Proximal myotonic myopathy with MRI white matter abnormalities of the brain.

abstract：:In patients with cranial dystonia, we compared the effects of central anticholinergic, peripheral anticholinergic, and placebo treatments in a double-blind crossover study. One of the nine patients who completed the study improved markedly with central anticholinergic therapy. The three treatments were indistinguishab...

journal_title：Neurology

authors： Nutt JG,Hammerstad JP,deGarmo P,Carter J

更新日期：1984-02-01 00:00:00

abstract：:We have demonstrated the reliability of a noninvasive method for successfully localizing the intracranial origin of the EEG. The dipole localization method (DLM) is a computer-assisted, mathematical method based on electrical field theory and is similar to localization methods currently used by electrocardiologists. I...

journal_title：Neurology

authors： Smith DB,Sidman RD,Flanigin H,Henke J,Labiner D

更新日期：1985-12-01 00:00:00

abstract：:This is a report on hepatic adverse events associated with dantrolene therapy. All cases reported to the manufacturer are included, from all sources, through 1987. Of 122 cases containing sufficient data to analyze, 47 patients had asymptomatic transaminase elevations, 12 had additional mild (less than or equal to 2.5...

journal_title：Neurology

authors： Chan CH

更新日期：1990-09-01 00:00:00

abstract：:We compared the number of new gadolinium-enhancing and T2-weighted lesions on six monthly MRI scans in eight patients with relapsing multiple sclerosis before and during treatment with weekly intramuscular interferon beta-1a (Avonex; 30 microg). MRI activity was modestly reduced during treatment (p = 0.016). The treat...

journal_title：Neurology

authors： Waubant E,Goodkin DE,Sloan R,Andersson PB

更新日期：1999-09-11 00:00:00

abstract：:We detected crossed cerebellar as well as uncrossed basal ganglia and thalamic diaschisis in Alzheimer's disease by positron emission tomography (PET) using 18F-fluorodeoxyglucose. We studied a series of 26 consecutive, clinically diagnosed Alzheimer cases, including 6 proven by later autopsy, and compared them with 9...

journal_title：Neurology

authors： Akiyama H,Harrop R,McGeer PL,Peppard R,McGeer EG

更新日期：1989-04-01 00:00:00

abstract：:We identified the cytokines interleukin-1 beta (IL-1 beta), tumor necrosis factor (TNF), and interleukin-6 (IL-6) by specific radioimmunoassays in the CSF of patients with multiple sclerosis (MS) and other neurologic diseases (OND). There was a high incidence of detectable IL-1 beta in patients with active MS compared...

journal_title：Neurology

authors： Hauser SL,Doolittle TH,Lincoln R,Brown RH,Dinarello CA

更新日期：1990-11-01 00:00:00

abstract：OBJECTIVE:The natural history of succinic semialdehyde dehydrogenase (SSADH) deficiency in adulthood is unknown; we elucidate the clinical manifestations of the disease later in life. METHODS:A 63-year-old man with long-standing intellectual disability was diagnosed with SSADH deficiency following hospitalization for ...

journal_title：Neurology

authors： Lapalme-Remis S,Lewis EC,De Meulemeester C,Chakraborty P,Gibson KM,Torres C,Guberman A,Salomons GS,Jakobs C,Ali-Ridha A,Parviz M,Pearl PL

更新日期：2015-09-08 00:00:00

abstract：:Intravenous tissue plasminogen activator (tPA) is an effective therapy for treatment of acute ischemic stroke when administered within 3 hours of symptom onset. Three large randomized clinical trials that have attempted to extend the time window for tPA treatment beyond 3 hours have failed to demonstrate convincing ev...

journal_title：Neurology

authors： Albers GW

更新日期：2001-01-01 00:00:00

abstract：:A young girl who initially presented with jaw hypomobility at age 4 years subsequently developed signs of a slowly progressive myopathy. Rods were found in all sampled muscles, and were associated with fibrous contractures in the temporomandibular joint muscles. Rods in fibers undergoing necrosis displayed a pattern o...

journal_title：Neurology

authors： Powers JM,Young GF,Bass EB Jr,Reed FE Jr

更新日期：1980-09-01 00:00:00

abstract：OBJECTIVE:To determine the incidence of spinocerebellar ataxia (SCA) types 1, 2, 3, 6, and 7 and Friedreich's ataxia (FA) among a large panel of ataxia families. BACKGROUND:The ataxias are a clinically and genetically heterogeneous group of neurodegenerative diseases that variably affect the cerebellum, brainstem, and...

journal_title：Neurology

authors： Moseley ML,Benzow KA,Schut LJ,Bird TD,Gomez CM,Barkhaus PE,Blindauer KA,Labuda M,Pandolfo M,Koob MD,Ranum LP

更新日期：1998-12-01 00:00:00

abstract：OBJECTIVE:To verify the previously reported association between long-term use of β2-adrenoreceptor (β2AR) agonist and antagonist with reduced and increased risk of Parkinson disease (PD), respectively. METHODS:We obtained odds ratios (ORs) associating time of β2AR agonist and antagonist use with PD risk in nationwide ...

journal_title：Neurology

authors： Hopfner F,Wod M,Höglinger GU,Blaabjerg M,Rösler TW,Kuhlenbäumer G,Christensen K,Deuschl G,Pottegård A

更新日期：2019-07-09 00:00:00

abstract：OBJECTIVE:To report the clinical and pelvic CT findings in six patients with obturator mononeuropathy caused by cancer. DESIGN:A clinical case series of six patients followed for 2 months to 10 years (one patient lost to follow-up). SETTING:Three referral centers. PATIENTS:Three men and three women, ages 52 to 81 ye...

journal_title：Neurology

authors： Rogers LR,Borkowski GP,Albers JW,Levin KH,Barohn RJ,Mitsumoto H

更新日期：1993-08-01 00:00:00

abstract：OBJECTIVE:To clarify the genetic, clinicopathologic, and neuroimaging characteristics of patients with hereditary diffuse leukoencephalopathy with spheroids (HDLS) with the colony stimulating factor 1 receptor (CSF-1R) mutation. METHODS:We performed molecular genetic analysis of CSF-1R in patients with HDLS. Detailed ...

journal_title：Neurology

authors： Konno T,Tada M,Tada M,Koyama A,Nozaki H,Harigaya Y,Nishimiya J,Matsunaga A,Yoshikura N,Ishihara K,Arakawa M,Isami A,Okazaki K,Yokoo H,Itoh K,Yoneda M,Kawamura M,Inuzuka T,Takahashi H,Nishizawa M,Onodera O,Kakita

更新日期：2014-01-14 00:00:00

abstract：:Pathology and imaging studies have shown that patients with Parkinson disease (PD) have a prolonged period of uncertain duration when vulnerable neuronal populations are degenerating, but typical motor symptoms have not yet developed. This provides both an opportunity-it may be best to test new medications and, ultima...

journal_title：Neurology

authors： Marek K,Jennings D

更新日期：2009-02-17 00:00:00

abstract：OBJECTIVE:To identify the disease-causing gene of a family with upper limb predominant, slowly progressive amyotrophic lateral sclerosis (ALS), which was diagnosed as flail arm syndrome (FAS). METHODS:After causation of 24 known ALS genes was excluded by targeted next-generation sequencing, whole-exome sequencing was ...

journal_title：Neurology

authors： Liu Q,Shu S,Wang RR,Liu F,Cui B,Guo XN,Lu CX,Li XG,Liu MS,Peng B,Cui LY,Zhang X

更新日期：2016-10-25 00:00:00

abstract：BACKGROUND:Previous studies have examined the role of APOE variation in multiple sclerosis (MS), but have lacked the statistical power to detect modest genetic influences on risk and disease severity. The meta- and pooled analyses presented here utilize the largest collection, to date, of MS cases, controls, and famili...

journal_title：Neurology

authors： Burwick RM,Ramsay PP,Haines JL,Hauser SL,Oksenberg JR,Pericak-Vance MA,Schmidt S,Compston A,Sawcer S,Cittadella R,Savettieri G,Quattrone A,Polman CH,Uitdehaag BM,Zwemmer JN,Hawkins CP,Ollier WE,Weatherby S,Enzinger C

更新日期：2006-05-09 00:00:00

abstract：:Using a radioreceptor technique, we assayed serum trihexyphenidyl levels in patients with dystonia being treated chronically with high dosage. We found a significant correlation between total daily dose and the daily lowest (trough) serum levels. There was no relationship between serum levels and therapeutic response ...

journal_title：Neurology

authors： Burke RE,Fahn S

更新日期：1985-07-01 00:00:00

abstract：:We carried out an open study of the effects of large doses (12 to 16 gm per day) of oral choline on medically intractable human complex partial seizures (CPS). Marked increases of plasma choline concentration (75 to 300%) in three subjects were associated with (1) shorter duration of CPS, (2) less postseizure fatigue,...

journal_title：Neurology

authors： McNamara JO,Carwile S,Hope V,Luther J,Miller P

更新日期：1980-12-01 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：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: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：:Phenytoin suspension (PHY-S) is reported to settle, resulting in uneven drug distribution and variable patient dosing. We designed this study to determine the rate of settling and the amount of agitation needed to resuspend the preparation. To determine the rate of settling, we thoroughly shook three bottles of PHY-S ...

journal_title：Neurology

authors： Sarkar MA,Garnett WR,Karnes HT

更新日期：1989-02-01 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 three patients with acute chiasmal visual field loss caused by hemorrhage within an intrachiasmal arteriovenous malformation. One patient had five episodes; vision improved three times without treatment and twice after surgical decompression. Surgical intervention restored vision in two of the three patien...

journal_title：Neurology

authors： Lavin PJ,McCrary JA 3rd,Roessmann U,Ellenberger C Jr

更新日期：1984-08-01 00:00:00

abstract：:Because hereditary hyperekplexia results from a defect in the glycine receptor, we studied in five patients several spinal inhibitory pathways that are thought to use either glycine or gamma-aminobutyric acid as a neurotransmitter. Three patients had a mutation in the alpha1 subunit of the glycine receptor, whereas tw...

journal_title：Neurology

authors： Floeter MK,Andermann F,Andermann E,Nigro M,Hallett M

更新日期：1996-03-01 00:00:00

abstract：OBJECTIVE:To model the temporal trajectory of β-amyloid accumulation using serial amyloid PET imaging. METHODS:Participants, aged 70-92 years, were enrolled in either the Mayo Clinic Study of Aging (n = 246) or the Mayo Alzheimer's Disease Research Center (n = 14). All underwent 2 or more serial amyloid PET examinatio...

journal_title：Neurology

authors： Jack CR Jr,Wiste HJ,Lesnick TG,Weigand SD,Knopman DS,Vemuri P,Pankratz VS,Senjem ML,Gunter JL,Mielke MM,Lowe VJ,Boeve BF,Petersen RC

更新日期：2013-03-05 00:00:00

abstract：:We studied the effect of acetazolamide on plasma potassium in normals and in two patients with hyperkalemic periodic paralysis. Administration of acetazolamide for 48 hours lowered mean plasma potassium in normals from 4.01 to 3.56 mEq per liter (p less than 0.001) and in the patients from 4.55 to 4.00 mEq per liter (...

journal_title：Neurology

authors： Riggs JE,Griggs RC,Moxley RT 3rd,Lewis ED

更新日期：1981-06-01 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：OBJECTIVE:To assess whether faster cognitive decline in elders without dementia is associated with decreased risk of cancer mortality. METHODS:In this population-based, prospective study of 2,627 people without dementia aged 65 years and older (Neurological Disorders in Central Spain), a 37-item version of the Mini-Me...

journal_title：Neurology

authors： Benito-León J,Romero JP,Louis ED,Bermejo-Pareja F

更新日期：2014-04-22 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