Severely Mutilating Focal Dystonia in an Adolescent

Dystonia is a syndrome of sustained muscle contractions, frequently causing repetitive movements or abnormal postures. They are classified by age of onset, by aetiology and by distribution. Younger the age of onset, the more Likely that the dystonia will become severe and involve multiple parts of the body (Marsden et al., 1976). Approximately one third of all patients with dystonia have symptomatic dystonia (Fahnet al., 1987). In primary or idiopathic dystonia the only neurological abnormality is the presence of dystonia, and a familial pattern may be seen. The importance of personal examination of family members for the presence of dystonia has been emphasized (Zeman andDyken, 1968). Focal dystonias are often considered to be a milder expression of torsion dystonia as compared to generalized dystonia. The initial picture is at times bizzare and emotional factors are known to aggravate the condition. Hence they tend to be often misdiagnosed as primary psychiatric illness (Lesser and Fahn, 1978). We report an adolescent with oromandibular dystonia leading to severe self mutilation and discuss some of the issues in classification and assessment.