Abstract：
:Among 234 hemophiliacs, 14 patients (5.9%) with intracranial and 1 patient (0.4%) with intramedullary hemorrhage were observed and examined. The average age at the time of hemorrhage was 17 years in patients with severe hemophilia and 43 years in patients with moderate hemophilia. The patients with mild hemophilia and intracranial bleeding were 5 and 58 years old, respectively. Eight intracerebral, 3 subdural and 4 subarachnoid hemorrhages were encountered. In 7 cases bleeding was posttraumatic and in 5 it occurred spontaneously. In 2 patients hemorrhage followed a sudden increase of intracranial pressure. In 1 patient reliable data could not be obtained. Four of the 8 patients with intracerebral bleeding died; all survivors displayed some residual neurological abnormalities. All patients suffering from subarachnoid bleeding recovered completely. No serious complications were encountered after 3 lumbar punctures, 5 cerebral angiographies and after 1 myelography. Only in 1 case with an acquired inhibitor did a local hematoma develop at the site of puncture for the angiography. The same diagnostic procedures should be performed after substitution therapy with anti-hemophiliac plasma in a hemophiliac without inhibitor as in non-hemophiliacs if intracranial or intramedullar bleeding is suspected. In hemophiliacs with an anticoagulant, angiography or lumbar puncture should be performed only in exceptional cases.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Mamoli B,Sonneck G,Lechner Kdoi
10.1007/BF00313358subject
Has Abstractpub_date
1976-01-14 00:00:00pages
143-54issue
2eissn
0340-5354issn
1432-1459journal_volume
211pub_type
杂志文章abstract：:Extra- and intraneural ganglionic cysts rarely involved peripheral nerves. They are found in the neighbourhood of large joints. Intraneural cysts prefer the deep peroneal nerve and cause intermittent pain and severe nerve damage. The ulnar nerve is affected most often at the wrist. There are different types of distal ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00314606
更新日期：1975-06-09 00:00:00
abstract：:Acute relapses of multiple sclerosis (MS) are treated with intravenous methylprednisolone (IVMP), which speeds recovery from exacerbation. It is known that IVMP suppresses the immunological activation which occurs during an acute attack of MS. However, the specific target genes affected by this therapy remain obscure....
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-004-0516-y
更新日期：2004-10-01 00:00:00
abstract：:It has been approximately 50 years since neurologists were introduced to the entities, "progressive supranuclear palsy" and "corticobasal degeneration". Since the two seminal publications, there have been significant advancements in our understanding of these two neurodegenerative diseases, particularly the fact that ...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-015-7682-y
更新日期：2015-03-01 00:00:00
abstract：:Dopamine agonists have been proved safe and effective as initial therapy in early stages of Parkinson's disease. Prospective long-term clinical trials demonstrated that initial symptomatic treatment with a dopamine agonist is associated with a significantly reduced risk for motor complications in comparison with levod...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-004-1605-7
更新日期：2004-09-01 00:00:00
abstract：:Recent reports suggest the possible beneficial effects of haemopoietic stem cell transplantation (HSCT) in autoimmune diseases such as multiple sclerosis (MS). The definition of the risk/benefit ratio for such a treatment is perceived as a major issue for the neurological community worldwide. The First Consensus Confe...
journal_title：Journal of neurology
pub_type： 指南,杂志文章,实务指引
doi：10.1007/s004150050605
更新日期：2000-05-01 00:00:00
abstract：:We have performed genetic screening on the skeletal muscle chloride channel gene (CLCN1) in Taiwanese population. A total of four patients with myotonia congenita (MC) together with 106 normal individuals were examined. All 23 exons of the CLCN1 gene were analysed by direct sequencing of PCR products to detect the nuc...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-004-0383-6
更新日期：2004-06-01 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：: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：:We investigated the effect of chronic mechanical compression of the cervical spinal cord on the number of spinal accessory motoneurons in 25 tiptoe-walking Yoshimura mice. The animals had calcified deposits in the atlantoaxial membrane at the C1-C2 vertebral level, compressing the spinal cord posterolaterally. Motoneu...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150050076
更新日期：1997-04-01 00:00:00
abstract：:Premature atherosclerosis and cardiac complications have been reported among the systemic manifestations of cerebrotendinous xanthomatosis (CTX), a rare bile acid disorder with predominantly neurological features. In some cases, myocardial infarction has been the cause of sudden death. We examined nine CTX patients to...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150050274
更新日期：1998-11-01 00:00:00
abstract：:Cerebral hemodynamics play a pivotal role in stroke pathogenesis. Transcranial Doppler (TCD) studies demonstrated the importance of cerebral vasomotor reactivity (VMR) on the outcome of carotid artery occlusion (CAO). So far, positron emission tomography represents the best technique for detecting both hemodynamic and...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-006-0244-6
更新日期：2006-11-01 00:00:00
abstract：OBJECTIVE:We characterised the clinical and neuro-otological characteristics of patients with Susac syndrome. METHODS:The medical records of 30 patients with Susac syndrome were reviewed for details of their clinical presentation and course, neuro-otological symptoms, investigation results including audiology and vest...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-10086-0
更新日期：2020-12-01 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：:We studied the diagnostic value of computed tomographic angiography (CTA) in the posterior circulation as a noninvasive substitute for intra-arterial digital subtraction angiography (DSA). We prospectively investigated 103 patients with acute stroke in the posterior circulation. All patients underwent CTA and Doppler ...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章
doi：10.1007/s004150070089
更新日期：2000-10-01 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：:The symptomatology of 18 patients with the severest clinical course after subarachnoid hemorrhage (SAH) is described and analyzed. Seventeen patients died, five with an acute bulbar syndrome with cardiac arrest, and 12 with irreversible breakdown of brain function. One patient had an apallic syndrome with minimal sign...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313005
更新日期：1979-01-01 00:00:00
abstract：:Botulinum neurotoxin (BoNT) is an effective treatment for cervical dystonia (CD). Long-term changes of several variables, including the dose of BoNT, in these patients is largely unknown. We reviewed the clinical charts of 275 patients with CD treated with BoNT type A (BoNT-A) for at least 5 years since 1989 at ten te...
journal_title：Journal of neurology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00415-010-5880-1
更新日期：2011-06-01 00:00:00
abstract：BACKGROUND:Multiple sclerosis (MS) notably affects adults of working age. For persons with MS (PwMS), being employed enhances their quality of life and it may be regarded as an indicator of overall functioning. Thus, ensuring work participation in PwMS is of general public health interest. OBJECTIVE:To examine relevan...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-09973-3
更新日期：2020-10-01 00:00:00
abstract：:Acanthocytosis occurs because of ultrastructural abnormalities of the erythrocyte membranous skeleton resulting in reduced membrane fluidity. At least three hereditary neurological conditions are associated with it, although as yet the pathogenesis of the neurological features is unknown. In abetalipoproteinaemia, an ...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s004150170241
更新日期：2001-02-01 00:00:00
abstract：:Four patients from three unrelated families, with clinical and electrophysiological findings compatible with the diagnosis of hereditary motor and sensory neuropathy, are presented. The molecular analysis showed that the affected individuals were homozygous for the mutation in the X25 gene, characteristic of Friedreic...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-002-0902-2
更新日期：2002-11-01 00:00:00
abstract：:Isolated floccular infarction is extremely rare, and impairments of the vestibulo-ocular reflex (VOR) have not been explored in humans with isolated floccular lesions. The purpose of this study was to examine and report selective impairment of VOR in response to high acceleration using head impulse in a patient with i...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-013-6837-y
更新日期：2013-06-01 00:00:00
abstract：:Eight cases of malignancies with concurrent motor neuron disease (MND), derived from an unselected population representing all cases of MND diagnosed during a 15-year period in two Italian provinces, were studied to verify the existence of paraneoplastic forms of the disease. No statistically significant association b...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00314238
更新日期：1988-07-01 00:00:00
abstract：BACKGROUND:Dermic fibroblasts have been proposed as a potential genetic-ALS cellular model. This study aimed to explore whether dermic fibroblasts from patients with sporadic-ALS (sALS) recapitulate alterations typical of ALS motor neurons and exhibit abnormal DNA-damage response. METHODS:Dermic fibroblasts were obtai...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-09704-8
更新日期：2020-05-01 00:00:00
abstract：BACKGROUND:Cognitive impairment is a debilitating symptom experienced by ~ 50% of multiple sclerosis patients, with processing speed (PS) and memory most affected. Until now, the field has considered cognition in a binary fashion: patients are designated as impaired or not impaired. This designation is typically arrive...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-018-8747-5
更新日期：2018-03-01 00:00:00
abstract：:Two patients are reported with a chronic progressive illness characterized by dementia, ataxia and spasticity. There were no myoclonic jerks and both had normal electroencephalograms (EEG). Pathological findings in three brain biopsies were those of viral meningoencephalitis with perivenous demyelination. Serological ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313048
更新日期：1979-09-01 00:00:00
abstract：BACKGROUND:Transcranial direct current stimulation (tDCS) has been investigated as a tool for dysphagia recovery after stroke in several single-center randomized controlled trials (RCT). OBJECTIVE:The aim of this investigation was to quantitatively evaluate the effect of tDCS on dysphagia recovery after a stroke utili...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-10142-9
更新日期：2021-01-01 00:00:00
abstract：:The geste antagoniste is a voluntary maneuver that temporarily reduces the severity of dystonic posture or movements. It is a classical feature of focal and particularly cervical dystonia. However, the precise historical aspects of geste antagoniste still remain obscure. The goals of this review were (1) to clarify th...
journal_title：Journal of neurology
pub_type： 历史文章,杂志文章
doi：10.1007/s00415-011-6380-7
更新日期：2012-08-01 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：OBJECTIVES:To determine demographic or disease-related factors that may influence the severity of autonomic dysfunction in idiopathic Parkinson's disease (IPD). METHODS:532 patients with IPD aged between 55 and 75 years were included. Severity of autonomic dysfunction was assessed using a 9-item autonomic dysfunction ...
journal_title：Journal of neurology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00415-005-0909-6
更新日期：2005-12-01 00:00:00
abstract：:Lipids were extracted from brains of a patient with membranous lipodystrophy (ML) and three normal patients and the neutral lipid and sphingolipid constituents were investigated. The storage of a large amount of free fatty acid was observed in the ML brain, but no cholesterol ester was found. Total lipid, cholesterol ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313947
更新日期：1979-03-22 00:00:00
