Randomized trials of dichlorphenamide in the periodic paralyses. Working Group on Periodic Paralysis.

abstract：:Because of the association of D-penicillamine (DP) therapy with myasthenia gravis, we have studied long-term DP treatment in five inbred strains of mice with doses comparable to those used in patients with rheumatoid arthritis. No clinical weakness or anti-acetylcholine receptor (AChR) antibody developed with up to 6 ...

journal_title：Annals of neurology

authors： Bever CT Jr,Dretchen KL,Blake GJ,Chang HW,Penn AS,Asofsky R

更新日期：1984-07-01 00:00:00

abstract：:Charcot-Marie-Tooth (CMT) neuropathies are inherited neuromuscular disorders caused by a length-dependent neurodegeneration of peripheral nerves. More than 900 mutations in 60 different genes are causative of the neuropathy. Despite significant progress in therapeutic strategies, the disease remains incurable. The inc...

journal_title：Annals of neurology

authors： Bouhy D,Timmerman V

更新日期：2013-09-01 00:00:00

abstract：:Gene therapy methods have continued to develop rapidly, and many initial limitations that hampered clinical application have been overcome. Thus serious consideration of clinical application of gene therapy is warranted for selected disorders in which the pathogenesis is well defined. Parkinson's disease has been the ...

journal_title：Annals of neurology

authors： Kang UJ,Nakamura K

更新日期：2003-01-01 00:00:00

abstract：:A unique phenotype of Waardenburg-Hirschsprung disease (WS4) accompanied by peripheral neuropathy and central dysmyelination has been recognized recently in association with SOX10 mutations. We report an infant boy with lethal congenital hypomyelinating neuropathy and WS4 who had a heterozygous SOX10 mutation (Q250X)....

journal_title：Annals of neurology

authors： Inoue K,Shilo K,Boerkoel CF,Crowe C,Sawady J,Lupski JR,Agamanolis DP

更新日期：2002-12-01 00:00:00

abstract：:This study assessed whether or not levodopa induces dyskinesias in normal (ie, unlesioned) squirrel monkeys. All six animals treated twice daily with levodopa (15 mg/kg with carbidopa by oral gavage) for two weeks developed choreoathetoid dyskinesias, whereas none of the vehicle-treated animals displayed any abnormal ...

journal_title：Annals of neurology

authors： Togasaki DM,Tan L,Protell P,Di Monte DA,Quik M,Langston JW

更新日期：2001-08-01 00:00:00

abstract：OBJECTIVE:To assess the associations of blood biomarkers of carbohydrate, lipid, and apolipoprotein metabolisms with the future risk of amyotrophic lateral sclerosis (ALS). METHODS:In the Apolipoprotein-related MOrtality RISk study, we enrolled 636,132 men and women during 1985-1996 in Stockholm, Sweden, with measurem...

journal_title：Annals of neurology

authors： Mariosa D,Hammar N,Malmström H,Ingre C,Jungner I,Ye W,Fang F,Walldius G

更新日期：2017-05-01 00:00:00

abstract：:Existing and emerging viral central nervous system (CNS) infections are major sources of human morbidity and mortality. Treatments of proven efficacy are currently limited predominantly to herpesviruses and human immunodeficiency virus (HIV). Development of new therapies has been hampered by the lack of appropriate an...

journal_title：Annals of neurology

authors： Nath A,Tyler KL

更新日期：2013-09-01 00:00:00

abstract：:We report the use of a new stable isotope-labeled form of levodopa (LD) to examine in vivo central LD metabolism in Parkinson's disease (PD). Eight patients representing a wide spectrum of disease severity were administered 50 mg of carbidopa orally followed in 1 hour by an intravenous bolus of 150 mg of stable isotop...

journal_title：Annals of neurology

authors： Durso R,Evans JE,Josephs E,Szabo GK,Evans BA,Handler JS,Jennings D,Browne TR

更新日期：1997-09-01 00:00:00

abstract：:Hemophilus influenzae is the most common cause of bacterial meningitis in children, and a high percentage of survivors are at risk for long-term sequelae. To explore the mechanisms responsible for these sequelae, a neonatal rat model was used to define the behavioral, electrophysiological, and biochemical changes foll...

journal_title：Annals of neurology

authors： Konkol RJ,Chapman L,Breese GR,Collier AM,Kilts C,Finley C,Vogel RR,Mailman RB,Bendeich EG

更新日期：1987-04-01 00:00:00

abstract：:The incidence of periventricular-intraventricular hemorrhage (PV-IVH) in a group of 460 preterm infants with birth weight less than 2,250 gm, studied by cranial ultrasonography, was 39%. Sixty-four (36%) of the infants with periventricular-intraventricular hemorrhage had, in addition, periventricular intraparenchymal ...

journal_title：Annals of neurology

authors： McMenamin JB,Shackelford GD,Volpe JJ

更新日期：1984-03-01 00:00:00

abstract：:Eighteen-month-old identical twins with the muscle histological characteristics of congenital fiber type disproportion are reported. The fiber typing system of pH-dependent adenosine triphosphatase was used to analyze the size and percentage of type 1, 2A, and 2B fibers in muscle tissue obtained by needle biopsy. Both...

journal_title：Annals of neurology

authors： Curless RG,Nelson MB

更新日期：1977-12-01 00:00:00

abstract：:We studied the impact of cerebral amyloid angiopathy on tissue plasminogen activator-induced cerebral hemorrhages in APP23 transgenic mice. Results show that the intravenous administration of tissue plasminogen activator in APP23 mice leads to an increase in cerebral amyloid angiopathy-associated microhemorrhages and ...

journal_title：Annals of neurology

authors： Winkler DT,Biedermann L,Tolnay M,Allegrini PR,Staufenbiel M,Wiessner C,Jucker M

更新日期：2002-06-01 00:00:00

abstract：:Plasma valproate concentrations were monitored prospectively in 54 previously untreated adult patients with epilepsy. Dose and plasma concentration were highly correlated. Adverse effects were common in association with plasma levels above 100 micrograms/ml. In patients suffering tonic-clonic seizures without focal sy...

journal_title：Annals of neurology

authors： Turnbull DM,Rawlins MD,Weightman D,Chadwick DW

更新日期：1983-07-01 00:00:00

abstract：:We describe the clinical and oculographic findings in 4 families with episodic ataxia and interictal nystagmus (EA-2) linked to chromosome 19p. Episodes varied from pure ataxia to combinations of symptoms suggesting involvement of the cerebellum, brainstem, and cortex. Some affected individuals exhibited a progressive...

journal_title：Annals of neurology

authors： Baloh RW,Yue Q,Furman JM,Nelson SF

更新日期：1997-01-01 00:00:00

abstract：:Indirect evidence suggests that an autoimmune response to myelin basic protein (MBP) may be involved in the pathogenesis of multiple sclerosis (MS). In MS, several reports have suggested that restricted T-cell populations respond to MPB, as in inbred rodents with the MS disease model experimental allergic encephalomye...

journal_title：Annals of neurology

authors： Joshi N,Usuku K,Hauser SL

更新日期：1993-09-01 00:00:00

abstract：:We measured the intrinsic mechanical properties and protein content of single skinned muscle fibers obtained from patients who had Duchenne muscular dystrophy. To check for possible nonspecific changes caused by muscle disease per se, we also studied the properties of muscle fibers obtained from patients exhibiting se...

journal_title：Annals of neurology

authors： Horowits R,Dalakas MC,Podolsky RJ

更新日期：1990-06-01 00:00:00

abstract：:Aggregated tau proteins constitute the basic matrix of neuronal inclusions specific to numerous neurodegenerative disorders. Monodimensional and two-dimensional Western blot analyses performed on cortical brain homogenates allowed discrimination between disease-specific tau protein profiles. These observations raised ...

journal_title：Annals of neurology

authors： Delacourte A,Sergeant N,Wattez A,Gauvreau D,Robitaille Y

更新日期：1998-02-01 00:00:00

abstract：:Clinical evidence is presented to support the contention that the sternocleidomastoid muscle is innervated primarily by undecussated fibers from the ipsilateral hemisphere. Stroke patients often show contralateral hemiparesis accompanied by weakness in head turning to the side of the paresis. ...

journal_title：Annals of neurology

authors： Balagura S,Katz RG

更新日期：1980-01-01 00:00:00

abstract：:The characteristic pathological features of Huntington's disease (HD) are neostriatal atrophy and neuronal loss. Although neuroradiological studies often show caudate atrophy in patients with moderate HD, frequently no caudate atrophy is found early in the illness. There have been no quantitative reports to date on in...

journal_title：Annals of neurology

authors： Harris GJ,Pearlson GD,Peyser CE,Aylward EH,Roberts J,Barta PE,Chase GA,Folstein SE

更新日期：1992-01-01 00:00:00

abstract：:Hippocampal depth electrodes are often used to localize seizure onset in patients who may have temporal lobe epilepsy (TLE). A number of features of the spontaneous seizures and of their ictal onset patterns can be analyzed from these recordings. We compared a number of the typical electroencephalographic (EEG) change...

journal_title：Annals of neurology

authors： Williamson A,Spencer SS,Spencer DD

更新日期：1995-11-01 00:00:00

abstract：:Using placebo data from 3 randomized multiple sclerosis (MS) trials with uniform inclusion criteria, we investigated heterogeneity of Expanded Disability Status Scale (EDSS) progression by geographical areas. Our analysis revealed a significantly lower EDSS progression in Eastern European countries (10.8%) compared wi...

journal_title：Annals of neurology

authors： Bovis F,Signori A,Carmisciano L,Maietta I,Steinerman JR,Li T,Tansy AP,Sormani MP

更新日期：2018-10-01 00:00:00

abstract：:Guillain-Barré syndrome (GBS) is an immune-mediated neuropathy, in which leukocytes and humoral components of the immune system proposedly initiate localized inflammation. An important pathogenic role for anti-GM1 ganglioside antibodies has been suggested. Therefore, we evaluated anti-GM1 IgG antibody-induced leukocyt...

journal_title：Annals of neurology

authors： van Sorge NM,van den Berg LH,Geleijns K,van Strijp JA,Jacobs BC,van Doorn PA,Wokke JH,van de Winkel JG,Leusen JH,van der Pol WL

更新日期：2003-05-01 00:00:00

abstract：:The North American study of plasmapheresis in Guillain-Barré syndrome (GBS) included early, standardized electrodiagnostic testing in 210 of the 245 patients. To determine the types of abnormalities and the relation to outcome, we analyzed the prospectively collected motor conduction data obtained during the first 30 ...

journal_title：Annals of neurology

authors： Cornblath DR,Mellits ED,Griffin JW,McKhann GM,Albers JW,Miller RG,Feasby TE,Quaskey SA

更新日期：1988-04-01 00:00:00

abstract：:In CAG repeat diseases, affected neurons possess many cytoplasmic granules immunopositive for expanded polyglutamine stretches. Electron microscopic immunohistochemistry showed that the granules corresponded to lysosomes of primitive type. The results suggest that, in addition to the ubiquitin/proteasome pathway, muta...

journal_title：Annals of neurology

authors： Yamada M,Tsuji S,Takahashi H

更新日期：2002-10-01 00:00:00

abstract：OBJECTIVE:Fast oscillations (FOs) were first explored from scalp electroencephalographic (EEG) data from hypsarrhythmia in West syndrome (infantile spasms) to investigate the meaning of FOs in this epileptic encephalopathy. METHODS:In 17 infants with West syndrome, we conservatively detected fast frequency peaks that ...

journal_title：Annals of neurology

authors： Kobayashi K,Akiyama T,Oka M,Endoh F,Yoshinaga H

更新日期：2015-01-01 00:00:00

abstract：OBJECTIVE:Huntington's disease (HD) is a monogenic, fully penetrant neurodegenerative disorder, providing an ideal model for understanding brain changes occurring in the years prior to disease onset. Diffusion tensor imaging (DTI) studies show widespread white matter disorganization in the early premanifest stages (pre...

journal_title：Annals of neurology

authors： Zhang J,Gregory S,Scahill RI,Durr A,Thomas DL,Lehericy S,Rees G,Tabrizi SJ,Zhang H,TrackOn-HD investigators.

更新日期：2018-10-01 00:00:00

abstract：:A prospective clinical and biochemical study on the effects of treatment with haloperidol has been performed in seven patients with Tourette syndrome. Pretreatment cerebrospinal fluid levels of homovanillic acid (CSF HVA) were significantly reduce in all patients, whereas 5-hydroxyindoleacetic acid was reduced in only...

journal_title：Annals of neurology

authors： Singer HS,Butler IJ,Tune LE,Seifert WE Jr,Coyle JT

更新日期：1982-10-01 00:00:00

abstract：:Guillain-Barré syndrome (GBS) is a recognized entity for which the basis for diagnosis is descriptive in our present state of knowledge. Diagnosis rests upon pattern recognition of the clinical picture plus other features including elevated cerebrospinal fluid protein level, electrophysiological changes of marked slow...

journal_title：Annals of neurology

authors： Asbury AK

更新日期：1981-01-01 00:00:00

abstract：OBJECTIVE:Epilepsy is a common neurological disorder characterized by recurrent seizures often unresponsive to pharmacological treatment. Brain inflammation is considered a crucial etiopathogenetic mechanism of epilepsy that could be targeted to control seizures. Specific inflammatory mediators overexpressed in human e...

journal_title：Annals of neurology

authors： Librizzi L,Noè F,Vezzani A,de Curtis M,Ravizza T

更新日期：2012-07-01 00:00:00

abstract：:A patient with generalized convulsions noted that seizures were reliably precipitated by mental arithmetic. The interictal electroencephalogram revealed only a mild, diffuse, nonspecific disturbance, but bursts of generalized epileptiform activity with no obvious clinical expression accompanied efforts at mental arith...

journal_title：Annals of neurology

authors： Wilkins AJ,Zifkin B,Andermann F,McGovern E

更新日期：1982-06-01 00:00:00