Huntington, Parkinson, Alzheimer, SLA: principali analogie e differenze

Huntington, Parkinson, Alzheimer, SLA: principali analogie e differenze

 

Neurodegenerative diseases have in common the selective death of cells of the nervous system responsible for the control of movements and cognition. The main neurodegenerative diseases are Huntington, Parkinson, Alzheimer and Amyotrophic Lateral Sclerosis (ALS).

The cause of these diseases is still unknown, with the sole exception of Huntington's, whose genetic cause was discovered in 1993. Huntington disease is by definition a disease in which the presence of a single mutation is a necessary cause and sufficient to determine its occurrence. The other neurodegenerative diseases, on the other hand, while recognizing some rare genetic and hereditary forms, have a multifactorial origin as a cause, i.e. attributable to the interaction of genetic factors and environmental factors that have not yet been fully identified.

Huntington and Parkinson fall into the category of so-called movement disorders, in which the fine regulation of motor control is impaired.

Huntington disease owes its name to the American neurologist George Huntington (1850-1916) and represents the prototype of the spectrum of hyperkinetic motor disorders, i.e. from excess movement. However, although they are the most evident, motor disorders play only a modest part in the disease, being able to be accompanied by pictures of cognitive and neuro-psychiatric dysfunction of considerable impact on aspects of autonomy and quality of life of the affected persons and family members.

Parkinson disease owes its name to the English neurologist James Parkinson (1755-1824) and instead falls within the "opposite" spectrum of motor disorders, i.e. those in which we find a significant slowing of movements accompanying the classic Parkinsonian tremor. Also in this pathology, non-motor disorders such as reduced sense of smell, sleep disorders, mood disorders and constipation are added to the clinical picture.

Alzheimer owes its name to the German neurologist and neuropathologist Alois Alzheimer (1864-1915): it is the main form of neuro-degenerative dementia and is characterized by progressive cognitive disorders such as memory impairment, difficulty in finding words, episodes of disorientation both in time and space, difficulty in organizing thoughts and actions.

Amyotrophic Lateral Sclerosis (ALS) described for the first time in a coherent and organic way by the French neurologist Jean-Martin Charcot (1825-1893) is a disease that manifests itself with a progressive paralysis up to the complete involvement of all skeletal muscles (excluding ocular ones) and secondary muscle loss, up to the loss of motor neurons that direct the motor impulse from the brain to the muscle.

Some of these diseases fall under the category of rare diseases but nevertheless, without going too far back, various examples of famous people suffering from similar disorders can be named: Woody Guthrie from Huntington, Michael J. Fox from Parkinson, Ronald Reagan from Alzheimer's and Stephen Hawking from Amyotrophic Lateral Sclerosis (ALS).

To date, there is no curative therapy for any of these diseases.

The role of research is, therefore, crucial and can only be successful with the collaboration of all the actors involved, from patients, to researchers, to pharmaceutical industries, to governments and regulatory authorities.

Funding research is equally important.