Abstract:
OBJECTIVE:Childhood arterial ischaemic stroke (AIS) is rare, but causes significant morbidity and mortality. We aimed to investigate incidence, age-dependent clinical presentation, and risk factors and to discuss the medical care situation in Germany. METHODS:This prospective epidemiological study was conducted via ESPED (Erhebungseinheit für Seltene Pädiatrische Erkrankungen in Deutschland), a hospital-based German nation-wide surveillance unit for rare pediatric diseases. Children aged 28 days-18 years with first AIS between January 2015 and December 2017 were included. RESULTS:In the 3-year period, 164 children were reported. Incidence showed peaks in infants, children < 2 years of age, and adolescents (12-18 years), with a significant male predominance observed in adolescents only. Independent of age, most children (91%) presented with focal symptoms, particularly with acute hemiparesis. The occurrence of seizures in infants (57%) and more nonspecific symptoms in school-children and adolescents (54%) is considered noteworthy. Prothrombotic states (34%), cardiac disorders (29%), and arteriopathies (19%) were the most frequently identified risk factors. The majority of children (72/131, thus 55%) were discharged home after acute care phase. At time of discharge, most common neurological symptoms were hemiparesis (42%), facial palsy (15%), and speech disturbance (12%). CONCLUSION:This study provides population-based data of childhood AIS which may be useful for further research. The improvement of acute stroke management is needed for children, but also the standardization of post-stroke care in the outpatient setting has to be structured. Considering the higher stroke incidence in (male) adolescents, it is advisable to combine research activities in adolescents and young adults.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Gerstl L,Weinberger R,Heinen F,Bonfert MV,Borggraefe I,Schroeder AS,Tacke M,Landgraf MN,Vill K,Kurnik K,Sorg AL,Olivieri Mdoi
10.1007/s00415-019-09508-5subject
Has Abstractpub_date
2019-12-01 00:00:00pages
2929-2941issue
12eissn
0340-5354issn
1432-1459pii
10.1007/s00415-019-09508-5journal_volume
266pub_type
杂志文章abstract::PNPLA6 mutations, known to be associated with the development of motor neuron phenotypes, have recently been identified in families with Boucher-Neuhäuser syndrome. Boucher-Neuhäuser is a rare autosomal recessive syndrome characterized by the co-occurrence of cerebellar ataxia, hypogonadotropic hypogonadism, and chori...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7516-3
更新日期:2014-12-01 00:00:00
abstract::The SOX10 transcription factor is involved in development of neural crest derivatives and fate determination in glial cells. SOX10 mutations have been found in patients with intestinal aganglionosis and depigmentation with deafness (Waardenburg-Hirschsprung). Associated neurological signs have been reported in some ca...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150170159
更新日期:2001-06-01 00:00:00
abstract::Patients with multiple system atrophy (MSA) often have evidence of compromised gastrointestinal motility. Ghrelin is a gut hormone that influences gastrointestinal motility in humans. The aim of this study was to determine whether ghrelin secretion is affected in MSA patients, and to investigate the relation between g...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-6944-9
更新日期:2013-08-01 00:00:00
abstract::Cardiac conduction and/or rhythm abnormalities (CCRA) are the most frequent and life-threatening complications in DM1. In order to determine prevalence, incidence, characteristics, age of onset and predictors of CCRA, CCRA progression and sudden cardiac death (SCD) in DM1, we collected ECG/24hECG-Holter data from a ye...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8773-3
更新日期:2018-04-01 00:00:00
abstract:OBJECTIVE:To investigate the efficacy of acupuncture on stroke recovery compared to an inert placebo. DESIGN:Placebo-controlled, randomised, clinical trial. SETTING:Post-stroke rehabilitation wards in five NHS hospitals in the UK. SUBJECTS:Patients between 4 and 10 days after their first stroke. INTERVENTIONS AND O...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s00415-008-0790-1
更新日期:2008-06-01 00:00:00
abstract::Local administration of botulinum toxin (BoTx) inhibits presynaptic acetylcholine release. All cholinergically innervated muscles and glands can be paralyzed accordingly. Studies on the application of BoTx in multiple sclerosis have shown good results for focal spasticity, in particular, of the extremities. The first ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-006-1104-0
更新日期:2006-02-01 00:00:00
abstract::Mutations in C19orf12 have been recently identified as the molecular genetic cause of a subtype of neurodegeneration with brain iron accumulation (NBIA). Given the mitochondrial localization of the gene product the new NBIA subtype was designated mitochondrial membrane protein-associated neurodegeneration. Frequent fe...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6521-7
更新日期:2012-11-01 00:00:00
abstract::An 18-year-old man suffered from acute Sydenham chorea appearing coincidently with beta-haemolytic streptococcal throat infection. Imaging techniques documented lesions of basal ganglia and substantia nigra. In the early course of the disease vascular lesions may be important pathogenetic mechanisms of this acquired m...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00858729
更新日期:1993-02-01 00:00:00
abstract::A patient is described who developed complex partial seizures with secondary generalization 3 years after a severe viral encephalitis with a CT and EEG identified lesion in the left insular cortex and its surrounding structures. When the seizures first occurred CT and MRI as well as repeated interictal conventional EE...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314461
更新日期:1989-07-01 00:00:00
abstract:BACKGROUND:Spontaneous cervical artery dissections (sCAD) are often preceded by infections. However, existing data about inflammatory parameters remained inconsistent. Remarkably, concurrent information about the coagulation system, whose affection seems also reasonable to cause ischaemic events, are still lacking in s...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8696-4
更新日期:2018-02-01 00:00:00
abstract:OBJECTIVES:The mechanisms of early neurologic deterioration (END) and prevention strategies for END are not completely understood. The aim of this study was to investigate the association between CYP2C19*2 variants and END, and the effectiveness of antiplatelet therapy for prevention of END according to CYP2C19*2 genot...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s00415-018-8998-1
更新日期:2018-10-01 00:00:00
abstract:BACKGROUND:Cerebral venous thrombosis (CVT) is associated with intracranial hemorrhage. AIM:To identify clinical and imaging features of CVT-associated intracranial hemorrhage. We hypothesized that higher clot burden would be associated with a higher risk of intracranial hemorrhage. METHODS:We performed a retrospecti...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10008-0
更新日期:2020-11-01 00:00:00
abstract:BACKGROUND:Spinocerebellar ataxias (SCAs) are rare dominantly inherited neurodegenerative disorders that lead to severe disability and premature death. OBJECTIVE:To better characterize the natural history of the most common SCAs, SCA1, SCA2, SCA3 and SCA6, we performed a meta-analysis of literature to determine diseas...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-020-09815-2
更新日期:2020-04-07 00:00:00
abstract::Although central facial paresis (CFP) is a major symptom of stroke, there is a lack of studies on the motor and non-motor disabilities in stroke patients. A prospective cohort study was performed at admission for inpatient rehabilitation and discharge of post-stroke phase of 112 patients (44% female, median age: 64 ye...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9099-x
更新日期:2019-01-01 00:00:00
abstract::We describe the case of a 40-year-old male patient who had presented clinically as primary lateral sclerosis for the past 10 years and neuropathologically as diffuse Lewy body disease (DLBD). Neuropathology demonstrated DLBD as an almost ubiquitous disorder of the neuronal cytoskeleton. ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00887816
更新日期:1995-01-01 00:00:00
abstract::Dementia is reversible in a minority of patients, and these should be diagnosed but without subjecting the majority with irreversible disease to an excessive set of investigations. Should a battery of ancillary investigations be performed routinely in dementia? Or can these tests be carried out as clinically indicated...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150050311
更新日期:1999-02-01 00:00:00
abstract:INTRODUCTION:Intracranial lipomas are rare, mostly congenital lesions. Sporadic case reports suggest an association with focal epilepsy. METHODS:All admissions to our epilepsy monitoring unit who had had brain MRI were reviewed for intracranial lipomas during 6 consecutive years. RESULTS:Five patients with intracrani...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0065-7
更新日期:2006-05-01 00:00:00
abstract:BACKGROUND:Conflicting findings have been reported on the prognostic significance of serum 25-hydroxyvitamin D level in patients with stroke. The objective of this meta-analysis was to evaluate the prognostic utility of serum 25-hydroxyvitamin D in stroke patients. METHODS:PubMed and Embase databases were systematical...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-019-09599-0
更新日期:2020-11-01 00:00:00
abstract:OBJECTIVES:There are varying reports on whether monoclonal gammopathy of undetermined significance-associated neuropathy (MGUSN) patients are distinguishable from those with chronic inflammatory demyelinating polyneuropathy (CIDP) and whether specific MGUSN subclasses are associated with specific clinical phenotypes. ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7357-0
更新日期:2014-08-01 00:00:00
abstract::Idiopathic hypereosinophilic syndrome (IHES) is a primary haematological condition characterised by persistent, otherwise unexplained hypereosinophilia sufficient to cause organ damage. Various neurological complications are reported, but very few have mentioned CNS pathology and none has included CNS vasculitis. Our ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-015-7720-9
更新日期:2015-05-01 00:00:00
abstract:CONTEXT:Polyneuropathy is a common disease and is more prevalent (at least 3 %) in elderly people. However, routine neurological examination of healthy elderly people may show distal sensory loss and absent tendon reflexes, which can obscure the distinction from polyneuropathy. OBJECTIVE:To investigate the relation be...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析
doi:10.1007/s00415-005-0064-0
更新日期:2006-05-01 00:00:00
abstract:OBJECTIVES:The outcome of aphasia at 3 months is variable in patients with moderate/severe stroke. The aim was to predict 3-month aphasia outcome using prediction models including initial severity in addition to the interaction between lesion size and location at the acute phase. METHODS:Patients with post-stroke apha...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09259-3
更新日期:2019-06-01 00:00:00
abstract::Palatal myoclonus is thought to occur after damage to certain brain-stem structures, and with a delay following the causative lesion. A case of palatal myoclonus, probably of epileptic nature, is described. ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00839145
更新日期:1992-04-01 00:00:00
abstract::Cerebral palsy (CP) continues to be a major problem in India. The present study provides an insight into the various clinical and neuroradiological correlates of CP. The study included 102 children with CP and was subjected to magnetic resonance imaging (MRI) of the brain. Forty-seven (46%) patients belonged to the 1-...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5782-2
更新日期:2011-03-01 00:00:00
abstract:PURPOSE:Epileptogenic foci exhibit disturbed function at the level of the benzodiazepine receptor. The aim of our study was to investigate the incidence of focal reductions of temporal benzodiazepine receptor binding (BRB) as assessed by scintigraphy with 123I-iomazenil in patients with denovo temporal lobe epilepsy (T...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150170136
更新日期:2001-07-01 00:00:00
abstract:BACKGROUND:One of the goals in treating subarachnoid hemorrhage patients is to prevent or minimize vasospasm-induced ischemia. Intracerebral microdialysis is a rapidly developing tool to monitor physiological and pathophysiological changes in chemical processes associated with ischemia. OBJECTIVE:To determine the diag...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-003-1079-z
更新日期:2003-07-01 00:00:00
abstract::The aim of the study was to identify the main factors that impact mobility impairment in multiple sclerosis (MS) patients in Italy. Clinicians from a large number of Italian MS centers took part in a Delphi process aimed at obtaining consensus statements among the participants. Large consensus was obtained for stateme...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-7230-6
更新日期:2014-03-01 00:00:00
abstract::The Richards-Rundle syndrome (RRS) is characterized by hearing loss, mental deterioration, ataxia, primary hypogonadism and autosomal recessive transmission. In a sibship of six members we found two sisters with RRS together with baldness, impaired GH and PRL secretion after stimulation and different degrees of impair...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313645
更新日期:1984-01-01 00:00:00
abstract:OBJECTIVE:To determine the response to treatment and the long-term outcome of patients with the antisynthetase syndrome associated with anti-Jo-1-antibodies. PATIENTS AND METHODS:A total of 12 patients with histologically proven myositis and anti-Jo-1-autoantibodies were evaluated over a mean follow-up period of 66.4 ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0449-5
更新日期:2004-07-01 00:00:00
abstract::Pompe disease is an autosomal recessive disorder in which deficiency of the lysosomal enzyme acid alpha-glucosidase results in the accumulation of glycogen mostly in muscle tissues. Several reports suggest a higher incidence of intracranial vascular abnormalities (IVAs) in this condition, as well as brain microbleeds ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8601-1
更新日期:2017-10-01 00:00:00