Frequently asked questions

How to know whether it is an inherited disorder or not ? Will other family members develop this disorder as well ?
Even if most of the primary dystonias are considered as inherited, only a few mutations have been identified as responsible for the inherited forms of dystonia. When the clinical expression of the disease is compatible with one of the genetical forms of dystonia, blood samples are taken after written informed consent of the patient or his parents (for children) and molecular analysis will be performed to check for the already identified mutations. The molecular analysis will check for the presence of these mutations. After confirmation of the genetical disease, if the relatives of the patient want to get informed about the risks to develop the same disease, they will be directed to the Genetic Council.
Which is the cost of the treatment and who pays?
The cost of the treatment is about 30 000 Euros and includes only (exclusively) the price of the devices (electrodes and neurostimulators). The price for hospitalization has to be added. Every 2 to 4 years the price for a new stimulator has to be considered when the stimulator reaches its end of life. The costs of the devices as well as the hospitalization are free of charge for patients insured by the French Social Security, but the number of patients that can be operated per year is limited. The University Hospital of Montpellier assures an extra-number of surgeries which allow to treat sometimes patients with a very severe clinical condition. For foreigners, the costs of treatment and hospitalization have to be insured by the patient or the Social Department (Security) of their country.
Which is the postoperative prognosis of chronical electrical stimulation for the different types of dystonia?
The results obtained by chronic electrical stimulation of the Gpi are best for primary dystonia (78% of improvement of the post-operative clinical scores compared to the pre-operative scores after two years of stimulation). As already mentioned, the functional rehabilitation in primary dystonia is nearly complete and allows the return to a normal daily living. In secondary dystonias, because of the diversity of etiologies, each case has to be analyzed independently. We obtained nevertheless a satisfactory control of painful muscle spasms as well as of the swallowing troubles with improvement of the quality of life.
How long is the hospital stay and the delay for obtaining a clinical response or an improvement by stimulation ?
The hospital stay is about 3 or 4 weeks and depends on the clinical status and its improvement under stimulation after surgery. The clinical response to DBS varies in patients even when presenting the same type of dystonia. There are patients already considerably improved at the and of the hospital stay. For others, improvement takes more time. It can even be completed during upto 3 years following surgery. The efficacy of stimulation will first be evaluated after six weeks.
Are implanted devices compatible with normal daily living ? (Mobile phone, sport,...)
The implanted devices will not modify the daily living of the patients. Quickly after surgery, the implanted devices will be neglected by the patient who will focuse on the clinical changes due to the stimulation. The presence of the implanted devices is compatible with the use of mobil phones, sport, shopping,...
How do patients perceive the clinical changes and how do they tolerate them?
At the beginning, patients are surprised and disturbed by the clinical changes occurred under stimulation. They focus extremely on the detection of the most discrete change of their symptoms. Finally, after a delay of adaptation, the clinical improvement of their clinical status is lived in a very positive way.
What is the lifespan of the stimulators? How to change them?
The lifespan of the stimulators is estimated between 2 and 4 years. This length is largely dependent on the stimulation level of each patient. When the stimulators reach the end of life, the patient will be rehospitalized. Surgery will be performed as soon as possible (few days depending on the clinical deterioration and the surgery planning) to remove the used stimulators and to reimplant the new ones. A few hours thereafter, stimulation will be turned on again.
How to know that stimulators reached their end of life?
When stimulators are exhausted, dystonic symptomes will gradually reappear.
Is pregnancy possible for a patient who underwent surgery for deep brain stimulation?
The devices implanted for deep brain stimulation are compatible with a pregnancy. The decision to assume a pregnancy is more influenced by the clinical and genetical context than by the presence of the implanted devices.
Has the treatment to be followed during life time?
The first patient underwent surgery 7 years ago. This is our longest follow-up for this treatment which is supposed to be a life time treatment.