This Week's Eye-Openers

AJOCR

Acalabrutinib as Promising Monotherapy for CLL with Optic Nerve Infiltration

Bilateral optic disc edema in a patient with longstanding leukemia raises concern for direct nerve infiltration, which is a rare, but devastating vision-threatening complication. There is currently no established standardized treatment protocol, making management difficult.

A 68-year-old man with chronic lymphocytic leukemia (CLL), diagnosed 13 years prior and not on medical treatment, presented with five days of blurred vision and floaters in his left eye. Visual acuity was 20/20 in both eyes. Fundus examination showed grade III optic disc edema with peripapillary flame hemorrhages in the right eye, grade II edema in the left, and vitreous haze in both eyes. Optical coherence tomography showed nerve fiber layer swelling, infiltrates along the Henle fiber layer in the right eye, and subfoveal fluid in the left. Visual fields showed bilateral blind spot enlargement. MRI of the brain and orbits was normal, and an infectious and uveitis workup was negative.

Because a biopsy was unlikely to change management, he was diagnosed with presumed CLL optic nerve infiltration and started on acalabrutinib, 100 mg twice daily, without steroids or other adjunctive treatment. Optic disc edema and vitreous haze improved within five days and resolved completely by two months. Vision and optic nerve appearance remained stable at one year, with only mild residual disc edema in the right eye.

Acalabrutinib is a second-generation Bruton tyrosine kinase inhibitor that crosses the blood-brain barrier. Although acalabrutinib is already standard therapy for progressive CLL, this case suggests it may also be sufficient monotherapy for optic nerve infiltration. Treatment may help avoid biopsy, intrathecal chemotherapy, or corticosteroids in select patients when there is a high degree of clinical suspicion and spared vision.

A 67-year-old man presents to your glaucoma clinic for his annual exam, accompanied by his wife. He has a history of primary open-angle glaucoma, and you have prescribed latanoprost to achieve a target IOP of less than 18 mmHg. He has a history of Graves' eye disease, for which he is currently taking methimazole. During the exam, he tells you that he recently began experiencing double vision. The symptoms first began about 2 weeks ago, and if he covers one eye, the double vision goes away. His wife also comments that he has looked “tired” recently, and she has noticed his eyelids drooping on several occasions. He denies any muscle weakness. Given this history, you are suspicious of new-onset ocular myasthenia gravis (MG).

Which of the following statements is INCORRECT regarding this situation?

A. Up to 20% of patients who present with isolated ocular MG may progress to generalized MG, but this usually occurs within the first 2 years.
B. The history of thyroid disease should increase your suspicion of myasthenia gravis.
C. You should add timolol to his glaucoma regimen, as it will help lower IOP and reduce symptoms associated with myasthenia gravis.
D. The underlying pathogenesis of myasthenia gravis involves antibodies against acetylcholine receptors.

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