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West Nile Encephalitis Associated with Recurrent Strokes Kate Hoppock DO, Timothy Mikesell DO, Ashwini Peruri MD, Lynn Klassman Advocate Lutheran General Hospital MRA Carotids wo/w Contrast: Stenoses at the origins of the cervical internal carotid arteries bilaterally. Diffuse diminished caliber of the cervical left internal carotid artery. Very poor filling/nofilling of bilateral M1 segments. Introduction: In 2012 the CDC reported 5,674 cases of West Nile Virus (WNV) infections in the United States, 56% classified as neuroinvasive disease. As of July 2013, 31 cases of WNV were reported with 35% classified as neuroinvasive disease. This case report is of a man with acute ischemic strokes associated with WNV and is aimed at raising awareness of this rare association. QuickTime™ and a decompressor are needed to see this picture. MRI Brain WO/W Contrast: Extensive infarctions in both hemispheres anteriorly mostly and moderate infarctions in the left cerebellum. Within the frontal infarctions are scattered small foci of blood products. Initial Presentation: 58 year old male brought to the ED complaining of a severe headache, neck stiffness, and confusion. Pt was in usual state of health until 1 week prior to admission after returning from a hiking trip in Wisconsin. The patient initially developed a headache, neck stiffness, intermittent nausea with no emesis and subsequently developed fevers and difficulty with balance. Upon questioning he did recall a mosquito bite to the neck during one of his hikes, but did not recall any other insect bites. Patient denied any rashes, extremity numbness, tingling, or weakness, changes in vision, chest pain, dyspnea, abdominal pain, vomiting, or diarrhea on initial presentation. His past medical history was significant for a pituitary adenoma s/p resection in 2007 as well as HTN and DM controlled without medications On initial physical exam, the patient was febrile, nonfocal, but was noted to have difficulty with coordination and gait. CT without contrast brain was negative for acute changes and lumbar puncture was unrevealing demonstrating a modest pleocytosis. Emperic antibiotics and antivirals were started along with high dose steroids and a work up was initiated. On day 3 of his hospitalization, the patient developed subtle right arm weakness and right facial droop, prompting further imaging. MRI brain revealed numerous punctate infarctions in both hemispheres along the distribution of the middle cerebral artery along with an MRA head/neck demonstrating narrowing at the carotid terminus bilaterally suggestive of an emboli or vascular abnormality. A comprehensive “young stroke workup” was then pursued. IR Carotid Cerebral Angiogram: Lack of normal visualization and contrast opacification of the distal internal carotid arteries--concerning for vasculitis. Figure 1. First MRI of admission: MRI brain wo/w contrast--Numerous punctate regions of acute cerebral infarctions involving the cerebral hemispheres bilaterally in the middle cerebral artery distribution. QuickTime™ and a decompressor are needed to see this picture. Cardioembolic: Transthoracic and transesophageal echo were negative for endocarditis. Hypercoaguable: Normal protein S and C, antithrombin III, thrombin, fibrinonogen, factor 7, plasminogen, lupus anticoagulant, cryoglobulin, MTHFR, phosphatidylserine, beta-2 glycoprotein, cardiolipin. On the day of the biopsy, Serum and CSF were found to be positive for CSF WNV Ab IgM Given the patient’s grave prognosis, artifical support was withdrwan and the patient passed away 12 days after presentation. Final autopsy results demonstrated “acute encephalitic changes, pancortical vascular congestion and edema, along with left cerebellar infarction. WNV has previously been implicated as causing meningitis, encephalitis, and demyelinating neuropathies. Based on review of the literature, this is the second case report of WNV associated with a stroke. Detection and sharing of WNV associated ischemic stroke cases will potentially lead to the development of treatment strategies to decrease the morbidity of this neuroinvasive disease. References Infectious: Negative for hepatitis, HIV, HSV, Lyme disease, varicella, RPR, T. pallidum, EBV, CMV. Blood cultures remained negative. WNV serologies from CSF and Serum were still pending. During work up the patient was treated with methylpredisone 1 g daily x 3 days and continued on Acyclovir until HSV serologies were negative. Emperic antibiotics were discontinued as no source was identified. Patient continued to deteriorate and imaging was again repeated. Brain, right frontal leptomeninges, right frontal, right frontal subcortex all demonstrated benign tissue without specific pathologic changes. Brain, right frontal: Benign cortical tissue with ischemic changes **The biopsies were essentially normal on histopathology. There were focal ischemic changes, but lacked increased chronic inflammation, perivascular chronic inflammation, neuronophagia, or evidence of CNS angiitis. Conclusion: Work Up: Autoimmune: No abnormalities including ANA, RF, MPO, lupus anticoagulant panel, C3, C4, CRP, Pathologic Diagnosis: 1.Kanagarajan K, Ganesh S, Alakhras M, et al. West Nile Virus Infection Presenting as Cerebellar Ataxia and Fever: Case Report. Southern Medical Association 2003;96 vol.6: 600601 Figure 2. Second MRI: MRI brain wo/w contrast--Extensive infarctions in both hemispheres anteriorly. Given the patients rapid decline, and unknown etiology, the decision was made to obtain a tissue diagnosis and cerebral and leptomeningeal biopsies were obtained. After the procedure, the patient remained on mechanical ventilation and did not regain consciousness. 2.CDC. West Nile Virus Disease and Other Arboviral Diseases United States, 2011. MMWR 2012;61; 510-514 3.Carson PJ, Konewko P, Wold KS et al. Long-Term Clinical and Neuropsychological Outcomes of West Nile Virus Infection. Clinical Infectious Diseases 43 (6);723-730 4.Alexander J, Lasky A, et al, Stroke Associated with Central Nervous System Vasculitis After West Nile Virus Infection. Journal of Child Neurology 2006; 21:623-625 5.Gilden D, Lipton H, Wolf, et al. Two patients with Unusual Forms of Varicella-Zoster Virusc Vasculopathy. New England Journal of Medicine 2002; 347:1500-1503