Abstract:
:We report the effects of intravenous gamma-globulin (IVGG) in severe Guillain-Barré syndrome (GBS) in a small open study. Seven patients were given IVGG (0.4 g/kg/day) for 5 consecutive days. At the start of treatment all had progressing limb weakness and none could stand unaided. Within 24 h all but one patient started to improve, without the expected plateau phase, and progress was maintained with a mean time to independent walking of 14 days. One patient started treatment 9 h prior to ventilation for 15 days but was walking independently 31 days after admission. This study provides further evidence that IVGG is a promising therapeutic alternative to plasmapheresis in GBS. The rapidity of the response to IVGG raises the possibility that a single dose regimen might be equally or more effective.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Jackson MC,Godwin-Austen RB,Whiteley AMdoi
10.1007/BF00838447subject
Has Abstractpub_date
1993-01-01 00:00:00pages
51-3issue
1eissn
0340-5354issn
1432-1459journal_volume
240pub_type
杂志文章abstract::Corticospinal lesions cause impairments in voluntary motor control. Recent findings suggest that some degree of voluntary control may be taken over by a compensatory pathway involving the reticulospinal tract. In humans, evidence for this notion mainly comes from StartReact studies. StartReact is the acceleration of r...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9027-0
更新日期:2018-11-01 00:00:00
abstract::The posterior reversible encephalopathy syndrome (PRES) is a neurological disorder of (sub)acute onset characterized by varied neurological symptoms, which may include headache, impaired visual acuity or visual field deficits, disorders of consciousness, confusion, seizures, and focal neurological deficits. In a major...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-016-8377-8
更新日期:2017-08-01 00:00:00
abstract::There is growing evidence that intravenous immunoglobulins (IVIG) are effective in some neuroimmunological disorders of childhood. This short review summarizes the evidence-based indications and recommendations of IVIG therapy in these disorders. Despite considerable efforts to define the role and mechanisms of IVIG, ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-006-5014-y
更新日期:2006-09-01 00:00:00
abstract::Movement-associated cortical potentials (MP) associated with thumb-opposition or fist-clenching were studied in 20 patients with unilateral cerebral lesions and in 8 healthy subjects. MP was abnormal in 16 patients. The most frequent abnormality was an attenuation of the N component or readiness potential, recorded on...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00312541
更新日期:1975-07-02 00:00:00
abstract::The neuropsychiatric symptoms and behavioral disorders affecting Parkinson's disease (PD) patients are common and disabling. A PD-specific interview-based 12-item scale, the Scale for Evaluation of Neuropsychiatric Disorders in Parkinson's Disease (SEND-PD), has been developed to assess the severity of neuropsychiatri...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-012-6490-x
更新日期:2012-11-01 00:00:00
abstract::Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder of progressive heterotopic ossification (HO) caused by a recurrent activating mutation of ACVR1/ALK2, a bone morphogenetic protein (BMP) type I receptor. FOP is characterized by progressive HO, which is associated with inflammation in the setting o...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6563-x
更新日期:2012-12-01 00:00:00
abstract::An 8-year-old boy with galactose-1-phosphate uridyl transferase (GALT) deficiency presented with hypotonia, muscle hypotrophy, hepatomegaly, bilateral cataract and mild mental retardation. Two brothers showed a GALT activity consistent with a homozygotic condition and both parents were found to be heterozygotes for th...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00838160
更新日期:1993-05-01 00:00:00
abstract::Advances in molecular biology have resulted in novel therapy for neurofibromatosis 2-related (NF2) tumours, highlighting the need for robust outcome measures. The disease-focused NF2 impact on quality of life (NFTI-QOL) patient questionnaire was assessed as an outcome measure for treatment in a multi-centre study. NFT...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-014-7303-1
更新日期:2014-05-01 00:00:00
abstract::An autopsy case of Creutzfeld-Jacob disease with widespread amyloid plaques is reported. A 45-year-old Japanese man, whose father had died of a similar disease, had a 5-year illness characterized by progressive cerebellar signs. Mental changes and brain-stem signs developed in the late stage. Myoclonus frequently occu...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314329
更新日期:1989-03-01 00:00:00
abstract:: ...
journal_title:Journal of neurology
pub_type: 评论,杂志文章
doi:10.1007/s00415-018-9098-y
更新日期:2018-12-01 00:00:00
abstract::Predicting unfavorable outcome is of paramount importance in clinical decision making. Accordingly, we designed this multinational study, which provided the largest case series of tuberculous meningitis (TBM). 43 centers from 14 countries (Albania, Croatia, Denmark, Egypt, France, Hungary, Iraq, Italy, Macedonia, Roma...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-015-7651-5
更新日期:2015-01-01 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::Intrathecal synthesis of immunoglobulin G (IgG) specific to Treponema pallidum, ssp. pallidum, or to the human immunodeficiency virus 1 (HIV-1) was investigated in patients with contemporary treponemal and HIV-1 infections. Using a T. pallidum and an HIV-1 IgG enzyme-linked immunosorbent assay, specific antibody units...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314359
更新日期:1988-03-01 00:00:00
abstract::Disability in multiple sclerosis (MS) patients is associated with white matter (WM) and gray matter (GM) pathology, and both processes contribute differently over the disease course. Total and regional GM volume loss can be imaged via voxel-based morphometry (VBM). Here, we retrospectively analyzed a group of 213 MS p...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8114-3
更新日期:2016-06-01 00:00:00
abstract::Although seizures in brain tumor patients are common, the knowledge on optimal anti-seizure therapy in this patient group is limited. An observational study was carried out using a database of all patients from the neuro-oncology service during the period 2000-2005, with data on seizure characteristics, therapy with A...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-5156-9
更新日期:2009-09-01 00:00:00
abstract:BACKGROUND:Recently, therapeutic attempts to control motor choreatic hyperkinesia of Huntington's disease (HD) by means of pallidal deep brain stimulation (Gp-DBS) were successful. With respect to the clinical effects of Gp-DBS in juvenile hypokinetic-rigid HD (jHD; Westphal variant), only one single-case has been repo...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8880-1
更新日期:2018-07-01 00:00:00
abstract::Dementia has a reversible cause in some cases, and these should be diagnosed without over-investigating the many patients with irreversible disease. We prospectively studied the prevalence of reversible dementia in a memory clinic, determined the added value of investigations compared with clinical examination and ass...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/pl00007724
更新日期:1997-01-01 00:00:00
abstract:OBJECTIVE:To characterize the progression of the cognitive involvement in patients affected by myotonic dystrophy type 1 (DM1) by a longitudinal neuropsychological follow-up study. METHODS:In a previous study we documented an ageing-related decline of frontal and temporal cognitive functions in juvenile/adult forms of...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0017-5
更新日期:2008-11-01 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::Knowledge of restless legs syndrome (RLS) has greatly increased in recent years due to the many advances that have been made in diagnosis, management and genetics. Tools have been developed that facilitate the diagnosis and treatment of RLS, in particular the essential diagnostic criteria for RLS have been refined, se...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-0134-9
更新日期:2009-04-01 00:00:00
abstract::We report a case of polycythemia vera with chorea in which the brain metabolism and dopamine system were investigated using 2-[(18)F]fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) and (99m)Tc-labeled tropane dopamine transporter ((99m)Tc-TRODAT-1) single photon emission computed tomography (SPECT). Al...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6069-y
更新日期:2011-11-01 00:00:00
abstract::Neuroinflammation (microglial activation) and subclinical nigrostriatal dysfunction have been reported in subjects at risk of Parkinsonism. Eight non-manifesting carriers (NMCs) of LRRK2 G2019S mutation had 11C-PK11195 and 18F-DOPA PET to assess microglial activation and striatal dopamine system integrity, respectivel...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09830-3
更新日期:2020-08-01 00:00:00
abstract::Corticobasal degeneration (CBD) was first described by Rebeiz et al. in 1967, and was called corticodentatonigral degeneration with neuronal achromasia [1]. Since then, our knowledge of the clinical features and underlying tau pathology has grown tremendously. Clinical antemortem diagnosis of CBD pathology remains cha...
journal_title:Journal of neurology
pub_type: 历史文章,杂志文章
doi:10.1007/s00415-017-8644-3
更新日期:2018-02-01 00:00:00
abstract:OBJECTIVE:To report the clinical features and long-term outcome of 22 newly diagnosed paraneoplastic patients with GABAB receptor antibodies (GABABR-Abs). METHODS:Retrospective clinical study of CSF-confirmed cases of GABABR-Abs encephalitis. RESULTS:We identified 22 patients (4 female) with GABABR-Abs, with a median...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9132-0
更新日期:2019-01-01 00:00:00
abstract::Circulating and cerebrospinal fluid (CSF) neurofilament light chain (NfL) levels represent a reliable indicator of disease activity and axonal damage in different neuroinflammatory conditions. Recently, high CSF NfL levels have been detected in active autoimmune encephalitis, as opposed to significant lower levels aft...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09306-z
更新日期:2019-07-01 00:00:00
abstract::The aim of this work was to determine whether survival changed during 2002-2009 at a French amyotrophic lateral sclerosis (ALS) center. We included all patients with ALS who were seen consecutively at the center from January 2002-May 2009. Participants were followed from date of first visit through death, date of cens...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6403-4
更新日期:2012-09-01 00:00:00
abstract::Two patients are described, in whom MRI detected unilateral signal abnormalities in the region of the inferior olivary nuclei, suggesting an early stage of olivary hypertrophy. MRI was performed 4 and 7 weeks respectively after traumatic brain-stem injury. Palatal myoclonus was concomitantly observed in one patient, w...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00867352
更新日期:1993-07-01 00:00:00
abstract:INTRODUCTION:Duchenne muscular dystrophy (DMD) is induced by a wide spectrum of mutations such as exon deletions, duplications and small mutations in the dystrophin gene. This is the first study on the mutational spectrum in a cohort of DMD children from India, with an emphasis to compare the mutations in familial and ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09380-3
更新日期:2019-09-01 00:00:00
abstract::Gait variability has potential utility as a predictive measure of dysfunction in Parkinson's disease (PD). Current understanding implicates non-dopaminergic pathways. This study investigated the explanatory characteristics of gait variability in PD on and off medication under single and dual task conditions. Fifty peo...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5789-8
更新日期:2011-04-01 00:00:00
abstract::Multiple System Atrophy (MSA) and idiopathic Parkinson's disease (PD) can be difficult to distinguish. There is an ongoing debate about the diagnostic value of the growth-hormone response to clonidine (CGH-test) in PD and MSA. We investigated whether the CGH-test can identify individual patients in the early stages of...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-002-0809-y
更新日期:2002-09-01 00:00:00