A three-generation family in which 5 members were diagnosed with focal dystonia and 3 with tics, Tourette syndrome, and hyperactivity is reported from the Radcliffe Infirmary, Oxford, and Queen Elizabeth Hospital, Birmingham, England. One with dystonic head tremor subsequently died of a motor neuron disease. The findings of dystonia and Tourette syndrome in 8 members of a single family suggest a common “susceptibility gene” and an etiologic relationship for these disorders. [1]

COMMENT. A relationship between some cases of Tourette syndrome and focal dystonia, suggested by previous scattered reports in the literature, is strengthened by this family of 8 affected members, and may indicate a sharing of predisposing genes. TS is frequently familial, and may be inherited as an autosomal dominant trait. Focal dystonias are genetically heterogeneous and can be inherited as autosomal dominant traits with incomplete penetrance. The family described in which TS and dystonia cosegregate may be genetically distinct from other forms of TS and focal dystonia. It is of interest that hyperactivity occurred in one patient, who was “always on the go,” and ADHD is a frequent comorbid disorder with TS. Choreiform movements sometimes coexist with ADHD (Prechtl syndrome), but focal dystonia is a rare and subtle finding.

We have recently observed a family in which a 2 year old is diagnosed with idiopathic dyskinesia, including dystonic movements, and a 5 year old has ADHD (Keating G, Millichap JG. Division of Neurology, Children’s Memorial Hospital, unpublished observations).

Tizanidine (Zanaflex) therapy in Tourette syndrome and ADHD. When administered alone or in combination with conventional therapies, tizanidine benefited 63 patients (ages 4-19 years), and side effects, especially drowsiness and fatigue, were fewer and less severe than those with clonidine, another centrally acting a2-adrenergic agonist. [2]