ALS, sometimes called Lou Gehrig's disease or Motor Neuron Disease (MND), is characterized by degeneration of a select group of nerve cells and pathways in the brain and spinal cord, leading to progressive paralysis of the muscles.

ALS involves the loss of motor nerve cells. The nerves affected are in the spinal cord and those that travel to the voluntary muscles, with weakness and wasting in the arms, legs and mouth, throat and respiratory system. The loss of nerve cells results in atrophy, or wasting of the muscles served by those cells.

Although symptoms of ALS usually present on one side of the body, both sides are involved and the effects usually become more symmetrical as the disorder progresses.

ALS does not discriminate. Anyone can get ALS--male or female of any race. It usually becomes evident as one approaches middle age. There is a very rare form transmitted from generation to generation and a very rare juvenile form.

ALS progresses relentlessly. There is no recovery or reversal and few plateaus; it is merely a rapid decline in motor capacity. For many, there is little impairment of the intellect and the senses remain intact.

Approximately 2000 Canadians live with ALS at any one time. Ninety percent of people with ALS will die within six years and the progression of the disease will remove them from society for much of that time. Two to three Canadians die of ALS every day.

What can be done?
Nation-wide, ALS clinics employ a team approach to the treatment of disease symptoms and assisting the person with ALS to live as fully as possible. Along with neurologists and other physicians, the team may include a physiatrist, respiratory therapist, occupational therapist, physiotherapist, dietitian, speech-language pathologist, social worker and pastoral care provider.

ALS Societies across the country make a valuable contribution, providing information and referral, access to specialized equipment in a timely manner, support groups for all concerned and advocacy for those affected by the disease.

These teams help those affected with ALS to make decisions that will assist in the management of ALS and to improve quality of life at each stage. Care-giving and caregiver support become vital as the person quickly progresses from independence to dependence.

ALS research in Canada is advancing toward treatment and a cure and research funding is increasing. For example, the ALS Society of Canada's participation in the Neuromuscular Research Partnership, working with the Muscular Dystrophy Association of Canada and the Canadian Institutes of Health Research, has funded nearly $6 million of research in the past two years.

Internationally respected, Canadian researchers are focussing on several areas including proteomics (the study of protein chemistry) to determine the cause of cell death and developing trials of potentially useful drug combinations.

These initiatives in stimulating research and provision of care will eventually result in increased longevity for those with ALS, with improved quality of life, and the hope of a cure for this devastating disease.

More information is available from the ALS Society of Canada site--www.als.ca.

Kathryn M. Yorkston, Ph.D., BC-NCD, Department of Rehabilitation Medicine, University of Washington, Seattle, WA.
David Beukelman, Ph.D., Department of Special Education and Communication Disorders, University of Nebraska, Lincoln, Munroe-Meyer Institute for Genetics and Rehabilitation, University of Nebraska, Omaha, NE.
Laura Ball, Ph.D., Munroe-Meyer Institute for Genetics and Rehabilitation, University of Nebraska, Omaha, NE.

Summary
This article describes intervention for dysarthria associated with amyotrophic lateral sclerosis (ALS). Five critical periods are presented including a stage with normal speech, detectable speech disturbance, behavioural intervention, use of augmentative communication, and loss of useful speech. Intervention strategies at each of these stages are outlined with the goal of maintaining functional communication regardless of the severity of dysarthria.

ALS is a rapidly progressive degenerative disease of unknown etiology involving the motor neurons of both the brain and spinal cord.¹ The symptoms characteristic of ALS are generally classified by site of involvement (that is, upper motor neuron versus lower motor neuron) and by whether spinal nerves (those innervating the arms and legs) or bulbar nerves (those innervating the muscles of speech and swallowing) are involved.

Nariman Malik, BSc
Contributing Author,
Geriatrics & Aging.

Lou Gehrig: A Brief History
Lou Gehrig was born June 19, 1903 in New York City. He played for the New York Yankees from 1923 to 1939 and was one of the most famous first basemen in the history of major league baseball.¹ The man known as the 'iron horse of baseball' and 'Columbia Lou' was originally recruited for only two games in 1923.² However, this durable athlete went on to play in 2,130 consecutive games.³ In fact, he never missed a game until he voluntarily benched himself on May 2, 1939.

Gehrig had an impressive career. He had a lifetime batting average of .340, hit 493 home runs and was a four-time winner of the Most Valuable Player award.³ He was also inducted into the Baseball Hall of Fame. The 1938 season had proven to be a bad one for Gehrig as he was not playing up to his usual standard. During spring training for the 1939 season, he began having trouble getting power behind the ball and had difficulty with his movements.² Unhappy with his performance, Gehrig voluntarily benched himself.

Six weeks later, Gehrig was referred to the renowned Mayo Clinic where he was diagnosed with amyotrophic lateral sclerosis (ALS). Gehrig was never told his true diagnosis and was unaware that the outcome was fatal. Only his wife and a few of her confidantes knew the true nature of Gehrig's illness.

In 1869, french neurologist Jean-Martin Charcot first described a rapidly progressive, fatal neuromuscular disease. This disease, amyotrophic lateral sclerosis, or Lou-Gehrig's disease, is a neurodegenerative disorder that affects the patient's motor neurons; typically the patient is paralyzed or deceased within 2 to 5 years of the initial diagnosis. Currently, approximately 3000 Canadians suffer from this tragic disease.

Andrew Eisen MD, FRCPC
Professor and Head, Division of
Neurology, University of British Columbia,
Head of the Neuromuscular Diseases Unit,
Vancouver General Hospital

Amyotrophic lateral sclerosis (ALS) is a prototypic neurodegeneration of the aging nervous system. It has a worldwide incidence of about 2 per 100,000 members of the population and a prevalence of 4&endash;7 per 100,000. As is true of both Parkinson's and Alzheimer's disease, the incidence of ALS is increasing proportional to the increasing longevity of the population. Information regarding the specific incidence of ALS in the elderly (aged 75 years and older) is sparse. The apparent decrease in incidence of this disease in patients older than 70 years reflects mortality from competing diseases in later life.

The etiopathogenesis of ALS is complex and multi-factorial.