Death of Rob Burrow: what is motor neurone disease, which led to the death of the famous rugby player at the age of 41 ?

Maladie du motoneurone : décès du rugbyman Rob Burrow

Rob Burrow, dont le décès a été annoncé dimanche par son ancien club de rugby, les Rhinos de Leeds, s’était vu diagnostiqué une maladie du motoneurone en 2019.

Charcot disease is the most common of these rare diseases with dramatic consequences. In the case of Rob Burrow, did intensive rugby play a role in the onset of the disease ?

We learned on Sunday June 2 of the death at the age of 41 of Rob Burrow, former British rugby union star. It was the Leeds Rhinos club with whom he had won numerous trophies which announced the sad news. Doctors diagnosed him with motor neuron disease in 2019.

What are motor neuron diseases ?

These are rare neurodegenerative diseases – 2,300 new cases per year in France – characterized by a progressive loss of motor neurons. They lead to motor problems and progressive paralysis which vary depending on the extent of the damage and the motor neuron affected. The most common of these diseases, 90% of cases, is amyotrophic lateral sclerosis (ALS), also called Charcot disease.

D’rapid evolution – ALS patients die on average within 30 months of the onset of symptoms – it is characterized by:

A decrease in muscular strength which leads to paralysis of the limbs, then the trunk and the respiratory muscles; Amyotrophy, or muscular atrophy; Spasticity: involuntary muscle stiffness.

Other motor neuron diseases include:

primary lateral sclerosis: degeneration of the motor neurons of the cerebral cortex without damage to the motor neurons of the spinal cord. This absence of damage to the motor neurons of the spinal cord differentiates it from ALS; Progressive muscular atrophy is characterized by isolated damage to the lower motor neuron, including progressive flaccid weakness, muscle atrophy, fasciculations (brief muscle contractions) and reduced or even absent tendon reflexes. Progressive bulbar palsy affects the neuronal network of the lower brainstem and manifests itself by paralysis of the muscles of the tongue, pharynx, larynx and atrophy of the tongue. Kennedy disease or X-linked bulbospinal muscular atrophy affects men in adulthood. It combines progressive muscle weakness and hormonal disorders. It is due to a genetic anomaly located in the gene coding for the androgen receptor, explains AFM Téléthon.

No curative medicine

There are currently no curative drugs for motor neuron diseases. Treatment is symptomatic and multidisciplinary (physiotherapy, psychological support, non-invasive respiratory ventilation…). Riluzole is the only drug available that can save ALS patients a few months of life.

Risk factors are genetic, environmental – even if they remain poorly known and difficult to identify. Occupational exposure to lead could play a role as well as exposure to pesticides in agriculture.

Is rugby to blame ?

Can the intensive practice of rugby play a role in the appearance of motor neurone disease ? In question, the French study rugby.com.

In 2022, researchers from the University of Glasgow (Scotland) published the results of the study in the British medical journal ;a study on the brains of rugby players. They examined the medical records, over 30 years, of 412 former international players aged 30 and over, which they compared to those of 1,236 individuals from the general population.

Results: rugby players were twice as likely to suffer from dementia. Even more serious, they were 15 times more likely to suffer from motor neurone disease than the general population. Scientists had called for "radical changes" in rugby to reduce the risk of head impacts and traumatic brain injuries, such as stopping any contact training during the competitive season or limit the number of matches in which players must participate.