SIR: Meige's syndrome is a focal dystonia involving blepharospasm and oromandibular dystonia. It is one of the extrapyramidal syndromes that starts after long-term use of dopamine receptor antagonists and is a very difficult condition to treat.
Mr. A., age 46 years, was diagnosed with schizophrenia by DSM-IV criteria and had been treated for 17 years with neuroleptics and anticholinergics. At age 44, he first noticed the presence of blepharospasm (excessive blinking, difficulty in seeing, facial grimacing) and oromandibular dystonia (difficulty in speaking or eating), which were diagnosed as Meige's syndrome induced by long-term multiple antipsychotic medication. At the time of diagnosis of Meige's syndrome, he was receiving methotrimeprazine 25 mg/day, haloperidol 6 mg/ day, perphenazine 8 mg/day and trihexyphenidyl hydrochloride 6 mg/day. His medication was gradually decreased to perphenazine 8 mg/day and trihexyphenidyl 4 mg/day, yet his extrapyramidal symptoms persisted.
Almost one year later, his blepharospasm became extremely severe and his eyelids closed more tightly. As a result, he had to discontinue vision-dependent activities such as reading and had to take leave from work. Mr. A. was admitted to our hospital, and olanzapine 10 mg/ day was added to the drug regimen. Two weeks later, perphenazine (8 mg/day) and trihexyphenidyl (6 mg/day) were withdrawn. After one week of treatment with olanzapine monotherapy, oromandibular dystonia improved significantly. After another couple of weeks, the improvement of oromandibular dystonia was more evident. In addition, there was a gradual decrease in the movements of his facial muscles, and his blepharospasm almost completely disappeared. Olanzapine was also as effective as the previous regimen in controlling his psychosis. Mr. A. was discharged after one month of hospitalization and was able to return to work.
Although a variety of treatments have been proposed for Meige's syndrome, none have proved completely efficacious.
+1 The recent so-called atypical neuroleptics represent a new class of antipsychotic drugs that only rarely cause extrapyramidal side effects. There have been a few case reports of the improvement of tardive dystonia with risperidone. However, there are also reports of de novo tardive dystonia presumably caused by risperidone.
+2,+3 Clozapine has been reported as an effective treatment for tardive dystonia and Meige's syndrome.
+4 Unfortunately, some patients cannot be treated with clozapine because of its life-threatening side effect of agranulocytosis.
Olanzapine is a serotonin-dopamine receptor antagonist with an affinity to neuroreceptors similar to that of clozapine. As far as we know, this is the second case report of successful treatment of Meige's syndrome by olanzapine monotherapy.
+5 One might argue that the remission of Meige's syndrome was simply due to the withdrawal of perphenazine and trihexyphenidyl. Although we believe such a possibility is implausible because rapid recovery from persistent and severe tardive dystonia is unusual after the withdrawal of neuroleptic agents, a drug-free observation period would have been necessary to definitely confirm the therapeutic effect of olanzapine for Meige's syndrome.
This case report suggests that olanzapine may have a beneficial effect in treating neuroleptic-induced Meige's syndrome. Further case series and, if possible, randomized placebo-controlled trials are necessary to confirm this possibly favorable effect of olanzapine.