How to treat the disease
There are various ways of treating the disease. Current treatments can be effective for some common symptoms such as depression, anxiety and personality disorders. Involuntary movements do not necessarily have to be blocked by the use of medication, because patients often are not aware of chorea and because this would reduce their autonomy. Some doctors may prescribe drugs when these are absolutely necessary. In many cases, patients react better when medicines are kept to a minimum.
Recent studies have shown that neuroleptics, especially traditional neuroleptics with a high incidence of side effects, worsen the severity of the disease course and patients’ autonomy. In addition, drugs that are effective in one phase of the disease may not be effective in another. For all these reasons it is advisable to consult a neurologist who is experienced in Huntington's chorea and has a consolidated knowledge in the use of psychotropic drugs; not all neurologists, in fact, do have an adequate understanding of such a complex disease.
The family doctor plays an important role, but it is possible that - as it is a rare disease - he or she does not have much experience with Huntington's Chorea. In this case, it is essential to consult a neurologist who can offer the best treatment according to the conditions of the affected person. Patients and family members play a critical role in monitoring the efficiency of each treatment and care. It may be useful to consult a physiotherapist and a speech therapist to improve the quality of life at an early stage. Proper physiotherapy should include the training of coordination manoeuvres in an attempt to rehabilitate voluntary movements such as walking, dressing, buttoning a jacket, playing with the ball, etc...
Nutrition is important for everyone's life, but it plays a fundamental role in people affected by Huntington's Chorea. These individuals have a high caloric need in order to maintain body weight. Often the best advice and emotional support can come from those who have already been involved in this problem. The mutual help that a person can find in other relatives of patients can greatly contribute to the acceptance of the disease.
Suggested therapeutic protocols, after a visit conducted by a specialist doctor.
Presymptomatic phase
Intervene only if there are emotional disorders as for any anxiety or depressive disorder regardless of the disease. Physical and mental training are useful.
Presymptomatic phase 'close' to onset
Intervene only with supplements if there are no disturbances related to the emotional sphere. Or as above. Use low dosages of creatine (3gr) possibly in cycles.
Initial steps
Use of neuroleptic drugs only in the presence of a behavioral disorder requiring it. Examples of possible protocols: 1. Olanzapine + Valproate; 2. Olanzapine + Valproate + Pimozide at low doses; associated with benzodiazepines and SSRIs if necessary. DO NOT use drugs to act on choreic hyperkinesias at this stage. Riluzole might be of help. It is a drug prescribed for ALS, a rare disease other than HD, but not for HD. A study has shown that patients do not improve with Riluzole. Another study showed that patients may get worse if uptake occurs at the level of brain metabolism. Many studies have shown efficacy in animal and cell models. Where possible, given the good tolerability, it should be suggested at this stage if health care institutions are willing to grant it.
Advanced stages
Use Clozapine if the behavior disorder is resistant to the neuroleptics listed above. If hyperkinesias are very intense, consider using low doses of Xenazine. High dosages of xenazine may cause depression, suicide risk and worsening of dystonia and stiffness over time.
Always combine supplements and, if necessary, supplementary meals, Acqua Gel for swallowing disorders, semolina or homogenized food. All these preparations should be prescribed cost-free.
