Cerebrospinal fluid biomarkers predict frontotemporal dementia trajectory.

Abstract:

Objective:The prognostic value of cerebrospinal fluid neurofilament light chain, total tau, phosphorylated tau181, and amyloid beta1-42 was examined in frontotemporal dementia subtypes. Methods:We compared baseline biomarkers between 49 controls, 40 patients with behavioral variant frontotemporal dementia, 24 with semantic variant primary progressive aphasia, and 26 with nonfluent variant primary progressive aphasia. Linear mixed effect models were used to assess the value of baseline biomarkers in predicting clinical and radiographic change in patient cohorts over multiple yearly follow up visits. Results:Neurofilament light chain concentrations were lowest in controls. Elevated baseline neurofilament light chain predicted faster worsening in clinical severity, frontotemporal volume and frontotemporal fractional anisotropy in patients with behavioral variant frontotemporal dementia and nonfluent variant primary progressive aphasia. High total tau similarly predicted faster progression in nonfluent variant primary progressive aphasia. In behavioral variant frontotemporal dementia, higher phosphorylated tau181 predicted faster clinical progression whereas lower amyloid beta1-42 predicted faster volumetric and fractional anisotropy reduction. Neurofilament light chain and phosphorylated tau181 were of greater predictive value in patients with tau pathology as compared to TDP-43 pathology. Baseline neurofilament light chain correlated with baseline clinical severity and frontotemporal volume in behavioral variant frontotemporal dementia. Baseline total tau correlated with baseline clinical severity in semantic variant primary progressive aphasia. Interpretation:High cerebrospinal fluid neurofilament light chain predicts more aggressive disease in behavioral variant frontotemporal dementia and nonfluent variant primary progressive aphasia. Total tau, phosphorylated tau181, and amyloid beta1-42 also predict some measures of disease aggressiveness in frontotemporal dementia.

journal_name

Ann Clin Transl Neurol

authors

Ljubenkov PA,Staffaroni AM,Rojas JC,Allen IE,Wang P,Heuer H,Karydas A,Kornak J,Cobigo Y,Seeley WW,Grinberg LT,Spina S,Fagan AM,Jerome G,Knopman D,Boeve BF,Dickerson BC,Kramer J,Miller B,Boxer AL,Rosen HJ

doi

10.1002/acn3.643

subject

Has Abstract

pub_date

2018-09-20 00:00:00

pages

1250-1263

issue

10

issn

2328-9503

pii

ACN3643

journal_volume

5

pub_type

杂志文章
  • Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis.

    abstract:Objective:Area postrema (AP) syndrome (defined as: nausea and/or emesis and/or singultus at onset of brainstem dysfunction) comprises complex pathophysiologic mechanisms triggered by different entities. The first objective was to assess the frequency of AP syndrome as a clinical feature in brainstem encephalitis (BE). ...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.666

    authors: Zeiner PS,Brandhofe A,Müller-Eschner M,Steinmetz H,Pfeilschifter W

    更新日期:2018-10-31 00:00:00

  • Age of onset determines intrinsic functional brain architecture in Friedreich ataxia.

    abstract:OBJECTIVE:Friedreich ataxia (FRDA) is the commonest hereditary ataxia in Caucasians. Most patients are homozygous for expanded GAA triplet repeats in the first intron of the frataxin (FXN) gene, involved in mitochondrial iron metabolism. Here, we used magnetoencephalography (MEG) to characterize the main determinants o...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.50966

    authors: Naeije G,Wens V,Coquelet N,Sjøgård M,Goldman S,Pandolfo M,De Tiège XP

    更新日期:2020-01-01 00:00:00

  • Impact of stroke mechanism in acute basilar occlusion with reperfusion therapy.

    abstract:Objective:We aimed to evaluate the impact of underlying mechanism of basilar artery (BA) occlusion on the outcomes after endovascular therapy (EVT) for reperfusion and the outcome factors associated with each mechanism, and to identify radiologic parameters enabling to distinguish the underlying mechanism. Methods:Fro...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.536

    authors: Lee WJ,Jung KH,Ryu YJ,Kim JM,Lee ST,Chu K,Kim M,Lee SK,Sohn CH,Roh JK

    更新日期:2018-01-30 00:00:00

  • The role of ventriculoperitoneal shunting in patients with supratentorial glioma.

    abstract:OBJECTIVES:To assess the impact of ventriculoperitoneal (VPS) in patients with glioma. METHODS:Retrospective review of patients with grade II-IV glioma who had VPS placement from January 1995 to November 2012. RESULTS:We identified 62 patients. At time of VPS, 41 had gait disturbance, 40 cognitive impairment and 16 u...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.17

    authors: de la Fuente MI,DeAngelis LM

    更新日期:2014-01-01 00:00:00

  • Serum neurofilament levels and patient-reported outcomes in multiple sclerosis.

    abstract:OBJECTIVE:Serum neurofilament light (sNfL) is a promising new biomarker in multiple sclerosis (MS). We explored the relationship between sNfL and health outcomes and resource use in MS patients. METHODS:MS patients with serum samples and health-outcome measurements collected longitudinally between 2011 and 2016 were a...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.51305

    authors: Galetta K,Deshpande C,Healy BC,Glanz B,Ziehn M,Saxena S,Paul A,Saleh F,Collins M,Gaitan-Walsh P,Castro-Mendoza P,Weiner HL,Chitnis T

    更新日期:2021-01-25 00:00:00

  • Biomarkers for REM sleep behavior disorder in idiopathic and narcoleptic patients.

    abstract::To search for discriminating biomarkers, 30 patients with idiopathic rapid-eye-movements sleep behavior disorder (iRBD) were compared with 17 patients with RBD within narcolepsy type 1. Both groups underwent extensive examinations, including skin biopsy searching for phosphorylated α-synuclein deposits and whole-night...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.50833

    authors: Antelmi E,Pizza F,Donadio V,Filardi M,Sosero YL,Incensi A,Vandi S,Moresco M,Ferri R,Marelli S,Ferini-Strambi L,Liguori R,Plazzi G

    更新日期:2019-09-01 00:00:00

  • Presymptomatic white matter integrity loss in familial frontotemporal dementia in the GENFI cohort: A cross-sectional diffusion tensor imaging study.

    abstract:Objective:We aimed to investigate mutation-specific white matter (WM) integrity changes in presymptomatic and symptomatic mutation carriers of the C9orf72,MAPT, and GRN mutations by use of diffusion-weighted imaging within the Genetic Frontotemporal dementia Initiative (GENFI) study. Methods:One hundred and forty muta...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.601

    authors: Jiskoot LC,Bocchetta M,Nicholas JM,Cash DM,Thomas D,Modat M,Ourselin S,Rombouts SARB,Dopper EGP,Meeter LH,Panman JL,van Minkelen R,van der Ende EL,Donker Kaat L,Pijnenburg YAL,Borroni B,Galimberti D,Masellis M,Tartagl

    更新日期:2018-07-11 00:00:00

  • Evaluation of SORD mutations as a novel cause of Charcot-Marie-Tooth disease.

    abstract::Biallelic mutations in the sorbitol dehydrogenase (SORD) encoding gene were recently identified as a common genetic cause in autosomal-recessive CMT patients. Here, we investigated the clinical, genetic, and electrophysiological characteristics of three CMT patients with biallelic SORD mutations from a Chinese cohort....

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.51268

    authors: Yuan RY,Ye ZL,Zhang XR,Xu LQ,He J

    更新日期:2021-01-01 00:00:00

  • CCL2 and CXCL10 are associated with poor outcome after intracerebral hemorrhage.

    abstract:Objective:Intracerebral hemorrhage carries a high mortality and survivors are frequently left with significant disability. Immunological mechanisms may play an important role in hemorrhage-induced brain injury, however, research linking these mechanisms with clinical outcome remains limited. We aim to identify serum in...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.595

    authors: Landreneau MJ,Mullen MT,Messé SR,Cucchiara B,Sheth KN,McCullough LD,Kasner SE,Sansing LH,Serum Markers After Spontaneous Cerebral Hemorrhage (SMASCH) Investigators.

    更新日期:2018-07-03 00:00:00

  • Systemic chemotherapy decreases brain glucose metabolism.

    abstract:OBJECTIVE:Cancer patients may experience neurologic adverse effects, such as alterations in neurocognitive function, as a consequence of chemotherapy. The mechanisms underlying such neurotoxic syndromes remain poorly understood. We here describe the temporal and regional effects of systemically administered platinum-ba...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.121

    authors: Horky LL,Gerbaudo VH,Zaitsev A,Plesniak W,Hainer J,Govindarajulu U,Kikinis R,Dietrich J

    更新日期:2014-10-01 00:00:00

  • Translocator protein in late stage Alzheimer's disease and Dementia with Lewy bodies brains.

    abstract:OBJECTIVE:Increased translocator protein (TSPO), previously known as the peripheral benzodiazepine receptor (PBR), in glial cells of the brain has been used as a neuroinflammation marker in the early and middle stages of neurodegenerative diseases, such as Alzheimer's disease (AD) and Dementia with Lewy Bodies (DLB). I...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.50837

    authors: Xu J,Sun J,Perrin RJ,Mach RH,Bales KR,Morris JC,Benzinger TLS,Holtzman DM

    更新日期:2019-08-01 00:00:00

  • New phenotype of DCTN1-related spectrum: early-onset dHMN plus congenital foot deformity.

    abstract:OBJECTIVE:To describe the clinical and genetic features of two patients with different phenotypes due to various Dynactin 1 (DCTN1) gene mutations and further explore the phenotype-genotype relationship. METHODS:Patient 1 is a 23-year-old man with congenital foot deformity and life-long distal muscle weakness and atro...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.50985

    authors: Tian WT,Liu LH,Zhou HY,Zhang C,Zhan FX,Zhu ZY,Chen SD,Luan XH,Cao L

    更新日期:2020-02-01 00:00:00

  • Predictors of response to opicinumab in acute optic neuritis.

    abstract:Objective:The objective of this study was to evaluate prespecified and post hoc analyses in RENEW subgroups to identify participants more likely to benefit from opicinumab. Methods:RENEW assessed the efficacy/safety of opicinumab versus placebo in participants with a first unilateral acute optic neuritis (AON) episode...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.620

    authors: Cadavid D,Balcer L,Galetta S,Aktas O,Ziemssen T,Vanopdenbosch LJ,Leocani L,Freedman MS,Plant GT,Preiningerova JL,Ziemssen F,Massacesi L,Chai Y,Xu L,RENEW Study Investigators.

    更新日期:2018-08-15 00:00:00

  • Repeat expansion scanning of the NOTCH2NLC gene in patients with multiple system atrophy.

    abstract:OBJECTIVE:Trinucleotide GGC repeat expansion in the 5'UTR of the NOTCH2NLC gene has been recognized as the pathogenesis of neuronal intranuclear inclusion disease (NIID). Previous studies have described that some NIID patients showed clinical and pathological similarities with multiple system atrophy (MSA). This study ...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.51021

    authors: Fang P,Yu Y,Yao S,Chen S,Zhu M,Chen Y,Zou K,Wang L,Wang H,Xin L,Hong T,Hong D

    更新日期:2020-04-01 00:00:00

  • Central role of Th2/Tc2 lymphocytes in pattern II multiple sclerosis lesions.

    abstract:OBJECTIVE:Multiple sclerosis (MS) is a disease of the central nervous system with marked heterogeneity in several aspects including pathological processes. Based on infiltrating immune cells, deposition of humoral factors and loss of oligodendrocytes and/or myelin proteins, four lesion patterns have been described. Pat...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.218

    authors: Planas R,Metz I,Ortiz Y,Vilarrasa N,Jelčić I,Salinas-Riester G,Heesen C,Brück W,Martin R,Sospedra M

    更新日期:2015-09-01 00:00:00

  • Accuracy of FGF-21 and GDF-15 for the diagnosis of mitochondrial disorders: A meta-analysis.

    abstract:OBJECTIVE:Given their diverse phenotypes, mitochondrial diseases (MDs) are often difficult to diagnose. Fibroblast growth factor 21 (FGF-21) and growth differentiation factor 15 (GDF-15) represent promising biomarkers for MD diagnosis. Herein we conducted a meta-analysis to compare their diagnostic accuracy for MDs. M...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.51104

    authors: Lin Y,Ji K,Ma X,Liu S,Li W,Zhao Y,Yan C

    更新日期:2020-07-01 00:00:00

  • Novel mutations in DNA2 associated with myopathy and mtDNA instability.

    abstract::The maintenance of mitochondrial DNA (mtDNA) relies on proteins encoded by nuclear genes. Mutations in their coding sequences result in heterogenous clinical presentations featuring mtDNA instability in affected tissues. DNA2 is a multi-catalytic protein involved in the removal of single strand DNA during mtDNA replic...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.50888

    authors: Ronchi D,Liu C,Caporali L,Piga D,Li H,Tagliavini F,Valentino ML,Ferrò MT,Bini P,Zheng L,Carelli V,Shen B,Comi GP

    更新日期:2019-09-01 00:00:00

  • Urate is closely linked to white matter integrity in multiple system atrophy.

    abstract:OBJECTIVE:We aimed to investigate the association of the serum urate level with cortical thickness and white matter integrity in multiple system atrophy (MSA). METHODS:We recruited 75 MSA patients and 42 controls who underwent brain MRI and measured serum urate level at baseline. Using cortical thickness and tract-bas...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.51073

    authors: Yoo HS,Chung SJ,Lee YH,Ye BS,Sohn YH,Kwon H,Lee PH

    更新日期:2020-06-01 00:00:00

  • The NRF2 pathway as potential biomarker for dimethyl fumarate treatment in multiple sclerosis.

    abstract:Objective:Immunological studies have demonstrated a plethora of beneficial effects of dimethyl fumarate (DMF) on various cell types. However, the cellular and molecular targets are incompletely understood and response markers are scarce. Here, we focus on the relation between nuclear factor (erythroid-derived 2)-like 2...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.553

    authors: Hammer A,Waschbisch A,Kuhbandner K,Bayas A,Lee DH,Duscha A,Haghikia A,Gold R,Linker RA

    更新日期:2018-04-16 00:00:00

  • Dissociated multimodal hubs and seizures in temporal lobe epilepsy.

    abstract:OBJECTIVE:Brain connectivity at rest is altered in temporal lobe epilepsy (TLE), particularly in "hub" areas such as the posterior default mode network (DMN). Although both functional and anatomical connectivity are disturbed in TLE, the relationships between measures as well as to seizure frequency remain unclear. We ...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.173

    authors: Douw L,DeSalvo MN,Tanaka N,Cole AJ,Liu H,Reinsberger C,Stufflebeam SM

    更新日期:2015-04-01 00:00:00

  • Aquatic training in MS: neurotherapeutic impact upon quality of life.

    abstract::Three fundamental principals associated with aquatic therapy differentiate it with respect to exercise on land, and in air. These are buoyancy (reduction in weight of the body within the buoyant medium of water), viscosity (a "drag force" is generated when moving within water, when compared with the same movement in a...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.220

    authors: Frohman AN,Okuda DT,Beh S,Treadaway K,Mooi C,Davis SL,Shah A,Frohman TC,Frohman EM

    更新日期:2015-08-01 00:00:00

  • Acute vitreoretinal trauma and inflammation after traumatic brain injury in mice.

    abstract:Objective:Limited attention has been given to ocular injuries associated with traumatic brain injury (TBI). The retina is an extension of the central nervous system and evaluation of ocular damage may offer a less-invasive approach to gauge TBI severity and response to treatment. We aim to characterize acute changes in...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.523

    authors: Evans LP,Newell EA,Mahajan M,Tsang SH,Ferguson PJ,Mahoney J,Hue CD,Vogel EW 3rd,Morrison B 3rd,Arancio O,Nichols R,Bassuk AG,Mahajan VB

    更新日期:2018-02-26 00:00:00

  • Optically pumped magnetoencephalography in epilepsy.

    abstract::We demonstrate the first use of Optically Pumped Magnetoencephalography (OP-MEG) in an epilepsy patient with unrestricted head movement. Current clinical MEG uses a traditional SQUID system, where sensors are cryogenically cooled and housed in a helmet in which the patient's head is fixed. Here, we use a different typ...

    journal_title:Annals of clinical and translational neurology

    pub_type:

    doi:10.1002/acn3.50995

    authors: Vivekananda U,Mellor S,Tierney TM,Holmes N,Boto E,Leggett J,Roberts G,Hill RM,Litvak V,Brookes MJ,Bowtell R,Barnes GR,Walker MC

    更新日期:2020-03-01 00:00:00

  • Early alteration of the locus coeruleus in phenotypic variants of Alzheimer's disease.

    abstract::Neuropathological studies showed early locus coeruleus (LC) neuronal loss associated with tauopathy in Alzheimer's Disease (AD). We used the LC signal intensity (LC-I) on 3T MRI to assess the LC integrity in AD (n = 37) and controls (n = 17). The LC-I was decreased in AD regardless of typical (amnesic) and atypical pr...

    journal_title:Annals of clinical and translational neurology

    pub_type: 临床试验,杂志文章

    doi:10.1002/acn3.50818

    authors: Olivieri P,Lagarde J,Lehericy S,Valabrègue R,Michel A,Macé P,Caillé F,Gervais P,Bottlaender M,Sarazin M

    更新日期:2019-07-01 00:00:00

  • POLG2 deficiency causes adult-onset syndromic sensory neuropathy, ataxia and parkinsonism.

    abstract:OBJECTIVE:Mitochondrial dysfunction plays a key role in the pathophysiology of neurodegenerative disorders such as ataxia and Parkinson's disease. We describe an extended Belgian pedigree where seven individuals presented with adult-onset cerebellar ataxia, axonal peripheral ataxic neuropathy, and tremor, in variable c...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.361

    authors: Van Maldergem L,Besse A,De Paepe B,Blakely EL,Appadurai V,Humble MM,Piard J,Craig K,He L,Hella P,Debray FG,Martin JJ,Gaussen M,Laloux P,Stevanin G,Van Coster R,Taylor RW,Copeland WC,Mormont E,Bonnen PE

    更新日期:2016-11-16 00:00:00

  • Plasma neuronal exosomal levels of Alzheimer's disease biomarkers in normal aging.

    abstract::Plasma neuronal exosomal levels of pathogenic Alzheimer's disease (AD) proteins, cellular survival factors, and lysosomal proteins distinguish AD patients from control subjects, but changes in these exosomal proteins associated with normal aging have not been described for cognitively intact subjects. Plasma neuronal ...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.309

    authors: Abner EL,Jicha GA,Shaw LM,Trojanowski JQ,Goetzl EJ

    更新日期:2016-04-13 00:00:00

  • The Multiple Faces of Spinocerebellar Ataxia type 2.

    abstract::Spinocerebellar ataxia type 2 (SCA2) is among the most common forms of autosomal dominant ataxias, accounting for 15% of the total families. Occurrence is higher in specific populations such as the Cuban and Southern Italian. The disease is caused by a CAG expansion in ATXN2 gene, leading to abnormal accumulation of t...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章,评审

    doi:10.1002/acn3.437

    authors: Antenora A,Rinaldi C,Roca A,Pane C,Lieto M,Saccà F,Peluso S,De Michele G,Filla A

    更新日期:2017-08-10 00:00:00

  • Suppression of agrin-22 production and synaptic dysfunction in Cln1 (-/-) mice.

    abstract:OBJECTIVE:Oxidative stress in the brain is highly prevalent in many neurodegenerative disorders including lysosomal storage disorders, in which neurodegeneration is a devastating manifestation. Despite intense studies, a precise mechanism linking oxidative stress to neuropathology in specific neurodegenerative diseases...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.261

    authors: Peng S,Xu J,Pelkey KA,Chandra G,Zhang Z,Bagh MB,Yuan X,Wu LG,McBain CJ,Mukherjee AB

    更新日期:2015-11-06 00:00:00

  • Heritability of alpha and sensorimotor network changes in temporal lobe epilepsy.

    abstract:OBJECTIVE:Electroencephalography (EEG) features in the alpha band have been shown to differ between people with epilepsy and healthy controls. Here, in a group of patients with mesial temporal lobe epilepsy (mTLE), we seek to confirm these EEG features, and using simultaneous functional magnetic resonance imaging, we i...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.51032

    authors: Yaakub SN,Tangwiriyasakul C,Abela E,Koutroumanidis M,Elwes RDC,Barker GJ,Richardson MP

    更新日期:2020-05-01 00:00:00

  • A single-center SCN8A-related epilepsy cohort: clinical, genetic, and physiologic characterization.

    abstract:OBJECTIVE:Pathogenic variants in SCN8A, encoding the voltage-gated sodium (Na+) channel α subunit Nav1.6, is a known cause of epilepsy. Here, we describe clinical and genetic features of all patients with SCN8A epilepsy evaluated at a single-tertiary care center, with biophysical data on identified Nav1.6 variants and ...

    journal_title:Annals of clinical and translational neurology

    pub_type: 杂志文章

    doi:10.1002/acn3.50839

    authors: Zaman T,Abou Tayoun A,Goldberg EM

    更新日期:2019-08-01 00:00:00