Apraxia of lid opening

Apraxia of lid opening (ALO) is a disabling syndrome characterized by an inability to open the eyes at will. It is frequently found in association with blepharospasm (BSP), with which it may be readily confused. ALO has been called by a variety of names and attributed to a number of different mechanisms. The syndrome is not widely recognized and has been the subject of few articles; there has been no major review. Herein are reviewed the arguments surrounding its name, and its clinical features, pathophysiology, and treatment in light of the current medical literature are discussed. Goldstein and Cogan’ have used the term apraxia of lid opening for the nonparalytic abnormality characterized by “difficulty in initiating the act of lid elevation” in four patients with basal ganglia disease. Earlier, Schilde? described a difficulty of eye opening in two patients who also had difficulty in closing their eyes, and Riese3 reported ALO in a patient with a frontotemporal injury. One of the patients reported by Schilder had Huntington’s chorea, a disease also present in one of Goldstein and Cogan’s patients. Subsequent reports emphasized the presence of the syndrome in extrapyramidal diseases such as multiple system a t r ~ p h y , ~ Parkinson’s d i ~ e a s e , ~ ~ MPTP intoxi~at ion,~ and in particular, progressive supranuclear palsy (PSP).4,7Jo-14 Difficulty or inability to open the eyes on command consistent with ALO was previously reported in seven patients during the acute stage of strokes involving the nondominant hemi~phere.’~-*~ In five of these patients, lid function recovered within l month of onset. ALO is also associated with acanthocyto~is,’~ Wilson’s disease,20 and motor neuron disease.21 BoghenZ2 first drew attention to the association between ALO and BSP. The occurrence of ALO in BSP was 7% in one study7 and 10% in another.23 Tolosa et al.24 found ALO in 12 of 22 consecutive patients with BSP. The majority of reported cases of ALO occur in association with BSP. ALO can also occur in isolation.7,25-27 Apraxia of lid opening: the term. The suitability of the term ALO was challenged by Lepore and Duvo i~ in .~ To them, the presence of extrapyramidal disease in their patients disqualified the syndrome as an apraxia. This objection is countered by the syndrome’s occurrence in the absence of basal ganglia disease and the fact that its neuroanatomic basis and pathophysiology are unknown. A number of different designations, none of which is a truly adequate substitute, are suggested as being more appropriate than ALO. One of these, focal eyelid d y ~ t o n i a , ~ is nonspecific and is based on presumed evidence that the syndrome is dystonic in nature. Others, such as involuntary levator palpebrae inhibition (ILPI),4 blepharocolysis (eyelid inhibition),28 pretarsal b l epharo~pasrn ,~~ and atypical blepharospasmz6 are based on the mistaken assumption that the syndrome is due to a single mechanism. Freezingz9 and akinesia of lid function30 are terms used in relation to Parkinson’s disease and were proposed before researchers realized that the majority of the patients lack extrapyramidal signs such as akinesia and rigidity. Moreover, although “freezing” is a vivid descriptive term, other qualifications such as “in the closed position,” are required to describe the phenomenon adequately. Despite that when applied to the syndrome under consideration “apraxia” doesn’t have its customary connotation of a dissociation between language interpretive areas and the motor cortex, ALO is probably still the most useful designation for this syndrome. As others have n~ ted , l~ -~O it is brief, clear, and in common use, and addresses the main feature of the syndrome, namely the inability to open the eyes at will with preservation of the ability to open them and to keep them open at other times. “ALO” is consistent with the common meaning of the term “apraxia,” which is used when a patient “cannot perform a motor action to command despite both an adequate understanding of the action and the elementary ability to carry it As is the case for