Journal of Neurology, Neurosurgery, and Psychiatry 1988;51:767-772 Blepharospasm: a review of 264 patients

SUMMARY The natural history and response to different treatments have been evaluated in 264 patients with blepharospasm. The mean age of onset was 55 8 years and there was a female pre-ponderance of 1 8 to 1. Dystonia elsewhere was found in 78 % of patients, usually in the cranial-cervical region, and appeared to follow a somatotopic progression. A family history of blepharo-spasm or dystonia elsewhere was found in 9-5 % of cases, which suggests a genetic predisposition. Ocular lesions preceded the onset of blepharospasm in 12- 1 % of cases. The response to drugs was inconsistent, although initial improvement was experienced by one fifth of patients treated with anticholinergics. Twenty-nine bilateral facial nerve avulsion operations were performed with benefit in 27 cases; but recurrences appeared in 22, on average one year after surgery. Botulinum toxin injections were performed in 151 patients. Significant improvement was achieved in 118 cases. Mean duration of benefit was 9-2 weeks. Transient ptosis and diplopia were the commonest side effects. Blepharospasm,I repetitive involuntary sustained contractions of orbicularis oculi, is now believed to be a neurological disease. As a result of increased public-ity and general awareness of this disabling condition, more and more patients are being recognised throughout the world. Prevalence data are limited, but a survey of the Mayo Clinic register estimated a figure of about 5 per 100,000 (Nutt, unpublished observations). There may therefore be some 3,000 affected individuals in the United Kingdom. To assist neurologists and ophthalmologists in advising pa-tients with blepharospasm on the possible precip-itants and inheritance of their illness, its natural history and chances of remission, and the outcome of the various treatment options for this condition, we present here a review of 264 patients seen a