International Journal of Neuroscience, 120, 680–682, 2010 Copyright © 2010 Informa Healthcare USA, Inc. ISSN: 0020-7454 print / 1543-5245 online DOI: 10.3109/00207454.2010.510915 Tolosa-Hunt Syndrome in a Patient with Autoimmune Hemolytic Anemia Pietro Biagio Carrieri, Silvana Montella, Maria Petracca, Giovanni Cerullo, and Andrea Elefante Department of Neurological Sciences, University of Naples Federico II, Naples, Italy ABSTRACT Tolosa-Hunt syndrome is a steroid responsive painful opthalmoplegia due to a nonspeciic inlammation of the cavernous sinus. Autoimmune hemolytic anemia is caused by antibodies directed against unmodiied autologous red cells. They are both rare conditions. Here we describe the simultaneous occurrence of Tolosa-Hunt syndrome and severe hemolytic crisis in the same patient. KEYWORDS: Autoimmune hemolytic anemia, international headache society criteria, magnetic resonance imaging, pathogenesis, steroids, Tolosa-Hunt syndrome INTRODUCTION Tolosa-Hunt syndrome (THS) is a steroid responsive painful ophthalmoplegia due to a nonspeciic inlam- mation of the cavernous sinus or superior orbital is- sure, whose etiology remains obscure (Hunt, Meagher, Lefever, & Zeman, 1961). Autoimmune hemolytic anemia (AIHA) is an un- common form of anemia, which is caused by antibodies directed against unmodiied autologous red cells (Va- lent & Lechner, 2008). On the basis of registry data and respective estimates, not more than 5–10 patients with AIHA per year are diagnosed in larger hemato- logical centres in the western world (Serrano, 1992). Most AIHA is caused by warm antibodies (WA-AIHA), whereas cold antibodies (CA-AIHA) are less commonly detected. The diagnosis is based on the presence of ane- mia, signs of hemolysis with reticulocytosis, low hap- toglobin, increased lactate dehydrogenase, elevated in- direct bilirubin, and a positive direct antiglobulin test (Coombs test) (Valent & Lechner, 2008). Here we describe a case of THS in the course of chronic idiopathic CA-AIHA, suggesting that THS could be a clinical manifestation of CA-AIHA or share with it an immunological pathogenesis. The simultane- Received 4 May 2010. Address correspondence to Pietro Biagio Carrieri, MD, Department of Neurological Sciences, University of Naples “Federico II”, Via S. Pansini 5, 80131 Naples, Italy. E-mail: pbcarrieri@libero.it ous occurrence of these two conditions in the same pa- tient was never described in literature before. Our patient, a 40-year-old female, was admitted to the Department of Neurological Sciences of the Fed- erico II University because of a 1-week history of left periorbital pain, which is described as an unremitting, steady, boring pain, widespread to the ipsilateral frontal and temporal regions. After 3 days, the patient com- plained the appearance of horizontal diplopia and ptosi. Three months before, after an episode of hemolytic ane- mia, a diagnosis of CA-AIHA was made. The patient was initially started on 2 mg/kg methylprednisolone, but after 2 weeks the hemoglobin level was only marginally improved. Therefore, a second-line treatment with a weekly dose of 375 mg/m 2 rituximab was started for 4 weeks. The hemoglobin level increased to 10.8 g/dL 1 week after the irst dose and normalized after the fourth dose, with clinical remission. At examination on admis- sion to our department, the patient presented anisoco- ria, delayed light relex, paralysis of the third, fourth, and sixth cranial nerve, hypoesthesia, and pain in the distribution of the irst branch of the ifth cranial nerve, all on the left side. Laboratory tests revealed an eleva- tion of erythrocyte sedimentation rate (120 mm/hr, nor- mal value 0–20) and C-reactive protein (70 mg/mL). Red blood cells count was 2.62 × 10 6 /μL (normal value 4.0–5.4) and hemoglobin value was 9.70 g/dL (12.0–16.0), ferritin was 762 ng/mL (9–120), lactate de- hydrogenase was 644 U/L (227–450), haptoglobin was 26 mg/dL (50–300), and total bilirubin was 2.65 mg/dL (0.2–1.1) with increased indirect bilirubin. Glycemic 680