Surgical treatment of intractable neonatal-onset seizures: the role of positron emission tomography.

abstract：OBJECTIVE:Prospective memory (PM) describes the ability to fulfill previously planned intentions and is highly dependent on executive functions. Previous studies have shown deficits in executive functions in patients with juvenile myoclonic epilepsy (JME) and in their unaffected siblings. JME has a strong genetic predi...

journal_title：Neurology

authors： Wandschneider B,Kopp UA,Kliegel M,Stephani U,Kurlemann G,Janz D,Schmitz B

更新日期：2010-12-14 00:00:00

abstract：:A clinical procedure for determining contrast sensitivity was performed on patients with suspected sella area masses. The test consisted of a series of six plates, each containing a bar pattern of fixed sinusoidal spatial frequency in which contrast varies along the length of the bar. Patients with compressive lesions...

journal_title：Neurology

authors： Kupersmith MJ,Siegel IM,Carr RE

更新日期：1981-05-01 00:00:00

abstract：:Genetic factors are important in Alzheimer disease (AD) and Parkinson disease but have not been well characterized in Lewy body dementia (LBD). The authors obtained family history in patients from an autopsy series of AD and LBD and in living healthy controls. A family history of dementia was more common in both LBD a...

journal_title：Neurology

authors： Woodruff BK,Graff-Radford NR,Ferman TJ,Dickson DW,DeLucia MW,Crook JE,Arvanitakis Z,Brassler S,Waters C,Barker W,Duara R

更新日期：2006-06-27 00:00:00

abstract：OBJECTIVE:To characterize disease severity and distribution of disability in pediatric-onset multiple sclerosis (POMS) and to develop an optimized modeling scale for measuring disability, we performed a multicenter retrospective analysis of disability scores in 873 persons with POMS over time and compared this to previ...

journal_title：Neurology

authors： Santoro JD,Waltz M,Aaen G,Belman A,Benson L,Gorman M,Goyal MS,Graves JS,Harris Y,Krupp L,Lotze T,Mar S,Moodley M,Ness J,Rensel M,Rodriguez M,Schreiner T,Tillema JM,Waubant E,Weinstock-Guttman B,Hurtubise BF,Roal

更新日期：2020-09-29 00:00:00

abstract：BACKGROUND:Multiple sclerosis (MS) is a neurodegenerative disease associated with marked impairments in health-related quality of life (HRQoL). Although standard clinical end points such as the Expanded Disability Status Scale and annualized relapse rate remain useful in assessing MS activity and severity, these measur...

journal_title：Neurology

authors： Miller D,Rudick RA,Hutchinson M

更新日期：2010-04-27 00:00:00

abstract：:First-degree relatives of narcoleptic subjects (probands) may have sleep pathology related to the transmission of the disorder through their family members. The authors examined four groups: probands (n = 96), first-degree relative (n = 337), environmental reference (n = 85), and general population (n = 6,694) groups....

journal_title：Neurology

authors： Ohayon MM,Okun ML

更新日期：2006-08-22 00:00:00

abstract：OBJECTIVE:To evaluate the risk of recurrent intracranial hemorrhage (ICH) in patients on vitamin K antagonists (VKAs) after a first episode of ICH. METHODS:The Cerebral Haemorrhage in patients Restarting Oral Anticoagulant Therapy (CHIRONE) Study collected data of patients eligible for the study from the database of 2...

journal_title：Neurology

authors： Poli D,Antonucci E,Dentali F,Erba N,Testa S,Tiraferri E,Palareti G,Italian Federation of Anticoagulation Clinics (FCSA).

更新日期：2014-03-25 00:00:00

abstract：:We investigated the role of ceruloplasmin in the antioxidative process in the brain in a patient with hereditary ceruloplasmin deficiency (HCD). Immunohistochemistry revealed an accumulation of Nepsilon-(carboxymethyl) lysine (CML) in basal ganglia of the HCD brain. In vitro study disclosed that ceruloplasmin inhibite...

journal_title：Neurology

authors： Tajima K,Kawanami T,Nagai R,Horiuchi S,Kato T

更新日期：1999-08-11 00:00:00

abstract：:Retraction of the lower eyelid is a useful sign of disease. It is an early manifestation of weakness of the facial muscles, occurring with myopathies, myasthenia, and upper and lower motor neuron facial paresis. Rarely, lower and upper lid retraction occur without proptosis in patients with Graves disease. Lower lid r...

journal_title：Neurology

authors： Cohen MM,Lessell S

更新日期：1979-03-01 00:00:00

abstract：OBJECTIVE:To examine metabolism during exercise in 2 patients with muscle phosphorylase kinase (PHK) deficiency and to further define the phenotype of this rare glycogen storage disease (GSD). METHODS:Patient 1 (39 years old) had mild exercise-induced forearm pain, and EMG showed a myopathic pattern. Patient 2 (69 yea...

journal_title：Neurology

authors： Preisler N,Orngreen MC,Echaniz-Laguna A,Laforet P,Lonsdorfer-Wolf E,Doutreleau S,Geny B,Akman HO,Dimauro S,Vissing J

更新日期：2012-01-24 00:00:00

abstract：:The authors studied the tolerability of subcutaneous interferon beta-1a (IFNbeta-1a) in 51 patients with early-onset multiple sclerosis. The most frequent systemic adverse effects were flu-like symptoms in 65%. Laboratory abnormalities included asymptomatic leukopenia (27%) and elevated hepatic transaminases (35%). Tr...

journal_title：Neurology

authors： Pohl D,Rostasy K,Gärtner J,Hanefeld F

更新日期：2005-03-08 00:00:00

abstract：:Increased urinary levels of the polyamines, putrescine, spermidine, and spermine are present in Duchenne muscular dystrophy (DMD) patients. Alterations in excretion are detectable when based on nanomoles per milligram creatinine, micromoles per 24-hour urine collection, or nanomoles per kilogram body weight per 24-hou...

journal_title：Neurology

authors： Russell DH,Stern LZ

更新日期：1981-01-01 00:00:00

abstract：:Seven patients had pendular, vertical, isolated movements of the eyes soon after severe pontine strokes. The movements had a frequency of about 2 Hz and amplitudes of 3 to 40 degrees. Other rhythmic body movements at similar frequencies occurred in four patients after a delay of 6 weeks to 9 months. The character and ...

journal_title：Neurology

authors： Keane JR

更新日期：1986-01-01 00:00:00

abstract：OBJECTIVE:To combine MRI-based cortical morphometry and diffusion white matter tractography to describe the anatomical correlates of repetition deficits in patients with primary progressive aphasia (PPA). METHODS:The traditional anatomical model of language identifies a network for word repetition that includes Wernic...

journal_title：Neurology

authors： Forkel SJ,Rogalski E,Drossinos Sancho N,D'Anna L,Luque Laguna P,Sridhar J,Dell'Acqua F,Weintraub S,Thompson C,Mesulam MM,Catani M

更新日期：2020-02-11 00:00:00

abstract：:Functional decline for each decade at symptom onset and need for cane, walker, or wheelchair were assessed in 78 biopsy-proved patients with sporadic inclusion body myositis. Patients with disease onset between 40 and 59 years used a walker after 10.2 +/- 5.8 years, whereas those with disease onset between 60 and 79 y...

journal_title：Neurology

authors： Peng A,Koffman BM,Malley JD,Dalakas MC

更新日期：2000-07-25 00:00:00

abstract：:The authors carried out a three-phase door-to-door survey in Atahualpa, Ecuador to assess epidemiologic and pathogenetic mechanisms of stroke. They found 10 stroke patients among 1,568 individuals aged > or =15 years (crude prevalence, 638 per 100,000). There was only one incident case (incidence, 64 per 100,000). Six...

journal_title：Neurology

authors： Del Brutto OH,Idrovo L,Mosquera A,Navas C,Santibáñez R,Cuesta F,Díaz-Calderón E

更新日期：2004-11-23 00:00:00

abstract：:Neurologic complications of brain tissue-derived rabies vaccine may be chronic or progressive. Lymphocytes and serum from three patients with this form of encephalitis were studied. Two patients had positive lymphoproliferation to purified myelin, two had antibody to white matter, and one had antibody to myelin basic ...

journal_title：Neurology

authors： Hemachudha T,Griffin DE,Johnson RT,Giffels JJ

更新日期：1988-01-01 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：OBJECTIVE:Cerebral cavernous malformations (CCM) occur in familial and sporadic forms that cannot be distinguished by phenotype. Mutations in Krit1, a gene located at the CCM1 locus on chromosome 7q21, account for the majority of familial CCM cases. The authors investigated the role that mutations at the CCM1 locus pla...

journal_title：Neurology

authors： Reich P,Winkler J,Straube A,Steiger HJ,Peraud A

更新日期：2003-04-08 00:00:00

abstract：:The goal of pharmacologic therapy for patients with epilepsy is to suppress seizures without side effects. There is growing interest in developing antiepileptogenic drugs capable of preventing the onset of seizures in patients at high risk and inducing a permanent remission of seizures in patients with epilepsy, i.e.,...

journal_title：Neurology

authors： Schachter SC

更新日期：2002-11-12 00:00:00

abstract：:Although neurophysiologic doctrine has traditionally referred to "the" voltage-gated sodium channel, it is now clear that there are at least nine genes that encode molecularly and physiologically distinct sodium channels. Mutations of sodium channel genes provide a basis for genetic channelopathies. Dysregulated expre...

journal_title：Neurology

authors： Waxman SG

更新日期：2001-06-26 00:00:00

abstract：:Parry-Romberg syndrome (PRS) is a rare neurocutaneous disorder characterized by progressive facial hemiatrophy. In this study, 205 patients with PRS were surveyed using the Internet. Estimates of the frequency of limb involvement (19%), epilepsy (11%), and other clinical and etiologic features were obtained. There was...

journal_title：Neurology

authors： Stone J

更新日期：2003-09-09 00:00:00

abstract：OBJECTIVE:To analyze differences in functional brain images collected in patients with type 2 and type 1 narcolepsy compared to normal controls and the relationship among brain images, objective neuropsychologic tests, and sleep findings. METHODS:Data collection included comprehensive clinical investigation, study of ...

journal_title：Neurology

authors： Huang YS,Hsiao IT,Liu FY,Hwang FM,Lin KL,Huang WC,Guilleminault C

更新日期：2018-04-24 00:00:00

abstract：OBJECTIVE:To assess the role of CHCHD2 variants in patients with Parkinson disease (PD) and Lewy body disease (LBD) in Caucasian populations. METHODS:All exons of the CHCHD2 gene were sequenced in a US Caucasian patient-control series (878 PD, 610 LBD, and 717 controls). Subsequently, exons 1 and 2 were sequenced in a...

journal_title：Neurology

authors： Ogaki K,Koga S,Heckman MG,Fiesel FC,Ando M,Labbé C,Lorenzo-Betancor O,Moussaud-Lamodière EL,Soto-Ortolaza AI,Walton RL,Strongosky AJ,Uitti RJ,McCarthy A,Lynch T,Siuda J,Opala G,Rudzinska M,Krygowska-Wajs A,Barcikowska

更新日期：2015-12-08 00:00:00

abstract：:We have discovered a new Italian pedigree with autosomal-dominant ALS. The pedigree, at present, comprises 75 members distributed in five generations. ALS was diagnosed in eight patients. The mean +/- SD age of onset of the disease was 46.8 +/- 13.5 years, with a range of 29 to 63 years. The mean +/- SD duration of th...

journal_title：Neurology

authors： Rainero I,Pinessi L,Tsuda T,Vignocchi MG,Vaula G,Calvi L,Cerrato P,Rossi B,Bergamini L,McLachlan DR

更新日期：1994-02-01 00:00:00

abstract：:Serum ratios of monoclonal immunoglobulin (Ig) to total protein, monoclonal immunoglobulin to albumin, CSF/serum Ig-albumin index, and CSF to serum monoclonal immunoglobulin were obtained in 21 patients with multiple myeloma and 2 with Waldenström's macroglobulinemia. Twelve patients (3 with CNS complications) showed ...

journal_title：Neurology

authors： Hansotia P,Gani K,Friedenberg W

更新日期：1983-11-01 00:00:00

abstract：OBJECTIVE:The purpose of this cross-sectional study was to determine the prevalence and potential significance of stroke symptoms among end-stage renal disease (ESRD) patients without a prior diagnosis of stroke or TIA. METHODS:We enrolled 148 participants with ESRD from 5 clinics. Stroke symptoms and functional statu...

journal_title：Neurology

authors： Kurella Tamura M,Meyer JB,Saxena AB,Huh JW,Wadley VG,Schiller B

更新日期：2012-09-04 00:00:00

abstract：:We describe the clinical, pathologic, and ultrastructural findings in a case of juvenile onset neuronal ceroid-lipofuscinosis without visual symptoms or retinal abnormalities. The histochemical and ultrastructural characteristics of the neuronal lipopigment were similar to those in typical cases of neuronal ceroid-lip...

journal_title：Neurology

authors： Greenwood RS,Nelson JS

更新日期：1978-07-01 00:00:00

abstract：OBJECTIVE:To determine the minimum incidence and minimum prevalence rates of small-fiber neuropathy (SFN) in a well-defined region in the southern part of the Netherlands. METHODS:In this cross-sectional study with retrospective data collection, we used data of patients diagnosed with pure SFN at our Small Fiber Neuro...

journal_title：Neurology

authors： Peters MJ,Bakkers M,Merkies IS,Hoeijmakers JG,van Raak EP,Faber CG

更新日期：2013-10-08 00:00:00

abstract：OBJECTIVE:The assessment of behavioral disturbances in amyotrophic lateral sclerosis (ALS) is important because of the overlap with the behavioral variant of frontotemporal dementia (ALS-bvFTD). Motor symptoms and dysarthria are not taken into account in currently used behavioral questionnaires. We examined the clinime...

journal_title：Neurology

authors： Raaphorst J,Beeldman E,Schmand B,Berkhout J,Linssen WH,van den Berg LH,Pijnenburg YA,Grupstra HF,Weikamp JG,Schelhaas HJ,Papma JM,van Swieten JC,de Visser M,de Haan RJ

更新日期：2012-09-25 00:00:00