Most cases of dementia with hippocampal sclerosis may represent frontotemporal dementia.

abstract：BACKGROUND:X-linked adrenoleukodystrophy (X-ALD) has variants with widely different outcomes, hampering clinical counseling and evaluation of therapies. OBJECTIVE:To evaluate the degree to which MRI patterns can predict lesion progression. METHODS:Two hundred six boys and men with cerebral X-ALD (median age 12.2 year...

journal_title：Neurology

authors： Loes DJ,Fatemi A,Melhem ER,Gupte N,Bezman L,Moser HW,Raymond GV

更新日期：2003-08-12 00:00:00

abstract：OBJECTIVE:To investigate the association between early depressive behavior after stroke onset and occurrence of poststroke depression (PSD) at 3- and 12-month follow-up evaluations. METHODS:The study prospectively included 273 patients with first-ever single uncomplicated ischemic stroke. In the stroke unit, nurses sc...

journal_title：Neurology

authors： Carota A,Berney A,Aybek S,Iaria G,Staub F,Ghika-Schmid F,Annable L,Guex P,Bogousslavsky J

更新日期：2005-02-08 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：:We report a Jewish family of Yemenite origin in which three brothers born from a consanguineous marriage had juvenile parkinsonism. The DNA samples from three affected brothers and one healthy brother were analyzed for the linkage to markers covering the autosomal-recessive juvenile parkinsonism (AR-JP) locus. A perfe...

journal_title：Neurology

authors： Nisipeanu P,Inzelberg R,Blumen SC,Carasso RL,Hattori N,Matsumine H,Mizuno Y

更新日期：1999-10-22 00:00:00

abstract：:A case-control study was conducted to assess personal and family medical history and the appearance of Alzheimer's disease. We compared 98 men with clinically diagnosed Alzheimer's disease and 162 controls, matched by sex, year of birth, and town of residence. Family history of dementia and personal history of depress...

journal_title：Neurology

authors： Shalat SL,Seltzer B,Pidcock C,Baker EL Jr

更新日期：1987-10-01 00:00:00

abstract：OBJECTIVE:The authors imaged the medial longitudinal fasciculus (MLF) in 58 patients with MS and chronic internuclear ophthalmoparesis (INO) to determine which MRI technique best shows the characteristic lesion associated with this ocular motor syndrome. METHODS:Using quantitative infrared oculography, the authors det...

journal_title：Neurology

authors： Frohman EM,Zhang H,Kramer PD,Fleckenstein J,Hawker K,Racke MK,Frohman TC

更新日期：2001-09-11 00:00:00

abstract：OBJECTIVE:Current prehospital scales used to detect large vessel occlusion reveal very low endovascular thrombectomy (EVT) rates among selected patients. We developed a novel prehospital scale, the Madrid-Direct Referral to Endovascular Center (M-DIRECT), to identify EVT candidates for direct transfer to EVT-capable ce...

journal_title：Neurology

authors： Rodríguez-Pardo J,Riera-López N,Fuentes B,Alonso de Leciñana M,Secades-García S,Álvarez-Fraga J,Busca-Ostolaza P,Carneado-Ruiz J,Díaz-Guzmán J,Egido-Herrero J,Gil-Núñez A,Masjuan-Vallejo J,Real-Martínez V,Vivancos-Mora J,Díez

更新日期：2020-02-25 00:00:00

abstract：:A 47-year-old woman had myasthenia gravis with only moderate weakness and a consistently poor response to anticholinesterase medication. She was investigated with routine electrophysiologic studies and single-fiber electromyography. The studies showed a limited effect of anticholinesterase medication. There was a decr...

journal_title：Neurology

authors： Schwartz MS,Stålberg E

更新日期：1975-01-01 00:00:00

abstract：OBJECTIVE:Because age affects hormonal production differently in women compared with men, we sought to define sex and age interactions across a multiracial/ethnic population after intracerebral hemorrhage (ICH) to uncover evidence that loss of gonadal hormone production would result in loss of the known neuroprotective...

journal_title：Neurology

authors： James ML,Langefeld CD,Sekar P,Moomaw CJ,Elkind MSV,Worrall BB,Sheth KN,Martini SR,Osborne J,Woo D,ERICH Investigators.

更新日期：2017-09-05 00:00:00

abstract：:We determined the gene frequencies for the alleles of the HLA-linked complement markers C2, properdin factor B (BF), C4A (Rodgers) and C4B (Chido), and the red cell enzyme glyoxalase-I in 38 unrelated patients with senile dementia of the Alzheimer type, 42 patients with idiopathic Parkinson's disease, and 59 unaffecte...

journal_title：Neurology

authors： Nerl C,Mayeux R,O'Neill GJ

更新日期：1984-03-01 00:00:00

abstract：OBJECTIVE:To investigate clinical and economic consequences following generic substitution of one vs multiple generics of topiramate (Topamax; Ortho-McNeil Neurologics, Titusville, NJ). METHODS:Medical and pharmacy claims data of Régie de l'Assurance-Maladie du Québec from January 2006 to October 2007 were used. Patie...

journal_title：Neurology

authors： Duh MS,Paradis PE,Latrémouille-Viau D,Greenberg PE,Lee SP,Durkin MB,Wan GJ,Rupnow MF,LeLorier J

更新日期：2009-06-16 00:00:00

abstract：:We evaluated 85 patients with serologic evidence of Borrelia burgdorferi infection. Manifestations included encephalopathy (41), neuropathy (27), meningitis (2), multiple sclerosis (MS) (6), and psychiatric disorders (3). We performed lumbar punctures in 53, brain MRI in 33, and evoked potentials (EPs) in 33. Only pat...

journal_title：Neurology

authors： Halperin JJ,Luft BJ,Anand AK,Roque CT,Alvarez O,Volkman DJ,Dattwyler RJ

更新日期：1989-06-01 00:00:00

abstract：:The presence of the APOE epsilon 4 allele encoding apolipoprotein E4 (apoE4) is the major genetic risk factor for late-onset Alzheimer's disease (AD). However, the molecular and cellular mechanisms by which APOE epsilon 4 renders AD risk are unclear. In this report, we present genetic evidence that an apoE receptor, L...

journal_title：Neurology

authors： Kang DE,Saitoh T,Chen X,Xia Y,Masliah E,Hansen LA,Thomas RG,Thal LJ,Katzman R

更新日期：1997-07-01 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：OBJECTIVE:To characterize the clinical course of pathologically diagnosed hippocampal sclerosis dementia (HSD). BACKGROUND:Dementia associated with HSD is incompletely characterized. Previous studies suggest similarities to both Alzheimer disease (AD) and frontotemporal dementia (FTD). METHODS:Case-control analysis o...

journal_title：Neurology

authors： Blass DM,Hatanpaa KJ,Brandt J,Rao V,Steinberg M,Troncoso JC,Rabins PV

更新日期：2004-08-10 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 assess the available evidence for the diagnostic accuracy of CSF testing for protein 14-3-3 in patients with suspected sporadic Creutzfeldt-Jakob disease (sCJD). METHODS:The authors performed a systematic review of the available literature from 1995 to January 1, 2011, to identify articles involving patie...

journal_title：Neurology

authors： Muayqil T,Gronseth G,Camicioli R

更新日期：2012-10-02 00:00:00

abstract：:We determined the horizontal and vertical components of brain displacement on coronal MRIs in 10 patients with acute supratentorial masses. The vertical distances from the superior sagittal sinus to the pontomesencephalic junction (PMJ) and from the vertical midline of the brain to the PMJ did not differ from measurem...

journal_title：Neurology

authors： Ropper AH

更新日期：1989-05-01 00:00:00

abstract：OBJECTIVES:To estimate patient-acceptable symptom state (PASS) cut points for myasthenia gravis (MG) health scales. METHODS:We conducted an electronic survey that included the Myasthenia Gravis Impairment Index (MGII), EuroQol 5-Dimension (EQ5D), and a simple PASS question. PASS-anchored thresholds were estimated for ...

journal_title：Neurology

authors： Mendoza M,Tran C,Bril V,Katzberg HD,Barnett C

更新日期：2020-09-22 00:00:00

abstract：OBJECTIVE:To describe the phenotypic spectrum and genetics of periventricular nodular heterotopia (PNH) caused by FLN1 mutations in four men. BACKGROUND:X-linked PNH caused by FLN1 mutations (MIM #300049) implies prenatal or early postnatal lethality in boys and 50% recurrence risk in daughters of affected women. MET...

journal_title：Neurology

authors： Guerrini R,Mei D,Sisodiya S,Sicca F,Harding B,Takahashi Y,Dorn T,Yoshida A,Campistol J,Krämer G,Moro F,Dobyns WB,Parrini E

更新日期：2004-07-13 00:00:00

abstract：:The authors investigated whether the lack of effect of tirilazad on clinical outcome in patients with acute ischemic stroke is explained by failure of tirilazad to reduce infarct volume. Overall, tirilazad had no significant effect on infarct volume. In the subgroups of male patients and of those with a cortical infar...

journal_title：Neurology

authors： van der Worp HB,Kappelle LJ,Algra A,Bär PR,Orgogozo JM,Ringelstein EB,Bath PM,van Gijn J,TESS Investigators.,TESS II Investigators.

更新日期：2002-01-08 00:00:00

abstract：:Intracerebral hemorrhage (ICH) represents 8 to 15% of all strokes in the United States and 20 to 30% of all strokes in Japan and China. Although ICH represents a relatively small fraction of total strokes, it is a formidable disease, with a 30-day mortality rate two- to sixfold higher than that for ischemic stroke. Fu...

journal_title：Neurology

authors： Fayad PB,Awad IA

更新日期：1998-09-01 00:00:00

abstract：:Mutations in DJ-1 are a cause of autosomal recessive parkinsonism. Polymorphism of genes implicated in hereditary forms of parkinsonism may be a predisposing factor in sporadic Parkinson's disease (PD). The authors analyzed whether a polymorphism (g.168_185del) within exon 1 of DJ-1 contributes to the risk of sporadic...

journal_title：Neurology

authors： Eerola J,Hernandez D,Launes J,Hellström O,Hague S,Gulick C,Johnson J,Peuralinna T,Hardy J,Tienari PJ,Singleton AB

更新日期：2003-10-14 00:00:00

abstract：:We describe the clinical, imaging, and pathologic findings in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). The patient experienced her first stroke-like episode at age forty-four. Brain MRI, obtained at symptom onset, at 3 weeks, and at 1 year, revealed migr...

journal_title：Neurology

authors： Clark JM,Marks MP,Adalsteinsson E,Spielman DM,Shuster D,Horoupian D,Albers GW

更新日期：1996-01-01 00:00:00

abstract：OBJECTIVES:To examine the illusional sensation of movement evoked by vibration of an immobilized arm. METHODS:Patients with idiopathic focal dystonia were compared with those with idiopathic PD and with patients with dystonia secondary to PD. A 100-Hz transcutaneous vibratory stimulus was applied to the biceps brachii...

journal_title：Neurology

authors： Rome S,Grünewald RA

更新日期：1999-11-10 00:00:00

abstract：OBJECTIVE:Dravet syndrome is a rare neurodevelopmental disorder characterized by seizures and other neurologic problems. SCN1A mutations account for ∼80% of cases. Animal studies have implicated mutation-related dysregulated cortical inhibitory networks in its pathophysiology. We investigated such networks in people wi...

journal_title：Neurology

authors： Stern WM,Sander JW,Rothwell JC,Sisodiya SM

更新日期：2017-04-25 00:00:00

abstract：OBJECTIVES:More than 30 different rare mutations, including copy number variants (CNVs), in the amyloid precursor protein gene (APP) cause early-onset familial Alzheimer disease (EOFAD), whereas the contribution of common APP variants to disease risk remains controversial. In this study we systematically assessed the r...

journal_title：Neurology

authors： Hooli BV,Mohapatra G,Mattheisen M,Parrado AR,Roehr JT,Shen Y,Gusella JF,Moir R,Saunders AJ,Lange C,Tanzi RE,Bertram L

更新日期：2012-04-17 00:00:00

abstract：:Family studies of primary torsion dystonia have used the diagnostic categories of definite, probable, and possible dystonia for gene mapping and identification, but the validity of this hierarchical classification is not known. The authors assessed 147 DYT1 GAG deletion carriers and 113 blood-related noncarriers from ...

journal_title：Neurology

authors： Bressman SB,Raymond D,Wendt K,Saunders-Pullman R,De Leon D,Fahn S,Ozelius L,Risch N

更新日期：2002-12-10 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：BACKGROUND:Infection with HIV can result in a debilitating CNS disorder known as HIV dementia (HIV-D). Since the advent of highly active antiretroviral therapy (HAART), the incidence of HIV-D has declined, but the prevalence continues to increase. In this new era of HIV-D, traditional biomarkers such as CSF viral load ...

journal_title：Neurology

authors： Bandaru VV,McArthur JC,Sacktor N,Cutler RG,Knapp EL,Mattson MP,Haughey NJ

更新日期：2007-05-01 00:00:00