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Motor neuron disease

The motor neurone diseases (MND) are a group of progressive neurological disorders that destroy motor neurons, the cells that control voluntary muscle activity such as speaking, walking, breathing, and swallowing. Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease, progressive muscular atrophy (PMA), spinal muscular atrophy (SMA), progressive bulbar palsy (PBP) and primary lateral sclerosis (PLS) are all motor neurone diseases. more...

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MND is the term often used internationally while ALS is often used in the United States (where it is also known as Lou Gehrig's disease, after the legendary baseball player) to cover all forms of MND. It was first described by Jean-Martin Charcot, a French neurologist, in 1869 and in France the disease is therefore known as Maladie de Charcot (Charcot's disease).

Signs and symptoms

Characteristic symptoms of MND include gradual weakening, wasting away, and uncontrollable twitching of the muscles; spasticity or stiffness in the arms and legs; and overactive tendon reflexes. Sensation, intellect, memory, and personality are not affected in MND. In some types of MND, such as ALS, muscle weakness is progressive and eventually leads to death when the muscles that control breathing no longer work. Other types of MND progress slowly and last over a lifetime.

In adults, symptoms usually appear after age forty, and may be similar to those of other diseases, making diagnosis difficult. In children, particularly in inherited forms of the disease, symptoms are present from birth.


The diagnosis of ALS is established based on the history of the patient and the findings on neurological examination. There is no diagnostic test for ALS. Electromyography (EMG) examination are useful to demonstrate the diffuse loss of motor neurones innervating muscles of extremities, face and abdomen and to rule out other disorders that may mimic ALS, but interpretation of the result is not necessarily straight forward. A set of diagnostic criteria called the El Escorial criteria have been defined by the World Federation of Neurologists and are widely used by neurologists and ALS researchers.

Clinically, upper motor neuron damage signs (such as spasticity, brisk reflexes and Babinski signs) can be found, while the lower motor neurones demonstrate weakness and muscle atrophy. Weakness of bulbar musculature can also be seen (difficulty breathing, swallowing, coughing, or speaking).

Neuroimaging examinations are usually performed to rule out alternative causes, such as a mass lesion of upper parts of spinal cord



Nonhereditary (also called sporadic) MND are caused by unknown factors. Nonhereditary MND include ALS, progressive bulbar palsy, pseudobulbar palsy, primary lateral sclerosis, progressive muscular atrophy, and post-polio syndrome. There are no specific tests to diagnose the MND.

About 90% of cases of MND are "sporadic", meaning that the patient has no family history of ALS and the case appears to have occurred sporadically in the community. The cause of sporadic ALS is unknown, though genetic factors are suspected to be important in determining an individual's susceptibility to disease. There is weak evidence to the suggestion that the onset can be triggered by a viral infection, but this is not widely believed in the ALS research community. The remaining 10% of cases are "familial", defined as more than one case of ALS in a family. Familial ALS is genetic in aetiology and the following genes are known to be linked to ALS: Cu/Zn superoxide dismutase SOD1, ALS2, NEFH(a small number of cases), senataxin (SETX) and vesicle associated protein B (VAPB).


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"Live to eat, eat to live": Nutritional support in motor neuron disease
From Canadian Journal of Dietetic Practice and Research, 10/1/00

Motor neuron disease (MND) is a motor system disease characterized by progressive, asymmetrical muscle weakness and wasting, with eventual paralysis. Atrophy may begin anywhere in the skeletal musculature and typically progresses to involve all of the striated muscles except extraocular and cardiac muscles. Three main clinical variants are usually identified: progressive muscular atrophy, progressive bulbar palsy (PBP) and amyotrophic lateral sclerosis (ALS).

Swallowing problems are associated with PBP and ALS. There is progressive loss of oral manipulation of food, associated with increasing weakness and atrophy of the tongue, masseter muscles and soft palate. All phases of swallowing become progressively compromised. Lingual sweeping may be ineffective and food is retained in the mouth after swallowing. Lip seal may also be ineffective during eating, causing oral loss of food. Nasal regurgitation may occur, in association with incompetent velopharyngeal closure. Many patients experience difficulty clearing secretions from the mouth; drooling is a common problem. Swallowing may be further compromised by the presence of thick mucous or a dry mouth. Esophageal abnormalities also occur with weak or absent peristaltic waves, simultaneous peristaltic waves and diffuse esophageal spasm. Cricopharyngeal spasm results in pooling in the pyriform sinuses.

Oral feeding becomes increasingly slow and difficult and may eventually become a high-risk activity due to inability to meet nutritional requirements and the risk of aspiration.

In recent years, percutaneous endoscopic gastrostomy (PEG) has become the intervention of choice when oral food intake becomes unsafe or inadequate. PEGs are not, however, the choice of all patients. A small but important group of patients choose to continue oral feeding.

We have observed that those patients who refuse PEG feeding are likely to be older (60+ years of age), live at home, have good family support, and/or have co-existing conditions (e.g., cancer, cardiac disease, mental illness). Reasons given for refusing PEG feeding include an unwillingness to give up the pleasure associated with eating (a "live to eat" personality), denial of swallowing problems, a desire to avoid invasive procedures, acceptance of impending death, and fear of the procedure (despite information and reassurance provided).

Management Strategies

Patients with MND require continuous management plans designed to minimize risk of aspiration, malnutrition and dehydration, while supporting continued enjoyment of food. An interdisciplinary approach with input from Nutrition, Speech Pathology and Occupational Therapy is most beneficial.

Texture Modification

Identification and avoidance of problem foods

Modification of food texture to facilitate oral management of food - soft/moist, cut-up, diced, minced, pureed

Modification of liquid consistency to minimize aspiration risk

Teach Heimlich manoeuver to caregivers) and self-Heimlich to patient

Nutritional Strategies

Use of nutritional supplements to ensure adequate energy and nutrient intake

Use of alternative fluid sources to ensure adequate hydration JELL-OS applesauce, ice cream

Snacking as an important contribution to nutritional intake

Therapy Techniques

Head position for airway protection

Chin tuck manoeuver to maximize airway protection and minimize aspiration risk

Double swallow to clear residue in the valleculae or pyriform sinuses Supraglottic swallow to facilitate

full laryngeal excursion and airway protection Thermal stimulation of oral cavity prior to eating

Behaviour Strategies

Thorough oral hygiene after meals and snacks to prevent aspiration of food residue

Smaller, more frequent meals to minimize fatigue of eating

Maintenance of consistent food temperature - hot or cold foods are easier to chew and swallow than tepid food

Introduction or elimination of highly seasoned foods

Leave last half inch of liquid in a cup to avoid chin tilt Reduce bite size

Reduce distractions while eating Adapted Tools

Adapted utensils for eating scoop plate, non-slip mat, raised plate, handle extensions, built-up handles, mobile arm support

Adapted drinking tools - "nosey" cup, lidded cup Summary Nutritional support in ALS/MND may involve use of a PEG, or patients may choose to continue oral feeding. Health care professionals are responsible for providing persons with ALS/MND information on available strategies for nutritional support; helping patients to understand treatment choices and their consequences; supporting a person Is decision, and developing an appropriate management plan responsive to changing needs. At the core of any management plan is the concept that food should remain a pleasure as well as a source of nutrition.

This information was originally presented as a poster at the 10`" International Symposium on ALS/MND, Vancouver (11/99).

Contact Information:

Elaine Cawadias RD Susan Carroll-Thomas, SLP(C) ALS Clinic, The Rehabilitation Centre Ottawa, ON 613-737-7350 (5528)

Copyright Dietitians of Canada Fall 2000
Provided by ProQuest Information and Learning Company. All rights Reserved

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