Cauda equina-conus medullaris syndrome as an isolated presenting symptom of intravascular large B-cell lymphoma: Case report and review of the literature

Intravascular large B-cell lymphoma (IVLBCL) is a rare non-Hodgkin lymphoma with variable clinical manifestations. Although neurological symptoms are common in patients with IVLBCL, isolated cauda equina-conus medullaris syndrome is rarely reported. We herein report a case of IVLBCL whose initial presentation was cauda equina-conus medullaris syndrome with neither dermatological nor hematological manifestations. A 54-year-old man without known immune-compromised state presented with progressive ascending numbness and weakness of bilateral legs and urine incontinence for 2 months. Lumbar-sacral magnetic resonance images showed gadolinium-enhanced conus medullaris and cauda equina nerve roots. Cerebrospinal fluid analysis revealed lymphocyte predominant pleocytosis and elevated protein level without malignant cells. Focal seizure and mental status changes followed several weeks later. Brain biopsy led to the diagnosis of IVLBCL. Conclusions: IVLBCL should be included in the differential diagnosis of patients with isolated cauda equina-conus medullaris syndrome. A survey of previously published cases in the literature also showed that early initiation of chemotherapy has better outcome. Neurology Asia 2014; 19(2) : 219 – 225 Address correspondence to: Dr. Chin-Hsien Lin, Department of Neurology, National Taiwan University Hospital, Taipei 100, Taiwan. Tel: +886-2-23123456 ext 65335, Fax: +886-2-23418395, E-mail: [email protected] INTRODUCTION Intravascular large B-cell lymphoma (IVLBCL) is a rare form of extranodal non-Hodgkin lymphoma. Disseminated disease with a variety of organ involvement complicates the clinical presentations and leads to diagnosis difficulty.1 The nervous system and skin are the most frequently involved organs in western patients but are uncommon in Asians.2 Asian patients with IVLBCL usually presents as bone marrow involvement with hematological manifestations.3,4 The reasons for the ethnic difference in clinical presentation are unclear.2-4 Isolated cauda equina or conus medullaris syndrome is rarely reported as the presenting symptom of IVLBCL.5 We report an IVLBCL case whose initial presentation was cauda equina-conus medullaris syndrome without other system involvement. We also reviewed the previously reported cases reported in the literature.