Lymphoma presenting as cranial nerve neuropathies in HIV-infected patients.

In the HIV-infected population, those with systemic NHL usually present with extranodal disease involving the bone marrow, meninges, or GI tract as well as with systemic symptoms. Patients with HIV infection and primary CNS lymphoma typically present with focal neurological symptoms and associated single or multiple contrast-enhancing lesions in the brain parenchyma. Isolated cranial nerve involvement is an uncommon presentation for lymphoma. We discuss here 2 patients who manifested isolated cranial neuropathy as their initial clinical presentation of lymphoma. CASE SUMMARIES Patient M M was a 38-year-old woman with a history of poorly controlled HIV infection diagnosed in 2000. She did not have a history of opportunistic infections despite a reported nadir CD4+ cell count of 36/μL. Multiple antiretroviral regimens had been prescribed for her, but her adherence was consistently poor. M presented to the HIV clinic complaining of 2 weeks of worsening blurry vision and diplopia associated with difficulty raising her left eyelid. Her CD4+ cell count was 18/μL and HIV RNA level was 23,000 copies/mL. Results of visual testing and opthalmoscopic examination were normal. She denied eye pain, headaches, neck stiffness, hearing loss, tinnitus, and confusion. She reported a recent hospitalization for fevers and cough, and at that time she was treated for presumed community-acquired pneumonia. She was admitted to the hospital for further evaluation. M’s examination revealed a low-grade fever (temperature of 38.3°C [101°F]) with a normal physical examination other than an isolated left-sided third cranial nerve palsy. Ophthalmological examination demonstrated a left eye ptosis, a dilated and fixed 5-mm left pupil, and nystagmus in the right upward and downward gaze. An initial laboratory evaluation with complete blood cell count, chemistries, and liver function tests revealed no leukocytosis, a normocytic anemia with a hemoglobin level of 9.7 g/dL (normal, 11.7 to 16.0) and mean corpuscular volume of 81 fL (normal, 81 to 100), and a lactate dehydrogenase level of 464 U/L (normal, 96 to 200). All other test results were within normal limits. Cerebrospinal fluid (CSF) analysis revealed no white blood cells, normal protein and glucose levels, and significantly increased Epstein-Barr virus (EBV) DNA levels (more than 200,000 copies/mL using polymerase chain reaction [PCR] assay). Culture and PCR test results for JC virus, Cytomegalovirus, and varicella in the CSF were negative. CSF cytology was negative for malignant cells. An MRI demonstrated focal enhancement of the left third cranial nerve. This finding was felt to be consistent with an inflammatory process although neoplastic involvement could not be ruled out. Her hospital course was significant for progression of the left-sided third cranial nerve palsy to involve the left-sided fourth, fifth, and sixth cranial nerves and the right-sided third cranial nerve. Positron emission tomography (PET)/CT scans obtained on hospital day 6 showed hypermetabolic nodes encasing the small bowel and mesenteric lymphadenopathy, with increased uptake in left paraspinal muscles, the sphenoidal sinus, and external iliac lymph nodes.