A CASE OF SPONTANEOUS BILATERAL CAROTID CAVERNOUS FISTULAS. S. Habib 1 , P.K. Han 1 ; 1 University of Pittsburgh, Pittsburgh, PA (Tracking ID #76354) LEARNING OBJECTIVES: 1) To recognize the clinical symptoms and signs of carotid cavernous fistula. 2) To describe an unusual presentation of carotid cavernous fistula. CASE INFORMATION: An 85-year-old woman presented with a 3±4 week history of progressive difficulty with eye opening, poor vision, and tinnitus. She denied headaches, ocular or orbital pain, diplopia, worsening of symptoms with activity, dizziness, focal weakness, or other recent illnesses. She had a history of recent falls, but no head trauma. Her past medical history was significant for hypertension, primary hyperparathyroidism, normocytic anemia, Paget's disease of bone, and a fifty pack-year smoking history. She was on ferrous sulfate but no other medications Physical examination revealed a blood pressure of 168/74 without orthostatic changes, mild conjunctival pallor, and bilateral complete ptosis. There was chemosis of the right eye and opthalmoplegia, with bilateral palsies of cranial nerves III, IV, and IV. Both pupils were mid-dilated and non-reactive to light. No other neurological deficits were noted. Diagnostic evaluation included a normal CT scan of the brain, while brain MRI demonstrated changes suggestive of bilateral carotid cavernous fistulas. Cerebral angiography was performed, which confirmed the presence of bilateral type A carotid cavernous fistulas. The patient then underwent trans-carotid endovascular embolization, which was unsuccessful. Unfortunately the patient developed right-sided hemiparesis a few days later, and subsequently declined further intervention. IMPLICATIONS/DISCUSSION: Carotid cavernous fistula is an unusual problem caused by an abnormal communication between the internal and/external carotid artery and the cavernous sinus. It is classically characterized by the triad of pulsating exophthalmos, ocular chemosis and an orbital bruit. Other features include orbital pain or tightness, pulsatile tinnitis, visual loss due to ischemia of optic nerve and retina, retinal vein congestion, and glaucoma. Ophthalmoplegia often results from pressure upon local cranial nerves; CN VI is affected most often, and CN III and IV are involved less frequently. Our patient did not present with the classic clinical triad, but with bilateral complete ophthalmoplegia and pulsatile tinnitis. Trauma is the most common cause of carotid cavernous fistula although 20 percent of cases are non-traumatic. Spontaneous bilateral carotid cavernous fistula, as observed in our patient, is a very rare entity. Risk factors for carotid cavernous fistulas include older women, mothers during childbirth, hypertension, atherosclerosis and collagen vascular diseases. A four-type classification of carotid cavernous fistulas based on the anatomy of the lesion has been proposed. Our patient had type A fistulas, presumably due to a rupture of an intra-cavernous carotid artery aneurysm. Early recognition and intervention is important to prevent permanent visual loss and ophthalmoplegia. Endovascular interventions (embolization and balloon occlusion) represent a successful treatment option although stroke is a well-recognized complication in 5±15 % of cases. LEARNING OBJECTIVES: 1, To recognize that autoimmune hepatitis is a rare complication following allogeneic stem cell transplantation. 2, To understand the pathophysiology of auto immune hepatitis folllowing stem cell transplantation. CASE INFORMATION: A 43 year-old white female developed Ph-chromosome positive chronic myeloid leukemia. She received cytoreductive therapy, followed by a 6/6 HLA matched allogeneic peripheral stem cell transplant (SCT). Tacrolimus and prednisone were continued for 18 months. One month after tacrolimus dose reduction, her serum AST and ALT levels were 723 and 965 respectively, but bilirubin and alkaline phosphatase were normal. An abdominal sonogram was normal. After the tacrolimus dose was increased to its baseline level, serum AST and ALT levels decreased to 45 and 60. Tacrolimus was then gradually reduced and eventually discontinued, which caused a rise in AST and ALT levels. She had no risk factors for viral hepatitis. Physical examination was normal except for Cushingoid appearance. CBC with differential were normal. Biochemical tests showed total bilirubin 0.7, AST 88, ALT 128, ALP 96, GGT 74, albumin 3.7, and total protein 9.8. Other biochemical profile including TSH, iron studies, ceruloplasmin, a 1 anti-trypsin and a feto protein were all within normal limits. ANA and anti thyroglobulin antibodies were positive whereas anti LKM, anti mitochondrial, anti smooth muscle antibodies were all negative. She had negative serology for EBV, CMV, HZV and hepatitis A, B, and C viruses. The histology was most consistent with autoimmune hepatitis. In situ hybridization study for Y chromosome established that immune reactive cells were exclusively of donor origin. There was no evidence of recurrent chronic myelogenous leukemia. Serum transaminase levels normalized with prednisone 20 mg a day but relapsed on dose reduction. Azathioprine was therefore added. Her transaminase levels have been normal for 28 months. IMPLICATIONS/DISCUSSION: This report documents a case of liver dysfunction, following SCT, which was indistinguishable from`definite' autoimmune hepatitis (baseline diagnostic score > 15) as defined by the modified criteria of International Autoimmune Hepatitis Group. To our knowledge, the present case represents the first report of this complication after allogeneic peripheral SCT. One intriguing feature of this case is that the target liver cells and immune reactive cells are of different origins. The donor origin of immune reactive cells was clearly established in our female patient by in-situ hybridization labeling of the Y chromosome. As the term`autoimmune hepatitis' does not accurately reflect the disease process, we propose the term`alloimmune hepatitis' for this syndrome. A 50 year old white male was admitted to our hospital in July with three days of fever, chills, headache, and neck stiffness. He worked as an air-conditioner repairman and spent a considerable amount of time shirtless, outdoors in East Texas. Physical examination was significant for fever of 102.4 degrees Fahrenheit, meningismus, and Kernig's sign. The patient was awake, alert, and oriented to person, place, and time. Initial CSF analysis revealed WBC 91 with 92% neutrophils and 8% monocytes; protein 81, glucose 68; gram stain revealed few WBCs, and was negative for bacteria; latex agglutination screen was negative for H. influenzae, S. pneumoniae, N. meningitis A, B, C and group B streptococcus. CBC revealed a WBC count of 10.6 with 70% neutrophils, 20% lymphocytes, and 10% monocytes. Within two days of admission, the patient became progressively more lethargic and delirious and was oriented only to person, month, and year. Repeat CSF analysis revealed WBC of 223 with 15% neutrophils, 59% lymphocytes, and 26% monocytes; glucose of 66 and protein of 81. CSF bacterial cultures remained negative. CSF studies for syphilis, tuberculosis, and herpes simplex virus were negative. CSF IgM was positive for West Nile virus. IMPLICATIONS/DISCUSSION: Until 1999, West Nile virus was found only in Africa, Asia, and the Middle East. Since that time, West Nile virus has infected over 3852 individuals in the U.S. in at least 39 states. West Nile virus is a single-stranded RNA Flavivirus virus that belongs to the Japanese encephalitis complex. Birds are the natural hosts of West Nile virus and mosquitoes, particularly of the culex species, act as vectors. Infection in humans typically occurs in late summer through early fall when mosquitoes are most predominant. Severe neurologic disease accompanying West Nile virus infection is rare. One out of every 150 infected patients develops meningitis or encephalitis. Age greater than 50, is the major risk factor for developing life-threatening neurologic disease. West Nile viral encephalitis is similar to other viral encephalitities and is characterized by fever, headache and an altered level of consciousness. CSF studies typically show a lymphocytic pleocytosis with elevated protein.