Myoclonus-dystonia: clinical and electrophysiologic pattern related to SGCE mutations.

abstract：:Resources of the Rochester Epidemiology Project were used to estimate prevalence rates for dementia on January 1, 1975, 1980, and 1985, and to evaluate trends over time. Prevalence rates increased with age for both men and women. There was no difference in the prevalence rates for January 1, 1975, and January 1, 1980,...

journal_title：Neurology

authors： Beard CM,Kokmen E,O'Brien PC,Kurland LT

更新日期：1995-01-01 00:00:00

abstract：OBJECTIVE:To characterize a kindred with a distinctive autosomal dominant neuromuscular disorder. BACKGROUND:The authors studied a large Italian family affected by a progressive neuromyopathy. Ten individuals over three generations were affected. The disease was characterized by onset from the late teens to early 50s ...

journal_title：Neurology

authors： Servidei S,Capon F,Spinazzola A,Mirabella M,Semprini S,de Rosa G,Gennarelli M,Sangiuolo F,Ricci E,Mohrenweiser HW,Dallapiccola B,Tonali P,Novelli G

更新日期：1999-09-11 00:00:00

abstract：:We studied 30 necropsy cases of vascular dementia with a lacunar state. Manifestations included dementia, lack of volition, emotional lability, small-stepped gait, dysarthria, urinary incontinence, grasp reflex, pyramidal signs, paraplegia in flexion, and akinetic mutism. Pathologically, there was diffuse incomplete s...

journal_title：Neurology

authors： Ishii N,Nishihara Y,Imamura T

更新日期：1986-03-01 00:00:00

abstract：OBJECTIVE:To estimate whole-brain microinfarct burden from microinfarct counts in routine postmortem examination. METHODS:We developed a simple mathematical method to estimate the total number of cerebral microinfarcts from counts obtained in the small amount of tissue routinely examined in brain autopsies. We derived...

journal_title：Neurology

authors： Westover MB,Bianchi MT,Yang C,Schneider JA,Greenberg SM

更新日期：2013-04-09 00:00:00

abstract：BACKGROUND:Tricyclic antidepressants (TCA) are often used in the treatment of painful polyneuropathy. Venlafaxine is a serotonin and weak noradrenaline reuptake inhibitor antidepressant with a different profile of other pharmacologic actions from those of TCA. OBJECTIVE:To test if venlafaxine would relieve painful pol...

journal_title：Neurology

authors： Sindrup SH,Bach FW,Madsen C,Gram LF,Jensen TS

更新日期：2003-04-22 00:00:00

abstract：:A model of latent infection by varicella-zoster virus (VZV) was obtained in the adult rat. Inoculation of VZV-infected cells in the skin led to infection of the peripheral nervous system. Latency was characterized by a long-lasting presence of the viral genome, of selected viral gene transcripts, and of at least one v...

journal_title：Neurology

authors： Sadzot-Delvaux C,Debrus S,Nikkels A,Piette J,Rentier B

更新日期：1995-12-01 00:00:00

abstract：:Although psychological distress and cognitive dysfunction are well documented in adults with multiple sclerosis (MS), they are poorly understood in children with the disease. Psychosocial difficulty experienced by children and adolescents with MS involves factors common to all chronic illnesses in children, as well as...

journal_title：Neurology

authors： MacAllister WS,Boyd JR,Holland NJ,Milazzo MC,Krupp LB,International Pediatric MS Study Group.

更新日期：2007-04-17 00:00:00

abstract：:The authors studied the association of an exon 4 (E4*epsilon2/3/4) and three promoter polymorphisms of APOE with disease course and severity stratified by gender in 221 patients with multiple sclerosis from two overlapping population-based prevalence cohorts. Women carriers of the E4*epsilon2 allele took longer to att...

journal_title：Neurology

authors： Kantarci OH,Hebrink DD,Achenbach SJ,Pittock SJ,Altintas A,Schaefer-Klein JL,Atkinson EJ,De Andrade M,McMurray CT,Rodriguez M,Weinshenker BG

更新日期：2004-03-09 00:00:00

abstract：:A 47-year-old woman had myasthenia gravis with only moderate weakness and a consistently poor response to anticholinesterase medication. She was investigated with routine electrophysiologic studies and single-fiber electromyography. The studies showed a limited effect of anticholinesterase medication. There was a decr...

journal_title：Neurology

authors： Schwartz MS,Stålberg E

更新日期：1975-01-01 00:00:00

abstract：OBJECTIVE:To perform an open-label, long-term efficacy and safety/tolerability study of vagus nerve stimulation (VNS) of 454 patients with refractory epilepsy. METHODS:Subjects were enrolled from five clinical trials of VNS between 1988 and 1995 after undergoing an implantation of a pulse generator in the chest and a ...

journal_title：Neurology

authors： Morris GL 3rd,Mueller WM

更新日期：1999-11-10 00:00:00

abstract：:Of 53 patients with drug-induced seizures seen in the last decade, 45% had single seizures, 40% had multiple convulsions, and 15% had status epilepticus. Generalized seizures with focal features were common, but simple partial (motor) seizures occurred in only two patients. Isoniazid, insulin, lidocaine, and psychotro...

journal_title：Neurology

authors： Messing RO,Closson RG,Simon RP

更新日期：1984-12-01 00:00:00

abstract：OBJECTIVE:To investigate the effect of age, sex, APOE4 genotype, and lifestyle enrichment (education/occupation, midlife cognitive activity, and midlife physical activity) on Alzheimer disease (AD) biomarker trajectories using longitudinal imaging data (brain β-amyloid load via Pittsburgh compound B PET and neurodegene...

journal_title：Neurology

authors： Vemuri P,Lesnick TG,Przybelski SA,Knopman DS,Machulda M,Lowe VJ,Mielke MM,Roberts RO,Gunter JL,Senjem ML,Geda YE,Rocca WA,Petersen RC,Jack CR Jr

更新日期：2016-03-22 00:00:00

abstract：BACKGROUND:Hypothalamic hamartomas (HHs), rare developmental abnormalities of the inferior hypothalamus, often cause refractory, symptomatic, mixed epilepsy, including gelastic seizures. We present 37 patients with HH who underwent transcortical transventricular endoscopic resection. METHODS:Between October 2003 and A...

journal_title：Neurology

authors： Ng YT,Rekate HL,Prenger EC,Wang NC,Chung SS,Feiz-Erfan I,Johnsonbaugh RE,Varland MR,Kerrigan JF

更新日期：2008-04-22 00:00:00

abstract：OBJECTIVE:3-Methylglutaconic aciduria type I is a rare inborn error of leucine catabolism. It is thought to present in childhood with nonspecific symptoms; it was even speculated to be a nondisease. The natural course of disease is unknown. METHODS:This is a study on 10 patients with 3-methylglutaconic aciduria type I...

journal_title：Neurology

authors： Wortmann SB,Kremer BH,Graham A,Willemsen MA,Loupatty FJ,Hogg SL,Engelke UF,Kluijtmans LA,Wanders RJ,Illsinger S,Wilcken B,Cruysberg JR,Das AM,Morava E,Wevers RA

更新日期：2010-09-21 00:00:00

abstract：:In multifocal motor neuropathy (MMN), little is known of the long-term effect of human immunoglobulin (HIG) infusions as a maintenance therapy. We report a patient who improved after an initial HIG infusion of 2 g/kg and continued to improve both clinically and electrophysiologically during maintenance HIG treatment c...

journal_title：Neurology

authors： Van den Berg LH,Franssen H,Wokke JH

更新日期：1995-05-01 00:00:00

abstract：:The authors interviewed 53 cluster headache patients who had used psilocybin or lysergic acid diethylamide (LSD) to treat their condition. Twenty-two of 26 psilocybin users reported that psilocybin aborted attacks; 25 of 48 psilocybin users and 7 of 8 LSD users reported cluster period termination; 18 of 19 psilocybin ...

journal_title：Neurology

authors： Sewell RA,Halpern JH,Pope HG Jr

更新日期：2006-06-27 00:00:00

abstract：BACKGROUND:Open clinical trials indicate that low doses of pergolide, a long-acting D1 and D2 dopamine agonist, lead to a reduction in the symptoms of restless legs syndrome (RLS) with subjective improvement in sleep quality. OBJECTIVE:To assess the therapeutic efficacy of pergolide in improving sleep and subjective m...

journal_title：Neurology

authors： Wetter TC,Stiasny K,Winkelmann J,Buhlinger A,Brandenburg U,Penzel T,Medori R,Rubin M,Oertel WH,Trenkwalder C

更新日期：1999-03-23 00:00:00

abstract：OBJECTIVE:Small-cell lung carcinoma (SCLC) and limbic encephalitis are recognized γ-aminobutyric acid-B receptor (GABABR) autoantibody accompaniments. We sought to determine in a diagnostic serology laboratory the frequency and accompaniments (neurologic, oncologic, and serologic) of GABABR-immunoglobulin G (IgG). MET...

journal_title：Neurology

authors： Jeffery OJ,Lennon VA,Pittock SJ,Gregory JK,Britton JW,McKeon A

更新日期：2013-09-03 00:00:00

abstract：OBJECTIVE:To describe the effects of the anti-tumor necrosis factor neutralizing antibody, infliximab, and the antiproliferative immunosuppressant, mycophenolate mofetil, in refractory neurosarcoidosis. METHODS:We treated patients with biopsy-proven sarcoidosis and CNS involvement, who had failed treatment with steroi...

journal_title：Neurology

authors： Moravan M,Segal BM

更新日期：2009-01-27 00:00:00

abstract：OBJECTIVE:To investigate whether sex and race differences exist in dementia diagnosis risk associated with traumatic brain injury (TBI) among older veterans. METHODS:Using Fine-Gray regression models, we investigated incident dementia diagnosis risk with TBI exposure by sex and race. RESULTS:After the exclusion of ba...

journal_title：Neurology

authors： Kornblith E,Peltz CB,Xia F,Plassman B,Novakovic-Apopain T,Yaffe K

更新日期：2020-09-29 00:00:00

abstract：:We have described four cases of medial plantar neuropathy that was confirmed by the near-nerve sensory nerve conduction study. The common compression site in medial plantar neuropathy is at the entrance to the fibromuscular tunnel behind the navicular tuberosity, distal to the tarsal tunnel, where Tinel's sign is loca...

journal_title：Neurology

authors： Oh SJ,Lee KW

更新日期：1987-08-01 00:00:00

abstract：:We studied 20 patients with an acute parietal stroke with hemisensory disturbances but no visual field deficit and no or only slight motor weakness, without thalamic involvement on CT or MRI and found three main sensory syndromes. (1) The pseudothalamic sensory syndrome consists of a faciobrachiocrural impairment of e...

journal_title：Neurology

authors： Bassetti C,Bogousslavsky J,Regli F

更新日期：1993-10-01 00:00:00

abstract：OBJECTIVES:To assess the association of established multiple sclerosis (MS) risk variants in 3,254 African Americans (1,162 cases and 2,092 controls). METHODS:Human leukocyte antigen (HLA)-DRB1, HLA-DQB1, and HLA-A alleles were typed by molecular techniques. Single nucleotide polymorphism (SNP) genotyping was conducte...

journal_title：Neurology

authors： Isobe N,Gourraud PA,Harbo HF,Caillier SJ,Santaniello A,Khankhanian P,Maiers M,Spellman S,Cereb N,Yang S,Pando MJ,Piccio L,Cross AH,De Jager PL,Cree BA,Hauser SL,Oksenberg JR

更新日期：2013-07-16 00:00:00

abstract：OBJECTIVE:To determine how memory-encoding tasks elicit functional perfusion change in subjects with amnestic mild cognitive impairment (aMCI). METHODS:Twelve subjects with aMCI and 14 age-matched cognitively normal (CN) subjects were recruited for this study. Arterial spin-labeling perfusion MRI (ASL-MRI) was employe...

journal_title：Neurology

authors： Xu G,Antuono PG,Jones J,Xu Y,Wu G,Ward D,Li SJ

更新日期：2007-10-23 00:00:00

abstract：OBJECTIVE:Parkinson disease (PD), a devastating neurodegenerative disorder, affects motor abilities and cognition as well. It is not clear whether the proapoptotic protein, Bid, is involved in tumor necrosis factor death receptor I (TNFRI)-mediated destructive signal transduction pathways such as cell dysfunction or ne...

journal_title：Neurology

authors： Jiang H,He P,Adler CH,Shill H,Beach TG,Li R,Shen Y

更新日期：2012-10-23 00:00:00

abstract：OBJECTIVE:To assess whether hippocampal sclerosis (HS) severity is mirrored at the level of large-scale networks. METHODS:We studied preoperative high-resolution anatomical and diffusion-weighted MRI of 44 temporal lobe epilepsy (TLE) patients with histopathologic diagnosis of HS (n = 25; TLE-HS) and isolated gliosis ...

journal_title：Neurology

authors： Bernhardt BC,Fadaie F,Liu M,Caldairou B,Gu S,Jefferies E,Smallwood J,Bassett DS,Bernasconi A,Bernasconi N

更新日期：2019-05-07 00:00:00

abstract：:We describe a case of West Nile virus (WNV) infection in an HIV-infected patient who presented with an isolated flaccid monoparesis of the right upper extremity. To our knowledge, this is the first reported case of flaccid paralysis caused by WNV infection in an HIV-infected patient. We then review the medical literat...

journal_title：Neurology

authors： Torno M,Vollmer M,Beck CK

更新日期：2007-02-13 00:00:00

abstract：OBJECTIVE:To identify the metabolic signature of the various levels of cognitive deficits in amyotrophic lateral sclerosis (ALS) using 18F-2-fluoro-2-deoxy-d-glucose-PET (18F-FDG-PET). METHODS:A total of 170 ALS cases consecutively enrolled at the ALS Center of Turin underwent brain 18F-FDG-PET and were classified as ...

journal_title：Neurology

authors： Canosa A,Pagani M,Cistaro A,Montuschi A,Iazzolino B,Fania P,Cammarosano S,Ilardi A,Moglia C,Calvo A,Chiò A

更新日期：2016-01-05 00:00:00

abstract：OBJECTIVE:Brain changes occurring early in HIV infection are not well characterized. The Chicago Early HIV Infection Study aimed to evaluate the presence and extent of structural brain alterations using quantitative MRI. METHODS:Forty-three HIV and 21 control subjects were enrolled. Mean length of infection was estima...

journal_title：Neurology

authors： Ragin AB,Du H,Ochs R,Wu Y,Sammet CL,Shoukry A,Epstein LG

更新日期：2012-12-11 00:00:00

abstract：:Permanent paralysis was induced in dogs by a 500 gram centimeter force injury on the spinal cord, and drug treatments were given 1 hour after injury and were continued for 3 days. Dogs were evaluated for 90 days. Isotonic saline or mannitol administration were ineffective in reversing the paralysis. In dogs receiving ...

journal_title：Neurology

authors： De La Torre JC,Johnson CM,Goode DJ,Mullan S

更新日期：1975-06-01 00:00:00