Natural history and predictors of survival in progressive supranuclear palsy.

Abstract:

BACKGROUND:Progressive supranuclear palsy is a neurodegenerative disorder characterized by high functional disability and rapidly progressive dependency. The predictors of survival are still unclear. METHODS:The predictors of survival were evaluated in a group of clinically diagnosed PSP patients, focusing primarily on extensive cognitive assessment. RESULTS:The mean survival time from symptom onset was 8.25±3.0years. Sex, age at onset, education, occupation and severity of extrapyramidal symptoms did not correlate with survival. The only factor associated with a shorter life expectancy in our cohort was the presence of dementia at diagnosis. Impairment of executive functions was the best predictor of an unfavorable outcome. CONCLUSIONS:Our findings suggest that dementia and executive functions need to be evaluated in order to define survival probability in PSP patients.

journal_name

J Neurol Sci

authors

Cosseddu M,Benussi A,Gazzina S,Manes MA,Dell'Era V,Cristillo V,Turrone R,Alberici A,Borroni B

doi

10.1016/j.jns.2017.09.043

subject

Has Abstract

pub_date

2017-11-15 00:00:00

pages

105-107

eissn

0022-510X

issn

1878-5883

pii

S0022-510X(17)34349-6

journal_volume

382

pub_type

杂志文章
  • Depletion of mitochondrial DNA in leukocytes of patients with poly-Q diseases.

    abstract::Polyglutamine (poly-Q) diseases are late-onset neurodegenerative disorders arising from the expansion of an unstable CAG repeat in the affected gene, which is translated to a tract of glutamine residues. This kind of mutant proteins may be aggregated and accumulated, and thereby enhance cellular oxidative stress. In o...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2007.07.016

    authors: Liu CS,Cheng WL,Kuo SJ,Li JY,Soong BW,Wei YH

    更新日期:2008-01-15 00:00:00

  • Accelerometer cut-points derived during over-ground walking in persons with mild, moderate, and severe multiple sclerosis.

    abstract:BACKGROUND:There has been increased interest in objectively quantifying time spent in moderate-to-vigorous physical activity (MVPA) using accelerometry as an outcome among persons with multiple sclerosis (MS). This requires development of a cut-point for interpreting the rate of accelerometer output based on its associ...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2014.02.024

    authors: Sandroff BM,Riskin BJ,Agiovlasitis S,Motl RW

    更新日期:2014-05-15 00:00:00

  • No association between APOE ε 4 allele and multiple sclerosis susceptibility: a meta-analysis from 5472 cases and 4727 controls.

    abstract:BACKGROUND:Apolipoprotein E (APOE) gene ε4, 2 alleles have been reported to be associated with multiple sclerosis (MS), but results were conflicting. In order to derive a more precise estimation of the associations, a meta-analysis was performed. METHODS:The PubMed, EBSCO and BIOSIS databases were searched to identify...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章,meta分析

    doi:10.1016/j.jns.2011.05.040

    authors: Xuan C,Zhang BB,Li M,Deng KF,Yang T,Zhang XE

    更新日期:2011-09-15 00:00:00

  • Cognitive conditions of pathologically confirmed dementia with Lewy bodies and Parkinson's disease with dementia.

    abstract::The relationship between dementia with Lewy bodies (DLB) and Parkinson's disease with dementia (PDD) has been insufficiently described, and it is still problematic. Twenty-nine cases of DLB and 10 cases of PDD were investigated in the present study. DLB cases disclosed a significantly older disease onset and shorter d...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(03)00220-x

    authors: Horimoto Y,Matsumoto M,Nakazawa H,Yuasa H,Morishita M,Akatsu H,Ikari H,Yamamoto T,Kosaka K

    更新日期:2003-12-15 00:00:00

  • Reliability of TMS-related measures of tibialis anterior muscle in patients with chronic stroke and healthy subjects.

    abstract::A lack of normative data for transcranial magnetic stimulation (TMS)-related measures of the lower limb muscles in patients with stroke prevents us from understanding whether changes in TMS-related measures are induced by treatment or are due to their variability and/or the natural evolution of the disease. The purpos...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2011.01.004

    authors: Cacchio A,Paoloni M,Cimini N,Mangone M,Liris G,Aloisi P,Santilli V,Marrelli A

    更新日期:2011-04-15 00:00:00

  • Sporadic adult-onset distal myopathy with rimmed vacuoles, 15-18 nm tubulofilaments and extensive rod formation.

    abstract::Starting after the age of 35 years this German man had a slowly progressive distal myopathy greater in the legs than in the arms. First he realized gait difficulties with reduced ankle dorsiflexion. Serum creatine kinase activity was normal. Muscle biopsy studies showed myopathic changes, rimmed vacuoles and the prese...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(96)00264-x

    authors: Sieb JP,VonOertzen J,Tolksdorf K,Dörfler P,Kappes-Horn K,Jerusalem F

    更新日期:1997-02-27 00:00:00

  • Refractory status epilepticus secondary to CNS vasculitis, a role for epilepsy surgery.

    abstract::Central nervous system (CNS) vasculitis is a rare group of disorders that affect vessels of the brain parenchyma and meninges. It presents with headache, cognitive changes, or seizures, yet without aggressive management, it carries a high degree of morbidity and mortality. Refractory status epilepticus (SE) has been r...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2011.11.029

    authors: Atkinson M,Atkinson B,Norris G,Shah A

    更新日期:2012-04-15 00:00:00

  • Cup to disc ratio by optical coherence tomography is abnormal in multiple sclerosis.

    abstract:OBJECTIVE:To identify and characterize cup to disc ratio (CDR) and related optic nerve head abnormalities in multiple sclerosis (MS) using spectral domain optical coherence tomography (OCT). BACKGROUND:While CDR is routinely assessed by ophthalmologists in the evaluation of glaucoma, CDR and related optic nerve head m...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2010.12.011

    authors: Syc SB,Warner CV,Saidha S,Farrell SK,Conger A,Bisker ER,Wilson J,Frohman TC,Frohman EM,Balcer LJ,Calabresi PA

    更新日期:2011-03-15 00:00:00

  • Platelet APP isoform ratios in asymptomatic young adults expressing an AD-related presenilin-1 mutation.

    abstract::The Alzheimer's disease (AD) related amyloid precursor protein (APP) is stored, cleaved and released similarly from neurons and from platelets. We have reported that the proportion of 120-130 to 110 kDa carboxyl-cleaved APP present in the platelets of AD patients is significantly lower than that of platelets of age-ma...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(00)00483-4

    authors: Baskin F,Rosenberg RN,Iyer L,Schellenberg GD,Hynan L,Nee LE

    更新日期:2001-01-15 00:00:00

  • Rare novel CYP2U1 and ZFYVE26 variants identified in two Pakistani families with spastic paraplegia.

    abstract:BAKGROUND:Hereditary Spastic paraplegias (HSPs) are a clinically and genetically heterogeneous group of degenerative disorders characterized by progressive spasticity and weakness of the lower limbs. This study aimed to identify causative gene variants in two unrelated consanguineous Pakistani families presented with 2...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2020.116669

    authors: Bibi F,Efthymiou S,Bourinaris T,Tariq A,Zafar F,Rana N,Salpietro V,Houlden H,Raja GK,SYNaPS Study Group.,Saeed S,Minhas NM

    更新日期:2020-04-15 00:00:00

  • Neuropathological evaluation and apolipoprotein E gene polymorphism analysis in diffuse Lewy body disease.

    abstract::We performed a quantitative neuropathological investigation of senile plaques and neurofibrillary tangles in the brains of 14 Japanese patients with diffuse Lewy body disease (DLBD), and examined apolipoprotein E (APOE) gene polymorphism in these patients. Most DLBD brains had as many senile plaques as those with Alzh...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(95)00312-p

    authors: Kawanishi C,Suzuki K,Odawara T,Iseki E,Onishi H,Miyakawa T,Yamada Y,Kosaka K,Kondo N,Yamamoto T

    更新日期:1996-03-01 00:00:00

  • Trends in epilepsy diagnosis and surgery in western China during 2009-2017.

    abstract:OBJECTIVE:Despite the strong evidence for the efficacy of surgery to treat temporal lobe epilepsy, the number of such surgeries has plateaued in western countries. This study examined trends in the number of epilepsy cases diagnosed, number of surgeries performed, and certain characteristics of surgery patients in west...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2019.06.026

    authors: Qiu X,Lai W,Kinoshita M,Zhang L,Li H,Peng A,Li W,Chen L

    更新日期:2019-08-15 00:00:00

  • Longitudinal course of mild parkinsonian signs in elderly people: A population-based study in Japan.

    abstract::We aimed to clarify the longitudinal course of mild parkinsonian signs (MPS) and their association with dementia and functional disability by conducting a comprehensive epidemiological study, including brain MRI, and assessments of cognition, depression, and sleep, in people aged ≥65years living in Ama-cho. We diagnos...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2016.01.016

    authors: Wada-Isoe K,Tanaka K,Uemura Y,Nakashita S,Tajiri Y,Tagashira S,Yamamoto M,Yamawaki M,Kishi M,Nakashima K

    更新日期:2016-03-15 00:00:00

  • Comparative efficacy and safety of multiple antiplatelet therapies for secondary prevention of ischemic stroke or transient ischemic attack: A network meta-analysis.

    abstract:BACKGROUND:Antiplatelet therapies for secondary prevention of ischemic stroke or transient ischemic attack (TIA) is a highly active research topic with five critical drugs obtained by visual analysis. We aimed to compare and rank multiple antiplatelet therapies using a network meta-analysis. METHODS:Relevant medical d...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章,meta分析

    doi:10.1016/j.jns.2019.02.037

    authors: Xiang RW,Han RB,Yang JY,Zhao MY,Zhao QC,Chen HS,Zhao FQ,Sun ZY,Zhao T,Song TY

    更新日期:2019-04-15 00:00:00

  • Single dose of methylphenidate improves cognitive performance in multiple sclerosis patients with impaired attention process.

    abstract:BACKGROUND AND PURPOSE:Attention is one of the major cognitive domains adversely affected in multiple sclerosis (MS). The aim of the current study was to determine the effect of a single dose of methylphenidate on cognitive performance of MS patients with significant attention deficit. METHODS:In a double-blind placeb...

    journal_title:Journal of the neurological sciences

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1016/j.jns.2008.08.025

    authors: Harel Y,Appleboim N,Lavie M,Achiron A

    更新日期:2009-01-15 00:00:00

  • Epstein-Barr virus and multiple sclerosis.

    abstract::Epstein-Barr virus (EBV) is a human DNA herpesvirus infecting more than 90% of the world's population. EBV is the etiological agent of infectious mononucleosis (Pfeiffer's disease). Furthermore, diverse malignancies such as Burkitt and Hodgkin lymphoma have been associated with EBV. More recently, a possible role for ...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章,评审

    doi:10.1016/j.jns.2009.03.028

    authors: Pohl D

    更新日期:2009-11-15 00:00:00

  • Primary angiitis of the central nervous system: report of eight cases from a single Italian center.

    abstract::The primary angiitis of the central nervous system (PACNS) is a rare and potentially fatal form of vasculitis with unknown etiology. Headache and encephalopathy are the most frequent symptoms. Neuroimaging plays an important role in the diagnosis, but the pattern of abnormal findings is not specific. In some cases bra...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2011.05.014

    authors: Pizzanelli C,Catarsi E,Pelliccia V,Cosottini M,Pesaresi I,Puglioli M,Moretti P,Tavoni A

    更新日期:2011-08-15 00:00:00

  • Hydroxychloroquine reduces microglial activity and attenuates experimental autoimmune encephalomyelitis.

    abstract:BACKGROUND:Microglial activation is thought to be a key pathophysiological mechanism underlying disease activity in all forms of MS. Hydroxychloroquine (HCQ) is an antimalarial drug with immunomodulatory properties that is widely used in the treatment of rheumatological diseases. In this series of experiments, we explo...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2015.08.1525

    authors: Koch MW,Zabad R,Giuliani F,Hader W Jr,Lewkonia R,Metz L,Wee Yong V

    更新日期:2015-11-15 00:00:00

  • Epidermal innervation: changes with aging, topographic location, and in sensory neuropathy.

    abstract::In previous work we demonstrated little effect of aging on the density and spatial pattern of epidermal innervation, however, this was restricted to two sites proximal and distal in the leg. To expand on these observations, we used punch skin biopsy in ten healthy controls to examine the variation in intra-epidermal n...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(99)00063-5

    authors: Lauria G,Holland N,Hauer P,Cornblath DR,Griffin JW,McArthur JC

    更新日期:1999-04-01 00:00:00

  • Visual deprivation elicits subclinical postural inflexibilities in early Parkinson's disease.

    abstract:BACKGROUND:Postural instability is often experienced in the late stages of PD and is a marker of disease progression. Little information is available on the role of visual inputs as an adaptive strategy to compensate for postural instability in PD. The purpose of this study was to determine visual dependency for postur...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2015.01.022

    authors: Panyakaew P,Anan C,Bhidayasiri R

    更新日期:2015-02-15 00:00:00

  • Efficacy of low-dose, add-on therapy of clobazam (CLB) is produced by its major metabolite, N-desmethyl-CLB.

    abstract::We evaluated the efficacy of low-dose, add-on therapy of CLB in adults with refractory epilepsy. 28 patients were included: 12 with temporal lobe epilepsy (TLE), 14 with extratemporal lobe epilepsy (ETLE) and 2 with symptomatic generalized epilepsy (SGE). CLB was added with the initial dose of 2.5 mg/day and increased...

    journal_title:Journal of the neurological sciences

    pub_type: 临床试验,杂志文章

    doi:10.1016/j.jns.2007.05.025

    authors: Kinoshita M,Ikeda A,Begum T,Terada K,Shibasaki H

    更新日期:2007-12-15 00:00:00

  • Friedreich ataxia with minimal GAA expansion presenting as adult-onset spastic ataxia.

    abstract::Around a quarter of Friedreich ataxia (FA) patients, despite being homozygous for GAA expansion within the FRDA gene, show atypical presentations. Our aim is to describe the case of three brothers with long-term follow-up suffering from late onset FA manifested with spastic ataxia. The three patients belong to a famil...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(01)00681-5

    authors: Berciano J,Mateo I,De Pablos C,Polo JM,Combarros O

    更新日期:2002-02-15 00:00:00

  • Peripheral inflammation is associated with decreased veratridine-induced release of GABA in the rat ventrocaudal periaqueductal gray: microdialysis study.

    abstract::Systemic administration of opiates or direct injection of opioid peptides into the periaqueductal gray (PAG) produces a profound antinociception which is thought to be associated with inhibition of neuronal activity in the PAG. This inhibitory effect has been postulated to result from opiate inhibition of GABAergic ne...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/s0022-510x(98)00327-x

    authors: Renno WM,Beitz AJ

    更新日期:1999-03-01 00:00:00

  • Effect of immunotherapy in myelitis with atopic diathesis.

    abstract:OBJECTIVE:A recent nationwide survey of myelitis with atopic diathesis in Japan disclosed that the disease frequently shows a chronic persistent course. A neuropathological study of the spinal cord also revealed chronic active inflammation. Since the effects of various immunotherapies have not been studied extensively ...

    journal_title:Journal of the neurological sciences

    pub_type: 临床试验,杂志文章

    doi:10.1016/j.jns.2004.08.001

    authors: Murai H,Arahata H,Osoegawa M,Ochi H,Minohara M,Taniwaki T,Tobimatsu S,Mihara F,Tsuruta Y,Inaba S,Kira J

    更新日期:2004-12-15 00:00:00

  • New molecular findings in congenital myopathies due to selenoprotein N gene mutations.

    abstract::Selenoprotein N-related myopathy (SEPN1-RM) is an early-onset muscle disorder that can manifest clinically as congenital muscular dystrophy with spinal rigidity and can result in specific pathological entities such as multiminicore disease, desmin-related myopathy with Mallory body-like inclusions, and congenital fibe...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2010.09.011

    authors: Cagliani R,Fruguglietti ME,Berardinelli A,D'Angelo MG,Prelle A,Riva S,Napoli L,Gorni K,Orcesi S,Lamperti C,Pichiecchio A,Signaroldi E,Tupler R,Magri F,Govoni A,Corti S,Bresolin N,Moggio M,Comi GP

    更新日期:2011-01-15 00:00:00

  • Spontaneous and evoked electrical discharges from a central demyelinating lesion.

    abstract::Recordings have been made from afferent fibres shown to traverse a focal demyelinating lesion induced in the dorsal columns of the cat by the direct micro-injection of lysophosphatidyl choline. Many fibres were spontaneously active, discharging with steady frequencies between 15 and 45 impulses per second or dischargi...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(82)90168-x

    authors: Smith KJ,McDonald WI

    更新日期:1982-07-01 00:00:00

  • Intrathecal upregulation of IFN-gamma and MIP-1beta in juvenile muscular atrophy of the distal upper extremity.

    abstract::Juvenile muscular atrophy of the distal upper extremity (JMADUE) is associated with airway allergy and hyperIgEaemia, suggesting the involvement of immunological processes. In this study we aimed to clarify the changes in various cytokines/chemokines in cerebrospinal fluid (CSF) from JMADUE patients. We simultaneously...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2008.07.020

    authors: Tanaka M,Ishizu T,Ochi H,Kawano Y,Ohyagi Y,Kira J

    更新日期:2008-12-15 00:00:00

  • Association of interleukin-6-572G/C gene polymorphisms in the Cantonese population with intracranial aneurysms.

    abstract::Recent studies have demonstrated that cytokines play an important role in the pathogenesis of intracranial aneurysms (IAs). Interleukin-6 (IL-6) is an important proinflammatory cytokine. In our study, we investigated the association of genetic variants within the gene encoding interleukin-6-572G/C (IL-6-572G/C) with I...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/j.jns.2011.03.036

    authors: Zhang G,Tu Y,Feng W,Huang L,Li M,Qi S

    更新日期:2011-07-15 00:00:00

  • Safety of nitrous oxide administration in patients with Charcot-Marie-Tooth disease.

    abstract::Nitrous oxide is routinely administered to children and adults with Charcot-Marie-Tooth disease (CMT) as an anaesthetic for procedures such as nerve conduction studies and maintenance for general anaesthesia. However it is listed as a 'moderate to significant' risk of potential toxicity and worsening neuropathy in peo...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章,评审

    doi:10.1016/j.jns.2007.12.004

    authors: Isbister GK,Burns J,Prior F,Ouvrier RA

    更新日期:2008-05-15 00:00:00

  • Methods for producing a reproducible crush in the sciatic and tibial nerve of the rat and rapid and precise testing of return of sensory function. Beneficial effects of melanocortins.

    abstract::A procedure for placing a crush lesion in the sciatic and tibial nerve of the rat based on anatomical landmarks is described. These crush lesions are used to study the process of regeneration of peripheral nervous tissue and the beneficial effects of melanocortins on speed and quality of nerve regeneration. A new prec...

    journal_title:Journal of the neurological sciences

    pub_type: 杂志文章

    doi:10.1016/0022-510x(86)90109-7

    authors: De Koning P,Brakkee JH,Gispen WH

    更新日期:1986-07-01 00:00:00