Meige's syndrome in dementia with Lewy bodies

A complicating factor in the management of dementia with Lewy bodies (DLB) is the profound sensitivity of patients to antipsychotic treatment¹, which in turn may initiate or enhance movement disorders. Florid psychotic symptoms may, however, develop before DLB can be diagnosed.

CASE HISTORY

A woman aged 60 came to psychiatric attention for the first time when referred for advice on depression and morbid jealousy which had existed for many months. She had also experienced hallucinations and misidentification delusions. Initially she was treated with an antipsychotic (trifluoperazine 2 mg twice daily) and an antidepressant (amitriptyline 125 mg daily). Over the next few months there were repeated episodes of falling and syncope with fluctuating confusion, and a drop in her Mini-Mental State examination score indicated rapid deterioration in her cognitive abilities. Therefore, trifluoperazine was replaced with the newer atypical antipsychotic olanzapine, 5 mg daily. The results of all investigations were normal except for a CT scan of the brain, which showed moderate cerebral atrophy. Dementia with Lewy bodies was diagnosed. Although the depressive and psychotic symptoms had been improved by treatment, this benefit was overshadowed a few months later by the development of persistent involuntary bilateral blinking resulting from repetitive and prolonged contractions of the orbicularis oculi muscles (blepharospasm). Soon afterwards she began to experience an oromandibular dystonia characterized by spasms of the lower face muscles, especially those surrounding the mouth. The choreoathetotic movements seen in tardive dyskinesia were absent, and there was no known family history of movement disorders or Huntington's chorea. Although the antipsychotic medication had been stopped after development of the dystonia, the symptoms gradually worsened and became very distressing. In addition to dementia with Lewy bodies, she was deemed to have Meige's syndrome.

COMMENT

Meige's syndrome is a segmental cranial dystonia that occurs in primary or secondary forms². There is some evidence for involvement of the basal ganglia along with an imbalance in the dopaminergic system³.

Up to 25% of brains in Meige's syndrome show Lewy bodies, and some workers have proposed that the syndrome is part of the clinical spectrum of Lewy body disease⁴. Indeed, very occasionally Meige's syndrome has been reported to precede the development of Parkinson's disease⁵. Secondary Meige's syndrome, however, is not restricted to those with primary neurodegenerative disorders since it has also been diagnosed in patients receiving antipsychotic medications⁶. DLB patients, who are known to be highly sensitive to these dopamine receptor blockers, may be particularly at risk of developing secondary Meige's syndrome, since they are already prone to various parkinsonian and movement disorders. Although newer atypical antipsychotics such as olanzapine and risperidone seem less apt to cause movement disorders and better tolerated in elderly patients, DLB patients may still be vulnerable to their effects. Our patient was receiving an atypical antipsychotic immediately before developing Meige's syndrome.

Meige's syndrome has been earlier reported in patients with parkinsonism, but to our knowledge this is the first case specific to DLB. In such patients, persistent involuntary blinking of the eyes (the commonest initial presentation of Meige's syndrome) is an ominous sign that should lead to early withdrawal of the offending agent. The spectrum of Lewy body disease should also be considered in individuals with persistent involuntary blinking even if they are not receiving a dopamine receptor blocker.