Oromandibular dystonia

The oromandibular dystonia ( Orofacial dystonia, Latin os "mouth"; mandible " lower jaw (bone )"; facies "face", gr dys "bad", "wrong"; Latin tonus " voltage " ) is a neurological disorder, when it comes to persistent, tonic spasms of the muscles of the lower half of the face and of the lower jaw. The affected muscles involuntarily tense, creating a hindrance in speaking and eating may result. In addition, frequently occur pain. It belongs to the group of dystonias.

Epidemiology

Conservative projections for oromandibular dystonia of a prevalence of 2/100.000 inhabitants from. When combined with spasms of the facial muscles of the upper half of the face ( Meige syndrome), the prevalence is about 7/100.000.

Etiology

The majority of oromandibular dystonia is considered " idiopathic ", the cause is unknown so. In addition, however, the tardive dystonia play ( late effect of treatment with neuroleptics ) play an important role. If the dystonia occurs in children or adolescents to be reckoned with symptomatic cause and a subsequent spread to other parts of the body frequently. In some cases, the structural changes in the basal ganglia or the brainstem were detected. Furthermore, the genetic predisposition plays a role.

Appearance

It is mainly to persistent tension of the muscles of the mouth, the chin and floor of the mouth. While dystonia of facial muscles usually affect predominantly social or cosmetically, dystonia of the jaw muscles can lead to disability in eating and speaking, as well as significant tooth wear.

Some patients may by certain movements or maneuvers (eg, counter- hold with one finger) the tension temporarily solve (gesture antagonistique ). Pain of cramped muscles are often especially those involving the muscles of mastication.

Therapy

Method of choice today is the therapy with botulinum toxin A. The treatment of facial muscles is the easiest, the muscles of the jaw closing slightly more complicated. The problem remains the treatment of oromandibular dystonia of the jaw opening type. Placing the injection into the covered by the lower jaw bone muscle (musculus lateral pterygoid ) is complicated and the effect due to the involvement of muscles in the area of floor of the mouth and throat often unsatisfactory.

For further drug therapies are especially L -dopa and Trihexyphenydil, baclofen, neuroleptics and tetrabenazine available. However, these substances are in effect often unsatisfactory and not admitted to a large extent for the indication (off label use ).

Finally, as a surgical procedure is the surgical transection of the corresponding nerves available. With careful diagnosis and appropriate symptoms, the effect can be excellent, but the effect is on the one hand connected to a surgical procedure and on the other irreversible. In particularly debilitating cases may eventually be considered a deep brain stimulation, which, however, only in specialized centers is possible.

In individual cases, physiotherapy and electrical stimulation (TENS ) have been described as useful. Massages, neck ties, rails, etc. usually lead to a worsening of dystonia. The learning of relaxation techniques can be helpful especially if dystonia under the psychological tension significantly increased.