Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM.

abstract：OBJECTIVE:To evaluate the efficacy and tolerability of lamotrigine (LTG) for the treatment of pain in HIV-associated sensory neuropathies. METHODS:In a randomized, double-blind study, patients with HIV-associated distal sensory polyneuropathy (DSP) received LTG or placebo during a 7-week dose escalation phase followed...

journal_title：Neurology

authors： Simpson DM,McArthur JC,Olney R,Clifford D,So Y,Ross D,Baird BJ,Barrett P,Hammer AE,Lamotrigine HIV Neuropathy Study Team.

更新日期：2003-05-13 00:00:00

abstract：:We tested the efficacy of coenzyme Q10 (ubidecarenone, CoQ10) therapy in patients with Kearns-Sayre syndrome and other mitochondrial myopathies with chronic progressive external ophthalmoplegia (CPEO). We treated seven patients for 1 year with daily oral administration of 120 mg of CoQ10. Throughout the treatment most...

journal_title：Neurology

authors： Bresolin N,Bet L,Binda A,Moggio M,Comi G,Nador F,Ferrante C,Carenzi A,Scarlato G

更新日期：1988-06-01 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：:The authors have developed an MG activities of daily living (ADL) profile (MG-ADL)-a simple eight-question survey of MG symptoms. In 254 consecutive encounters with established MG patients, the authors compared scores from the MG-ADL to the quantitative MG score (QMG)-a standardized, reliable scale used in clinical tr...

journal_title：Neurology

authors： Wolfe GI,Herbelin L,Nations SP,Foster B,Bryan WW,Barohn RJ

更新日期：1999-04-22 00:00:00

abstract：:We investigated the prevalence of Parkinson's disease and other types of parkinsonism in a Sicilian population using a door-to-door two-phase approach. This design called for the administration of a brief screening instrument to all subjects who, on November 1, 1987, were residents of Terrasini (Palermo Province), San...

journal_title：Neurology

authors： Morgante L,Rocca WA,Di Rosa AE,De Domenico P,Grigoletto F,Meneghini F,Reggio A,Savettieri G,Castiglione MG,Patti F

更新日期：1992-10-01 00:00:00

abstract：:Primary lateral sclerosis (PLS) is a diagnosis of exclusion in patients with progressive spinobulbar spasticity and could be part of the clinical spectrum of ALS. Unlike ALS, which is familial in 5 to 10% of the cases, PLS has been described as a sporadic disorder in adults. The authors report two patients with PLS fr...

journal_title：Neurology

authors： Brugman F,Wokke JH,Vianney de Jong JM,Franssen H,Faber CG,Van den Berg LH

更新日期：2005-05-24 00:00:00

abstract：OBJECTIVE:Kennedy disease (KD), or spinobulbomuscular atrophy, is a slowly progressive inherited neurodegenerative disorder, marked by prominent fasciculations that typically precede the development of other symptoms. Although the genetic basis of KD relates to triplet (CAG) repeat expansion in the androgen receptor (A...

journal_title：Neurology

authors： Vucic S,Kiernan MC

更新日期：2007-11-06 00:00:00

abstract：OBJECTIVE:We sought to determine the association of dietary factors and risk of cognitive decline in a population at high risk of cardiovascular disease. METHODS:Baseline dietary intake and measures of the Mini-Mental State Examination were recorded in 27,860 men and women who were enrolled in 2 international parallel...

journal_title：Neurology

authors： Smyth A,Dehghan M,O'Donnell M,Anderson C,Teo K,Gao P,Sleight P,Dagenais G,Probstfield JL,Mente A,Yusuf S,ONTARGET and TRANSCEND Investigators.

更新日期：2015-06-02 00:00:00

abstract：BACKGROUND:Because of the relatively poor results of frontal lobe epilepsy (FLE) surgery, identification of prognostic factors for surgical outcome is of great importance. METHODS:To identify predictive factors for FLE surgery, we analyzed the data of 61 patients (mean age at surgery 19.2) who had undergone presurgica...

journal_title：Neurology

authors： Janszky J,Jokeit H,Schulz R,Hoppe M,Ebner A

更新日期：2000-04-11 00:00:00

abstract：:We studied the effect of CSF drainage on cerebral blood flow (CBF) in normal pressure hydrocephalus (NPH) and non-NPH dementia using the 133Xenon inhalation technique. Dementia patients had lower CBF than matched elderly normals. Flow values for NPH and non-NPH patients did not differ before or after CSF drainage. CBF...

journal_title：Neurology

authors： Kushner M,Younkin D,Weinberger J,Hurtig H,Goldberg H,Reivich M

更新日期：1984-01-01 00:00:00

abstract：:A double-blind crossover study with imipramine was conducted in 10 patients with absence and myoclonic-astatic seizures who had not responded to conventional medications. Imipramine produced a significant initial decrease in seizure frequency in 5 of the 10 patients, and in 2 patients the beneficial effect was maintai...

journal_title：Neurology

authors： Fromm GH,Wessel HB,Glass JD,Alvin JD,Van Horn G

更新日期：1978-09-01 00:00:00

abstract：OBJECTIVES:Laminin alpha2 deficiency presents at birth with muscle weakness, hypotonia, and usually asymptomatic white matter signal on MRI. Few patients with laminin alpha2 deficiency have been described with seizures and structural brain abnormalities. The reason for the variation in the severity of the clinical phen...

journal_title：Neurology

authors： Pegoraro E,Fanin M,Trevisan CP,Angelini C,Hoffman EP

更新日期：2000-10-24 00:00:00

abstract：OBJECTIVE:To devise a neurophysiologic strategy to select X-linked Charcot-Marie-Tooth neuropathy syndrome (CMTX) families for connexin 32 mutation screening. BACKGROUND:Once the common chromosome 17 DNA duplication (CMT1A syndrome) has been excluded, clinical features are not sufficiently distinctive to select which ...

journal_title：Neurology

authors： Nicholson GA,Yeung L,Corbett A

更新日期：1998-11-01 00:00:00

abstract：OBJECTIVE:Coccidioides species are the most common etiologic agents of chronic meningitis in regions endemic for coccidioidomycosis. Occasionally, even short-term travel to endemic regions results in the acquisition of meningeal disease, so awareness of this complication of coccidioidomycosis is important even in nonen...

journal_title：Neurology

authors： Drake KW,Adam RD

更新日期：2009-11-24 00:00:00

abstract：:We studied three patients with Leigh's syndrome (LS) and cytochrome c oxidase (COX) deficiency. Biochemical studies in brain, muscle, heart, liver, kidney, and fibroblasts disclosed a generalized COX deficiency. Kinetic studies of COX activity in brain mitochondria showed a low Vmax and a normal Km for reduced cytochr...

journal_title：Neurology

authors： Lombes A,Nakase H,Tritschler HJ,Kadenbach B,Bonilla E,DeVivo DC,Schon EA,DiMauro S

更新日期：1991-04-01 00:00:00

abstract：:Apraxia is the loss of the ability to perform learned skilled movements correctly. In right-handers, apraxia and aphasia are most frequently associated with left-hemisphere lesions. When they are dissociated, however, aphasia is more common in the absence of apraxia than vice versa. There are two hypotheses that can a...

journal_title：Neurology

authors： Foundas AL,Henchey R,Gilmore RL,Fennell EB,Heilman KM

更新日期：1995-07-01 00:00:00

abstract：:PET-demonstrated decreases in [11C]flumazenil binding occur in anterior mesial temporal structures on the side of epileptogenesis in unilateral mesial temporal lobe epilepsy. We performed quantitative autoradiography on anterior mesial and lateral temporal specimens from 11 subjects with unilateral mesial temporal lob...

journal_title：Neurology

authors： Burdette DE,Sakurai SY,Henry TR,Ross DA,Pennell PB,Frey KA,Sackellares JC,Albin RL

更新日期：1995-05-01 00:00:00

abstract：:We enrolled 11 patients with secondary progressive MS in a randomized single-masked cross-over study of plasma exchange (PE) in combination with azathioprine 2 mg/kg. PE was performed once a week for 4 weeks and thereafter every second week for 20 weeks (14 treatments). Eight patients completed the whole trial, and th...

journal_title：Neurology

authors： Sørensen PS,Wanscher B,Szpirt W,Jensen CV,Ravnborg M,Christiansen P,Schreiber K,Nordenbo A

更新日期：1996-06-01 00:00:00

abstract：BACKGROUND:Abeta42-immunization reduces plaque burden and improves cognition in transgenic mouse models of Alzheimer disease (AD). This phase 1 study evaluated the safety, tolerability, and immunogenicity of AN1792 (human aggregated Abeta42) in patients with mild to moderate AD. METHODS:Twenty patients were enrolled i...

journal_title：Neurology

authors： Bayer AJ,Bullock R,Jones RW,Wilkinson D,Paterson KR,Jenkins L,Millais SB,Donoghue S

更新日期：2005-01-11 00:00:00

abstract：OBJECTIVE:To evaluate the integrity of the dorsal striatal dopaminergic innervation in rigid and choreic Huntington disease (HD). BACKGROUND:Some patients with HD have an akinetic-rigid phenotype. It has been suggested that nigrostriatal in addition to striatal pathology is present in this subgroup. The authors sought...

journal_title：Neurology

authors： Bohnen NI,Koeppe RA,Meyer P,Ficaro E,Wernette K,Kilbourn MR,Kuhl DE,Frey KA,Albin RL

更新日期：2000-05-09 00:00:00

abstract：:Outcome after carpal tunnel surgery was studied retrospectively in 32 patients with peripheral neuropathy and carpal tunnel syndrome. Nocturnal paresthesias were almost universally relieved, followed in order of responsiveness by pain, numbness, and weakness. Twenty-five of 28 patients said they would have the surgery...

journal_title：Neurology

authors： Morgenlander JC,Lynch JR,Sanders DB

更新日期：1997-10-01 00:00:00

abstract：OBJECTIVE:To describe the characteristics of late-onset multiple sclerosis (MS) (LOMS, 50+ years) vs adult-onset MS (AOMS, 16 to <50 years) and examine prognosis and associated risk factors. METHODS:Patients with definite MS, onset prior to July 1988, registered with a BCMS clinic before July 1998, with at least one E...

journal_title：Neurology

authors： Tremlett H,Devonshire V

更新日期：2006-09-26 00:00:00

abstract：OBJECTIVE:To use the Unified Batten Disease Rating Scale (UBDRS) to measure the rate of decline in physical and functional capability domains in patients with juvenile neuronal ceroid lipofuscinosis (JNCL) or Batten disease, a neurodegenerative lysosomal storage disorder. We have evaluated the UBDRS in subjects with JN...

journal_title：Neurology

authors： Kwon JM,Adams H,Rothberg PG,Augustine EF,Marshall FJ,Deblieck EA,Vierhile A,Beck CA,Newhouse NJ,Cialone J,Levy E,Ramirez-Montealegre D,Dure LS,Rose KR,Mink JW

更新日期：2011-11-15 00:00:00

abstract：:We report a family with Machado-Joseph disease (MJD) that was previously diagnosed with dentatorubral-pallidoluysian atrophy (DRPLA), on the basis of the neuropathologic findings. Because the clinical and pathologic reevaluation strongly suggested a diagnosis of MJD, we conducted a genetic study in the family. Two pat...

journal_title：Neurology

authors： Sakai T,Antoku Y,Kawakami H,Maruyama H,Nakamura S,Tanaka K

更新日期：1996-04-01 00:00:00

abstract：:We studied a patient in a minimally conscious state using PET and cognitive evoked potentials. Cerebral metabolism was below half of normal values. Auditory stimuli with emotional valence (infant cries and the patient's own name) induced a much more widespread activation than did meaningless noise; the activation patt...

journal_title：Neurology

authors： Laureys S,Perrin F,Faymonville ME,Schnakers C,Boly M,Bartsch V,Majerus S,Moonen G,Maquet P

更新日期：2004-09-14 00:00:00

abstract：:The present study sought to evaluate the validity and generality of the Mini-Mental State Examination (MMSE) and its subsection scores. We gave the MMSE and other neuropsychological tests to 51 patients with probable Alzheimer's disease. On the basis of correlational and factor analyses, overall performance on the MMS...

journal_title：Neurology

authors： Giordani B,Boivin MJ,Hall AL,Foster NL,Lehtinen SJ,Bluemlein LA,Berent S

更新日期：1990-12-01 00:00:00

abstract：OBJECTIVE:To determine serial MRI and CT abnormalities around calcified cysts due to cysticercosis in previously treated patients during periods of seizure activity. BACKGROUND:Some patients with calcified lesions due to cysticercosis have seizures. How and why seizures occur in this setting are unknown. METHODS:Thre...

journal_title：Neurology

authors： Nash TE,Patronas NJ

更新日期：1999-09-11 00:00:00

abstract：:The clinical and angiographic correlations of small deep infarcts seen on computed tomography (CT) scan were studied in 297 consecutive patients. Some of these showed the features described by Fisher as part of the lacunar syndrome. In a large number, a source of emboli from either a cardiac or a carotid source was hi...

journal_title：Neurology

authors： Pullicino P,Nelson RF,Kendall BE,Marshall J

更新日期：1980-10-01 00:00:00

abstract：:The authors describe a case of neurologic involvement in mild hyperphenylalaninemia (HPA), not due to tetrahydrobiopterin (BH(4)) deficiency, with low levels of monoamine neurotransmitter metabolites in CSF. The combined BH(4)-Phe loading test suggested a BH(4) response, confirmed by clinical improvement after BH(4) t...

journal_title：Neurology

authors： Bonafé L,Blau N,Burlina AP,Romstad A,Güttler F,Burlina AB

更新日期：2001-09-11 00:00:00

abstract：OBJECTIVE:To compare the effectiveness of upper extremity virtual reality rehabilitation training (VR) to time-matched conventional training (CT) in the subacute phase after stroke. METHODS:In this randomized, controlled, single-blind phase III multicenter trial, 120 participants with upper extremity motor impairment ...

journal_title：Neurology

authors： Brunner I,Skouen JS,Hofstad H,Aßmus J,Becker F,Sanders AM,Pallesen H,Qvist Kristensen L,Michielsen M,Thijs L,Verheyden G

更新日期：2017-12-12 00:00:00