What is Huntington's disease? - CBD for a better quality of life

Huntington's disease (also known as Huntington's chorea) is a rare, neurodegenerative disease of the central nervous system that is steadily progressive. It is hereditary and causes a gradual destruction of cells in certain brain areas.

The result is the gradual deterioration of physical, mental, and psychological functions. The disease usually breaks out between the ages of 35 and 45, but in rare cases, it can also occur in early childhood or old age. The course of the disease varies from patient to patient.

Huntington's disease is named after the American physician George Huntington, who described it in 1872 and was the first to recognize that it was a hereditary disease. The disease is also called Huntington's chorea or Morbus Huntington and was formerly known as St. Vitus' Dance. The name Chorea (Greek: Chorea = dance) comes from the typical movements of the disease, which can vaguely resemble a dance.

Since in medicine "chorea" refers to sudden, varied, and involuntary movements of various muscles, and since chorea movements are only part of the disease, the term Huntington's disease is now used more frequently than Huntington's chorea.

Huntington's disease manifests primarily through movement disorders, emotional disturbances, and cognitive impairments. The most noticeable symptoms are sudden, uncontrollable movements of individual muscles, entire limbs, or the trunk.

However, at the beginning of the disease, psychological abnormalities are usually in the foreground: for example, patients are depressed or increasingly irritable and aggressive to disinhibited. Some notice a loss of mental abilities and thus develop increasing anxiety.

Finally, the deterioration of motor functions follows in conjunction with the increase in uncontrolled movements. These movement disorders can occur at rest or impair other movements in action.

Initially, it is often possible to incorporate the exaggerated and unwanted movements into meaningful sequences of movements, which, for example, creates an exaggerated gesture for the observer. The tongue and pharyngeal muscles can also be affected. As a result, speech in many cases appears incomprehensible and choppy, and sounds are emitted explosively.

Huntington's disease is a genetically determined disease that is inherited. Children whose one parent carries the affected gene have a 50% chance of inheriting this gene and developing the disease. Therefore, this risk should be carefully weighed regarding family planning.

If parents or siblings are affected, a suspected diagnosis can be made based on the typical symptoms and course. Through the combination of family medical history and neurological examinations, the disease can be diagnosed at a very early stage.

However, further neurological and psychiatric examinations are still necessary to rule out other diseases that cause similar symptoms. These examinations should ideally be carried out by an experienced doctor at one of the Huntington centers.

The disease can finally be confirmed by a molecular genetic examination that detects the altered gene. This gene test is not a diagnosis of the time of onset of the disease. At-risk individuals can be tested from the age of 18. If the gene alteration is present, there is no way to prevent the onset of the disease. It runs relentlessly and fatally.

So far, Huntington's disease cannot be causally treated, meaning there is no possibility of a cure with current knowledge. Therefore, the main goal of therapy is to treat the emerging symptoms. However, even this is only possible to a limited extent and primarily through medication, physical and occupational therapy, and speech therapy. For excessive uncontrolled movements, neuroleptics are prescribed, and for depressive moods, antidepressants.

Since the symptoms can vary greatly, the treating physician must adapt the therapy individually for each patient. These various treatment methods aim to maintain the independence of those affected for as long as possible and to improve their quality of life.

Around the world, researchers are looking for medications and procedures that intervene in the disease process. Although research has made enormous progress in the basics over the last 20 years, researchers have not yet achieved a breakthrough despite special medications and therapy techniques. Therefore, it is understandable that affected individuals are pleased with even the smallest reports of success. However, these successes are often only noted in animal experiments.

Affected individuals can seek advice and help from national and international Huntington self-help organizations, as well as from regional Huntington self-help groups, which exist in many places. These endeavor to inform those affected and to organize mutual assistance for affected individuals, relatives, and at-risk individuals.

Since few doctors have experience with this rare disease, there are special Huntington centers and outpatient clinics where one can consult knowledgeable specialists. These specialists are also listed on the websites of the self-help organizations.

In the last decade, scientists have begun to research the cannabis plant to find new treatment options for diseases such as Huntington's disease.

Cannabis has great potential as a therapeutic agent due to its ability to regulate the nervous system. CBD is particularly interesting because it not only stimulates the endocannabinoid system in one direction but also ensures that the system as a whole can work more efficiently. This is extremely important because mice with Huntington's disease have been shown to have lower CB1 activity and higher CB2 activity.

However, research is still in its infancy, and there are only a handful of clinical studies that have investigated the relationship between CBD and Huntington's disease. Therefore, much more research and studies are needed to understand the extent to which CBD can help people diagnosed with Huntington's disease.

Nevertheless, CBD's ability to regulate both neurological and immune functions makes it an ideal candidate for future treatment options for Huntington's disease. There is growing evidence that CBD has the ability to slow the progression of the disease and improve the quality of life of those affected.¹

We eagerly await developments in the coming years when high-quality clinical studies on humans are available on this topic.