Huntington's Disease: Signs And Treatment
Huntington's disease, a genetic disorder, causes gradual breakdown of nerve cells, affecting one's physical as well as mental capabilities. Over time, one loses the ability to move or perform physical tasks, like walking, eating, or speaking, and to think or reason clearly. Depression, anxiety, and suicidal tendencies take over. There's no cure, but Ayurveda identifies the symptoms with vata dosha imbalance and can offer some relief.
Huntington’s disease (HD) is a genetic disorder that causes gradual breakdown of nerve cells, especially in special cells called the basal ganglia situated in the brain.1
This disease affects the physical as well as the mental capabilities of an individual over time. In people with this condition, the huntingtin protein depletes the most basic energy-giving molecule utilized by every cell in our body – adenosine triphosphate (ATP). This depletion of energy molecules produces the symptoms of Huntington’s disease.
Who Is Prone To Huntington’s?
Male or female, you have a 50% risk of developing Huntington’s if even one of your parents has it. But if you don’t inherit it, your child won’t.
Huntington’s disease affects both sexes and people of all races equally. It is an autosomal dominant disorder, which means that if even one parent carries the gene responsible for the condition, the children are likely to inherit it. Every child of a parent suffering from Huntington’s has a 50% chance of carrying the faulty gene. If the child does not inherit the disease gene, he or she will neither develop this condition nor pass it on to the next generation.2
Signs To Look Out For
Huntington’s disease is a neurodegenerative condition and a person with this disorder will face difficulties in motor functions, cognitive deterioration, and psychiatric symptoms.3
This includes both faulty voluntary actions and involuntary movements like jerking movements or trembling (chorea). Muscular problems such as rigidity, muscle contractures (dystonia), difficulty in swallowing, difficulty in communicating via speech, and trouble with gait and balancing are also common.
You might have difficulty swallowing food or speaking coherently.
You may obsessively cling to a thought, behavior, or action.
These include difficulty in organizing, planning, and focusing on tasks, tendency to get stuck on a thought, a behavior, or an action, action without thinking (impulsiveness), difficulty in learning new things, and lack of awareness of one’s behavior and abilities.4
You can have alternating episodes of depression and mania and behave impulsively.
Depression is the most common psychiatric symptom of Huntington’s disease as a result of the damage dealt out to the brain. Depression manifests as irritability, sadness or apathy, insomnia, fatigue, and thoughts of death or suicide. Other psychiatric signs include obsessive-compulsive behavior, mania, overactivity, impulsive behavior, inflated self-esteem, and alternating episodes of depression and mania.5
Stages Of Huntington’s
Because a cure hasn’t yet been found for the disease, it is important to identify the disease in its early stages so that the patient can plan ahead and manage day-to-day living.67
Symptoms include nervous twitches in the face or fingers.
The patient may get involuntary twitches in the fingers and toes and even the face and have difficulty performing tasks that require a complicated set of motions. It’s also tough for the patient to think through a complicated task, plan, and organize. Depression and anxiety are common, and there might even be a tendency toward suicide. Speech slurring and difficulty swallowing food may also be seen.
The patient might lose weight because of his or her eating difficulties.
Chorea becomes more visible in this stage, and the patient has trouble maintaining balance, which makes him or her walk in a staggered manner and often suffer falls. Swallowing becomes yet more difficult, leading to weight loss. It also becomes increasingly difficult to think clearly and perform daily activities. There is also a peak in suicidal tendencies.
Advanced Or Late Stage
The patient’s movement and speech are both very slow.
The chorea subsides but it is difficult to perform voluntary actions because of problems of rigid muscles, prolonged muscle contractions, which cause twisting movements, and slowness of movement. Cognitive abilities decline increasingly, and response may be slower than before, but the patient can still understand speech and recognize loved ones. Depression is replaced by apathy, and the patient is less aggressive or suicidal in this stage. Other physical problems outweigh the emotional or cognitive difficulties. The patient might need assistance for everything in this stage.
Which Age Group Does Huntington’s Affect?
Generally, the signs and symptoms of Huntington’s disease start appearing at the age of 30–40 years, but the early 20s are a possibility too.
Treatment For Huntington’s Disease
Treatment has to be multi-pronged to manage the many aspects of the disease, like speech, motor functions, or cognitive abilities.
No treatment can cure the disease as the roots lie in the genes. What a doctor can do is take care of the physical symptoms and the mental challenges. Medicines too can be prescribed to take care of symptoms like chorea and the psychiatric illnesses.
It can help the person with Huntington’s manage behavioral problems and facilitate effective communication among family members.
Huntington’s disease impairs the control over the muscles of the mouth and throat. A speech therapist can help the patient speak clearly in later stages of the disease. The therapist will use communication tools like alphabet boards, picture boards, and other electronic devices to coach the patient. Speech therapists can also address challenges with muscles used in eating or swallowing.
A physical therapist can teach safe exercises that improve strength, flexibility, balance, and coordination. These exercises can help the person retain mobility for as long as possible and reduce the risk of falls greatly. In the later stages, when a walker or wheelchair is required, the physical therapist can guide the patient on appropriate use and posture.8
An occupational therapist can help with assistive devices that improve functional abilities – say, using handrails at home, or special utensils for eating and drinking.
Creatine slows down the progression of Huntington’s disease and vitamin E helps in the early stages.
Studies have found that a drug called creatine, which helps replace the lost ATP in the cells, can improve the condition of people with Huntington’s disease. In fact, it even slows down the progression of this debilitating disease.9
Vitamin E has shown promise as a potential dietary supplement to help slow down the progress of the condition in early stages. However, it hasn’t been found to be effective in more advanced stages.10
The Alternative Effect
The Ayurveda Route
Ayurveda suggests that the symptoms are caused chiefly by an imbalance of the vata dosha and are similar to those of tandava roga.
According to Ayurvedic expert Janardhana Hebbar, the symptoms of Huntington’s arise from a vata dosha imbalance, with secondary involvement of pitta dosha. The symptoms are similar to those of a neuromuscular condition called tandava roga. He prescribes Ayurvedic treatments like panchakarma therapy to bring the doshas in balance.
Turmeric and ginger have been found to help control the symptoms.
Alternative therapy such as Ayurveda can help alleviate symptoms and bring relief. A study found that herbs like brahmi, ginkgo biloba, ashwagandha, turmeric, gotu kola, and ginger have been found to have potential benefits in controlling symptoms of Huntington’s disease and slowing down its progression through their anti-inflammatory and anti-oxidant properties.11
Procedures like nasya and shirostalam are part of treatment.
Another case study showed how an Ayurvedic regimen including medication and procedures like nasya, shirostalam, abhyanga, and basti improved the symptoms of chorea in a 47-year-old woman with Huntington’s.12 Nasya is the instillation of medicated oils through the nasal passage. For shirostalam, a medicinal paste is applied on the head and kept tied with a leaf for 8 hours. Abhyanga is massage with aromatic herbal oils to induce healing at a cellular level. Basti or enema therapy is one of the panchakarma or five-pronged detoxification therapies.
One study also confirmed improvements in chorea through the use of the homeopathic medicine Belladonna.13 Thus, a multidisciplinary approach involving various specialties and branches of medicine can be most beneficial for patients.14
References [ + ]
|1.||↑||Schroll, Henning, and Fred H. Hamker. “Basal Ganglia dysfunctions in movement disorders: What can be learned from computational simulations.” Movement Disorders 31, no. 11 (2016): 1591-1601.|
|2.||↑||Sutovsky, Stanislav, Tomas Smolek, Irina Alafuzoff, Andrej Blaho, Vojtech Parrak, Peter Turcani, Michal Palkovic, Robert Petrovic, Michal Novak, and Norbert Zilka. “Atypical Huntington’s disease with the clinical presentation of behavioural variant of frontotemporal dementia.” Journal of Neural Transmission: 1-11.|
|3.||↑||Rodrigues FB, Wild EJ. Psychogenic non-epileptic seizures in early Huntington’s disease. Pract Neurol. 2016 Jun 21. pii: practneurol-2016-001423.|
|4.||↑||Mörkl, Sabrina, Nicole J. Müller, Claudia Blesl, Leonora Wilkinson, Adelina Tmava, Walter Wurm, Anna K. Holl, and Annamaria Painold. “Problem solving, impulse control and planning in patients with early-and late-stage Huntington’s disease.” European Archives of Psychiatry and Clinical Neuroscience 266, no. 7 (2016): 663-671.|
|5.||↑||Abbott SM, Videnovic A. Chronic sleep disturbance and neural injury: links to neurodegenerative disease. Nat Sci Sleep. 2016 Jan 25;8:55-61. eCollection 2016. Review.|
|6.||↑||Stages of Huntington’s Disease. Huntington’s Outreach Project for Education, at Stanford|
|7.||↑||Santini, Veronica E., MD, and Sharon Sha, MD. Stages of Huntington’s Disease and Treatment|
|8.||↑||Rollnik, J. D. “[Rehabilitation in Huntington’s Disease].” Fortschritte der Neurologie-Psychiatrie 83, no. 6 (2015): 334-343.|
|9.||↑||Hersch, S. M., S. Gevorkian, K. Marder, C. Moskowitz, A. Feigin, M. Cox, P. Como et al. “Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2′ dG.” Neurology 66, no. 2 (2006): 250-252.|
|10.||↑||McHugh, Paul R., and Susan E. Foistein. “Trial of du-Tocopherol in Huntington’s Disease.” Am J Psychiatry 1, no. 52 (1995): 1771.|
|11.||↑||Choudhary, Sunayna, Puneet Kumar, and Jai Malik. “Plants and phytochemicals for Huntington’s disease.” Pharmacognosy reviews 7, no. 14 (2013): 81.|
|12.||↑||Girish, K. J., Amit Kumar, Devendra Kaushik, and Diwakar P. Joshi. “Management of Huntington’s Disease through Ayurveda–A Case Study.” Journal of Ayurveda and Holistic Medicine (JAHM) 3, no. 3 (2015): 53-57.|
|13.||↑||Tomlinson, Paul J. “The treatment of Huntington’s chorea with belladonna alkaloids.” Psychiatric Quarterly 21, no. 3 (1947): 447-452.|
|14.||↑||Veenhuizen, Ruth B., Branda Kootstra, Wilma Vink, Janneke Posthumus, Pleuntje van Bekkum, Margriet Zijlstra, and Jelleke Dokter. “Coordinated multidisciplinary care for ambulatory Huntington’s disease patients. Evaluation of 18 months of implementation.” Orphanet journal of rare diseases 6, no. 1 (2011): 1.|