The effect of oral and intravenous methylprednisolone treatment on subsequent relapse rate in multiple sclerosis.

We investigated the effect of oral and intravenous methylprednisolone treatment on subsequent relapse rate in patients with multiple sclerosis. Following a double blind trial designed to compare the effect of oral and intravenous methylprednisolone treatment on promoting recovery from acute relapses of multiple sclerosis, 80 patients were followed for two years with six-monthly assessments during which all subsequent relapses were recorded. The annual relapse rate was slightly higher in the oral compared with the intravenous methylprednisolone-treated patients (1.

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 limited data is available on the impact such treatments have on quality of life. This paper reports on the development of an ALS-specific measure, the forty item ALS assessment questionnaire (ALSAQ-40).

Hugh Cairns, Dorothy Russell and the first pleomorphic xanthoastrocytoma?

The 40-year survival of a patient with an unusual astrocytoma, excised in 1930, has been re-evaluated by study of the original microfilmed records, histological slides and paraffin-wax embedded tissue blocks. The operation and neuropathological examination were carried out by Mr Hugh Cairns and Dr Dorothy Russell, in the very early days of neurosurgery. Review of the original tissue specimen with immunocytochemistry has revealed that the tumour was a pleomorphic xanthoastrocytoma, the earliest recorded example.

What is primary lateral sclerosis?

Primary lateral sclerosis (PLS) is a rare degenerative disorder of the upper motor neuron. Its nosological status and relationship to other motor neuron syndromes, especially amyotrophic lateral sclerosis (ALS), is uncertain. Diagnostic criteria have been proposed.

Consensus guidelines for the design and implementation of clinical trials in ALS. World Federation of Neurology committee on Research.

Background: In 1994 consensus guidelines were developed for conducting clinical trials in ALS. With growing experience in clinical trials, it has become clear that a number of further guidelines were needed.

Methods: Under the auspices of the World Federation of Neurology Committee on Research, a multinational group of neurologists, statisticians, patient advocates, representatives from the pharmaceutical industry as well as regulatory agencies developed consensus about a number of revisions to the existing guidelines during a 2 day conference in April 1998.

An algorithm for ALS diagnosis and management.

The diagnosis of ALS is often delayed, probably largely because the disease is relatively uncommon. A practical approach to diagnosis, with a simple algorithm for investigation, might improve diagnostic sensitivity in the early stages of the disease. It is clear that the first step in the diagnostic algorithm must be referral to a neurologist, which implies recognition by the primary care physician that the illness is neurologic in origin.

Extrapyramidal involvement in amyotrophic lateral sclerosis: backward falls and retropulsion.

Three patients with sporadic amyotrophic lateral sclerosis (ALS) presented with a history of backward falls. Impaired postural reflexes and retropulsion accompanied clinical features of ALS. Hypokinesia, decreased arm swing, and a positive glabellar tap were noted in two of these three patients.

Paraneoplastic painful ulnar neuropathy.

A 58-year-old woman developed painful, bilateral ulnar neuropathy in conjunction with small cell lung carcinoma and high serum titer of anti-Hu antibody. An incidental stage I plasma cell dyscrasia, with immunoglobulin G kappa monoclonal protein, was also present. Electropysiological assessment excluded a generalized neuropathy, and nerve biopsy showed marked loss of myelinated and small unmyelinated fibers, without inflammatory changes or amyloid deposition.

I.v. immunoglobulin reduces circulating proinflammatory cytokines in Guillain-Barré syndrome.

Background: Treatment with human i.v. immunoglobulin (IVIg) modifies the course of Guillain-Barré syndrome (GBS), but its specific mode of action is unknown.

IgM paraproteinemia in a patient with primary lateral sclerosis.

Primary lateral sclerosis is an uncommon, distinct clinical entity. We report a patient with primary lateral sclerosis in whom investigations revealed an IgM monoclonal gammopathy, raised CSF protein and persistently high ESR. A number of reports suggest that lymphoproliferative disorders, paraproteinemia and clinico-pathological syndromes mimicking motor neuron diseases may be associated.

Dopaminergic therapy in acute encephalitis lethargica.

We describe a patient with an acute illness, with clinical features characteristic of encephalitis lethargica, who responded repeatedly to trials of an apomorphine infusion and subsequently to oral levodopa therapy. This observation implies a direct acute neurotropic effect of the encephalitis on nigral dopaminergic cells. Dopaminergic medication may therefore be helpful in the acute stage of encephalitis lethargica as well as in the delayed post-encephalitic parkinsonian phase of the syndrome.

The European Amyotrophic Lateral Sclerosis Health Profile Study. ALS-HPS Steering Group.

Quality of life is increasingly regarded as an important outcome measure in the evaluation of treatment regimes. The last decade has seen an enormous growth in the application of measures designed to assess quality of life in a vast array of medical specialities. However, the use of such measures in neurology has been limited and is virtually non-existent in amyotrophic lateral sclerosis (ALS).

Riluzole has no acute effect on motor unit parameters in ALS.

Riluzole is the only drug to have been approved for the treatment of amyotrophic lateral sclerosis (ALS/MND). Its mechanism of action is complex and includes actions on NMDA and kainate receptors and modulation of voltage gated Na channels. In ALS, its effects on measurable parameters of the motor units utilising current neurophysiological techniques are unknown.

Early diagnosis of ALS/MND.

With the advent of a specific biological therapy for ALS there is an increasing imperative for early diagnosis. As new, more effective therapies become available, this will become more important. It is intuitively probable that early therapy will prevent disability in this otherwise relentlessly progressive disorder.

Quality of life in multiple sclerosis in France, Germany, and the United Kingdom. Cost of Multiple Sclerosis Study Group.

Objective: To assess the quality of life (QoL) of patients with multiple sclerosis in France, Germany, and the United Kingdom with a cross sectional study.

Methods: Patients were classified into three severity groups according to the expanded disability severity scale (EDSS); stage I, II, and III, corresponding to mild (EDSS 1.0-3.

Sacral reflexes: physiology and clinical application.

Sacral reflexes consist of motor responses in the pelvic floor and sphincter muscles evoked by stimulation of sensory receptors in pelvic skin, anus, rectum, or pelvic viscera. These responses may be elicited by physical or electrical stimuli. They have been used in research studies of the pathophysiology of pelvic floor and anorectal disorders and many have been recommended for diagnostic use.

Fasciculation potentials: a study of amyotrophic lateral sclerosis and other neurogenic disorders.

We studied fasciculation potentials in amyotrophic lateral sclerosis (ALS), and in other neurogenic disorders, in strength and single-fiber electromyography-matched muscles. Benign fasciculations were studied in 3 normal subjects. Fasciculations were more stable and easier to recruit voluntarily in the early phase of ALS; later, fasciculations were more unstable, more complex, and less likely to be voluntarily recruited.

Hypokalaemic myopathy in alcoholism.

We describe the clinical and pathological features of a patient with an acute painless proximal myopathy due to hypokalaemia associated with alcoholism. There was an excellent response to treatment with potassium supplements. The importance of recognition of low potassium states in alcohol-dependent patients with muscular weakness is emphasized.

Faecal incontinence in myotonic dystrophy.

Two siblings with myotonic dystrophy presented for treatment of faecal incontinence. The pathophysiology of this functional disorder is described with the results of anorectal manometry, EMG, and biopsy of smooth and striated muscle of the anorectal sphincters. Both medical and surgical management of the incontinence was unsatisfactory in the long term.

Fasciculations: what do we know of their significance?

Fasciculations are observed in patients with neurogenic disorders and in healthy individuals. Depending on the associated clinical symptoms and signs, they may signify the presence of a variety of disorders of the lower motor neuron. Divergent and occasionally conflicting opinions prevail regarding the aetiology, pathogenesis, clinical significance, neurophysiological characteristics and the physiological site of origin of fasciculations.

Circulating tumor necrosis factor-alpha correlates with electrodiagnostic abnormalities in Guillain-Barré syndrome.

Autoimmune damage to peripheral nerves, mediated by activated T lymphocytes and macrophages, underlies the pathogenesis of inflammatory demyelination in Guillain-Barré syndrome. Both T lymphocytes and macrophages secrete tumor necrosis factor-alpha, a cytokine that exerts toxic effects on myelin, Schwann cells, and endothelial cells. The reportedly high serum levels of this cytokine in patients with Guillain-Barré syndrome may reflect the degree of immune activation rather than a direct pathogenic effect.

Health outcome and quality-of-life measurements in amyotrophic lateral sclerosis.

Quality of life has been used as a primary outcome measure in the treatment of cancer and cardiovascular disease, and as a secondary outcome measure in therapy of Parkinson's disease. However, it has been relatively neglected in studies of amyotrophic lateral sclerosis (ALS). Although there is need for the development of an ALS-specific quality-of-life measure, it will be necessary, nonetheless, to continue to use generic measures in order to ensure comparability of measurement between disease states.

Randomised trial of oral and intravenous methylprednisolone in acute relapses of multiple sclerosis.

Background: An intravenous rather than oral course of methylprednisolone is often prescribed for treating acute relapses in multiple sclerosis (MS) despite the lack of evidence to support this route of administration. Our double-blind placebo-controlled randomised trial was designed to compare the efficacy of commonly used intravenous and oral steroid regimens in promoting recovery from acute relapses in MS.

Methods: 42 patients with clinically definite relapse in MS received oral, and 38 intravenous, methylprednisolone.