Ptosis, blepharospasm, and apraxia of eyelid opening secondary to putaminal hemorrhage

Case report.

A 60-year-old left-handed hypertensive woman without any history of neurologic events was admitted with acute onset of right-sided weakness. She was alert and oriented with fluent but dysarthric speech. She had no evidence of orofacial or limb apraxia. She had a central right-sided facial weakness and a right hemiparesis. There was no neglect or sensory loss.

The pupils were 3 mm, symmetrical, and reactive. Extraocular movements were full, including vertical saccades. There was no local corneal disease. During the first 2 days, she rarely opened her eyes spontaneously even when engaged in conversation. She would attempt to contract her frontalis muscles to raise her eyelids. The patient was aware of her difficulty in opening her eyes but was frustrated, as attempts to open her lids with her nonparalyzed hand would provoke reflex blepharospasm. Voluntary closure of eyes was normal after 2 days, although voluntary opening remained slow. Following the initial 2 days, she could open her eyes slowly after blinking but there was bilateral ptosis.

A CT scan done at time of admission revealed a left putaminal hemorrhage with mass effect on the posterior limb of the capsule (figure). The blepharospasm subsided over 2 days and the AEO improved over 8 days. The ptosis was improved but still present when she was seen 4 weeks later. She was ambulatory with mild residual hemiparesis. There was no evidence of extrapyramidal disease.

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Figure. Noncontrast CT scan shows left putaminal hemorrhage with mass effect on the posterior limb of the internal capsule.

Discussion.

The pathophysiologic substrate for disorders of eyelid movements is not well known. Bilateral eyelid opening in awake patients by unilateral stimulation in the prefrontal, occipital, and rarely the precentral cortices has been described.3 Recently, the basal ganglia have been shown to play an important role in production of the blink reflex.4

Apraxia of eyelid opening has been defined as a nonparalytic inability to open eyes at will in the absence of orbicularis oculi contraction, and is usually associated with forceful frontalis muscle contraction.5 Ocular motor nerve or ocular sympathetic dysfunction are not present. The presence of AEO with nondominant hemispherical infarction2 and rostral brainstem disease6 suggests disruption in supranuclear pathways linking these structures. Apraxia of eyelid opening is also a feature of extrapyramidal diseases, such as progressive supranuclear palsy, indicating the role of the basal ganglia in the production of this syndrome.6 Reflex blepharospasm or excessive eyelid closure that is provoked by attempts to hold the eyelids open is seen with nondominant hemispheric infarction1 as well as brainstem lesions.7

Cerebral ptosis, previously thought to be due to mass effect on the brainstem, has since been described following hemispheric infarctions.1,2 These reports suggest that supranuclear control of the levator palpebrae superioris may be lateralized to the nondominant hemisphere.1,2 The severity of ptosis does not always correspond with side of weakness.

Different eyelid abnormalities may coexist in the same patient. Our patient was unique in that she exhibited three different eyelid abnormalities—AEO, reflex blepharospasm, and bilateral ptosis. A similar constellation of signs has been previously described in a patient with a right-sided frontotemporal hemorrhage.1 To our knowledge, these eyelid abnormalities have not been reported with putaminal hemorrhage. Combinations of ptosis and AEO have been described with nondominant hemisphere infarction, as well as with capsular infarction.2 Apraxia of eyelid opening has been reported to occur in up to 10% of cases of blepharospasm.5

The majority of left-handed people tend to have left hemisphere dominance. However, the presence of these eyelid movement abnormalities, similar to other reported cases,1,2 as well as the lack of language abnormalities leads us to suggest that the lesion in our left-handed patient was in her nondominant hemisphere. The coexistence of these three abnormalities in this patient suggests that they share a common supranuclear pathway linking the nondominant cerebral hemisphere to the brainstem and involving the basal ganglia. The improvement in these signs, coinciding with the improvement of the hemiparesis, raises the possibility of capsular involvement in the production of these eyelid abnormalities. Alternatively, the intact hemisphere could have taken over this function. This presentation also indicates that the control of eyelid movements is lateralized, most likely to the nondominant hemisphere, accounting for the development of these abnormalities with unilateral lesions.