LETTER TO THE EDITOR Intracranial hypertension as the presenting symptom of duodenal cancer Çağdaş Erdoğan & Eylem Değirmenci & Nedim Ongun & Levent Sinan Bir Received: 15 December 2011 / Accepted: 27 February 2012 / Published online: 14 March 2012 # Springer-Verlag 2012 Dear Editor, Intracranial hypertension (IH) is a poorly understood syn- drome that most commonly presents with headache, vision changes, and papilledema [1–3]. The pathophysiology of IH remains poorly defined. Based on the current understanding of cerebrospinal fluid flow, contributors may include cere- brospinal fluid overproduction or under-absorption, or vas- cular congestion resulting in resistance to the outflow of cerebrospinal fluid [3, 4]. IH has been reported in young oncology patients; however, IH associated with duodenal cancer was never reported before [4, 5]. Here, we report on a case of duodenal cancer in which IH was the presenting symptom. A 50-year-old man was admitted to our hospital with headache, vomiting, and blurred vision, which he had suf- fered for the previous 15 days. His neurological examination revealed bilateral papilledema and decreased visual acuity (0.6 in the right eye and 0.8 in the left eye), so he was hospitalized for the differential diagnosis of elevated intra- cranial pressure. His cranial, orbital MRI, and MRI venog- raphy were normal, excluding mass effect or venous thrombosis. Blood tests were normal except for an elevated sedimen- tation rate, and the first CSF examination was normal except for mild elevation in the protein level, but the pressure was high (210 mmH20=). CSF histopathology demonstrated nonspecific inflammation. He was put on oral acetazolamide treatment, and diagnostic tests for the etiology of IH includ- ing vasculitic markers, coagulability factors, and infectious markers were unremarkable. Thorax and abdomen CT dem- onstrated multiple lymphadenopathies. He was then searched for sarcoidosis and malignity. Biopsy was achieved from the scalene lymph nodes. His CEA and CA-19.9 levels were mildly elevated. At the same time in his follow-up, he experienced dyspnea. Echocardiogram demonstrated pleural and pericardial effusion, and anti-inflammatory treatment was started. In his follow-up, his headache and visual com- plaints were worsened, so we administered a repeated lum- bar puncture. His second CSF tests also revealed nothing other than nonspecific inflammation, but this time the pres- sure was 700 mmH20. After the second lumbar puncture, the patient underwent lumboperitoneal shunt operation. His headache and visual acuity got better after the shunt opera- tion, but the pathology results of the lymph node biopsy showed metastatic involvement of malign epithelial signet- ring cells. He was searched for gastro-intestinal system tumors, and duodenal biopsy confirmed the diagnosis of signet-ring cell tumor. Signet-ring cell tumors rarely occur primarily in the central nervous system [6]. Gastrointestinal tumors have been reported to present with many different clinical symp- toms, and but neurological problems are respectively rare [7]. Recently, Lee et al. reported a patient with leptomenin- geal involvement due to signet-ring cell gastric cancer [8]. However, to our best knowledge this is the first case of duodenal signet-ring cell carcinoma presenting with IH. There are several pathogenetic mechanisms for a tumor to be the cause of elevated pressure. The first is a mass effect due to primary or metastatic tumors [6]. The other way is a spinal block caused by a spinal tumor. Our patient’ s cranial and spinal imagings were unremarkable. Other pathogenetic processes which cause elevated intracranial hypertension in the course of malignity are meningeal carsinomatosis and paraneoplastic syndromes [7, 9]; however, our patient’ s Ç. Erdoğan : E. Değirmenci (*) : N. Ongun : L. S. Bir Department of Neurology, School of Medicine, Pamukkale University, Denizli, Turkey 90-20020 e-mail: eylemteke@yahoo.com Graefes Arch Clin Exp Ophthalmol (2013) 251:1009–1010 DOI 10.1007/s00417-012-1987-7