NEUROMYELITIS OPTICA. I.P. Haque 1 ; 1 University of Virginia at Roanoke-Salem, Roanoke, VA LEARNING OBJECTIVE: Diagnose the Different Variants of Multiple Sclerosis including Neuromylelitis OpticaTreatment of Neuromyelitis Optica CASE INFORMATION: Neuromyelitis optica or Devic's syndrome is an uncommon neurological illness characterized by optic neuropathy and myelopathy. This case presents a 39 year old African-American female with new onset of visual changes, periocular pain, headache, malaise, lower extremity weakness, and back pain. The patient's symptoms began one week prior to admission. She previously presented with lower extremity pain and weakness six months prior and was suspected clinically to have multiple sclerosis and had resolution of her symptoms with corticosteroids. She had no changes on magnetic resonance imaging of brain and cerebral spinal fluid findings were nonspecific at the time. Her symptoms of visual changes and loss with periocular pain were not present upon this prior admission. On physical exam, she had papillitis, severe visual field defect, profoundly decreased lower extremity weakness and sensation, and Lhermitte's sign. On laboratory exam, cerebral spinal fluid showed increased protein and she had an elevated ESR. Magnetic resonance imaging of spine showed diffuse cervical cord swelling and cervical signal changes. This patient presented with bilateral optic neuritis and myelitis, known as Devic's syndrome or Neuromyelitis optica. She was treated with intravenous methylprednisolone and had gradual improvement of her symptoms. DISCUSSION: Devic's syndrome generally has a rapid onset of optic neuritis and myelitis. Patients with acute or subacute Devic's syndrome often respond to corticosteroids as this patient did. In Devic's syndrome, acute spinal cord lesions can demonstrate diffuse swelling extending over several levels or the entire cord in either a continuous or patchy distribution. The optic nerve and chiasm can show either demyelinating lesions and/or necrotizing lesions. Approximately 35 percent of patients have a monophasic illness and 55 percent develop relapses, and rarely patients have a fulminantly progressive course without relapses or a course typical of multiple sclerosis. (2) Identify the indications for valve replacement in IE. CASE INFORMATION: A 41-year-old white male with past medical history of migraine headaches presented to the emergency department for a seizure occurring in a restaurant. While sitting, the patient suddenly developed stiffness and ridgidity in the right arm then the left arm. He then collapsed and had a generalized seizure. According to an eyewitness the patient lost consciousness for 3±4 minutes, after which he was confused but without incontinence or focal weakness. Prior to this event the patient experienced low-grade fever as high as 1018F and myalgias for one week but no other symptoms. Physical examination revealed a young ill looking male; temperature was 378C. He was tachycardic and the blood preasure was 164/78 mmHg. Significant findings included abrasions over the left temple, evidence of an old infarct in the left parietal region thought to be embolic. Echocardiogram showed a dilated left ventricle with borderline left ventricular hypertrophy and good systolic function, severe aortic regurgitation and suspicion of an aortic valve vegetation. A transesophageal echocardiogram showed a bicuspid aortic valve with prolapse of one of the leaflets, 3+ aoritic incompetence and evidence of a vegetation 4±5 mm in size on one of the leaflets of the aortic valve. Blood cultures initially reveled Gram + Cocci in clusters later identified as coagulase negative Staphylococcus. All the cultures drawn later were negative for any growth. Because of evidence of embolic disease on the CT scan of the brain and the recent generalized seizure (representing multiple embolic events), aortic valve replacement was indicated. The patient had a St. Jude HB mechanical valve replacement. He had an uneventful post-operative course. He was started post-operatively on heparin and then later on Coumadin. Blood cultures and cultures from the aortic and mitral valves were negative for any growth. The culture from the aortic vegetation was also negative. The patient was continued on cefrtiaxone for 4 weeks to treat likely atypical organisms. DISCUSSION: When considering the differential diagnosis of a new onset seizure, IE is a relative uncommon cause, but should be considered when fever and/or a heart murmur is present. When IE is associated with seizures, the seizures usually occur later in the course of the disease rather than as part of the initial presentation as in this case. The bicuspid aortic valve in our patient was a risk factor of IE but it is important to recognize that one third of patients with IE have no identifiable predisposing cardiac lesions. Negative cultures occur in 2±5% patients with IE. The indication for valve replacement in this case was evidence of recurrent embolic events. Mechanical valve was chosen because of the patient's young age.