Abstract:
:The neuropathological findings in two siblings with Menkes' disease were compared with representative material obtained from lambs suffering from swayback (enzootic ataxia). The aim of the study was to demonstrate the similarity of lesions in a genetic and a nutritional form of copper deficiency in support of the view that all lesions in Menkes' disease could be ascribed to simple hypocupraemia. All lesions of Menkes' disease were shown to have their counterpart in swayback, with exception of the abnormal arborisations of the Purkinje cell dendrites. These have often been interpreted as malformations and cited in evidence of the prenatal origin of the cerebral lesions. They are, however, non-specific and similar lesions have been reported in conditions arising in later life. While there is abundant collateral evidence of disturbed copper metabolism in utero, the problem of the prenatal versus postnatal origin of cerebral damage remains unresolved.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Tan N,Urich Hdoi
10.1016/0022-510x(83)90192-2subject
Has Abstractpub_date
1983-12-01 00:00:00pages
95-113issue
1-3eissn
0022-510Xissn
1878-5883pii
0022-510X(83)90192-2journal_volume
62pub_type
杂志文章abstract:BACKGROUND:Multiple sclerosis (MS) and allergies are both considered to be related to imbalanced Th1 and Th2 immune responses. Previous studies evaluating the relationship between MS and allergies provide conflicting results. OBJECTIVE:To assess allergies and asthma as risk factors for MS and as predictors of MS relap...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,多中心研究
doi:10.1016/j.jns.2017.02.041
更新日期:2017-04-15 00:00:00
abstract::The effect of aluminum (Al) was studied in an in vitro system of cultured hippocampal neurons from fetal rats in a chemically defined medium. Neuronal aggregates interconnected by neurites were detected light and electron microscopically in control and Al-treated cultures. In the Al-treated cultures disruptions of the...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(89)90186-x
更新日期:1989-11-01 00:00:00
abstract::Thirteen months after castration of male rats the weight of their soleus muscles was lowered to 82% and their choline acetyltransferase (ChAc) activity to 83% of control values. The administration of testosterone lasting 5 weeks increased the weight of the soleus muscles of castrated animals by 19% and their ChAc acti...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(76)90008-3
更新日期:1976-03-01 00:00:00
abstract::Reversible cerebral vasoconstriction syndromes are characterized by prolonged but reversible vasoconstriction of the cerebral arteries, presenting typically with thunderclap headache, with or without focal neurological signs or symptoms. They resemble primary central nervous system vasculitis but it is critical to dif...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2008.08.034
更新日期:2009-01-15 00:00:00
abstract::A multidimensional quantitative EMG analysis method with averaged motor unit potentials (MUPs) was applied to a study of different stages of polymyositis. The duration parameters had a general tendency to increase in polymyositis. The only exception was the total MUP duration, which decreased in the onset stage of acu...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(82)90029-6
更新日期:1982-12-01 00:00:00
abstract::The present study was performed to investigate the effects of Ca(2+)/calmodulin-dependent protein kinase II (CaMKII) antisense oligodeoxynucleotides (ODNs) on the assembly of the CaMKII·GluR6·PSD-95 signaling module, GluR6 serine phosphorylation and c-Jun N-terminal kinase 3 (JNK3) activation. A further aim was to det...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.10.012
更新日期:2012-03-15 00:00:00
abstract::Cortical excitability to magnetic stimulation was investigated interictally in 10 patients with migraine with aura, 10 with migraine without aura and in 10 healthy volunteers. Thresholds, latencies and amplitudes of the magnetic-evoked potentials (MEPs) were measured from threshold to 100% stimulus intensity in 10% st...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(96)00044-5
更新日期:1996-07-01 00:00:00
abstract::We report two patients with numb clumsy hands due to cervical spondylotic spinal cord compression. It is proposed that arterial insufficiency in the watershed area between the central and radial arterial systems of the cervical cord explains the sensory findings in the upper extremities with preservation of motor func...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(96)00147-5
更新日期:1996-09-01 00:00:00
abstract:PURPOSE:T cells are important in the immunopathology of immune-mediated peripheral neuropathies (PNP) and activated vitamin D regulates the immune response through increasing the amount of regulatory T cells. An association between vitamin D deficiency and polyneuropathy has been stipulated; hence we assessed whether p...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.07.040
更新日期:2014-10-15 00:00:00
abstract::We describe a case of a 50-year-old man with bilateral shoulder girdle weakness caused by anterior subdural hygroma secondary to a previous spontaneous CSF leak. The CSF leak occurred and resolved 16 years prior to presenting with a 6-year progressive painless, asymmetric proximal muscle weakness involving both upper ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.02.029
更新日期:2011-06-15 00:00:00
abstract::Fast 60 degree head rotations of nine patients with cerebellar deficits were analysed and compared with those of nine normal subjects. The surface EMG activity from both Splenii capitis muscles were recorded. The triphasic pattern of reciprocal innervated neck muscles with regard to the duration, amplitude and onset o...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(97)00276-1
更新日期:1998-02-18 00:00:00
abstract::The kynurenine pathway (KP), the primary route of tryptophan degradation in mammalian cells, consists of many metabolites including kynurenic acid (KYNA), quinolinic acid (QUIN), 3-hydroxykynurenine (3-HK) and picolinic acid (PIC). The former two are neuroactive, while the latter two are molecules with pro-oxidants an...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2012.08.005
更新日期:2012-12-15 00:00:00
abstract::Unilateral facial spasms (UFS) are frequently caused by hemifacial spasm (HFS), a disorder that usually results from vascular loop compression at the root exit zone of the facial nerve. However, UFS can also be a manifestation of other conditions, including brainstem tumours or demyelination, post-Bell's synkinesis, l...
journal_title:Journal of the neurological sciences
pub_type: 信件
doi:10.1016/j.jns.2019.116532
更新日期:2019-12-15 00:00:00
abstract::Vascular dementia (VaD) has a great deal of overlap (in terms of features and symptoms) with Alzheimer's disease (AD). Mixed dementia, or AD with concomitant cerebrovascular disease (AD with CVD), is increasingly being recognized as a distinct clinical condition that occurs with substantial frequency. The robust evide...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/s0022-510x(02)00267-8
更新日期:2002-11-15 00:00:00
abstract::The clinical value and practical application of the electrically induced BC reflex was investigated in 40 patients with traumatic or compressive lesions of the conus medullaris or cauda equina. It was shown that the BC reflex was either absent or delayed depending upon the invovlement of the sacral 2--4 spinal and rad...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(79)90036-4
更新日期:1979-04-01 00:00:00
abstract:BACKGROUND:Several correlations between cognitive impairment (CI), radiologic markers and cognitive reserve (CR) have been documented in MS. OBIECTIVE:To evaluate correlation between CI and brain volume (BV) considering CR as possibile mitigating factor. METHODS:195 relapsing MS patients underwent a neuropsychologica...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2018.01.011
更新日期:2018-03-15 00:00:00
abstract::Ten unmedicated patients with Parkinson's disease received sub-threshold 5-Hz repetitive transcranial magnetic stimulation (rTMS) over the primary motor hand area (M1(HAND)) contralaterally to the more affected upper limb. Compared to a midfrontal sham-rTMS, real-rTMS over M1(HAND) was associated with a significant de...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章
doi:10.1016/s0022-510x(00)00370-1
更新日期:2000-09-15 00:00:00
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
更新日期:2020-04-15 00:00:00
abstract::We describe a 56-year-old man who had a progressive pseudobulbar palsy, spastic tetraparesis, forced laughing and disturbance of voluntary eyelid closure, and was clinically compatible with chronic progressive spinobulbar spasticity. Magnetic resonance images (MRI) revealed atrophy of the bilateral motor cortices and ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(90)90131-6
更新日期:1990-05-01 00:00:00
abstract:BACKGROUND AND AIMS:Subacute combined degeneration (SACD) of the spinal cord, characterized by degeneration of lateral and posterior columns, is often found in vitamin B12 deficiency. Our aim was to look for sensitivity of imaging in depicting the spinal cord abnormality in vitamin B12 deficient patients and to find an...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.05.020
更新日期:2014-07-15 00:00:00
abstract::The two-dimensional immunoelectrophoresis on cellulose acetate, already utilized for serum proteins, has been applied to CSF proteins. The technical modifications which allow the use of unconcentrated CSF are described. By utilizing a particularly suitable supporting medium, a good separation of proteins is achieved; ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(78)90203-4
更新日期:1978-07-01 00:00:00
abstract::Antibodies against myelin oligodendrocyte glycoprotein (MOG) mediate demyelination in experimental autoimmune encephalomyelitis (EAE) in different animal species and are implicated in the immunopathogenesis of multiple sclerosis (MS). In order to evaluate the anti-MOG response, we have analyzed the cerebrospinal fluid...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(03)00066-2
更新日期:2003-07-15 00:00:00
abstract::Nutritional deficiency may be the cause of a genuine optic neuropathy, sometimes associated with involvement of the peripheral nervous system. Nutritional optic neuropathies are usually bilateral, painless, chronic, insidious and slowly progressive. Most often, they present as a non-specific retrobulbar optic neuropat...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2007.06.038
更新日期:2007-11-15 00:00:00
abstract::Chronic relapsing experimental allergic encephalomyelitis has been induced in juvenile strain 13 guinea pigs with isologous spinal cord in Freund's complete adjuvant. Retention of antigen at the injection site and in the draining lymph nodes was studied by immunocytochemical staining with antiserum to myelin basic pro...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(84)90145-x
更新日期:1984-10-01 00:00:00
abstract::The present study tested aspects of a novel etiological/pathogenetic hypothesis of Alzheimer's disease (AD), which proposes that alterations in endocrine regulation of peripheral calcium/phosphate (Ca/PO4) homeostasis (e.g., by glucocorticoids, vitamin D, etc.) induce and/or reflect altered calcium homeostasis and neu...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(91)90261-5
更新日期:1991-12-01 00:00:00
abstract::The Mongolian gerbil, because of the uniqueness of the anatomical features of its circle of Willis, has become an increasingly useful model in the study of cerebrovascular disease. The present work defines acute changes at the ultrastructural level following ischemic insult. The pathomorphological responses include in...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(77)90190-3
更新日期:1977-08-01 00:00:00
abstract::The physiological and histological changes following injection of 10 microliter of proteinase K into the rat tibial nerve have been compared with those induced by proteinase K and specific inhibitor phenylmethylsulphonyl fluoride (PMSF) and also by rabbit experimental allergic neuritis (EAN) serum. Proteinase K and PM...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(87)90005-0
更新日期:1987-12-01 00:00:00
abstract::Intellectual problems are a recognized feature of neurofibromatosis 1 (NF1) but their aetiology is unknown. We investigated the frequency, nature, severity and cause of intellectual impairment in NF1. We undertook neurological and psychometric assessments on 103 patients with NF1 and 105 controls equated for age, sex ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(96)00022-6
更新日期:1996-06-01 00:00:00
abstract::Using special soft tissue X-ray investigations the authors have found that the increase in volume of the calf musculature in progressive muscular dystrophy does not seem to be in complete accordance with the criteria of pseudohypertrophy. It has been demonstrated that this structural alteration of the musculature does...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(79)90074-1
更新日期:1979-09-01 00:00:00
abstract::Data of 2399 brain weights and ages from populations before 1880, 1885 to 1900 and 1966 to 1976 were obtained from German anatomical and pathological institutes, analyzed with non-linear and multiple linear regression analyses and the results compared. The influence of the absolute age (sample period) on brain weights...
journal_title:Journal of the neurological sciences
pub_type: 历史文章,杂志文章
doi:10.1016/0022-510x(79)90202-8
更新日期:1979-02-01 00:00:00