Abstract:
:This study of frontotemporal dementia (FTD) was carried out to determine whether MR spectroscopy can provide an in vivo marker for the neuronal loss and gliosis that occur in this condition. We compared spectra in frontal and temporal regions known to be affected early in the course of the disease with spectra in the parietal lobe that is spared until late stages of FTD. We were interested in the relative concentrations of two compounds, NAA (a marker of neuronal integrity) and mI (a marker of gliosis), expressed as ratios to creatine (a relatively stable brain constituent). MR spectroscopy was performed on the temporal, parietal, and anterior cingulate cortices of five patients with the established semantic dementia form of FTD, two patients with the frontal form of FTD and 13 age matched controls. Structural MRI and neuropsychometry were also performed. Patients with FTD had reduced NAA/Cr in frontal and temporal, but not parietal lobes. The two patients with the frontal form of FTD had increased mI/Cr in their cingulate cortices. These data show for the first time that MR spectroscopy can reveal regionally selective abnormalities in patients with FTD. This opens up the possibility of using MR spectroscopy as a clinical tool to identify earlier presentations of the condition.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Coulthard E,Firbank M,English P,Welch J,Birchall D,O'Brien J,Griffiths TDdoi
10.1007/s00415-006-0045-ysubject
Has Abstractpub_date
2006-07-01 00:00:00pages
861-8issue
7eissn
0340-5354issn
1432-1459journal_volume
253pub_type
杂志文章abstract::Familial paroxysmal exercise-induced dyskinesia (PED) is a rare movement disorder that is mostly caused by mutations in the solute carrier family 2, member 1 (SLC2A1) gene and inherited in an autosomal dominant manner. Clinical, laboratory, and genetic studies were performed in three family members. The proband's symp...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7441-5
更新日期:2014-10-01 00:00:00
abstract::In this work, we investigated motor network structure in patients affected by essential tremor (ET) with or without resting tremor, using probabilistic tractography of the cerebello-thalamo-basal ganglia-cortical loop. Twenty-five patients with ET, twenty-two patients with ET associated with resting tremor (rET), and ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8553-5
更新日期:2017-09-01 00:00:00
abstract::A recently developed technique is used to study the permeation of water through membranes of human erythrocyte ghosts. The method, based on the difference of the indices of refraction of H2O and D2O, is briefly described. Comparative measurements on erythrocyte ghosts from normal donors and from patients with Huntingt...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313655
更新日期:1984-01-01 00:00:00
abstract::Thyronine (T4), triiodothyronine (T3), and reverse-triiodothyronine (rT3) levels were evaluated in cerebrospinal fluid (CSF) and in serum of 12 patients with definite amyotrophic lateral sclerosis (ALS) by specific radioimmunoassays. Circulating microsomal and thyroglobulin antibodies were also evaluated. In all patie...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314221
更新日期:1989-01-01 00:00:00
abstract::To define the incidence and type of neurological complications and associated factors, we reviewed 41 consecutive patients who had 45 procedures for liver transplantation. Encephalopathy occurred after 28 procedures (62%) with immediate onset and no significant recovery before death or re-transplantation in 11 (24%), ...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s004150170023
更新日期:2001-12-01 00:00:00
abstract::The objective of the study was to identify differences in treatment strategies for anti-NMDA receptor encephalitis based on specialty of treating physicians, geographic location, and years in practice. We conducted an anonymous worldwide electronic survey through the Practice Current section of Neurology® Clinical Pra...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8407-1
更新日期:2017-04-01 00:00:00
abstract:BACKGROUND:Dysarthria may be classified as flaccid, spastic, ataxic, hypokinetic, choreatic, dystonic, or mixed. We hypothesized that in routine neurological practice the reliability and accuracy of perceptual analysis alone in the classification of dysarthria is low and that this classification is mainly based on the ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0978-4
更新日期:2008-10-01 00:00:00
abstract:OBJECTIVE:To quantify spinal cord atrophy and its impact on clinical disability in spinocerebellar ataxia (SCA) type 3 and 6. METHODS:Atrophy of the upper spinal cord was assessed by high resolution T1-weighted MRI of patients with SCA3 (n = 14) and SCA6 (n = 10). Furthermore, two groups of age- and sex-matched health...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0907-6
更新日期:2008-08-01 00:00:00
abstract::We should like to emphasize the following points: 1. Apathy is defined here as a quantified and observable behavioral syndrome consisting in a quantitative reduction of voluntary (or goal-directed) behaviors; 2. Therefore, apathy occurs when the systems that generate and control voluntary actions are altered; 3. These...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-006-7012-5
更新日期:2006-12-01 00:00:00
abstract::Post-concussion symptoms (PCS) (such as headaches, irritability, anxiety, dizziness, fatigue and impaired concentration) are frequently experienced by patients who have sustained a minor head injury (MHI). The post-concussion syndrome has been defined as a clinical state where 3 or more symptoms persist for more than ...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s004150050254
更新日期:1998-09-01 00:00:00
abstract::Seventy-seven cases of the optic-spinal form of multiple sclerosis (OSMS) were collected from 6 institutes in 3 cities of Japan, and the clinical and MRI features were analyzed. Two-thirds of the OSMS patients had longitudinally extensive spinal cord MRI lesions (LESL), and had clinical features similar to those of re...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-006-0400-z
更新日期:2007-04-01 00:00:00
abstract::Recessive mutations in GJC2, the gene-encoding connexin 47 (Cx47), cause Pelizaeus-Merzbacher-like disease type 1, a severe dysmyelinating disorder. One recessive mutation (p.Ile33Met) has been associated with a much milder phenotype--hereditary spastic paraplegia type 44. Here, we present evidence that a novel Arg98L...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7429-1
更新日期:2014-10-01 00:00:00
abstract::We examined 199 consecutive patients who underwent 220 liver transplantations, to define the type, frequency and aetiology of posttransplant neurological complications and their prognostic value. We found neurological complications in 63 patients (32%), mostly involving the central nervous system. The most frequent co...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF02444004
更新日期:1996-02-01 00:00:00
abstract:OBJECTIVE:Spinal and bulbar muscular atrophy (SBMA) is caused by an abnormal expansion of the CAG repeat in the androgen receptor gene. This study aimed to systematically phenotype a German SBMA cohort (n = 80) based on laboratory markers for neuromuscular, metabolic, and endocrine status, and thus provide a basis for ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8790-2
更新日期:2018-05-01 00:00:00
abstract::Tibial muscular dystrophy (TMD) or Udd myopathy is an autosomal dominant distal myopathy with late onset, at first described in the Finnish population. We report here the first Italian cases of TTN mutated titinopathy. The proband, a 60 year-old female, had the first muscular signs at the age of 59 years, with difficu...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-5372-3
更新日期:2010-04-01 00:00:00
abstract::Fifteen patients with Sneddon's syndrome presenting since 1979 were re-examined. After a neurological examination, an orienting test of mental ability as well as an electroencephalogram were performed in all patients. In 10 of the 15 patients computed tomography of the brain was performed, too. Preceding reports and t...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314215
更新日期:1989-01-01 00:00:00
abstract::Based on the observation of bilateral temporoparietal hypoperfusion in Alzheimer's disease (AD), single photon emission computed tomography (SPECT) is advocated by some as a powerful diagnostic tool in the evaluation of demented patients. We studied whether routine brain SPECT in elderly, mildly demented outpatients i...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00868397
更新日期:1995-06-01 00:00:00
abstract::A patient with chronic relapsing inflammatory demyelinating polyneuropathy was successfully treated with plasma exchanges and cyclosporin A (CsA). Dynamometric measurements of hand force during the time of CsA treatment showed a highly significant correlation between hand force and CsA blood levels. The largest influe...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314752
更新日期:1990-08-01 00:00:00
abstract::Mitochondrial diseases are due to impairment of the mitochondrial respiratory chain. A plausible pathogenic mechanism leading to cellular dysfunction and phenotypic expression is oxidative stress, but there are surprisingly few clinical studies on this subject. Glutathione (GSH) deficiency has been reported in mitocho...
journal_title:Journal of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00415-009-5409-7
更新日期:2010-05-01 00:00:00
abstract::Neurological complications following rubella are only rarely encountered. However, in many cases severe neurological impairment may occur, leading to permanent disability. In a recent epidemic of rubella in Israel during the years 1978-1979, 20 patients with severe neurological complications have been seen. We report ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313402
更新日期:1982-01-01 00:00:00
abstract::The emotional and relationship problems associated with MS have not always been fully appreciated by the medical profession, which has tended to concentrate on the physical aspects of this disease. Yet the psychological problems of MS often cause more suffering than physical effects. ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150170223
更新日期:2001-03-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Mechanical thrombectomy (MT) improves early clinical outcome in patients with acute ischemic stroke but insights on determinants of long-term outcome after MT treatment are scarce. METHODS:Data from stroke patients with anterior circulation large vessel occlusion of a prospective MT registry (01...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09670-w
更新日期:2020-04-01 00:00:00
abstract::We recently reported findings that loss of cortical acetylcholinesterase (AChE) activity is greater in parkinsonian dementia than in Alzheimer's disease (AD). In this study we determined cognitive correlates of in vivo cortical AChE activity in patients with parkinsonian dementia (PDem, n = 11), Parkinson's disease wi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0971-0
更新日期:2006-02-01 00:00:00
abstract:BACKGROUND:Delayed ischaemic neurological deficit (DID) following subarachnoid haemorrhage from aneurysm rupture (aSAH) is a serious complication and a major cause of mortality and morbidity. No empirical estimates of resource use and costs of patients with delayed ischaemic deficit compared to those without have been ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-0034-z
更新日期:2009-03-01 00:00:00
abstract::Hearing deficits associated with cognitive impairment have attracted much recent interest, motivated by emerging evidence that impaired hearing is a risk factor for cognitive decline. However, dementia and hearing impairment present immense challenges in their own right, and their intersection in the auditory brain re...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-016-8208-y
更新日期:2016-11-01 00:00:00
abstract::Reduced level of consciousness is a common clinical finding in acutely sick patients. In the majority of cases a cause for the encephalopathy is readily identifiable,whilst in a minority the aetiology is more difficult to ascertain. Frequently the onset of encephalopathy is associated with, or follows, infection. The ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-006-0092-4
更新日期:2006-07-01 00:00:00
abstract::Parkinson's disease (PD) is a neurodegenerative disorder of the aging population with unknown etiopathogenesis. It is assumed that the underlying pathobiochemical processes comprise multifactorial and multigenetic disturbances leading to a progressive and devastating disorder without remission. Subtypes exist suggesti...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/pl00007777
更新日期:2000-09-01 00:00:00
abstract::Silver syndrome/SPG17 is a motor manifestation of mutations in the BSCL2 gene and usually presents as a complicated form of hereditary spastic paraplegia (HSP). We present clinical data, follow-up, and genetic results of seven patients with Silver syndrome/SPG17 including a family with a variable intrafamilial phenoty...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8301-2
更新日期:2017-01-01 00:00:00
abstract:OBJECTIVES:The outcome of aphasia at 3 months is variable in patients with moderate/severe stroke. The aim was to predict 3-month aphasia outcome using prediction models including initial severity in addition to the interaction between lesion size and location at the acute phase. METHODS:Patients with post-stroke apha...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09259-3
更新日期:2019-06-01 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