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The management of motor neurone disease (MND) has evolved rapidly over the last two decades. Although still incurable, MND is not untreatable. From an attitude of nihilism, treatments and interventions that prolong survival have been developed. These treatments do not, however, arrest progression or reverse weakness. They raise difficult practical and ethical questions about quality of life, choice, and end of life decisions.
Coordinated multidisciplinary care is the cornerstone of management and evidence supporting this approach, and for symptomatic treatment, is growing.1–3 Hospital based, community rehabilitation teams and palliative care teams can work effectively together, shifting emphasis and changing roles as the needs of the individuals affected by MND evolve. In the UK, MND care centres and regional networks of multidisciplinary teams are being established. Similar networks of MND centres exist in many other European countries and in North America.
Here, we review current practice in relation to diagnosis, genetic counselling, the relief of common symptoms, multidisciplinary care, the place of gastrostomy and assisted ventilation, the use of riluzole, and end of life issues.
Motor neurone disease (MND) is a synonym for amyotrophic lateral sclerosis (ALS).
MND comprises several syndromes (classical or Charcot ALS; progressive bulbar palsy—PBP; progressive muscular atrophy—PMA).
With the exception of primary lateral sclerosis (PLS) all these share the characteristic pathology of MND. The same applies to the “flail arm” and “flail leg” syndromes (table 1).
MND is used throughout this article, except in relation to the El Escorial criteria.
DIAGNOSIS: MAKING IT AND BREAKING IT
The average delay from onset of symptoms to diagnosis is about 14 months, about one third of expected survival. Occasionally, survival following diagnosis may be less than six months. The patient may already suspect the diagnosis …