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Eyelid motor abnormalities found in progressive supranuclear palsy are reviewed. Electrophysiological correlates of blepharospasm, levator inhibition (blepharokolysis) and supranuclear paralysis of lid closure are presented. Disorders of eyelid motility are not uncommon in progressive supranuclear palsy (PSP). They may be found in about one third of patients with this syndrome (Jackson et al., 1983; Golbe et al., 1989). This is not surprising since ocular and eyelid movements are highly coordinated, mainly in the vertical plane (Gordon, 1951; Kennard and Smith, 1963; Kennard and Glaser, 1964), and a supranuclear ophthalmoplegia with down gaze impairment is a cardinal feature of PSP (Steele et al., 1964). The spectrum of eyelid motor disorders described in PSP includes blinking abnormalities, lid retraction, blepharospasm, levator inhibition and supranuclear palsy of eye closure.

The response to different therapeutic methods was evaluated in a series of 264 patients with blepharospasm. The most effective drug therapy were anticholinergic agents, which resulted in the initial improvement of symptoms in 20% of treated patients. There was improvement in isolated cases treated with levodopa, dopaminergic agonists, neuroleptics, benzodiazepines and tricyclic antidepressants. Bilateral avulsion of the facial nerve was carried out in 29 patients; 27 of these (93%) improved. Spasms relapsed in 22 cases, after a mean period of 12 months. Myectomy of the orbicular muscle was performed in 8 patients. In only 2 cases some improvement was obtained. One hundred and fifty-one patients were treated with infiltrations of botulinum toxin A in the orbicular muscle. 78% of cases improved. The mean duration of the benefit of each injection was 9.2 weeks. The most common secondary effects were local (ptosis, diplopia) and transient.

Two hundred and seventy-two patients with idiopathic blepharospasm were reviewed to establish the role of local eye disease in their illness. The majority of patients (57%) had symptoms at the onset of their illness such as dryness of the eyes, grittiness, irritation or photophobia suggesting eye lid or ocular surface disease. Detailed ophthalmological examination at the time of presentation had been carried out in 170 of the 272 cases; 64% of these patients had ocular symptoms, and 40% had demonstrable ocular surface or eye lid pathology. Such pathology was usually bilateral, chronic and resistant to local treatment. Blepharospasm developed in these patients after a long latent period, often of many years. Unilateral pathology was acute, normally responded well to local treatment, but was followed by the development of bilateral blepharospasm usually within six months. Amongst all 272 patients, those without ocular symptoms at presentation rarely developed them subsequently; if they did, there were no abnormal signs. The data suggest that ophthalmological disorders may trigger idiopathic blepharospasm in a substantial proportion of cases predisposed to develop this condition.

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 preponderance 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 blepharospasm 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.