Journal of Neurology

http://link.springer.com/journal/415

List of Papers (Total 662)

Prehospital and intra-hospital time delays in posterior circulation stroke: results from the Austrian Stroke Unit Registry

Therapeutic effect of recombinant tissue-plasminogen activator (rt-PA) is time dependent. There is limited evidence whether localization of stroke within the posterior circulation (PCS) is associated with a treatment delay. We aimed to analyze within a nationwide multicenter cohort whether duration of pre- and intra-hospital patient management differs between patients with PCS...

Alzheimer’s disease in people with Down’s syndrome: the prospects for and the challenges of developing preventative treatments

People with Down’s syndrome (DS) are at high risk for developing Alzheimer’s disease (AD) at a relatively young age. This increased risk is not observed in people with intellectual disabilities for reasons other than DS and for this reason it is unlikely to be due to non-specific effects of having a neurodevelopmental disorder but, instead, a direct consequence of the genetics of...

The role of sleep on cognition and functional connectivity in patients with multiple sclerosis

Sleep disturbances are common in multiple sclerosis (MS), but its impact on cognition and functional connectivity (FC) of the hippocampus and thalamus is unknown. Therefore, we investigated the relationship between sleep disturbances, cognitive functioning and resting-state (RS) FC of the hippocampus and thalamus in MS. 71 MS patients and 40 healthy controls underwent...

Importance of cerebrospinal fluid analysis in the era of McDonald 2010 criteria: a German–Austrian retrospective multicenter study in patients with a clinically isolated syndrome

The majority of patients presenting with a first clinical symptom suggestive of multiple sclerosis (MS) do not fulfill the MRI criteria for dissemination in space and time according to the 2010 revision of the McDonald diagnostic criteria for MS and are thus classified as clinically isolated syndrome (CIS). To re-evaluate the utility of cerebrospinal fluid (CSF) analysis in the...

The frontotemporal syndrome of ALS is associated with poor survival

Thirty percent of ALS patients have a frontotemporal syndrome (FS), defined as behavioral changes or cognitive impairment. Despite previous studies, there are no firm conclusions on the effect of the FS on survival and the use of non-invasive ventilation (NIV) in ALS. We examined the effect of the FS on survival and the start and duration of NIV in ALS. Behavioral changes were...

External validation of a 3-step falls prediction model in mild Parkinson’s disease

The 3-step falls prediction model (3-step model) that include history of falls, history of freezing of gait and comfortable gait speed <1.1 m/s was suggested as a clinical fall prediction tool in Parkinson’s disease (PD). We aimed to externally validate this model as well as to explore the value of additional predictors in 138 individuals with relatively mild PD. We found the...

Is chronic ventilatory support really effective in patients with amyotrophic lateral sclerosis?

Most patients with amyotrophic lateral sclerosis (ALS) develop respiratory insufficiency in the advanced stage of their disease. Non-invasive ventilation (NIV) is commonly regarded to be a treatment that is effective in reducing these complaints. To assess whether the effect of NIV on gas exchange and quality of life (QOL) is different in patients with ALS versus without ALS. A...

Neuro-otology- some recent clinical advances

Vestibular disorders manifesting as vertigo, chronic dizziness and imbalance are common problems in neurological practice. Here, we review some recent interesting and important advances in diagnosis of vestibular disorders using the video head impulse test and in the management of benign positional vertigo and migrainous vertigo.

Severe axonal neuropathy is a late manifestation of SPG11

Complex hereditary spastic paraplegia (HSP) is a clinically heterogeneous group of disorders usually inherited in an autosomal recessive manner. In the past, complex recessive spastic paraplegias have been frequently associated with SPG11 mutations but also with defects in SPG15, SPG7 and a handful of other rare genes. Pleiotropy exists in HSP genes, exemplified in the recent...

Progression of motor subtypes in Huntington’s disease: a 6-year follow-up study

The objective of this study is to investigate the progression of predominantly choreatic and hypokinetic-rigid signs in Huntington’s disease (HD) and their relationship with cognitive and general functioning over time. The motor signs in HD can be divided into predominantly choreatic and hypokinetic-rigid subtypes. It has been reported in cross-sectional studies that...

Drug-associated progressive multifocal leukoencephalopathy: a clinical, radiological, and cerebrospinal fluid analysis of 326 cases

The implementation of a variety of immunosuppressive therapies has made drug-associated progressive multifocal leukoencephalopathy (PML) an increasingly prevalent clinical entity. The purpose of this study was to investigate its diagnostic characteristics and to determine whether differences herein exist between the multiple sclerosis (MS), neoplasm, post-transplantation, and...

Visual height intolerance and acrophobia: distressing partners for life

The course of illness, the degree of social impairment, and the rate of help-seeking behavior was evaluated in a sample of individuals with visual height intolerance (vHI) and acrophobia. On the basis of a previously described epidemiological sample representative of the German general population, 574 individuals with vHI were identified, 128 fulfilled the DSM-5 diagnostic...

Hearing and dementia

Hearing deficits associated with cognitive impairment have attracted much recent interest, motivated by emerging evidence that impaired hearing is a risk factor for cognitive decline. However, dementia and hearing impairment present immense challenges in their own right, and their intersection in the auditory brain remains poorly understood and difficult to assess. Here, we...

Variables associated with upper extremity function in patients with Duchenne muscular dystrophy

Preserving upper extremity (UE) function in patients with Duchenne muscular dystrophy (DMD) is extremely important as it is related to independence and quality of life. For clinical decision making, knowledge of variables associated with UE function is necessary. This knowledge is, however, limited. Therefore, this study aims to gain more insight into the variables associated...

Identifying Niemann–Pick type C in early-onset ataxia: two quick clinical screening tools

Niemann–Pick disease type C (NP-C) is a rare multisystemic lysosomal disorder which, albeit treatable, is still starkly underdiagnosed. As NP-C features early onset ataxia (EOA) in 85–90 % of cases, EOA presents a promising target group for undiagnosed NP-C patients. Here, we assessed the ability of the previously established NP-C suspicion index (SI) and a novel abbreviated ‘2/3...