We present an unusual case of chronic lymphocytic leukemia (CLL) causing infiltrative optic neuritis and intracranial hypertension.

Cases of CLL with optic nerve infiltration have been reported with good response to intrathecal methotrexate and/or systemic chemotherapy in the absence of intracranial hypertesion.  However, concomitant intracranial hypertension is unusual and poses treatment challenge.

A 53-year-old woman with history of untreated CLL (RAI stage III) presented with blurry vision, morning headaches, pulsatile tinnitus and transient visual obscurations. Eye examination was pertinent for 20/20 visual acuity (VA), full color vision, 0.3 log unit APD left eye and bilateral severe optic disc edema.

MRI orbits showed findings consistent with increased intracranial pressure and mild enhancement of the left optic nerve. Lumbar puncture revealed opening pressure of 47 cm H2O, protein of 67.5 mg/dl and 7 WBCs. Left eye VA continued to deteriorate with persistent disc edema, despite maximizing acetazolamide (4000mg daily) and furosemide.  Repeat lumbar puncture revealed opening pressure of 36 cm H2O, 38 WBCs with 95% lymphocytes, and protein of 110.5 mg/dl. Flow cytometry showed monoclonal B-cell population with CD19+ B-cells (27.1%). CSF studies for infectious and inflammatory conditions were negative. Repeat MRI orbit showed intense enhancement of intracanalicular and proximal intraorbital segment of left optic nerve.

She was treated with high dose IV methylprednisolone, intrathecal cytarabine (6 doses), and systemic chemotherapy with fludarabine, cyclophosphamide and rituximab. Disc edema improved bilaterally but she developed optic atrophy in left eye.

1. 1. Co-existence of the two conditions is explained by either: Primary idiopathic intracranial hypertension complicated by spread of CLL intracranially, or intracranial leukostatis from CLL leading to secondary intracranial hypertension.
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3. 2. Co-existence of intracranial hypertension and optic infiltration in CLL patients raises diagnostic and treatment challenge.
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5. 3. Efficacy and safety of Intrathecal cytarabine vs methotrexate in the presence of papilledema requires further studies.