Abstract:
BACKGROUND:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that still lacks reliable diagnostic biomarkers. This study aims to evaluate the diagnostic and prognostic potential of CSF total Tau (t-Tau), phospho-Tau (p-Tau) and p-Tau/t-Tau ratio in ALS patients using CSF neurofilament light (NFL) as the reference biomarker. METHODS:Eighty-five incident ALS, 30 ALS-mimicking (AM) diseases and 51 other non-neurodegenerative diseases (ONND) were included in the study. RESULTS:ALS patients had higher levels of CSF t-Tau and lower p-Tau/t-Tau ratio than AM (p = 0.005 and p = 0.006) and ONND (p < 0.001). CSF t-Tau levels discriminated ALS from AM with a sensitivity of 69% and specificity of 60%, and from ONND with a sensitivity of 88% and specificity of 51%. These values were lower than the accuracy of CSF NFL in ALS (sensitivity 86% and specificity 87% in distinguishing ALS from AM and sensitivity 83% and specificity 75% from ONND); CSF t-Tau correlated with progression rate and SNIP. CSF p-Tau did not show relation with any ALS clinical features. CSF NFL significantly correlated with all considered clinical parameters. High levels of CSF t-Tau and NFL were related to poor survival. CONCLUSION:CSF t-Tau showed no reliable diagnostic significance but the relation between the high levels of CSF t-Tau and short survival suggests the potential prognostic role of this biomarker in ALS. However, CSF NFL was confirmed to be the most reliable and efficient tool for diagnosis and prediction of clinical progression and survival in ALS patients.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Scarafino A,D'Errico E,Introna A,Fraddosio A,Distaso E,Tempesta I,Morea A,Mastronardi A,Leante R,Ruggieri M,Mastrapasqua M,Simone ILdoi
10.1007/s00415-018-9008-3subject
Has Abstractpub_date
2018-10-01 00:00:00pages
2353-2362issue
10eissn
0340-5354issn
1432-1459pii
10.1007/s00415-018-9008-3journal_volume
265pub_type
杂志文章abstract::Brains from 54 patients with organic dementia were examined systematically. As in previous investigations a predominance of Alzheimer type changes was observed. Seventeen patients showed Lewy bodies in the nucleus basalis, the substantia nigra and the locus coeruleus as well as other lesions of parkinsonian type. In 5...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313858
更新日期:1986-04-01 00:00:00
abstract::Production of reactive oxygen species after cerebral blood flow disruption may enhance tissue damage through multiple molecular pathways. Changes in nitric oxide (NO) metabolism and oxidative stress status were investigated in 47 patients with ischemic stroke by measuring plasma nitric oxide (NO) and peroxynitrite (ON...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-007-0700-y
更新日期:2008-01-01 00:00:00
abstract:BACKGROUND:Dermic fibroblasts have been proposed as a potential genetic-ALS cellular model. This study aimed to explore whether dermic fibroblasts from patients with sporadic-ALS (sALS) recapitulate alterations typical of ALS motor neurons and exhibit abnormal DNA-damage response. METHODS:Dermic fibroblasts were obtai...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09704-8
更新日期:2020-05-01 00:00:00
abstract:OBJECTIVE:Childhood arterial ischaemic stroke (AIS) is rare, but causes significant morbidity and mortality. We aimed to investigate incidence, age-dependent clinical presentation, and risk factors and to discuss the medical care situation in Germany. METHODS:This prospective epidemiological study was conducted via ES...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09508-5
更新日期:2019-12-01 00:00:00
abstract::Eye movements were studied in 13 patients with Friedreich's ataxia and correlated with MRI findings to investigate whether oculomotor abnormalities can be traced to cerebellar disturbances in this disease. One of the most prominent eye signs was fixation instability (square-wave jerks, SWJ.). Besides SWJ the patients ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00867423
更新日期:1995-08-01 00:00:00
abstract::The bulbocavernosus reflex (BCR) was examined in 39 normal potent men and in 252 patients with impaired potency of varying aetiology. For BCR evaluation minimum, maximum and mean latencies, the temporal dispersion in ten successive responses, together with minimum and maximum side differences from simultaneous recordi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314350
更新日期:1988-03-01 00:00:00
abstract::A major challenge in the diagnosis of disorders of consciousness is the differential diagnosis between the vegetative state (VS) and the minimally conscious state (MCS). Clinically, VS is defined by complete unawareness, whereas MCS is defined by the presence of inconsistent but clearly discernible behavioural signs o...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6734-9
更新日期:2013-04-01 00:00:00
abstract::Two cases of Addison's disease associated with myasthenia gravis are reported. This association has been described only rarely in the literature. In the first case, there were marked immunological abnormalities. It is most likely that the origin of the adrenocortical insufficiency in the second case is tuberculous. Th...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313835
更新日期:1985-01-01 00:00:00
abstract:OBJECTIVES:Lumbar spondylosis (LS) is a common spinal degenerative disorder which causes various types of lower urinary tract dysfunction (LUTD). However, it is not certain whether LS may cause urinary retention in elderly women. METHODS:In a period covering the past 3 years, we retrospectively reviewed: a) urodynamic...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0790-3
更新日期:2005-08-01 00:00:00
abstract::Genetic evidence indicates a central role of cerebral accumulation of beta-amyloid (Abeta) in the pathogenesis of Alzheimer's disease (AD). Beside presenilin 1 and 2, three other recently discovered proteins (Aph 1, PEN 2 and nicastrin) are associated with gamma-secretase activity, the enzymatic complex generating Abe...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0799-7
更新日期:2005-09-01 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which was thought to be untreatable. However, recent evidence in both experimental animals and men indicates that antiglutamatergic strategies are the first to have an influence on its pathogenesis and slow down the disease process. Since the eff...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF03161074
更新日期:2000-12-01 00:00:00
abstract::Atrial fibrillation (AF) is considered a predictor for severe stroke and poor outcome. The aim was to evaluate whether AF is associated with poor outcome in acute ischemic stroke (IS) patients treated with intravenous thrombolysis (IVT). In a retrospective study, 157 consecutive IS patients (98 males, mean age 67.3 +/...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5452-4
更新日期:2010-06-01 00:00:00
abstract::The last 5 years have seen rapid progress in Parkinson’s disease (PD) genetics, with the publication of a series of large-scale genome wide association studies for PD, and evaluation of the roles of the LRRK2 and GBA genes in the aetiology of PD. We are beginning to develop a coherent picture of the interplay of Mende...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-013-7003-2
更新日期:2014-02-01 00:00:00
abstract::The cases of identical twins with ataxia telangiectasia, early intellectual impairment and progressive spasticity are reported. Immunological tests revealed reduced levels of serum and salivary IgA, increased B cells, reduced T cells and raised alpha-fetoprotein. CT scan performed in one of the twins was normal. The p...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314163
更新日期:1986-10-01 00:00:00
abstract::We report two cases of unilateral spatial neglect associated with an isolated right frontal lobe lesion. Case 1 was a 59-year-old, right-handed man, who developed a left hemiplegia, disorientation, and frontal lobe neglect associated with a haemorrhagic contusion following a head injury. Case 2 was a 55-year-old, righ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF00866909
更新日期:1995-10-01 00:00:00
abstract::The symptomatology of 18 patients with the severest clinical course after subarachnoid hemorrhage (SAH) is described and analyzed. Seventeen patients died, five with an acute bulbar syndrome with cardiac arrest, and 12 with irreversible breakdown of brain function. One patient had an apallic syndrome with minimal sign...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313005
更新日期:1979-01-01 00:00:00
abstract::PNPLA6 mutations, known to be associated with the development of motor neuron phenotypes, have recently been identified in families with Boucher-Neuhäuser syndrome. Boucher-Neuhäuser is a rare autosomal recessive syndrome characterized by the co-occurrence of cerebellar ataxia, hypogonadotropic hypogonadism, and chori...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7516-3
更新日期:2014-12-01 00:00:00
abstract::Dimethyl fumarate (DMF), fingolimod (FTY) and teriflunomide (TFN) are oral disease-modifying therapies (DMTs) approved for relapsing-remitting multiple sclerosis (RRMS) whose efficacy and tolerability have been separately assessed in phase III trials. Conversely, little evidence exists about their head-to-head compari...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8595-8
更新日期:2017-11-01 00:00:00
abstract:BACKGROUND:To date, insertable cardiac monitors (ICMs) are the most effective method for the detection of occult atrial fibrillation (AF) in cryptogenic stroke. The overall detection rate after 12 months, however, is low and ranges between 12.4 and 33.3%, even if clinical predictors are considered. Ischemic stroke pati...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09524-5
更新日期:2019-12-01 00:00:00
abstract:BACKGROUND:Despite advances in the field, diagnosis and management of the wide spectrum of neurological events post allogeneic hematopoietic cell transplantation (alloHCT) remain challenging. Therefore, we investigated their incidence, diagnosis, management and long-term prognosis in alloHCT recipients. METHODS:We ret...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09372-3
更新日期:2019-08-01 00:00:00
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journal_title:Journal of neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1007/s00415-003-1097-x
更新日期:2003-07-01 00:00:00
abstract::Cerebral ischemia of 15 min was produced in rats by increasing intracranial pressure above the systolic blood pressure level by infusing artificial cerebrospinal fluid into the cisterna magna. Pulmonary edema was prevented by blocking the vasomotor response with a ganglioplegic agent, and reducing the fluid load by pe...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00316314
更新日期:1977-12-01 00:00:00
abstract::Psychiatric complaints are common in Parkinson's disease (PD), and have a significant influence in disease outcome and quality of life. Little attention has been paid to psychiatric symptoms at early stage disease. We aimed to screen a population of early stage PD patients for psychiatric symptoms and to study the rel...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6140-8
更新日期:2012-01-01 00:00:00
abstract::Rapid-eye-movement (REM) sleep behavior disorder (RBD) is a parasomnia characterized by complex motor activity associated with dreaming during REM sleep. RBD may be idiopathic or associated with various neurological diseases involving the brainstem. The association of RBD and limbic system impairment was unclear. We r...
journal_title:Journal of neurology
pub_type: 信件
doi:10.1007/s00415-009-5067-9
更新日期:2009-07-01 00:00:00
abstract:BACKGROUND:Gastrointestinal (GI) dysfunction is prevalent in Parkinson's disease (PD). Symptoms are evident throughout the disease course, affect the length of the GI tract and impact on patient quality of life and management. We clarify real-life differences in the frequency and severity of GI symptoms in a cohort of ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09723-5
更新日期:2020-05-01 00:00:00
abstract::Sera from 23 patients with acute Guillain Barré syndrome (GBS), 15 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and from 40 age-matched blood donors were analysed for antibodies to acidic glycosphingolipids from human brain and peripheral nerve. Antibodies to ganglioside LM1, the majo...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00315684
更新日期:1991-04-01 00:00:00
abstract::Extra- and intraneural ganglionic cysts rarely involved peripheral nerves. They are found in the neighbourhood of large joints. Intraneural cysts prefer the deep peroneal nerve and cause intermittent pain and severe nerve damage. The ulnar nerve is affected most often at the wrist. There are different types of distal ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314606
更新日期:1975-06-09 00:00:00
abstract::Cerebral hemodynamics play a pivotal role in stroke pathogenesis. Transcranial Doppler (TCD) studies demonstrated the importance of cerebral vasomotor reactivity (VMR) on the outcome of carotid artery occlusion (CAO). So far, positron emission tomography represents the best technique for detecting both hemodynamic and...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0244-6
更新日期:2006-11-01 00:00:00
abstract::In contrast to the distal sensory response, the somatosensory evoked response (SSER) is detectable in patients with A-alpha sensory fiber diseases such as Friedreich's ataxia. In four patients with this disease a combined histological and electro-physiological investigation of sensory propagation was performed. The su...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00312980
更新日期:1978-12-22 00:00:00
abstract::Myotonic Dystrophy Type 1 (DM1) and 2 (DM2) present with distinct though overlapping clinical phenotypes. Comparative imaging data on skeletal muscle involvement are not at present available. We used the novel technique of whole body 3.0 Tesla (T) Magnetic Resonance Imaging (MRI) to further characterize musculoskeleta...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0111-5
更新日期:2006-06-01 00:00:00