A novel autosomal dominant limb-girdle muscular dystrophy (LGMD 1F) maps to 7q32.1-32.2.

abstract：BACKGROUND:Computerized ambulatory monitoring provides real-time assessments of clinical outcomes in natural contexts, and it has been increasingly applied in recent years to investigate symptom expression in a wide range of disorders. The purpose of this study was to examine the feasibility and validity of this data c...

journal_title：Neurology

authors： Johnson EI,Sibon I,Renou P,Rouanet F,Allard M,Swendsen J

更新日期：2009-11-10 00:00:00

abstract：OBJECTIVE:To determine whether comorbidity is associated with clinical (relapses, disability worsening) and MRI outcomes in multiple sclerosis (MS) by conducting a secondary analysis of the CombiRx clinical trial. METHODS:CombiRx compared interferon beta-1a, glatiramer acetate, and the combination of these agents. For...

journal_title：Neurology

authors： Salter A,Kowalec K,Fitzgerald KC,Cutter G,Marrie RA

更新日期：2020-08-04 00:00:00

abstract：:Between the years 1964 and 1973, 225 patients with transient ischemic attacks (TIAs) due primarily to atherosclerosis were evaluated and treated. They have now been followed for from 3 to 14 years (average 5.5 years). As of 1976, 82 of the 225 patients were dead, 21 from cerebral infarction, 52 from heart disease and ...

journal_title：Neurology

authors： Toole JF,Yuson CP,Janeway R,Johnston F,Davis C,Cordell AR,Howard G

更新日期：1978-08-01 00:00:00

abstract：:Tizanidine, an imidazoline that acts as an agonist at alpha 2-adrenergic receptors, has been shown to be effective in reducing spasticity caused by MS. This multicenter study (14 sites) assessed the efficacy and safety of oral tizanidine in patients who had spinal cord injury of > 12 months' duration. Of the 124 patie...

journal_title：Neurology

authors： Nance PW,Bugaresti J,Shellenberger K,Sheremata W,Martinez-Arizala A

更新日期：1994-11-01 00:00:00

abstract：BACKGROUND:Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare multisystem disorder associated with plasma cell dyscrasia. There is increasing evidence that high-dose chemotherapy with autologous peripheral blood stem cell transplantation (Auto-PBSCT) is an efficacious t...

journal_title：Neurology

authors： Kuwabara S,Misawa S,Kanai K,Suzuki Y,Kikkawa Y,Sawai S,Hattori T,Nishimura M,Nakaseko C

更新日期：2008-11-18 00:00:00

abstract：:In 47 patients with Parkinson's disease, we studied the visual evoked potential (VEP) using the pattern-reversal checkerboard (CBVEP) and the grating (GVEP) stimuli before and after 3 months of L-dopa therapy. The P100 latency in both CBVEP and GVEP was prolonged before L-dopa therapy, usually commensurate with the de...

journal_title：Neurology

authors： Bhaskar PA,Vanchilingam S,Bhaskar EA,Devaprabhu A,Ganesan RA

更新日期：1986-08-01 00:00:00

abstract：:Of 101 patients with benign intracranial hypertension not related to vasculitis, neck stiffness occurred in 31, tinnitus in 27, distal extremity paresthesias in 22, joint pains in 13, low back pain in 5, and gait "ataxia" in 4. Symptoms resolved promptly upon lowering the intracranial pressure by lumbar puncture, and ...

journal_title：Neurology

authors： Round R,Keane JR

更新日期：1988-09-01 00:00:00

abstract：:We report a woman with a chronic inflammatory myopathy who developed muscle stiffness and dystonic postures of both upper extremities. Electromyography demonstrated that involuntary complex repetitive discharges of muscle caused the posture. Rare cases of dystonic posturing may be of peripheral, and not central system...

journal_title：Neurology

authors： Preston DC,Finkleman RS,Munsat TL

更新日期：1996-01-01 00:00:00

abstract：OBJECTIVE:To assess whether lifetime physical activity during work and leisure time is associated with an increased risk of developing ALS and to determine the association between physical activity and duration or age at onset of disease. METHODS:Patients referred to our clinic during the 1-year period 2001 to 2002 wh...

journal_title：Neurology

authors： Veldink JH,Kalmijn S,Groeneveld GJ,Titulaer MJ,Wokke JH,van den Berg LH

更新日期：2005-01-25 00:00:00

abstract：:Twenty-two patients were analyzed 2 or more years after corpus callosum section (9 partial, 13 total). Forty-one percent had class 1 outcome (elimination of secondarily generalized and complex partial seizures), 32% had class 2 outcome (elimination of secondarily generalized seizures), and 27% had class 3 outcome (no ...

journal_title：Neurology

authors： Spencer SS,Spencer DD,Williamson PD,Sass K,Novelly RA,Mattson RH

更新日期：1988-01-01 00:00:00

abstract：:The "modified host protein" model of scrapie proposes that the transmissible agent is composed of the degradation-resistant protein, Sp33-37, and that clinical and pathologic signs result from neurotoxic accumulations of this protein. Sp33-37 is an abnormal, amyloidogenic isoform of the normally occurring cellular pro...

journal_title：Neurology

authors： Bendheim PE,Brown HR,Rudelli RD,Scala LJ,Goller NL,Wen GY,Kascsak RJ,Cashman NR,Bolton DC

更新日期：1992-01-01 00:00:00

abstract：BACKGROUND:Findings from a small clinical study suggested that statins may counteract the therapeutic effects of interferon beta (IFNbeta) in patients with relapsing-remitting multiple sclerosis (RRMS). METHODS:We conducted a post hoc analysis of data from the Safety and Efficacy of Natalizumab in Combination With IFN...

journal_title：Neurology

authors： Rudick RA,Pace A,Rani MR,Hyde R,Panzara M,Appachi S,Shrock J,Maurer SL,Calabresi PA,Confavreux C,Galetta SL,Lublin FD,Radue EW,Ransohoff RM

更新日期：2009-06-09 00:00:00

abstract：OBJECTIVE:To compare characteristics between Black and White patients with amyotrophic lateral sclerosis (ALS) in order to identify disparities and phenotypic variability. METHODS:We performed database review for patients seen between 1997 and 2020 at the Emory ALS Center in Atlanta, Georgia. Patients with ALS were in...

journal_title：Neurology

authors： Brand D,Polak M,Glass JD,Fournier CN

更新日期：2020-12-28 00:00:00

abstract：:Patients with Parkinson's disease (PD) commonly complain of impaired visual function and difficulty reading, despite normal visual acuity. Although previous studies have evaluated contrast sensitivity, color vision, visuospatial processing, visual hallucinations, and ocular movements, none has systematically evaluated...

journal_title：Neurology

authors： Biousse V,Skibell BC,Watts RL,Loupe DN,Drews-Botsch C,Newman NJ

更新日期：2004-01-27 00:00:00

abstract：:A 53-year-old man presented with a painful, demyelinating sensorimotor peripheral neuropathy with lymphomatous infiltration on sural nerve biopsy, but no evidence of systemic lymphoma. The neuropathy responded to cytotoxic therapy. Seven years later he developed generalized lymphadenopathy due to B cell lymphoplasmacy...

journal_title：Neurology

authors： Ince PG,Shaw PJ,Fawcett PR,Bates D

更新日期：1987-07-01 00:00:00

abstract：OBJECTIVE:To evaluate the usefulness of ocular motor information in the early diagnosis of corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). METHODS:Seven PSP patients, six CBD patients, and three atypical CBD patients were followed longitudinally with repeated electrooculographic (EOG) recordi...

journal_title：Neurology

authors： Rivaud-Péchoux S,Vidailhet M,Gallouedec G,Litvan I,Gaymard B,Pierrot-Deseilligny C

更新日期：2000-03-14 00:00:00

abstract：:Proteins from single frozen sections of human muscle were separated by two-dimensional gel electrophoresis and detected by fluorography or Coomassie Blue staining. The major proteins were identical in different normal muscles obtained from either sex at different ages, and in Duchenne and myotonic dystrophy samples. C...

journal_title：Neurology

authors： Giometti CS,Danon MJ,Anderson NG

更新日期：1983-09-01 00:00:00

abstract：:Tracheostomy has been employed to release the airway obstruction at the glottic level and to prevent sudden death in patients with multiple system atrophy (MSA). However, sudden death is possible even after tracheostomy. Nocturnal polysomnography showed that the apnea-hypopnea index became higher after tracheostomy, a...

journal_title：Neurology

authors： Jin K,Okabe S,Chida K,Abe N,Kimpara T,Ohnuma A,Nomura H,Itoyama Y,Onodera H

更新日期：2007-05-08 00:00:00

abstract：:A 60-year-old man presented with progressive large fiber sensory loss in the right first three fingers and, to a lesser extent, in both fourth and fifth fingers. Electrophysiologic studies were characteristic of chronic sensory demyelinating polyneuropathy, a variant of chronic inflammatory demyelinating polyneuropath...

journal_title：Neurology

authors： Thonnard JL,Detrembleur C,Van den Bergh PY

更新日期：1997-07-01 00:00:00

abstract：:The epsilon 4 isoform of apolipoprotein E (Apo-E) may confer genetic susceptibility for familial and sporadic Alzheimer's disease (AD). Because dementia in AD and Parkinson's disease (PD) share many biologic and clinical features, we determined the Apo-E genotypes for 79 patients with PD, 22 of whom were demented, and...

journal_title：Neurology

authors： Marder K,Maestre G,Cote L,Mejia H,Alfaro B,Halim A,Tang M,Tycko B,Mayeux R

更新日期：1994-07-01 00:00:00

abstract：OBJECTIVE:The aims of this study were to examine executive dysfunction using an antisaccade (AS) task in normal elderly (NE) and patients with mild cognitive impairment (MCI) and Alzheimer disease (AD) as well as to evaluate the relationship between AS performance and cortical thinning within AD-associated regions. ME...

journal_title：Neurology

authors： Heuer HW,Mirsky JB,Kong EL,Dickerson BC,Miller BL,Kramer JH,Boxer AL

更新日期：2013-10-01 00:00:00

abstract：:In seven of eight patients with progressive multiple sclerosis subjected to long-term plasmapheresis in combination with azathioprine and pulsed prednisone therapy, we found modest improvement of neurologic function. There was no change in auditory and visual evoked responses or serum demyelinating activity. In six of...

journal_title：Neurology

authors： Dau PC,Petajan JH,Johnson KP,Panitch HS,Bornstein MB

更新日期：1980-10-01 00:00:00

abstract：:We investigated potential risk factors for Alzheimer's disease (AD) in a clinicopathologic study of 407 patients with definite AD, 100 non-Alzheimer dementia patients, and 50 normal subjects. The AD patients had more first-degree relatives with dementia than the non-AD dementia group (odds ratio of 1.85, 95% confidenc...

journal_title：Neurology

authors： Mendez MF,Underwood KL,Zander BA,Mastri AR,Sung JH,Frey WH 2nd

更新日期：1992-04-01 00:00:00

abstract：BACKGROUND:Previous studies have suggested a variety of factors that may be associated with the presence of hippocampal formation (HF) atrophy in patients with complex partial seizures (CPS), including a history of complex or prolonged febrile seizures (FS), age at seizure onset, and epilepsy duration. OBJECTIVE:To de...

journal_title：Neurology

authors： Theodore WH,Bhatia S,Hatta J,Fazilat S,DeCarli C,Bookheimer SY,Gaillard WD

更新日期：1999-01-01 00:00:00

abstract：:Free amino acid determinations in the cerebrospinal fluid of patients with Parkinson's disease or other extrapyramidal disorders showed that in both groups most amino acids were increased. Significantly increased amino acids were neutral and basic amino acids. Amino acid clusters were present and corresponded with the...

journal_title：Neurology

authors： Lakke JP,Teelken AW

更新日期：1976-05-01 00:00:00

abstract：:Two cases of giant-cell arteritis with cerebral arteritis are presented. The clinical situation and the suggestive but nonspecific angiographic features make the antemortem diagnosis possible. Greater awareness of this entity will facilitate its diagnosis and the institution of effective steroid therapy. ...

journal_title：Neurology

authors： Enzmann D,Scott WR

更新日期：1977-08-01 00:00:00

abstract：OBJECTIVES:We used functional MRI (fMRI), transcranial Doppler ultrasound, and visual evoked potentials (VEPs) to determine the nature of blood flow responses to functional brain activity and carbon dioxide (CO2) inhalation in patients with cerebral amyloid angiopathy (CAA), and their association with markers of CAA se...

journal_title：Neurology

authors： Peca S,McCreary CR,Donaldson E,Kumarpillai G,Shobha N,Sanchez K,Charlton A,Steinback CD,Beaudin AE,Flück D,Pillay N,Fick GH,Poulin MJ,Frayne R,Goodyear BG,Smith EE

更新日期：2013-11-05 00:00:00

abstract：OBJECTIVE:To estimate the prevalence of childhood ataxia resulting from both genetic and acquired causes. METHODS:A systematic review was conducted following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-analyses) statement. Five databases were searched for articles reporting a frequency measur...

journal_title：Neurology

authors： Musselman KE,Stoyanov CT,Marasigan R,Jenkins ME,Konczak J,Morton SM,Bastian AJ

更新日期：2014-01-07 00:00:00

abstract：:Alzheimer's disease is characterized by relative sparing of primary sensory and motor cortex and a lack of sensory or motor symptomatology. We report a case of presenile onset dementia accompanied by a slowly progressive hemiparesis. Autopsy examination showed severe pathologic involvement of somatosensory cortex with...

journal_title：Neurology

authors： Jagust WJ,Davies P,Tiller-Borcich JK,Reed BR

更新日期：1990-01-01 00:00:00

abstract：OBJECTIVE:To validate a neuropsychological algorithm for dementia diagnosis. METHODS:We developed a neuropsychological algorithm in a sample of 1,023 elderly residents of Cache County, UT. We compared algorithmic and clinical dementia diagnoses both based on DSM-III-R criteria. The algorithm diagnosed dementia when th...

journal_title：Neurology

authors： Tschanz JT,Welsh-Bohmer KA,Skoog I,West N,Norton MC,Wyse BW,Nickles R,Breitner JC

更新日期：2000-03-28 00:00:00