“Dystonic” body perception in childhood dystonia

Dystonia refers to a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements, or abnormal postures. Although the pathophysiology of the abnormal posture is still unknown and a wide variety of abnormal postures can be observed, recent psychophysical studies have revealed abnormalities in the way patients with focal and generalized dystonia judge the position of their bodies in space [Bove M, Brichetto G, Abbruzzese G, Marchese R, Schieppati M. Neck proprioception and spatial orientation in cervical dystonia. Brain 2004;127(Pt 12):2764–78; Molloy FM, Carr TD, Zeuner KE, Dambrosia JM, Hallett M. Abnormalities of spatial discrimination in focal and generalized dystonia. Brain 2003;126(Pt 10):2175–82; Anastasopoulos D, Nasios G, Psilas K, Mergner T, Maurer C, Lucking CH. What is straight ahead to a patient with torticollis? Brain 1998;121(Pt 1):91–101]. Most intriguingly, patients do not always recognize “straight ahead” in the way normal individuals do [Anastasopoulos D, Nasios G, Psilas K, Mergner T, Maurer C, Lucking CH. What is straight ahead to a patient with torticollis? Brain 1998;121(Pt 1):91–101]. We describe a patient with childhood-onset dystonia who consistently drew images of his unaffected family members with ‘dystonic’ postures without being aware that this posture is abnormal.