Apraxia of Lid Opening

Excerpt

Apraxia of lid opening (ALO), synonymous with apraxia of eyelid opening, is a nonmotor abnormality characterized by the patient's difficulty in eyelid elevation bilaterally. There is an inability of voluntary eye reopening without an orbicularis oculi spasm despite sustained frontalis contraction. In other words, ALO is a disorder characterized by a patient's difficulty in voluntarily opening their eyes despite an ability to do so and the absence of any ocular motor or muscular abnormalities. The definition is a misnomer, given that it rarely represents pure and true apraxia. On rare occasions, it can occur as an isolated idiopathic phenomenon.

Max Heinrich Lewandowsky, a renowned German physician and academician, first described ALO in his 1907 essay "Über apraxie des Lidschlusses." Later, Schilder, in 1927, reported 2 patients (1 with Huntington chorea), and Riese, in 1930, 1 patient with frontotemporal injury from a bullet. No other reports were found in the literature until 1965, when Goldstein and Cogan reported 4 patients (Huntington disease, parkinsonism, parkinsonian syndrome after cyanide attempted suicide, and cerebral diplegia).

ALO is often misdiagnosed due to its clinical subtlety and the lack of overt pathognomonic signs. ALO is distinct from blepharospasm, where involuntary muscle contraction forces the eyelids closed, and from ptosis, where a droop in the eyelid occurs due to muscle weakness or mechanical issues.

The eyelid opening mechanism involves a complex interaction of muscles and nerves. The levator palpebrae superioris muscle is primarily responsible for lifting the upper eyelid, and it is innervated by the superior division of the oculomotor nerve (cranial nerve III). The Müller's muscle, innervated by the sympathetic nervous system, also assists in eyelid elevation. Voluntary eyelid control is modulated by neural pathways that connect the basal ganglia, frontal lobes, and supplementary motor area, which are areas involved in initiating and planning voluntary movements. In ALO, the dysfunction appears to lie within these neural pathways rather than in the muscles themselves. The disorder is believed to originate from a disruption in the communication between the brain's intention to open the eyelid and the execution of that action. While the exact pathophysiological mechanism is not fully understood, it is associated with neurodegenerative conditions such as Parkinson disease, progressive supranuclear palsy, and other diseases that affect the basal ganglia.

Patients with ALO typically demonstrate effortful attempts to open their eyelids, often engaging in compensatory maneuvers such as manually lifting the eyelids or using exaggerated facial expressions. These actions suggest a disconnect between the intention to open the eyelids and the actual movement, a hallmark of apraxia. The condition can fluctuate throughout the day and may be influenced by factors such as fatigue, stress, or concentration. While ALO can be an isolated phenomenon, it is often seen as part of a broader neurological syndrome. It may initially present in conjunction with other Parkinsonian features before spreading to involve more extensive areas of motor control. In Parkinson disease, ALO is thought to be a manifestation of the widespread motor planning disruptions that characterize the disease.

The diagnosis of ALO is clinical, relying on the recognition of its characteristic features during examination. There are no specific tests to confirm ALO, and its diagnosis is one of exclusion. It is vital to distinguish ALO from other eyelid movement disorders, as the treatment and prognosis differ significantly. The management of ALO is challenging and is best approached with a multidisciplinary team, including neurologists and ophthalmologists. Treatment strategies may include optimizing therapy for underlying neurological conditions, using botulinum toxin injections to facilitate eyelid opening, or employing surgical interventions in refractory cases.

Understanding ALO's natural history and anatomical basis is crucial for proper diagnosis and treatment. Given its association with neurodegenerative diseases and its impact on patient's quality of life, ALO represents a significant clinical challenge that requires further research and education within the medical community. By increasing awareness and knowledge of this condition, healthcare professionals can better identify and manage this disabling and often overlooked disorder.