Ventilatory response in myotonic dystrophy.

abstract：:To study the incidence of abnormalities of the pyruvate (PDH) or ketoglutarate (KGDH) dehydrogenase complexes in patients with spinocerebellar degenerations, we measured the activities of PDH and KGDH in platelet-enriched preparations from the blood of 14 patients. Low PDH was found in 6 of the 14 patients; low KGDH w...

journal_title：Neurology

authors： Kark RA,Rodriguez-Budelli M

更新日期：1979-01-01 00:00:00

abstract：OBJECTIVE:To investigate the relationship between cerebral amyloid angiopathy (CAA), dementia, and cognitive function in an autopsy sample of 211 Japanese-American men from the population-based Honolulu-Asia Aging Study. METHODS:Starting in 1991, participants were assessed with the Cognitive Abilities Screening Instru...

journal_title：Neurology

authors： Pfeifer LA,White LR,Ross GW,Petrovitch H,Launer LJ

更新日期：2002-06-11 00:00:00

abstract：:Nine neurosurgical patients suffering from a variety of brain tumors were studied. Sodium valproate was given for 3 days before surgery. At craniotomy, sections of brain and samples of cerebrospinal fluid (CSF) and plasma were obtained and assayed for sodium valproate concentrations. Brain levels were 6.8 to 27.9% of ...

journal_title：Neurology

authors： Vajda FJ,Donnan GA,Phillips J,Bladin PF

更新日期：1981-04-01 00:00:00

abstract：OBJECTIVE:To examine the relationships between age at onset and duration of seizure disorder with severity of hippocampal sclerosis (HS) and cognitive functioning in patients with HS and unilateral temporal lobe epilepsy. METHODS:Twenty-six subjects had left temporal lobe seizure onset; 20 had right temporal onset. Me...

journal_title：Neurology

authors： Fuerst D,Shah J,Kupsky WJ,Johnson R,Shah A,Hayman-Abello B,Ergh T,Poore Q,Canady A,Watson C

更新日期：2001-07-24 00:00:00

abstract：OBJECTIVE:To identify neural sites associated with gaze-evoked tinnitus (GET), an unusual condition that may follow cerebellar-pontine angle surgery. METHODS:The authors examined eight patients with GET and used PET to map the neural sites activated by lateral gaze in them and seven age- and sex-matched control subjec...

journal_title：Neurology

authors： Lockwood AH,Wack DS,Burkard RF,Coad ML,Reyes SA,Arnold SA,Salvi RJ

更新日期：2001-02-27 00:00:00

abstract：:All but 6% of the subjects with relapsing remitting MS who were randomly assigned to receive glatiramer acetate or placebo for the 9-month controlled phase of the European/Canadian MRI trial entered an open-label extension with quarterly clinical and MRI evaluations for another 9 months. There was a 54% reduction in t...

journal_title：Neurology

authors： Wolinsky JS,Comi G,Filippi M,Ladkani D,Kadosh S,Shifroni G,European\/Canadian Glatiramer Acetate Study Group.

更新日期：2002-10-22 00:00:00

abstract：:Migraine does not increase the risk for complications of pregnancy for the mother or for the fetus: the incidences of toxemia, miscarriages, abnormal labour, congenital anomalies, and stillbirths are comparable to those of the general population. Several retrospective studies have shown a tendency for migraine to impr...

journal_title：Neurology

authors： Aubé M

更新日期：1999-01-01 00:00:00

abstract：OBJECTIVE:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary arteriopathy caused by mutations of the Notch3 gene. The disease is characterized by T2-hyperintense lesions (subcortical white matter lesions), T1-hypointense lesions (lacunar lesions), and T2...

journal_title：Neurology

authors： Viswanathan A,Gschwendtner A,Guichard JP,Buffon F,Cumurciuc R,O'Sullivan M,Holtmannspötter M,Pachai C,Bousser MG,Dichgans M,Chabriat H

更新日期：2007-07-10 00:00:00

abstract：OBJECTIVE:To report our experience delivering intrathecal nusinersen through cervical puncture in patients with spinal muscular atrophy (SMA) with no lumbar access. BACKGROUND:SMA is a neuromuscular disorder characterized by profound muscle weakness, atrophy, and paralysis due to degeneration of the anterior horn cell...

journal_title：Neurology

authors： Veerapandiyan A,Pal R,D'Ambrosio S,Young I,Eichinger K,Collins E,Westesson PL,Kwon J,Ciafaloni E

更新日期：2018-08-14 00:00:00

abstract：:In 2001, the authors described the clinical features of a genetically distinct autosomal dominant limb-girdle muscular dystrophy (LGMD; LGMD 1F). Using a genome-wide screen with more than 400 microsatellite markers, the authors identified a novel LGMD disease locus at chromosome 7q32.1-32.2. Within this chromosomal re...

journal_title：Neurology

authors： Palenzuela L,Andreu AL,Gàmez J,Vilà MR,Kunimatsu T,Meseguer A,Cervera C,Fernandez Cadenas I,van der Ven PF,Nygaard TG,Bonilla E,Hirano M

更新日期：2003-08-12 00:00:00

abstract：BACKGROUND:An association between primary hyperparathyroidism (PHP) and amyotrophic lateral sclerosis (ALS) has been noted; however, a causal relation between these disorders has not been confirmed. PATIENTS/METHODS:We report five patients (three men, two women) meeting El Escorial criteria for ALS who also had PHP. I...

journal_title：Neurology

authors： Jackson CE,Amato AA,Bryan WW,Wolfe GI,Sakhaee K,Barohn RJ

更新日期：1998-06-01 00:00:00

abstract：OBJECTIVE:This study explored the association between long-term epilepsy surgery outcome and changes in depressive symptoms. METHODS:Adults were enrolled between 1996 and 2001 in a multicenter prospective study to evaluate outcomes of resective epilepsy surgery. The extent of depressive symptoms and depression case st...

journal_title：Neurology

authors： Hamid H,Liu H,Cong X,Devinsky O,Berg AT,Vickrey BG,Sperling MR,Shinnar S,Langfitt JT,Walczak TS,Barr WB,Dziura J,Bazil CW,Spencer SS

更新日期：2011-11-29 00:00:00

abstract：BACKGROUND:Structural abnormalities were detected in bilateral primary sensorimotor areas in writer's cramp. Evidence in other primary dystonia, including blepharospasm and cervical dystonia, suggest that structural abnormalities may be observed in other brain areas such as the cerebellum in writer's cramp. OBJECTIVE:...

journal_title：Neurology

authors： Delmaire C,Vidailhet M,Elbaz A,Bourdain F,Bleton JP,Sangla S,Meunier S,Terrier A,Lehéricy S

更新日期：2007-07-24 00:00:00

abstract：OBJECTIVE:This prospective, randomized, controlled study was designed to investigate the safety, feasibility, and preliminary efficacy of long-term CSF drainage via a low-flow ventriculoperitoneal shunt in subjects suffering from AD. METHODS:Twenty-nine subjects selected for probable AD (National Institute of Neurolog...

journal_title：Neurology

authors： Silverberg GD,Levinthal E,Sullivan EV,Bloch DA,Chang SD,Leverenz J,Flitman S,Winn R,Marciano F,Saul T,Huhn S,Mayo M,McGuire D

更新日期：2002-10-22 00:00:00

abstract：BACKGROUND:Cerebral infarctions are common in older persons but their relationship with dementia and cognitive function remains controversial. METHODS:Participants were 164 older Catholic nuns, priests, and brothers who underwent annual clinical evaluation and brain autopsy at death. The authors quantified number and ...

journal_title：Neurology

authors： Schneider JA,Wilson RS,Cochran EJ,Bienias JL,Arnold SE,Evans DA,Bennett DA

更新日期：2003-04-08 00:00:00

abstract：OBJECTIVE:To study the long-term dual inhibitory effects of rivastigmine on acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE) in patients with AD. METHODS:Eleven patients with mild AD received rivastigmine for 12 months. Cholinesterase (ChE) activities in the CSF and plasma were assessed colorimetrically. ...

journal_title：Neurology

authors： Darreh-Shori T,Almkvist O,Guan ZZ,Garlind A,Strandberg B,Svensson AL,Soreq H,Hellström-Lindahl E,Nordberg A

更新日期：2002-08-27 00:00:00

abstract：:A 64-year-old man with diffuse atherosclerosis developed an organic mental syndrome, followed by hypothalamic symptoms. Autopsy showed an old large cystic infarct involving the left temporal lobe including the hippocampus, and atrophy of the ipsilateral fornix, mammillary body, hypothalamus, mammillothalamic tract, an...

journal_title：Neurology

authors： Torch WC,Hirano A,Solomon S

更新日期：1977-12-01 00:00:00

abstract：:Automatisms are commonly seen in epilepsy, either ictally or postictally. However, most automatisms are simple, with hand movements, mouth smacking, nose-rubbing, repetition of a single word, or coughing, grunting, or screeching. Complex automatisms are less common and striking. The authors report two cases of seizure...

journal_title：Neurology

authors： Doherty MJ,Wilensky AJ,Holmes MD,Lewis DH,Rae J,Cohn GH

更新日期：2002-11-12 00:00:00

abstract：:A specific form of deep cerebral hemisphere infarction was identified in 11 of 1,600 stroke register patients. Despite the CT finding of a deep striatocapsular lesion, these patients showed evidence of both cortical and capsular abnormalities. Angiography showed significant proximal carotid artery lesions as well as e...

journal_title：Neurology

authors： Bladin PF,Berkovic SF

更新日期：1984-11-01 00:00:00

abstract：:We used [18F]-2-fluoro-2-deoxy-D-glucose (FDG) and PET to study regional cerebral glucose utilization in seven patients with fatal familial insomnia (FFI), an inherited prion disease with a mutation at codon 178 of the prion protein gene. Four patients were methionine/methionine homozygotes at codon 129 (symptom durat...

journal_title：Neurology

authors： Cortelli P,Perani D,Parchi P,Grassi F,Montagna P,De Martin M,Castellani R,Tinuper P,Gambetti P,Lugaresi E,Fazio F

更新日期：1997-07-01 00:00:00

abstract：OBJECTIVE:To assess whether genetic subgroups in recent amyotrophic lateral sclerosis (ALS) trials responded to treatment with lithium carbonate, but that the treatment effect was lost in a large cohort of nonresponders. METHODS:Individual participant data were obtained from 3 randomized trials investigating the effic...

journal_title：Neurology

authors： van Eijk RPA,Jones AR,Sproviero W,Shatunov A,Shaw PJ,Leigh PN,Young CA,Shaw CE,Mora G,Mandrioli J,Borghero G,Volanti P,Diekstra FP,van Rheenen W,Verstraete E,Eijkemans MJC,Veldink JH,Chio A,Al-Chalabi A,van den Berg

更新日期：2017-10-31 00:00:00

abstract：:Recent positive trials have thrust acute cerebral perfusion imaging into the routine evaluation of acute ischemic stroke. Updated guidelines state that in patients with anterior circulation large vessel occlusions presenting beyond 6 hours from time last known well, advanced imaging selection including perfusion-based...

journal_title：Neurology

authors： Vagal A,Wintermark M,Nael K,Bivard A,Parsons M,Grossman AW,Khatri P

更新日期：2019-11-12 00:00:00

abstract：:We retrospectively evaluated the neurologic complications in 425 patients who underwent bone marrow transplant (BMT) (310 allogeneic, 115 autologous) for leukemia. Forty-six patients (11%) developed 47 central and three peripheral neurologic complications. The most common complications were cerebral hemorrhage (3.8%),...

journal_title：Neurology

authors： Graus F,Saiz A,Sierra J,Arbaiza D,Rovira M,Carreras E,Tolosa E,Rozman C

更新日期：1996-04-01 00:00:00

abstract：:Malformations of cortical development (MCD) account for a high proportion of medically resistant partial seizures in children and figure prominently in pediatric surgical series. In contrast to the results of epilepsy surgery for postnatally acquired lesions, seizure freedom in patients with MCD is less certain owing ...

journal_title：Neurology

authors： Duchowny M,Jayakar P,Levin B

更新日期：2000-08-08 00:00:00

abstract：OBJECTIVE:To test efficacy and safety of atorvastatin in subjects with clinically isolated syndrome (CIS). METHODS:Subjects with CIS were enrolled in a phase II, double-blind, placebo-controlled, 14-center randomized trial testing 80 mg atorvastatin on clinical and brain MRI activity. Brain MRIs were performed quarter...

journal_title：Neurology

authors： Waubant E,Pelletier D,Mass M,Cohen JA,Kita M,Cross A,Bar-Or A,Vollmer T,Racke M,Stüve O,Schwid S,Goodman A,Kachuck N,Preiningerova J,Weinstock-Guttman B,Calabresi PA,Miller A,Mokhtarani M,Iklé D,Murphy S,Kopetskie

更新日期：2012-04-10 00:00:00

abstract：OBJECTIVE:To assess the determinants of amyotrophic lateral sclerosis (ALS) phenotypes in a population-based cohort. METHODS:The study population included 2,839 patients with ALS diagnosed in Piemonte, Italy (1995-2015). Patients were classified according to motor (classic, bulbar, flail arm, flail leg, predominantly ...

journal_title：Neurology

authors： Chiò A,Moglia C,Canosa A,Manera U,D'Ovidio F,Vasta R,Grassano M,Brunetti M,Barberis M,Corrado L,D'Alfonso S,Iazzolino B,Peotta L,Sarnelli MF,Solara V,Zucchetti JP,De Marchi F,Mazzini L,Mora G,Calvo A

更新日期：2020-02-25 00:00:00

abstract：:The medical records of all children at our hospital with International Classification of Diseases 9th revision (ICD-9) codes 342, 433 to 438, or 767 from May 1999 to May 2004 were reviewed to assess whether they had stroke (any type) or, specifically, arterial ischemic stroke (AIS). Code accuracy ranged from 37 to 88%...

journal_title：Neurology

authors： Golomb MR,Garg BP,Saha C,Williams LS

更新日期：2006-12-12 00:00:00

abstract：OBJECTIVE:To determine the usefulness of the 14-3-3 test in patients with dementia of various causes. BACKGROUND:Recent reports have suggested that the detection of the 14-3-3 protein in the CSF of patients with Creutzfeldt--Jakob disease is a highly sensitive and specific marker of the disease that might be used as a...

journal_title：Neurology

authors： Burkhard PR,Sanchez JC,Landis T,Hochstrasser DF

更新日期：2001-06-12 00:00:00

abstract：OBJECTIVE:To determine the effect of erenumab, a human monoclonal antibody targeting the calcitonin gene-related peptide receptor, on health-related quality of life (HRQoL), headache impact, and disability in patients with chronic migraine (CM). METHODS:In this double-blind, placebo-controlled study, 667 adults with C...

journal_title：Neurology

authors： Lipton RB,Tepper SJ,Reuter U,Silberstein S,Stewart WF,Nilsen J,Leonardi DK,Desai P,Cheng S,Mikol DD,Lenz R

更新日期：2019-05-07 00:00:00

abstract：OBJECTIVE:To examine the relationship between carotid artery stenosis, other risk factors, and lacunar stroke. BACKGROUND:Carotid artery stenosis in patients presenting with lacune stroke may be coincidental or causal. The distinction by risk factor profile is uncertain. The risk and cause of subsequent stroke, and be...

journal_title：Neurology

authors： Inzitari D,Eliasziw M,Sharpe BL,Fox AJ,Barnett HJ

更新日期：2000-02-08 00:00:00