OBJECTIVE. To review evidences on the management of patients with motor neuron disease.

DATA SOURCES. PubMed literature searches from January 1982 up to January 2011.

STUDY SELECTION. Key words for literature search were "motor neuron disease review (MND)". Only the articles which concentrated on the ventilation, nutrition, cognitive or multidisciplinary approaches for motor neuron disease were included. Case reports were not included in the review. In addition, publications were identified using the World Wide Web from references in these papers. Only articles in English were considered.

DATA EXTRACTION. A total of 782 articles were retrieved using the key word search, of which 72 concentrated on ventilation, nutrition, cognitive or multidisciplinary approaches. From these, 43 articles were eventually included and formed the basis of this review.

DATA SYNTHESIS. Motor neuron disease is an adult-onset neurodegenerative disease that leads to weakness of limb, bulbar, and respiratory muscles. It displays an ethnic variation in incidence; 90% of cases are sporadic and 10% are familial. New diagnostic criteria have been proposed to increase diagnostic sensitivity. Proper clinical studies, electrophysiology, and neuroimaging are necessary before reaching a diagnosis of motor neuron disease. Riluzole remains the only disease-modifying drug approved for this disease; it prolongs life by 3 to 4 months. Multidisciplinary care units are important in the management of motor neuron disease patients. Non-invasive positive pressure ventilation prolongs life in motor neuron disease patients with respiratory failure. Enteral feeding is usually recommended for affected patients with malnutrition. Cognitive impairment is common in these patients, for whom a formal neuropsychiatric assessment is recommended. Appropriate palliative care is needed for these patients in order to improve their quality of dying.

CONCLUSION. Motor neuron disease is an incurable disease, for which a highly effective treatment is still pending. Symptomatic treatment remains the mainstay of management. A multidisciplinary approach embracing advances in non-invasive ventilation and gastrostomy can improve quality of life and extend the survival of motor neuron disease patients.

Key words: Amyotrophic lateral sclerosis; Motor neuron disease; Muscle weakness; Muscular atrophy; Riluzole

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