Objective:Anti–N-methyl-d-aspartate receptor encephalitis is a rare but emerging cause of autoimmune encephalitis. Our objective is to present a case of this rare disease while highlighting the importance of an aggressive search for underlying malignancy as well as the common mischaracterization of primary psychiatric illness that occurs in these patients.Methods:A young Caucasian female with no known psychiatric history presented with acute onset of seizures and psychosis.Results:Magnetic resonance imaging abdomen and pelvis showed a 6-mm ovarian teratoma which was not visualized on initial computed tomographic scans. Pathology was consistent with a mature teratoma. Both serum and cerebrospinal fluid N-methyl-d-aspartate receptor antibodies were positive.Conclusion:An exhaustive search for underlying malignancy and specifically ovarian teratoma in young women should be completed in these patients. Diagnosis often is delayed given the prominent psychiatric manifestations and providers should be aware and strongly consider this in younger women with acute onset of neuropsychiatric symptoms.

Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia. However, anti-RNAP III has not been associated with systemic sclerosis sine scleroderma. We describe a patient with an atypical presentation of anti-RNAP III positive systemic sclerosis sine scleroderma who presented without the typical features of anti-RNAP III disease. Instead, she presented with critical digital ischemia, pulmonary arterial hypertension, gastroesophageal reflux disease, interstitial lung disease, and no clinically detectable sclerodactyly.