Case Presentation: A 42 Y/O AAM with PMH of polysubstance abuse presented to ED with 2 weeks of worsening vertigo, headache, left facial weakness, blurred vision and new onset dysphagia. Physical exam was notable for complete left facial (VII) palsy, left abducence (VI) palsy and umbilicated facial rash. MRI of head revealed a 2.6×2.6×3.9 cm progressive brain lesion at the ponto-medullo-cerebellar junction, moderately compressing fourth ventricle. Ten days prior he had brain MRI at another hospital which showed significantly smaller 1.2×1.0 cm lesion suggestive of a demyelinating process such as multiple sclerosis. He deferred further work up at that time. Serum syphilis IgG came positive with a 1:4 titer and LP was significant for WBC 64 and lymphocytic pleocytosis. A diagnosis of neurosyphilis with gumma was entertained and the paitent was placed on IV penicillin. HIV testing was positive with a CD4 count of 55 cells/mL and viral load of 100,000. EBV CSF PCR returned elevated at 119,000. The patient initially declined further invasive testing; however he developed progressive dysphagia over the next few days and ultimately consented to brain biopsy. Brain biopsy was performed and pathology revealed EBV positive HIV associated large B-cell lymphoma. Stains for cd19, cd20, bcl2 were positive. The patient was started on IV decadron with plans for radiation and chemotherapy. To further define extent of disease, a bone marrow biopsy was performed which revealed no evidence of lymphoma. Unfortunately he developed aspiration and respiratory distress after anesthesia for bone marrow biopsy and expired
Discussion: Human Immunodeficiency Virus (HIV) can present in myriad ways, with duration of infection and degree of immunosuppression typically determining symptomatology. Likewise, patients with HIV are at risk for a number of AIDS defining illnesses (ADI) related to immunosuppression not typically seen in the general population. The most common ADI include opportunistic infections and Kaposi sarcoma, with other opportunistic malignancies representing less than two percent of ADI. We present a case of acute CN VI and VII palsy as the sentinel event prompting a diagnosis of HIV/AIDS and ultimately diffuse large B cell lymphoma (DLBCL).
Conclusions: The differential diagnosis for patients with cranial neuropathies is broad. Our case illustrates the importance of early recognition of immunocompromised state and tissue diagnosis to guide therapy. Diagnosis of DLBCL remains challenging because its clinical presentation could overlap with other HIV associated CNS lesions. DLBCL usually presents with supratentorial rather than brain stem lesions as with our patient. It is typically a late complication of long standing HIV infection in the setting of profound immunosuppression and rarely represents as an ADI. Brainstem involvement is characterized by aggressive nature, high rates of complications and fatality. Biopsy is always necessary to differentiate from other HIV associated lesions with markedly different treatments.