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Images in Neurology |

Burkitt Lymphoma Presenting as a Rapidly Evolving Cavernous Sinus Syndrome

Michael Rasper; Santosh Kesari, MD, PhD
Arch Neurol. 2008;65(12):1668. doi:10.1001/archneur.65.12.1668.
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A 33-year-old woman with a history of migraines presented to her primary care physician with progressive retro-orbital headache, diplopia, and blurry vision. She was immediately examined by a neurologist and admitted to a local hospital to rule out cerebral aneurysm. A magnetic resonance (MR) angiogram revealed no aneurysm, but a subsequent MR image showed bilateral enlargement and enhancement in the pituitary gland, cavernous sinus, and optic nerves (Figure). The patient was hospitalized. Results of serologic testing for Lyme disease and lumbar puncture were negative (cytological testing and measurement of erythrocyte sedimentation rate were not performed). She was diagnosed as having Tolosa-Hunt syndrome (granulomatous inflammation of the cavernous sinus) and was treated with oral prednisone. Her headaches and visual difficulties improved and she was discharged to home receiving tapered oral prednisone therapy. Three days after discharge, she awoke with headache and complete loss of vision in the left eye accompanied by nausea, vomiting, and abdominal pain. She was admitted to our hospital, where a computed tomogram of the torso showed multiple abdominal masses. Laboratory evaluations revealed elevated levels of D-dimer (3.162 μg/mL) (to convert to nanomoles per liter, multiply by 5.476) and lactate dehydrogenase (13.69 U/L) (to convert to microkatals per liter, multiply by 0.0167). Levels of antinuclear antibodies, antineutrophil cytoplasmic antibodies, and angiotensin-converting enzyme were all within reference ranges. Results of the biopsy of an abdominal mass were preliminarily consistent with non–Hodgkin lymphoma. Because of the progressive ophthalmoplegia and blindness, the cavernous sinuses were irradiated. The next day, the final biopsy finding was interpreted as Burkitt lymphoma (BL). Radiotherapy was stopped and the patient received chemotherapy. Unfortunately, her neurological and medical status deteriorated and a second MR image of the brain showed further enlargement of the cavernous sinus masses. She died shortly thereafter of numerous medical complications and resistant lymphoma.

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Axial (A) and coronal (B) gadolinium-enhanced magnetic resonance images of the cavernous sinus area showing bilateral enlarged sinuses with abnormal enhancement of the soft tissues that abut the intracranial segments of the optic nerves, consistent with lymphoma (arrows).

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