Case Presentation: A 30 year old male with history of recently diagnosed AIDS presented with altered mental status, fevers and 3 witnessed tonic-clonic seizures. His family reported that he had been complaining of progressive headaches and photophobia for several weeks and that he was diagnosed with AIDS. He was admitted 1 month prior for rash and pneumonia and again for headache and fevers. During the prior admissions, he was found to have zoster, PJP pneumonia and neurosyphilis. Head CT and MRI Brain were normal. He was discharged home with Penicillin G and started on HAART therapy with Triumeq(abacavir, dolutegravir and lamivudine). At a followup visit, he continue to have headache and complained of new diplopia, photophobia, and meningismus, leading to return to the ED. En route, he had three episodes of generalized tonic clonic seizure activity. He was afebrile but confused and somnolent. He had positive Brudzinski’s sign, photophobia, and thrush. A lumbar puncture(LP) was performed which showed pleocytosis with 60% Lymphocytes and 35% Polymorphonuclear cells (Previous hospital LP showed 45% lymphocytes and 35% polymorphonuclear cells). He was treated with Vancomycin, Rocephin, Ampicillin and Acyclovir. Lumbar puncture results found HSV in CSF by PCR, and an MRI showed bitemporal enhancement. The patient still endorsed nuchal rigidity and headache, and minimally improved photophobia. A CT Angiogram of the head and neck showed evolving ischemia in the thalamus and midbrain and arterial irregularity of the left Anterior Cerebral Artery(ACA) and bilateral Middle Cerebral Arteries. A six vessel cerebral angiogram showed severe diffuse vasculitis. The patient was immediately started on IV methylprednisolone. A repeat LP that was performed 4 days after admission returned positive for VZV DNA. Serum ANA, anti-SSA/SSB, c-ANCA, p-ANCA were negative. The patient slowly improved with resolution of nuchal rigidity, photophobia, and headaches. IV steroids were transitioned to oral prednisone. Within a few days, the patient was able to walk, eat, had no gross neurological deficits, and the he was discharged with IV Acyclovir to complete treatment for HSV and VZV and tapering prednisone for cerebral vasculitis.
Discussion: This case highlights the importance of persistence in identifying a diagnosis when clinical improvement is not observed. Although our patient was initially treated for neurosyphillis, the patient was discharged from the outside hospital without resolution or improvement of his presenting symptoms. VZV and HSV meningoencephalitis are not especially rare in the immunosuppressed individual, however their ability to cause vasculitis in the setting of AIDS and neurosyphillis is uncommon. This case presents a management dilemma for the use of high dose intravenous steroids in a patient where immunosuppresion with steroids is potentially dangerous. Given the acute and progressive nature of vasculitis and impending cerebral ischemia based on angiogram, use of corticosteroids was indicated and proved to be beneficial.
Conclusions: Immunosuppressed patients may have multiple CNS infections. Absence or inadequate clinical improvement with treatment should prompt investigation including repeat Lumbar punctures and MRI or CT of the brain. Although uncommon, VZV CNS infection has been associated with CNS vasculitis. Based on case reports, corticoteroids are beneficial in preventing morbidity and mortality from catastrophic intracerebral events due to vasculitis.