What is generalized dystonia?


Dystonia is a neurological condition characterized by intense, involuntary muscle contractions, provoking abnormal postures of the whole or part of the body. When these contractions are short and more rhythmical, the patient may appear to be trembling.

In many cases, dystonia is of genetic origin and may affect a person at any age.
Generalized dystonia is a form of dystonia involving practically the whole body. The onset of involuntary muscle contractions is not limited to a single muscle group or half the body, but may affect all four limbs, as well as the face, neck and spine.

However, certain areas may be spared. Indeed, dystonia is said to be generalized when it affects both sides of the body and at least one lower limb and its axis. Generalized dystonia may be focal (localized) initially or even limited to one limb, before progressing to previously unharmed areas.

Symptoms


The symptoms are very varied.

Here are some examples of focal dystonia :

  • Blepharospasm

The eyelids close repeatedly and uncontrollably, from a few seconds to several minutes.

  • Spasmodic torticollis or cervical dystonia

The spasms force the neck to turn to one side or be pulled forward or backward and it remains blocked.

  • Musicians’, writers’ or athletes’ cramp

The muscles of the hand, wrist or cheeks contract at the time of writing or playing.

  • Oromandibular dystonia

The jaw muscles make involuntary movements (teeth clenched or mouth open).

Our therapeutic offering


As for Parkinson’s disease, surgical treatment exists. Deep brain stimulation (DBS) was developed back at the beginning of the 90s by the French neurosurgeon and physician, Alim-Louis Benabid and has already made it possible to change the lives of 150,000 patients.

DBS is used in certain cases of chronic primary dystonia when medication is ineffective. This means implanting in the brain small electrodes which, by electrical stimulation, block the abnormal activity of the brain structures involved in dystonia. The electrode is linked to a neurostimulator generally implanted under the skin, near the collarbone or on the abdomen. This treatment is reversible because the electrical stimulation system can be switched off or removed.

Thanks to pre-surgical planning, the ROSA® robot becomes an efficient tool for optimizing the accuracy of the surgical operation required for dealing with this pathology.

Benefits and risks


Benefits

The results are encouraging for segmental dystonia. The first effects can be measured from 3 months post-op and the severity of the motor handicap is reduced by 60% over one year. For generalized primary dystonia, physicians have noted a 40% improvement in the 3 first months.

  • Increased safety: the installation of the electrodes and stimulators involves fewer risks than pallidotomy (surgical lesions),
  • Less invasive, and therefore less painful procedures,
  • When applied to young patients, this  technique does not cause irreversible damage to a developing brain,
  • A customized solution which allows the electrical currant to adapt to the intensity of the symptoms and individual requirements,
  • A totally reversible installation in case of failure.
     


Risks

The risks of DBS may be related to :

  • To the surgical operation : bleeding, headaches,
  • Side-effects,
  • To complications with the neurostimulator.

 

Consult your doctor for a complete list of benefits and risks, precautions, clinical results and other important medical information relative to deep brain stimulation.

The results obtained with brain stimulation are better as regards primary dystonia (78% improvement on the dystonia rating scale at 2 years post-op). For primary dystonia, functional recovery is more or less complete and allows the patient to return to his/her normal life. For secondary dystonia, each case must be envisaged separately, because these are particularly diverse diseases.

The duration of hospitalization is around 3 to 4 weeks. This duration varies depending on the patient’s condition and clinical improvement after the intervention. The response to stimulation is very variable from one patient to another. The effect of stimulation on the patient is judged after 6 weeks and may continue to develop for up to 3 years after the operation.

The implanted equipment does not change the patient’s life in any way. Patients forget their equipment very quickly after the operation, and may live perfectly normally.

The stimulation equipment is quite compatible with pregnancy. The decision to have a family depends more on the patient’s medical, genetic and neurological context rather than on the presence of equipment.

Yes. Patients will be on treatment for life.