Huntington disease: the disregarded recommendations on the use of neuroleptic drugs
by Ferdinando Squitieri
Neuroleptics are those drugs that act within the nervous system on the main chemical mediators, such as dopamine, those substances that enable nervous cell connections and exchange of information: in other words, those substances that promote the dialogue between different areas of our brain (connectivity) and allow the brain itself to send orders to the muscles (movement) and other organs.
Unlike minor tranquilizers, which are prescribed to control some dysfunctions such as anxiety, sleep and mood disorders (such as the so-called benzodiazepines or antidepressants), neuroleptic drugs have a more radical, incisive, prolonged and inhibitory effect and are used to control important psychiatric disorders or, in some cases, involuntary movements, as it happens in Huntington disease.
The scientific community has repeatedly recommended to be careful in prescribing these drugs in Huntington disease.
In recent years, there have been several recommendations which, unfortunately, have not been endorsed by the medical community as expected, in particular by neurologists and psychiatrists.
I would like to mention three main publications:
1 - the first describes the negative side effects of neuroleptics on movement, autonomy and cognitive process (Tedroff et al., Journal of Huntington Disease of 2015);
2 - the second is a guideline that calls for caution and suggests protocols to be followed (Bachoud-Levi et al., Parkinsonisms and Related Disorders 2019);
3 - the third, just published in the British Medical Journal group (Harris et al., Journal of Neurology, Neurosurgery and Psychiatry 2020), underlines that, even though there may be positive effects on involuntary movements, neuroleptics invariably and unequivocally led to a significant deterioration in the progression of the so-called cognitive symptoms (memory, orientation, executive functions) during the disease’s course. Cognitive alterations lead to dementia and are the main causes of reduced independence in Huntington disease patients’ lives.
On the side lines of what has been reported in the article and by the large number of publications over the years, I would like to add a personal clue: neuroleptic drugs are different from each other and, above all, the dosages prescribed and the association with other drugs, can make a huge clinical difference. Therefore, it is critical to pay attention to the neuroleptics’ use, as well as the other available drugs, with foresight taking into account non only the patient’s clinical condition we observe today, but also what we expect the disease progression will bring tomorrow. Symptoms vary from patient to patient and change over time in the same patient: today's choreic hyperkinesias will turn into dystonic movements, which will turn again into body slowness (which we name bradykinesia) and rigidity.
Therefore, to rage against involuntary movements (of which patients are almost of the time unaware or partially aware), neglecting their clarity of thought and orientation that allows people to correctly relate to each other is, in my opinion, a nearsighted perspective.
Moreover, prescribing drugs whose the specific indication is the treatment of involuntary movements (chorea) and not of the whole spectrum of Huntington disease’s symptoms, such as tetrabenazine, in patients who have scarcity of choreic hyperkinesias “slows down” their body (as many patients have said "frozen" their body) which is technically and professionally incongruous.
The suggestion is to not worsen the already critical conditions of patients by exacerbating the 'natural' progression of the disease, with symptoms induced by inappropriate drug therapies. We all shall follow the international scientific community’s recommendations, which always aim to protect patient’s health and well-being, regardless of any commercial interests.
Attached, the three scientific publications mentioned above.