Torsion dystonia

Torsion dystonia - a disease characterized by the violation of muscle tone and a kind of motor disorders.
In the basis of the disease is a defeat cells subcortical structures of the brain and dentate nucleus of a cerebellum. The disease occurs more often in the form of isolated cases, but installed and genetically determined predisposition: sometimes torsion dystonia sick members of the same family.
In the muscles of the trunk and extremities progress slowly tonic spasms leading to the curvature of the body by type of rotation around the longitudinal axis (torsion spasm, rotational spasm). These convulsions (see) cover the muscles of the back, neck, torso, pelvic girdle and limbs, resulting in bizarre positions which are followed by the curvature of the back, bending the body to the side or forward, throws back his head, hands are allocated back or to the power given to the body. These violent muscle spasms - hyperkinesia (see) come paroxysmal and grow sharply at each attempt to move, resulting in patients with great difficulty walking or even standing. At rest convulsions diminish and disappear during sleep. All other functions of the nervous system and psyche remain fully intact.
The disease usually begins at a young age (10-15 years) and progresses steadily. In addition to generalized forms are observed and local forms of torsion dystonia, in which the cramps cover isolated neck muscles - spastical wryneck (see) or one limb, simulating writing spasm (see). Local torsion dystonia begins at a later age and flows more easily.
Sometimes convulsions type torsion dystonia can be a manifestation not an independent disease, but a symptom of other diseases, epidemic encephalitis (see), hepato-lenticular degeneration (see), brain tumor.
Diagnosis, differential especially, is more difficult and sometimes only set postmortem.
The forecast is unfavorable: the disease is slowly progressing.
Treatment. Prescribe muscle relaxants (mellitin), anticholinergic drugs (Metamucil), adrenoblokatora (ornid, aldomet), but they do not always have a positive effect and in such cases a surgery - destruction of subcortical structures (see Stereotaxic method). When the local forms of torsion dystonia can be applied surgical intervention on the peripheral nerves (see Neurotomy, Radiotime).