Symptoms

Generalized Dystonia

Primary Generalized Dystonia is also known as Dystonia Musculorum Deformance (DMD). Most cases commence in childhood (age 0 to 13 years), though in some it may not appear till the teen years (age 13 to 20 years); adult onset is rare. Primary dystonia typically begins as a focal disturbance that progress either inexorably until it involves both sides of the body and leads to the patient’s death from respiratory difficulties as a result of truncal involvement or upto a point after which it becomes static. It is our observation that younger the age of onset, longer and more extensive the progress and vice versa, a conclusion also reached by other workers.

The various motor abnormalities in primary dystonia consist of phasic and/or tonic elements. Depending on sharing of irregular activities, primary dystonia may be defined as hemi-dystonic, generalized, focal or segmental and multi-focal primary dystonia, which affects two or more disjunct body sides, also occurs. The common treatment for dystonia contains of drugs like Trihexphenidyl Hydrochloride, Levodopa and Tetrabenzine. The role of surgery in dystonia is limited to patients who have failed to respond after certain time of medical treatment. Proper case selection is very important in achieving good surgical outcome. Patients who have primary dystonia or familial dystonia secondary to genetic abnormalities such as DYT-1 gene abnormality are the candidates most suitable for functional neurosurgical procedures.

Secondary dystonia occurs due to variety of causes including trauma, metabolic dysfunction, brain iron deposition (PKAN), infection, drug induced (tardive dyskinesias) etc. In several cases the cause may not be even known. Secondary dystonia are more resistant to treatment and may be more disabling. The diagnosis is established by a careful history and various investigations appropriate to each individual capacity.