LETTERS TO THE EDITORS

Tardive dystonia, a case report

Distonia tardia, um relato de caso

Dear Editor,

Meige's syndrome II/Brueghel's syndrome is a disabling spasm of the facial musculature consisting of primary blepharospasm followed by abnormal facial movement as yawning, jaw opening, and abnormal tongue movements.¹ We describe a 54-year-old man, whose delivery had been assisted by forceps. He was diagnosed with persistent delusion disorder in 1991 and began treatment with a combination of periciazine (up to 25 mg/day) and biperiden (2 mg/day). This treatment continued until 1995, when the patient began to complain of diurnal bruxism. This condition gradually worsened and, as a consequence, he cracked some teeth. A few months later blepharospasm began, followed by anterior neck spasm. In 1996, the patient began using risperidone (2 mg/day) and reported improvement of motor symptoms. The blepharo and neck spasms returned in 1998, and clozapine was prescribed. The patient reported improvement in doses of up to 300 mg/day. A year later, due to financial difficulties, this drug was suspended and he continued treatment with sulpiride (400 mg/day). In the next two years the dystonic movements worsened progressively due to the use of this medication, and involuntary tongue protrusion started. Severe speech impairment lead this patient to social reclusion and retirement, which was aggravated by the incapability to drive and frequent falls wile walking due to the visual impairment of the blepharospasm. In 2004, clozapine was restarted (100 mg/day) and combined with clonazepam (4 mg/day), resulting in an important improvement of the blepharo and neck spasm, but tongue protrusion persisted. Botulinum toxin was applied around the eyes and in the tongue. After the first application there was complete blepharospasm remission, although there was still a little unilateral ptosis and only a mild reduction of tongue protrusion. Four months later, after the second application, the result was a total remission of the blepharospasm with no ptosis, and an important partial remission of tongue protrusion. During the one-year follow-up the patient continued with the same difficulty in spoken articulation, but reported a gradual decrease in social limitations.

In our case, tardive dystonia (TD) began insidiously and progressed over years until it became static. TD runs a chronic course and spontaneous remission is uncommon even if the antipsychotics are discontinued.² TD also causes pain, physical and emotional disability as seen in this case.

Besides exposure to antipsychotics, other important risk factors for tardive dystonia in this case were a possible history of head injury at birth and male gender.³ Some cases of TD may represent late-onset congenital torsion dystonias or delayed-onset dystonia secondary to prenatal injury provoked or unmasked by antipsychotics.⁴

Clozapine has been found useful in TD, especially because of its anti-D1 action [2]. Lieberman et al. reported 43% improvement in 30 patients treated with clozapine.⁵ Treatment with botulinum toxin is justifiable in refractory patients. Tarsy et al. reported, in a series of 38 affected body regions among 34 patients, that 29 were moderately to markedly improved by botulinum toxin type A injections.⁶

In this case, social limitations of daily living and interaction caused by dystonic movements were a more severe impediment than the primary disease. This movement disorder seems to draw a progressive and independent course, in spite of the interruption of typical neuroleptics or the use of atypicals. Best results were obtained with the continued use of botulinum toxin.

André B Veras, Márcia Rozenthal, Antonio E Nardi

Institute of Psychiatry, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro (RJ), Brazil

References

1. Marsden CD. Blepharospasm-oromandibular dystonia syndrome (Brueghel's syndrome). A variant of adult-onset torsion dystonia? J Neurol Neurosurg Psychiatry. 1976;39(12):1204-9.

2. Trugman JM, Leadbetter R, Zalis ME, Burgdorf RO, Wooten GF. Treatment of severe axial tardive dystonia with clozapine: case report and hypothesis. Mov Disord.1994;9(4):441-6.

3. Adityanjee, Aderibigbe YA, Jampala VC, Mathews T. The current status of tardive dystonia. Biol Psychiatry. 1999;45(6):715-30.

4. Burke RE, Fahn S, Gold AP. Delayed-onset dystonia in patients with "static" encephalopathy. J Neurol Neurosurg Psychiatry. 1980;43(9):789-97.

5. Lieberman JA, Saltz BL, Johns CA, Pollack S, Borenstein M, Kane J. The effects of clozapine on tardive dyskinesia. Br J Psychiatry.1991;158:503-10.

6. Tarsy D, Kaufman D, Sethi KD, Rivner MH, Molho E, Factor S. An open-label study of botulinum toxin A for treatment of tardive dystonia. Clin Neuropharmacol.1997;20(1):90-3.

Financing: Inexistente

Conflict of interests: Inexistente

Publication Dates