Dystonia in corticobasal degeneration

OBJECTIVE: To characterize the clinical features, particularly dystonia. in patients with clinically diagnosed or pathologically proven corticobasal d egeneration (CBD). BACKGROUND: Although dystonia has been reported in many neurodegenerative d isorders, it has not been studied in CBD. Dystonia, often accompanied by pa inful rigidity and fixed contractures, is one of the most disabling feature s of CBD. METHODS: The medical records, imaging studies, and videotapes of 66 patient s who satisfied the clinical criteria of CBD, evaluated between 1988 and 19 98, were reviewed. The occurrence, nature, and distribution of dystonic fea tures were analyzed and correlated with other features of CBD. RESULTS: Of the 66 patients with CBD, 39 (59.0%) had dystonia. The mean age at onset of initial symptoms was 63.9 years (range 44-75). In 20 (51.0%) p atients, dystonic symptoms began in one arm, while 13 (33.0%) patients had initial leg involvement. At least one arm was affected in 36 (92.0%) dyston ic patients. Although 11 (28.0%) patients had leg dystonia, the leg was the predominant site of involvement in only 1 patient. Only 12 (31.0%) patient s had dystonia involving the head, neck, or trunk in the course of the dise ase. The diagnosis of CBD was confirmed in all 4 patients who had autopsies . CONCLUSION: In this large series of CBD patients we found that asymmetric l imb dystonia, particularly affecting one arm, is a common manifestation of CBD; dystonia may be the initial manifestation of this neurodegenerative di sorder. Axial or leg dystonia. without significant involvement of an arm, i s rare. There is no effective treatment for this relentless disorder, excep t for temporary relief of dystonia and pain, with local botulinum toxin inj ections. (C) 2001 Movement Disorder Society.