Primary central nervous system lymphomatoid granulomatosis: a case report
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LETTER TO THE EDITOR
Primary central nervous system lymphomatoid granulomatosis: a case report Yuanyuan Xiang 1 & Peng Yan 1 & Shan Li 1 & Yuan Xue 1 & Cuicui Liu 1 & Jifeng Li 1 & Qinjian Sun 1 & Yifeng Du 1 & Xiaohui Liu 1 Received: 17 June 2020 / Accepted: 13 October 2020 # Fondazione Società Italiana di Neurologia 2020
Background Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder associated with Epstein-Barr virus (EBV) infection and is characterized by an angiocentric and angiodestructive lymphoid infiltrate. Clinically, LYG typically involves the lung, followed by the central nervous system (CNS), skin, kidney, and liver [1]. CNS symptoms related to LYG include headache, seizure, disturbance of consciousness, dementia, impaired vision, etc. On rare occasions, isolated CNS involvement can occur, and its clinicopathological features have not been fully elucidated yet. We herein report our experience with a case of primary CNS-LYG and discuss the importance of identifying such rare entities in light of the relevant literature.
Case presentation In December 2013, a 47-year-old woman was admitted to our hospital for generalized seizure which started more than a month ago and slow response for 5 days. Lumbar puncture examination in the local hospital showed an opening pressure of 230mmH2O. Cerebrospinal fluid (CSF) analysis revealed leukocytosis (70 × 106/L; normal range, [0–8] × 106/L) with normal levels of glucose, protein, and chloride. She was diagnosed as viral encephalitis and was treated with ganciclovir, dexamethasone, and other drugs for 14 days. Her condition improved and she was discharged. However, the symptoms recurred 5 days ago, accompanied by slow response, unstable walking and headache, but still no fever. * Xiaohui Liu [email protected] 1
Department of Neurology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, No. 324 JingWu Road, Jinan 250021, Shandong, People’s Republic of China
On admission, there were no other positive signs except for weakened cortical sensation and clumsiness on alternating movements of the left hand in physical and neurological examination. The mini-mental state examination (MMSE) score was 22 points (high school education level). The erythrocyte sedimentation rate was increased (36 mm/h; normal range, [0–12] mm/h). Serum EBV-IgG was positive, while EBV-IgM was negative. Other laboratory assessments, such as complete blood count, blood biochemistry, liver and renal function, thyroid function, coagulation function, hepatitis virus, human immunodeficiency virus (HIV), tuberculosis, syphilis, antinuclear antibody, double stranded DNA, and tumor markers, were all negative. No obvious abnormalities were found in chest CT; bone marrow aspiration test; and abdominal, gynecological, and superficial lymph nodes’ ultrasound. Our lumbar puncture examination showed that the pressure was 175 mm H2O. CSF analysis showed that the leucocyte count was lower than before (26 × 106/L; normal range, [0–8] × 106/L; 10% monocytes, 60% large l
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