Abstract:
OBJECTIVE:White matter (WM) integrity of Spinocerebellar ataxia 2 (SCA2) is poorly understood, more so in the early stages of SCA2. In this study, we evaluated the microstructural integrity of the WM tracts with an emphasis on the nature of in vivo pathological involvement in early SCA2. MATERIALS AND METHODS:We evaluated the MRI images of 26 genetically proven SCA2 patients with disease duration <5 years and 24 age- and gender-matched healthy controls using tract-based spatial statistics (TBSS) to identify the WM tract changes and their clinico-genetic correlates (age at onset, duration of disease, ataxia severity and CAG repeat length) using standard methodology. RESULTS:The mean age at onset and duration of disease were 28.7 ± 8.51 years and 3.5 ± 0.69 months, respectively. The mean CAG repeat length was 42.5 ± 4.6, and the ataxia severity score was 16.1 ± 4.9. Altered DTI scalars signifying degeneration was present in the bilateral anterior thalamic radiation (ATR), corticospinal tract (CST), inferior fronto-occipital fasciculus (IFOF), superior and inferior longitudinal fasciculus (SLF and ILF), uncinate fasciculus (UF), cingulum, corpus callosum (CC), forceps major and forceps minor (corrected p < .05). DTI scalars representing demyelination was seen in the superior cerebellar peduncle (SCP) and cerebellar WM. There was a significant correlation of SARA score with axial diffusivity of the bilateral cingulum, ATR, CST, forceps minor, IFOF, ILF, SLF and SCP on the right side (corrected p < .05). CONCLUSION:Extensive WM involvement is present in early SCA2. The DTI scalars indicate degeneration and demyelination and may have clinical implications.
journal_name
Acta Neurol Scandjournal_title
Acta neurologica Scandinavicaauthors
Stezin A,Bhardwaj S,Khokhar S,Hegde S,Jain S,Bharath RD,Saini J,Pal PKdoi
10.1111/ane.13359subject
Has Abstractpub_date
2020-10-08 00:00:00eissn
0001-6314issn
1600-0404pub_type
杂志文章abstract::In a well-defined prospective series of 247 patients with a first-ever supratentorial brain infarct, 15 patients (6%; 95% confidence interval 3-9%) had a large striatocapsular infarct. Twelve (80%) had signs of cortical dysfunction, whereas risk factor profile, frequency of significant carotid stenosis, and frequency ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1992.tb05089.x
更新日期:1992-09-01 00:00:00
abstract:INTRODUCTION:Charcot-Marie-Tooth disease (CMT) is a heterogeneous inherited neuropathy. The number of known CMT genes is rapidly increasing mainly due to next-generation sequencing technology, at present more than 70 CMT-associated genes are known. We investigated whether variants in the DCTN2 could cause CMT. MATERIA...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12515
更新日期:2016-07-01 00:00:00
abstract::The concentrations of sulfur-containing amino acids (taurine, methionine, cystine, cystathionine, homocystine) in lumbar CSF from patients with consciousness disturbances and controls were measured, and it was investigated whether there was any correlation between the sequential changes of these amino acid concentrati...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1983.tb04848.x
更新日期:1983-12-01 00:00:00
abstract::The role of the ophthalmic artery (OA) as a collateral to the cerebral circulation in carotid occlusive disease is somewhat controversial. The aim of this study was to assess the importance of this collateral by comparing the results of transorbital Doppler ultrasonography and regional cerebral blood flow measurements...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1996.tb00012.x
更新日期:1996-05-01 00:00:00
abstract::Fifteen epileptic patients had their dose of anticonvulsant drugs changed twice, each time by 30-50 per cent of the initial medication. Before the dose change, the patients were given six especially adapted mental performance tests, which were designed to measure vigilance, reaction time and certain aspects of memory....
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1975.tb05827.x
更新日期:1975-10-01 00:00:00
abstract:OBJECTIVE:To evaluate the clinical course of patients with myasthenia gravis (MG) up to 3 months after double filtration plasmapheresis (DFP). MATERIAL AND METHODS:We recorded the MG score and measured the level of acetylcholine receptor antibody (AchRAb) at baseline and day 1 (D1), week 1 (W1), 1 month (M1), 2 (M2) a...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2003.00107.x
更新日期:2003-09-01 00:00:00
abstract::The association of cortical tremor, myoclonus and epileptic seizures has been reported in many Japanese and European families with different acronyms. We reviewed the familial cases presenting the clinical picture of autosomal dominant cortical tremor, myoclonus and epilepsy and analysed the phenotypic differences bet...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2005.00385.x
更新日期:2005-04-01 00:00:00
abstract:OBJECTIVE:Blepharoclonus (BLC) denotes a large amplitude, involuntary tremors of the orbicularis oculi muscles, observed during gentle closure of the eyelids. BLC may follow major head trauma. Four patients with Arnold-Chiari malformation (ACM) and BLC are described. MATERIALS AND METHODS:The first patient had facial ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2001.104002113.x
更新日期:2001-08-01 00:00:00
abstract::A 23-year-old patient suffering from mental deterioration was referred for CT study following her first epileptic fit. The study disclosed generalized atrophy and diffuse symmetric white matter hypodensities. Similar findings were found in her 13-year-old retarded sister. The diagnosis of metachromatic leukodystrophy ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1987.tb07891.x
更新日期:1987-01-01 00:00:00
abstract::Three patients at Stage II of subacute sclerosing panencephalitis (SSPE) were treated with semipurified alpha-interferon (IFN) using different combinations of intrathecal and intravenous routes: 1 x 10(6) IU of alpha-IFN were given every other day up to a total of 15 x 10(6) IU. Transient improvement of neurological s...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1988.tb03639.x
更新日期:1988-09-01 00:00:00
abstract::Vigabatrin (VGB), an irreversible inhibitor of gamma-aminobutyric acid (GABA) transaminase, is approved as adjunct treatment of refractory partial seizures as well as infantile spasms. Although VGB has been proven to be effective, its use is limited by the risk of retinopathy and associated peripheral visual field def...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0404.2012.01684.x
更新日期:2012-10-01 00:00:00
abstract::Rats with different levels of blood glucose concentration were exposed to 10 min of complete brain ischemia achieved by compression of neck vessels by a pneumatic cuff. All normoglycemic rats survived the ischemic period and made the best clinical recovery. Hyperglycemic rats died within 12 h. Seizure activity was obs...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:
更新日期:1978-07-01 00:00:00
abstract::L-Deprenyl, a specific monoamine oxidase subtype B inhibitor, has been reported a valuable adjunct to conventional treatment of Parkinsonism. A double-blind cross-over controlled study was performed in 19 parkinsonian patients with on-off type problems. Five mg L-Deprenyl per day reduced the number of on-off episodes....
journal_title:Acta neurologica Scandinavica
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0404.1985.tb03233.x
更新日期:1985-06-01 00:00:00
abstract::In this study levels of neuron-specific enolase (NSE), S-100 protein (S-100) and myelin basic protein (MBP) in cerebrospinal fluid (CSF) of children and adults with distinct neurological disorders were examined. A previous study from our department demonstrated age related reference values for these brain-specific pro...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1995.tb01696.x
更新日期:1995-09-01 00:00:00
abstract::Prognosticating the outcome of an unconscious patient continues to pose a serious challenge to the clinicians. BAERs are the latest addition to neurological diagnostic aids for predicting the outcome of coma. The relative value of BAERs in predicting the outcome of 15 comatosed meningoencephalitis patients has been st...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1984.tb07795.x
更新日期:1984-03-01 00:00:00
abstract:OBJECTIVE:White blood cells might have a pathogenetic role in ischemic vascular conditions. Several studies have suggested that increased adhesiveness of these cells could contribute to such a damage. The present study was undertaken in order to examine the adhesive properties of leukocytes in the peripheral blood of p...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:
更新日期:1997-08-01 00:00:00
abstract::A three generation family is presented in which rapidly progressive, early-onset Creutzfeldt-Jakob disease without typical EEG changes segregates as an autosomal dominant disease. An aspartic acid to asparagine mutation at codon 178 of the prion gene, PRNP, co-segregates with the disease. As expected, the disease alle...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2001.103003139.x
更新日期:2001-03-01 00:00:00
abstract::The activity of 7 mitochondrial enzymes, fumarase, NAD-malate dehydrogenase (MDH), citrate synthase (CS), valine dehydrogenase (VDH), succinate dehydrogenase (SDH), glutamate dehydrogenase (GDH), pyruvate dehydrogenase complex (PDHC) has been measured in platelet preparations from patients affected by Friedreich's ata...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1989.tb03849.x
更新日期:1989-08-01 00:00:00
abstract::A headache disorder with shortlasting, frequently occurring (6-18/24 hours) head pain attacks is reported. The pain is excruciatingly severe, unilateral (always on the same side), unaccompanied by visual phenomena, nausea/vomiting, but accompanied by nasal congestion and lacrimation on the symptomatic side. The maximu...
journal_title:Acta neurologica Scandinavica
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0404.1976.tb04788.x
更新日期:1976-08-01 00:00:00
abstract::Soleus H-reflexes during tonic contractions and isometric ramp contractions of the ankle extensors in spastic and healthy subjects were measured. During the tonic contractions, the H-reflex increased with the contraction level. The increase was highest in the patients (p < 0.001). The facilitation of the H-reflex duri...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1993.tb05352.x
更新日期:1993-11-01 00:00:00
abstract:OBJECTIVES:To describe clinical, electrophysiological and genetic data of five unrelated Sicilian pedigrees harbouring a heterozygous Ser78Leu mutation in the myelin protein zero (MPZ) extracellular domain. MATERIALS AND METHODS:Clinical, electrophysiological and genetic findings of 16 patients were reported. Polymorp...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2008.01021.x
更新日期:2008-11-01 00:00:00
abstract:INTRODUCTION:We planned a descriptive study on the incidence of intracranial gliomas spanning a 16-year period (1976-1991) in the Local Health Service 31 of Ferrara, Northern Italy. MATERIAL AND METHODS:We used a complete enumeration approach by reviewing all the possible sources of case collection available in the st...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1994.tb02730.x
更新日期:1994-11-01 00:00:00
abstract::EEG, regional cerebral blood flow (rCBF) and neuropathological autopsy data were studied in 17 patients with presenile dementia. RCBF was measured in 14 cases using the intra-arterial-133-Xenon clearance technique. The neuropathological study included semi-serial whole brain microscopical sections in which the severit...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1977.tb01414.x
更新日期:1977-08-01 00:00:00
abstract::Factors at presentation which influenced the course of the disease and response to treatment were assessed in 125 de novo patients with Parkinson's disease. Ninety-eight patients were available for re-assessment at 5 years. Older patients presented earlier after the onset of symptoms, deteriorated more rapidly, and we...
journal_title:Acta neurologica Scandinavica
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1111/j.1600-0404.1995.tb00480.x
更新日期:1995-12-01 00:00:00
abstract:OBJECTIVE:The aim was to investigate risk factors separately and in sum in relation to long-term mortality after arterial cerebral infarction in young adults. MATERIALS AND METHODS:Mortality in relation to the number of risk factors (smoking, hypertension, hypercholesterolemia, diabetes mellitus, myocardial infarction...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2012.01681.x
更新日期:2013-02-01 00:00:00
abstract:OBJECTIVES:In this follow-up study, we wanted to present the long-term outcome (5-21 years) in terms of seizure freedom, seizure reduction, and the cognitive development in the first 47 children who underwent epilepsy surgery at the University Hospital in Lund from 1991 to 2007. MATERIALS AND METHODS:All children who ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12154
更新日期:2013-12-01 00:00:00
abstract:OBJECTIVES:Therapy of acute relapses in multiple sclerosis with corticosteroids (CC) remains uncertain with respect to route, dosage and effectiveness. This makes the treatment of relapses a clinical field where 'shared decision-making (SDM)' could be of advantage for the patients. A prerequisite for SDM is the provisi...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0404.2004.00284.x
更新日期:2004-07-01 00:00:00
abstract:OBJECTIVES:When the vascular disorder of the cochleovestibular system is mentioned, the diagnosis is based on exclusion of other diseases. Since arteries of the cochleovestibular system cannot be directly visualized, physicians must deduce from the vascular risk factors and the vascular lesion of other territories to t...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2001.104002068.x
更新日期:2001-08-01 00:00:00
abstract::One cause of ischemic brain injury is free radical formation during recirculation. Allopurinol inhibits xanthine oxidase, an important source of free oxygen radicals. It is known that allopurinol pre-treatment has a protective action during cerebral ischemia. In the present study we exposed slices from the rat hippoca...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1991.tb04702.x
更新日期:1991-05-01 00:00:00
abstract:BACKGROUND:The quality-of-life (QoL) perception by Parkinson's disease (PD) patients and their caregivers (CG) has not been studied in depth. OBJECTIVE:To examine patient/proxy agreements on the PD QoL Questionnaire (PDQ-39), the Scale of Quality of Life of Care-Givers (SQLC) and the Multidimensional Caregiver Strain ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12726
更新日期:2017-08-01 00:00:00