Abstract:
:Hereditary spastic paraplegia (HSP) is a genetically heterogeneous group of neurodegenerative disorders characterized by progressive lower extremity weakness and spasticity. HSP pathology involves axonal degeneration that is most pronounced in the terminal segments of the longest descending (pyramidal) and ascending (dorsal columns) tracts. In this study, we compared spinal cord magnetic resonance imaging (MRI) in 13 HSP patients with four different types of autosomal dominant hereditary spastic paraplegia (SPG3A, SPG4, SPG6, and SPG8) with age-matched control subjects. The cross-section area of HSP subjects at cervical level C2 was 59.42 +/- 12.57 mm2 and at thoracic level T9 was 28.58 +/- 5.25 mm2. Both of these values were less than in the healthy controls (p < 0.001). The degree of cord atrophy was more prominent in patients with SPG6 and SPG8 who had signs of severe cord atrophy (47.60 +/- 6.58 mm2 at C2, 21.40 +/- 2.4 mm2 at T9) than in subjects with SPG3 and SPG4 (66.0 +/- 8.94 mm2 at C2, p < 0.02; 31.75 +/- 2.76 mm2 at T9, p < 0.001). These observations indicate that spinal cord atrophy is a common finding in the four genetic types of HSP. Spinal cord atrophy was more severe in SPG6 and SPG8 HSP subjects than in other types of HSP we studied. This may suggest a different disease mechanism with more prominent axonal degeneration in these two types of HSP when compared with HSP due to spastin and atlastin mutations.
journal_name
Neuroradiologyjournal_title
Neuroradiologyauthors
Hedera P,Eldevik OP,Maly P,Rainier S,Fink JKdoi
10.1007/s00234-005-1415-3subject
Has Abstractpub_date
2005-10-01 00:00:00pages
730-4issue
10eissn
0028-3940issn
1432-1920journal_volume
47pub_type
杂志文章相关文献
NEURORADIOLOGY文献大全abstract::One hundred and fifteen patients, suffering from sensorineural hearing loss were tested with a 1.5 T superconducting magnet. The authors describe utility of both T1-weighted multiple slice and T2-weighted multiple echo images for the evaluation of cerebello-pontine angle, internal auditory canal and their neurovascula...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/BF00340127
更新日期:1990-01-01 00:00:00
abstract:INTRODUCTION:The aim of this study was to evaluate the risks of endovascular therapy, aneurysm regrowth, recanalization and the need for reembolization. METHOD:A prospective analysis was performed on 211 aneurysms treated endovascularly from February 2000 to December 2003. Of these 211 aneurysms, 81 were asymptomatic ...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-006-0153-5
更新日期:2007-01-01 00:00:00
abstract:PURPOSE:Pituitary macroadenoma consistency can influence the ease of lesion removal during surgery, especially when using a transsphenoidal approach. Unfortunately, it is not assessable on standard qualitative MRI. Radiomic texture analysis could help in extracting mineable quantitative tissue characteristics. We aimed...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-020-02502-z
更新日期:2020-12-01 00:00:00
abstract:INTRODUCTION:The aim of this study is to investigate the relationship between abnormal retropharyngeal lymph nodes (RPLNs) and sinonasal malignancies among adults. METHODS:Magnetic resonance and computed tomography images from 89 patients over 20 years old who were all histopathologically confirmed to have sinonasal m...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-014-1428-x
更新日期:2014-12-01 00:00:00
abstract::Contrast enhancement of malignant gliomas and the development of peritumoral edema are thought to be due to a breakdown of the blood-brain barrier (BBB). The degree to which these two factors are related to each other or to the degree of damage to the BBB is unknown. Our purpose was to quantitatively correlate the deg...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s002340050421
更新日期:1997-05-01 00:00:00
abstract::Our aim was to determine whether MRI reliably shows pathology in patients with active otosclerosis (otospongiosis). We studied five patients with clinical and audiometric signs of this disorder and positive findings on high-resolution CT and tympanocochlear scintigraphy. Contrast enhancement of otospongiotic lesions w...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s002340050445
更新日期:1997-06-01 00:00:00
abstract::It is useful to evaluate the occlusive lesions of middle cerebral artery (MCA) occlusion with transcranial color-coded real-time sonography (TCCS). However, TCCS criteria for locating the site of the MCA occlusion has, as yet, remained unclear. The aim of the present study was to establish TCCS criteria for MCA occlus...
journal_title:Neuroradiology
pub_type: 临床试验,杂志文章
doi:10.1007/s00234-005-1364-x
更新日期:2005-04-01 00:00:00
abstract::Tuberous sclerosis complex (TSC) is an autosomal dominant condition clinically presenting with heterogenous clinical features. Multiple neuroradiological manifestations have been associated with TSC, such as tubers, radial migration lines, subependymal nodules, subependymal giant cell astrocytomas, and cyst-like lesio...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-021-02647-5
更新日期:2021-01-22 00:00:00
abstract::Inborn errors of metabolism can affect the cerebellum during development, maturation and later during life. We have established criteria for pattern recognition of cerebellar abnormalities in metabolic disorders. The abnormalities can be divided into four major groups: cerebellar hypoplasia (CH), hyperplasia, cerebell...
journal_title:Neuroradiology
pub_type: 杂志文章,评审
doi:10.1007/s002340050597
更新日期:1998-06-01 00:00:00
abstract:INTRODUCTION:To demonstrate intratumoral susceptibility effects in malignant brain tumors and to assess visualization of susceptibility effects before and after administration of the paramagnetic contrast agent MultiHance (gadobenate dimeglumine; Bracco Imaging), an agent known to have high relaxivity, with respect to ...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-007-0298-x
更新日期:2008-01-01 00:00:00
abstract:PURPOSE:Brain volume changes (BVC) on therapy in MS are being considered as predictor for treatment response at an individual level. We ought to assess whether adding BVC as a factor to monitor interferon-beta response improves the predictive ability of the (no) evidence of disease activity (EDA-3) and Río score (RS-3)...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-020-02604-8
更新日期:2020-11-25 00:00:00
abstract:INTRODUCTION:Diffusion tensor imaging (DTI) has been applied to characterize the pathological features of Alzheimer's disease (AD) in a mouse model, although little is known about whether these features are structure specific. Voxel-based analysis (VBA) and atlas-based analysis (ABA) are good complementary tools for wh...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-013-1195-0
更新日期:2013-08-01 00:00:00
abstract::In small arteriovenous malformations (AVM) with large hematomas, surgery remains the main therapeutic option. However, intraoperative identification of the AVM, feeders, and draining veins could be difficult in the environment of substantial intracerebral blood. In those selected cases, we use navigated computed tomog...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-004-1307-y
更新日期:2005-01-01 00:00:00
abstract::Bilateral conductive deafness due to congenital absence of the long process of the incus (CALPI) is rare. The authors present the case of a patient with bilateral CALPI. To the best of our knowledge, this is the first reported case preoperatively diagnosed as bilateral CALPI by computed tomography. Multislice computed...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-004-1200-8
更新日期:2004-10-01 00:00:00
abstract::Hypothalamic hamartomas are relatively rare, non-neoplastic congenital malformations. With conventional MR images alone, small hypothalamic hamartomas may be difficult to diagnose because of artifacts from cerebrospinal fluid. We present the usefulness of three-dimensional constructive interference in steady state seq...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-005-1340-5
更新日期:2005-03-01 00:00:00
abstract::A case of Marfan's syndrome is described in which progressive neurological signs and symptoms were produced by flexion of the head. Radiological examinations revealed hypermotility between the atlas and axis, as well as compression of the medulla oblongata by the odontoid process on flexion, since low positioned cereb...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/BF00518792
更新日期:1981-02-01 00:00:00
abstract::We report CT and MRI findings in two patients with ameloblastoma. One case had a multilocular mixed lesion and the other had a unilocular solid pattern. The second case is probably one of the youngest reported. CT and MRI can be used to delineate and show the extent of the tumour. ...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-001-0754-y
更新日期:2002-05-01 00:00:00
abstract::The imaging characteristics of concurrent spinal cord injury in children with hypoxic-ischaemic injury of the brain have not been described. We present the MRI findings of hypoxic-ischaemic injury of the brain and spinal cord following hypovolaemic shock in a 2-year-old girl. ...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/BF02278153
更新日期:1996-05-01 00:00:00
abstract:PURPOSE:The aim of this study was to describe imaging markers of decision-making under uncertain conditions in normal individuals, in order to provide baseline activity to compare to impaired decision-making in pathological states. METHODS:In this cross-sectional study, 19 healthy subjects ages 18-35 completed a novel...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-017-1949-1
更新日期:2018-01-01 00:00:00
abstract:INTRODUCTION:In the settings of stroke, a non-invasive high-resolution imaging modality to visualize the arterial intracranial circulation in the interventional lab is a helpful mean to plan the endovascular recanalization procedure. We report our initial experience with intravenously enhanced flat-detector CT (IV FDCT...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-011-0893-8
更新日期:2012-04-01 00:00:00
abstract:PURPOSE:Flame-shaped pseudo-occlusion of the extracranial internal carotid artery (ICA) is a flow-related phenomenon that creates computed tomographic angiography (CTA) and digital subtraction angiography (DSA) findings that mimic tandem intracranial-extracranial ICA occlusion or dissection. We aim to determine the dia...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-017-1882-3
更新日期:2017-10-01 00:00:00
abstract::A case of rare intraspinal sparganosis treated by surgical excision is described. In this 59-year-old male with paraparesis and voiding problem, an intradural mass was noted on myelogram. Magnetic resonance (MR) and computed tomographic appearance of spinal and associated cerebral lesion are illustrated and possible r...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/BF00596346
更新日期:1992-01-01 00:00:00
abstract::We report on our experience with cervical analgesic facet joint arthrography in 11 patients (30 joints), of whom nine had previous spinal surgery. In this type of patient we have found the procedure to be a diagnostic test as well as a preoperative guide to the neurosurgeon, but not a permanent therapy. ...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/BF02426454
更新日期:1990-01-01 00:00:00
abstract::The original version of this article unfortunately contained a referencing omission. Figure 11 is reused from the original publication of Figure 10 of Gunny and Lin [1]. ...
journal_title:Neuroradiology
pub_type: 已发布勘误
doi:10.1007/s00234-020-02443-7
更新日期:2020-07-01 00:00:00
abstract::We present cases of cervical cord compression in father and son with diaphyseal aclasia. Both patients were investigated with plain radiography, CT and MRI. We believe this to be the first report of spinal cord compression in first-degree relatives with diaphyseal aclasia. ...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/BF00593223
更新日期:1996-01-01 00:00:00
abstract::Herniated nuclear material of the cervical disc often perforates the posterior longitudinal ligament. Of 22 patients who were operated on by an anterior approach, 10 were of this type (the subligamentous type of cervical disc protrusion), while in the remaining 12 patients and 15 discs the protruded disc caused no tea...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/BF00548195
更新日期:1986-01-01 00:00:00
abstract:INTRODUCTION:Reflux venous signal on the brain and neck time-of-flight magnetic resonance angiography (TOF MRA) is thought to be related to a compressed left brachiocephalic vein. This study is aimed to assess the prevalence of venous reflux flow in internal jugular vein (IJV), sigmoid sinus/transverse sinus (SS/TS), a...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-013-1239-5
更新日期:2013-10-01 00:00:00
abstract::L5 radicular pain related to an epidural gas-containing pseudocyst is described in a 62-year-old female. Transient resolution of radicular pain was observed after CT-guided gas aspiration. Recurrent radicular pain led to surgical treatment; after operation the radicular pain disappeared. ...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/BF00340142
更新日期:1990-01-01 00:00:00
abstract::Information obtained from CT scan after contrast administration was evaluated in 59 consecutive stroke patients. CT scans before and after contrast administration were performed 3 days and 2 1/2 weeks after stroke. A plain CT scan was repeated 6 months later. Contrast enhancement was practically not seen on the first ...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/BF00339392
更新日期:1982-01-01 00:00:00
abstract::As the global COVID-19 pandemic evolves, our knowledge of the respiratory and non-respiratory symptoms continues to grow. One such symptom, anosmia, may be a neurologic marker of coronavirus infection and the initial presentation of infected patients. Because this symptom is not routinely investigated by imaging, ther...
journal_title:Neuroradiology
pub_type: 杂志文章
doi:10.1007/s00234-020-02554-1
更新日期:2021-01-01 00:00:00