Abstract Details

Title Optic Perineuritis as the initial neurological manifestation of CLL

Topic Neuro-ophthalmology/Neuro-otology

Presentation(s) Neuro-ophthalmology/Neuro-otology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 014

Background Chronic lymphocytic leukemia (CLL) is a B-cell neoplasm and is the most common adult leukemia diagnosis in the United States. CNS involvement is rare in CLL with symptoms such as headache, seizures, sensory changes, motor weakness, and meningitis. Optic perineuritis (OPN) can cause unilateral or bilateral optic neuropathy and can be due to a diverse spectrum of inflammatory, infectious, neoplastic, and toxic entities. Here we describe the first case report of a male with early stage CLL whose initial neurologic manifestation was OPN.

Objective To highlight optic perineuritis as a neurologic manifestation of CLL

Design/Methods Case report

Results 58-year-old male with Rai stage 0 CLL diagnosed 2.5 years ago presented with a four-day history of left eye visual loss, disc edema, a left relative afferent pupillary defect, and was diagnosed with optic neuritis clinically. Orbital MRI was classic for optic perineuritis demonstrating enhancement of the nerve sheath and adjacent orbital fat, sparing the optic nerve. Lumbar puncture showed normal opening pressure, WBC of 43 (100% lymphocytes), and elevated protein at 96. Flow cytometry showed a CD5+ monotypic B-cell population, mirroring the flow cytometry results of his peripheral blood. Diagnosis of optic perineuritis due to CNS CLL was determined. Treatment was initiated with IV methylprednisolone and Ibrutinib. Three weeks after beginning treatment, MRI orbits showed improvement of optic nerve sheath enhancement.

Conclusions Isolated involvement of the optic nerve sheath by CLL leading to OPN is very rare but can occur at the earliest stage of CLL (peripheral lymphocytosis). OPN is often initially misdiagnosed as optic neuritis, but accurate distinction between the two is important as they have different therapeutic and prognostic value. Contrasted MRI of the orbits accurately separates the two entities. Clinicians should now consider OPN as a diagnosis in a patient with CLL and vision loss.

Authors/Disclosures
Parakash Pratibhu, MD (Wellstar Neurology and Headache Center) PRESENTER	Dr. Pratibhu has nothing to disclose.
Prashant Natteru, MBBS (Mayo Clinic Health System)	Dr. Natteru has nothing to disclose.
Salman Tarique, MD (University of Mississippi Medical Center)	Dr. Tarique has nothing to disclose.
James L. Parker, MD (VA Medical Center)	Dr. Parker has nothing to disclose.