomized, double-blind controlled trial of an 8-week course of 11 plasma exchange (PE) treatments in exacerbations of MS. The control group received sham PE, and both groups received identical treatment with IM ACTH and oral cyclophosphamide. Serum IgG decreased in the PE a...

journal_title：Neurology

authors： Weiner HL,Dau PC,Khatri BO,Petajan JH,Birnbaum G,McQuillen MP,Fosburg MT,Feldstein M,Orav EJ

更新日期：1989-09-01 00:00:00

abstract：OBJECTIVE:To use a combined neurogenetic-neuroimaging approach to examine the functional consequences of preclinical dopaminergic nigrostriatal dysfunction in the human motor system. Specifically, we examined how a single heterozygous mutation in different genes associated with recessively inherited Parkinson disease a...

journal_title：Neurology

authors： van Nuenen BF,Weiss MM,Bloem BR,Reetz K,van Eimeren T,Lohmann K,Hagenah J,Pramstaller PP,Binkofski F,Klein C,Siebner HR

更新日期：2009-03-24 00:00:00

abstract：:Four patients with disabling action tremor in the setting of MS were treated with isoniazid (800 to 1200 mg per day). All patients showed significant improvement of the tremor, allowing more functional use of their extremities. To the best of our knowledge, this is the only medical treatment for this type of tremor. ...

journal_title：Neurology

authors： Sabra AF,Hallett M,Sudarsky L,Mullally W

更新日期：1982-08-01 00:00:00

abstract：:Lyme borreliosis (LB) causes a range of neurologic manifestations, the most common of which is facial nerve paralysis. To evaluate nervous system LB, we organized a neurologic collaborative study group in Suffolk County, NY, a region of high LB incidence. Between July and September 1989, LB serologies were performed o...

journal_title：Neurology

authors： Halperin JJ,Golightly M

更新日期：1992-07-01 00:00:00

abstract：:Concentrations of cyclic adenosine 3',5' monophosphate (cAMP) were significantly lower in parkinsonian patients than in controls, but concentrations of guanosine 3',5' monophosphate (cGMP) were not altered. Both cAMP and cGMP levels were lower in patients with more severe symptoms on the left side of the body. Somatos...

journal_title：Neurology

authors： Volicer L,Beal MF,Direnfeld LK,Marquis JK,Albert ML

更新日期：1986-01-01 00:00:00

abstract：:Juvenile myoclonic epilepsy (JME) is a distinct subform of idiopathic generalized epilepsy of adolescence. Linkage studies with Bf and serologic HLA markers in families of JME patients have shown a tight linkage on chromosome 6. We present a linkage analysis with HLA-DQ restriction fragment length polymorphisms on mor...

journal_title：Neurology

authors： Durner M,Sander T,Greenberg DA,Johnson K,Beck-Mannagetta G,Janz D

更新日期：1991-10-01 00:00:00

abstract：OBJECTIVE:To study the attributes of quantitative strength testing using hand-held dynamometry (HHD) as an efficacy measure in 2 large phase 3 amyotrophic lateral sclerosis (ALS) trials. METHODS:In the phase 3 trials of ceftriaxone and dexpramipexole, 513 and 943 patients, respectively, were enrolled in double-blind, ...

journal_title：Neurology

authors： Shefner JM,Liu D,Leitner ML,Schoenfeld D,Johns DR,Ferguson T,Cudkowicz M

更新日期：2016-08-09 00:00:00

abstract：OBJECTIVES:To determine the relative utility of 1H MRSI and hippocampal volumetry for the lateralization of mesial temporal lobe epilepsy (MTLE) in patients with intractable epilepsy. BACKGROUND:MTLE is the most common partial-onset seizure disorder in patients undergoing temporal lobe epilepsy surgery. MR volumetry a...

journal_title：Neurology

authors： Kuzniecky R,Hugg JW,Hetherington H,Butterworth E,Bilir E,Faught E,Gilliam F

更新日期：1998-07-01 00:00:00

abstract：BACKGROUND:Both genetic and environmental factors play a role in the pathophysiology of MS and may influence the clinical expression of the disease. OBJECTIVE:To determine the contribution of familial factors to the clinical expression of MS. METHODS:The French Multiple Sclerosis Genetics Group identified 87 sibling ...

journal_title：Neurology

authors： Brassat D,Azais-Vuillemin C,Yaouanq J,Semana G,Reboul J,Cournu I,Mertens C,Edan G,Lyon-Caen O,Clanet M,Fontaine B

更新日期：1999-05-12 00:00:00

abstract：:We tested the hypothesis that an anticardiolipin antibody (aCL) titer of > or = 10 IgG phospholipid (GPL) at the time of an index ischemic stroke is associated with an increased risk of subsequent thrombo-occlusive events or death. First-time ischemic stroke patients from the Antiphospholipid Antibodies and Stroke Stu...

journal_title：Neurology

authors：

更新日期：1997-01-01 00:00:00

abstract：:Using transcranial Doppler (TCD) ultrasonography in patients with high-grade (> or = 70%) internal carotid artery (ICA) stenosis, we examined the relation between the rate of TCD-detected silent microembolism of the ipsilateral middle cerebral artery and a history of recent (< 121 days) ischemic symptoms attributable ...

journal_title：Neurology

authors： Siebler M,Kleinschmidt A,Sitzer M,Steinmetz H,Freund HJ

更新日期：1994-04-01 00:00:00

abstract：:An autoimmune reaction to the endplate acetylcholine receptor (AChR) is thought to be responsible for the muscular weakness of myasthenia gravis (MG). However, the significance of antithymic antibodies and the thymic AChR-like protein is still uncertain. We transferred immunoglobulins (Igs) from patients with MG to mi...

journal_title：Neurology

authors： Dalkara T,Onur R,Ruacan SA,Topaktaş S

更新日期：1983-10-01 00:00:00

abstract：OBJECTIVE:Recent publications show that using imagery instructions, brain activation patterns indicating consciousness can be found in approximately 10% of patients with unresponsive wakefulness syndrome (UWS; previously called vegetative state). It is possible, however, that patients who cannot follow instructions (be...

journal_title：Neurology

authors： Yu T,Lang S,Vogel D,Markl A,Müller F,Kotchoubey B

更新日期：2013-01-22 00:00:00

abstract：:In this study, the 24-hour pattern of blood pressure (BP), heart rate, and urinary catecholamine (CA) excretion and the response of BP and plasma CA to the tilt test have been investigated in 13 untreated patients affected by Parkinson's disease (PD) and in 11 age-matched healthy controls. Seven of the 13 PD subjects ...

journal_title：Neurology

authors： Micieli G,Martignoni E,Cavallini A,Sandrini G,Nappi G

更新日期：1987-03-01 00:00:00

abstract：OBJECTIVE:To determine whether changes in cerebral structure are present after preeclampsia that may explain increased cerebrovascular risk in these women. METHODS:We conducted a case control study in women between 5 and 15 years after either a preeclamptic or normotensive pregnancy. Brain MRI was performed. Analysis ...

journal_title：Neurology

authors： Siepmann T,Boardman H,Bilderbeck A,Griffanti L,Kenworthy Y,Zwager C,McKean D,Francis J,Neubauer S,Yu GZ,Lewandowski AJ,Sverrisdottir YB,Leeson P

更新日期：2017-03-28 00:00:00

abstract：OBJECTIVE:To determine whether the cytokine tumor necrosis factor alpha (TNF-alpha) acts as a pain mediator in neuropathic pain in humans. BACKGROUND:In animal models, inflammatory cytokines such as TNF-alpha have been shown to facilitate neuropathic pain. METHODS:The expression of TNF-alpha was analyzed immunohistoc...

journal_title：Neurology

authors： Empl M,Renaud S,Erne B,Fuhr P,Straube A,Schaeren-Wiemers N,Steck AJ

更新日期：2001-05-22 00:00:00

abstract：OBJECTIVE:Unilateral temporoparietal injury may result in an attentional deficit for stimuli presented in contralesional space. Thus, bilateral temporoparietal degeneration associated with Alzheimer disease (AD) might result in a bilateral attentional disturbance. Tests for hemispatial neglect, however, primarily asses...

journal_title：Neurology

authors： Kim EJ,Lee BH,Seo SW,Moon SY,Jung DS,Park KH,Heilman KM,Na DL

更新日期：2007-09-11 00:00:00

abstract：:Primary deficiency of merosin is the cause of the classic form of congenital muscular dystrophy (CMD) accompanied by brain white matter abnormalities. We report a female infant with dystrophinopathy who was deficient in merosin in skeletal muscle. The patient had a phenotype of typical CMD and white matter abnormaliti...

journal_title：Neurology

authors： Tachi N,Ohya K,Chiba S,Matsuo M,Patria SY,Matsumura K

更新日期：1997-08-01 00:00:00

abstract：:With the use of the newer antidepressants beyond the traditional tricyclics and monoamine oxidase inhibitors, newer options in headache prophylaxis are provided as well as the potential for undesirable and even potentially life-threatening interactions between medications. In this article, four patient reports of a sp...

journal_title：Neurology

authors： Diamond S,Pepper BJ,Diamond ML,Freitag FG,Urban GJ,Erdemoglu AK

更新日期：1998-07-01 00:00:00

abstract：BACKGROUND:Antiplatelets (APL), angiotensin-converting enzyme (ACE) inhibitors (ACEI), and statins (STAT) are commonly used for stroke prevention. The authors examined whether combination therapy with these agents has additive protective effects in reducing ischemic stroke severity. METHODS:The authors retrospectively...

journal_title：Neurology

authors： Kumar S,Savitz S,Schlaug G,Caplan L,Selim M

更新日期：2006-04-25 00:00:00

abstract：:Inhibitory antibody to von Willebrand factor (vWF)-cleaving protease (ADAMTS13) was detected in a patient with intravascular lymphomatosis. The increased serum level of the antibody paralleled an increase in the expression of uncleaved vWF, which might cause microvascular thrombosis and platelet consumption. Malignant...

journal_title：Neurology

authors： Kawahara M,Kanno M,Matsumoto M,Nakamura S,Fujimura Y,Ueno S

更新日期：2004-11-09 00:00:00

abstract：:Juvenile-onset dystonia that improves after levodopa may occur in both dopa-responsive dystonia (DRD) and juvenile parkinsonism (JP), clinically similar conditions with different prognoses and management goals. The authors show normal striatal uptake of the dopamine transporter ligand FP-CIT with SPECT in a clinically...

journal_title：Neurology

authors： O'Sullivan JD,Costa DC,Gacinovic S,Lees AJ

更新日期：2001-01-23 00:00:00

abstract：:First-degree relatives of narcoleptic subjects (probands) may have sleep pathology related to the transmission of the disorder through their family members. The authors examined four groups: probands (n = 96), first-degree relative (n = 337), environmental reference (n = 85), and general population (n = 6,694) groups....

journal_title：Neurology

authors： Ohayon MM,Okun ML

更新日期：2006-08-22 00:00:00

abstract：:The P3 component of the auditory evoked potential has been shown to reflect "endogenous" processes related to cognition. This component was measured serially in seven patients who had confusional states or dementing illnesses that fluctuated in severity over time. The latency of P3 component reflected the clinical imp...

journal_title：Neurology

authors： Goodin DS,Starr A,Chippendale T,Squires KC

更新日期：1983-09-01 00:00:00

abstract：:Erythrocyte calcium transport as a function of free [Ca++] was examined using inside-out vesicles from eight pairs of patients with Duchenne dystrophy and age- and sex-matched controls. The Km for Ca++ of the transport protein and the Vmax were higher in the Duchenne vesicles in seven of the eight pairs. The mean Km a...

journal_title：Neurology

authors： Mollman JE,Cardenas JC,Pleasure DE

更新日期：1980-11-01 00:00:00