Journal of Neurology

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

List of Papers (Total 658)

N-acetylaspartic acid in cerebrospinal fluid of multiple sclerosis patients determined by gas-chromatography-mass spectrometry

Background Axonal degeneration is considered to play a major role in the development of clinical disability in multiple sclerosis (MS). N-AcetylAspartic Acid (NAA) is a neuron-specific marker constantly identified in MR-spectroscopy studies of the normal and MS brain. To our knowledge there are no studies available that evaluated NAA in cerebrospinal fluid (CSF) as a possible...

Too hot to handle: heat stroke in multiple system atrophy

Sirs: Autonomic dysfunction is a common feature of multiple system atrophy (MSA). It can be incapacitating for patients, leading to urinary urge, severe constipation, altered sweating, and orthostatic hypotension with falls due to syncope [1]. Here, we present an unusual complication of autonomic dysfunction in a patient with definite MSA that came to light during the unusual hot...

Spinal arteriovenous shunts presenting as intracranial subarachnoid haemorrhage

Background In approximately 5% of patients with intracranial subarachnoid haemorrhage (SAH), the cause is another than a ruptured aneurysm or perimesencephalic haemorrhage. One of these causes is a spinal arteriovenous shunt (SAVS). The aim of this study was to investigate the characteristics of patients with SAVS who present with intracranial SAH without symptoms and signs...

Neck pain in chronic whiplash syndrome treated with botulinum toxin. A double-blind, placebo-controlled clinical trial

Objectives Neck pain in chronic whiplash syndrome is a major burden for patients, healthcare providers and insurance companies. Randomized data on treatment of botulinum toxin in chronic whiplash syndrome are scarce. We conducted a randomized, placebo-controlled clinical trial to prove efficacy of botulinum toxin for neck pain in chronic whiplash syndrome. Methods 40 patients...

Tremor in multiple sclerosis

Tremor is estimated to occur in about 25 to 60 percent of patients with multiple sclerosis (MS). This symptom, which can be severely disabling and embarrassing for patients, is difficult to manage. Isoniazid in high doses, carbamazepine, propranolol and gluthetimide have been reported to provide some relief, but published evidence of effectiveness is very limited. Most trials...

Does modafinil enhance activity of patients with myotonic dystrophy?

We performed a double-blind placebo-controlled crossover study in 13 patients with myotonic dystrophy to address the question whether modafinil, known to improve hypersomnolence in myotonic dystrophy, may improve levels of activity as well. We used the Epworth Sleepiness Scale as a measure of hypersomnolence and a structured interview of the patient and the partner or housemate...

Genetics of human hydrocephalus

Human hydrocephalus is a common medical condition that is characterized by abnormalities in the flow or resorption of cerebrospinal fluid (CSF), resulting in ventricular dilatation. Human hydrocephalus can be classified into two clinical forms, congenital and acquired. Hydrocephalus is one of the complex and multifactorial neurological disorders. A growing body of evidence...

First aid in acute stroke

Objective First aid training is well established to teach the public how to recognize a medical emergency and take appropriate action. Though it is now handled as a high priority emergency stroke is not among the main topics of first aid. We investigated if first aid training may be useful for enhancing stroke awareness. Methods We developed a 15–20 minute teaching session about...

Cerebrospinal fluid ferritin in chronic hydrocephalus after aneurysmal subarachnoid hemorrhage

Objectives Subarachnoid hemorrhage (SAH) is a common cause of chronic hydrocephalus. Blood in the subarachnoid space is intracranially metabolized to bilirubin and iron, and free iron is thereafter detoxified by ferritin. However, no studies have reported the relationship between intracranial heme metabolism and chronic hydrocephalus after SAH. The goal of this prospective study...

Comparison of the impact of atrial fibrillation on the risk of early death after stroke in women versus men

BackgroundAtrial fibrillation (AF) is considered a predictive factor of poor clinical outcome in patients with an ischemic stroke (IS). This study addressed whether the impact of AF on the in-hospital mortality after first ever IS is different according to the patient’s gender.MethodsWe prospectively studied 1678 patients with first ever IS consecutively admitted to two...

Treatment of amyotrophic lateral sclerosis— What is the next step?

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which was thought to be untreatable. However, recent evidence in both experimental animals and men indicates that antiglutamatergic strategies are the first to have an influence on its pathogenesis and slow down the disease process. Since the effect of drugs is still small, this progress cannot only be seen...

Molecular factors underlying selective vulnerability of motor neurons to neurodegeneration in amyotrophic lateral sclerosis

Current research evidence suggests that genetic factors, oxidative stress and glutamatergic toxicity, with damage to critical target proteins and organelles, may be important contributory factors to motor neuron injury in amyotrophic lateral sclerosis (ALS). Various molecular and neurochemical features of human motor neurons may render this cell group differentially vulnerable to...

ISIS Survey: an international study on the diagnostic process and its implications in amyotrophic lateral sclerosis

In a number of ALS patients, a long delay between onset of symptoms and diagnosis is reported. In this international study (ISIS Survey) we have identified the pathway followed by ALS patients from first symptoms to diagnosis confirmation. Diagnostic data from a total of 201 patients with definite ALS from 6 countries (Argentina, Brazil, Germany, Italy, Spain and the USA) were...

Riluzole does not have an acute effect on motor thresholds and the intracortical excitability in amyotrophic lateral sclerosis

Intracortical excitability in amyotrophic lateral sclerosis (ALS) is impaired. The effectiveness of the glutamate antagonist riluzole (Rilutek®, Rhône-Poulenc Rorer) in ALS has been shown in clinical studies. In healthy subjects it modifies intracortical excitability in a frequently used double-stimulus paradigm of transcranial magnetic stimulation (TMS). Under riluzole...

How to improve the diagnostic process

The existing therapeutic options for treating amyotrophic lateral sclerosis (ALS) are relatively limited but, as therapies improve, there will be a need for early diagnosis of this rare but fatal neurogenerative disorder. Early diagnosis not only requires the utility of a good biological marker, it also requires medical personnel who are experienced to consider/establish a...

Development and validation of a short measure of health status for individuals with amyotrophic lateral sclerosis/ motor neurone disease: the ALSAQ-40

In recent years there has been an increased appreciation of the importance of measuring health status from the patient’s point of view, but until now no attempt has been made to develop an amyotrophic lateral sclerosis (ALS)-specific health status measure. The development of such an instrument is especially relevant now with the introduction of drugs that prolong life in ALS but...

Biological markers in amyotrophic lateral sclerosis: help or hindrance?

Biological markers play an important role in the development of the understanding of a disease, its diagnosis and treatment. This is particularly true of amyotrophic lateral sclerosis (ALS) at this time. We need better biological markers for the diagnosis, for improved understanding of the underlying pathogenetic mechanisms, and for assistance in new drug development. This review...

Getting the diagnosis right: Beyond El Escorial

The development and endorsement of the ‘El Escorial criteria’ by the World Federation of Neurology (WFN) for the diagnosis of amyotrophic lateral sclerosis (ALS) in 1990 and subsequent revision of these guidelines in April 1998 has provided physicians with a much needed tool in the secure diagnosis of ALS. However, even today, over 100 years since ALS was first described by...