Journal of Neurology

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

List of Papers (Total 608)

Update on the pharmacotherapy of cerebellar and central vestibular disorders

An overview of the current pharmacotherapy of central vestibular syndromes and the most common forms of central nystagmus as well as cerebellar disorders is given. 4-aminopyridine (4-AP) is recommended for the treatment of downbeat nystagmus, a frequent form of acquired persisting fixation nystagmus, and upbeat nystagmus. Animal studies showed that this non-selective blocker of ...

Vestibular assistance systems: promises and challenges

The handicap resulting from a bilateral vestibular deficit is often underestimated. In most cases the deficit settles gradually. Patients do not understand what is happening to them and have many difficulties to describe their symptoms. They have to consult several doctors with different medical specialties before diagnosis. Once the diagnosis is made there is no biological way to ...

DIZZYNET—a European network initiative for vertigo and balance research: visions and aims

Vertigo is one of the most common complaints in medicine. Despite its high prevalence, patients with vertigo often receive either inappropriate or inadequate treatment. The most important reasons for this deplorable situation are insufficient interdisciplinary cooperation, nonexistent standards in diagnostics and therapy, the relatively rare translations of basic science findings ...

Recent advances in amyotrophic lateral sclerosis

ALS is a relentlessly progressive and fatal disease, with no curative therapies available to date. Symptomatic and palliative care, provided in a multidisciplinary context, still remains the cornerstone of ALS management. However, our understanding of the molecular mechanisms underlying the disease has advanced greatly over the past years, giving new hope for the development of ...

The effect of shunt surgery on neuropsychological performance in normal pressure hydrocephalus: a systematic review and meta-analysis

We conducted a systematic review of the literature and used meta-analytic techniques to evaluate the impact of shunt surgery on neuropsychological performance in patients with normal pressure hydrocephalus (NPH). Twenty-three studies with 1059 patients were identified for review using PubMed, Web of Science, Google scholar and manual searching. Inclusion criteria were prospective, ...

Dementia and assisted suicide and euthanasia

The number of dementia patients requesting euthanasia in the Netherlands has increased over the past five years. The issue is highly controversial. In this contribution we discuss some of the main arguments: the nature of suffering, the voluntariness of the request and the role of the physician. We argue that society has a duty to care for patients who suffer from dementia and to ...

Normalisation of brain spectroscopy findings in Niemann–Pick disease type C patients treated with miglustat

Niemann–Pick disease type C (NP-C) is a fatal progressive neurolipidosis involving neuronal storage of cholesterol and gangliosides. Miglustat, an inhibitor of glycosphingolipid synthesis, has been approved to treat neurological manifestations in adults and children with NP-C. This open-label observational study in adults with confirmed NP-C evaluated the efficacy of miglustat (200 ...

Quantifying the burden of caregiving in Duchenne muscular dystrophy

Duchenne muscular dystrophy (DMD) is a rare pediatric neuromuscular disease associated with progressive muscle degeneration and extensive care needs. Our objective was to estimate the caregiver burden associated with DMD. We made cross-sectional assessments of caregiver health-related quality of life (HRQL) and burden using the EuroQol EQ-5D, a Visual Analogue Scale (VAS), the ...

Association between i.v. thrombolysis volume and door-to-needle times in acute ischemic stroke

Centralization of intravenous thrombolysis (IVT) for acute ischemic stroke in high-volume centers is believed to improve the door-to-needle times (DNT), but limited data support this assumption. We examined the association between DNT and IVT volume in a large Dutch province. We identified consecutive patients treated with IVT between January 2009 and 2013. Based on annualized IVT ...

A mixed treatment comparison to compare the efficacy and safety of botulinum toxin treatments for cervical dystonia

A systematic pair-wise comparison of all available botulinum toxin serotype A and B treatments for cervical dystonia (CD) was conducted, as direct head-to-head clinical trial comparisons are lacking. Five botulinum toxin products: Dysport® (abobotulinumtoxinA), Botox® (onabotulinumtoxinA), Xeomin® (incobotulinumtoxinA), Prosigne® (Chinese botulinum toxin serotype A) and Myobloc® ...

Prevalence of multiple sclerosis in the Lazio region, Italy: use of an algorithm based on health information systems

Compared with other areas of the country, very limited data are available on multiple sclerosis (MS) prevalence in Central Italy. We aimed to estimate MS prevalence in the Lazio region and its geographical distribution using regional health information systems (HIS). To identify MS cases we used data from drug prescription, hospital discharge and ticket exemption registries. Crude, ...

Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the Guidelines of the German Neurological Society

Myasthenia gravis (MG) is an autoimmune antibody-mediated disorder of neuromuscular synaptic transmission. The clinical hallmark of MG consists of fluctuating fatigability and weakness affecting ocular, bulbar and (proximal) limb skeletal muscle groups. MG may either occur as an autoimmune disease with distinct immunogenetic characteristics or as a paraneoplastic syndrome ...

Concomitant granule cell neuronopathy in patients with natalizumab-associated PML

Granule cell neuronopathy (GCN) is a rare JC virus infection of the cerebellar granule cell neurons in immunocompromised patients. On brain imaging, GCN is characterized by cerebellar atrophy which can be accompanied by infratentorial white matter lesions. The objective of this study is to investigate the prevalence of MRI findings suggestive of GCN in a large ...

An MRI-defined measure of cerebral lesion severity to assess therapeutic effects in multiple sclerosis

Assess the sensitivity of the Magnetic Resonance Disease Severity Scale (MRDSS), based on cerebral lesions and atrophy, for treatment monitoring of glatiramer acetate (GA) in relapsing-remitting multiple sclerosis (MS). This retrospective non-randomized pilot study included patients who started daily GA [n = 23, age (median, range) 41 (26.2, 53.1) years, Expanded Disability Status ...