Multicentric Castleman's disease with voltage-gated potassium channel antibody-positive limbic encephalitis: a case report

• Published in: BMC neurology Vol. 15; no. 1; p. 4
• Main Authors: Rao, Vikram R, Lim, Leland E, Fong, Dean, Garga, Nina I, Parko, Karen L
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 04.02.2015; BioMed Central
• Subjects: Antibodies - blood; Care and treatment; Case Report; Case studies; Castleman Disease - diagnosis; Development and progression; Diagnosis; Encephalitis; Health aspects; Humans; Limbic Encephalitis - diagnosis; Male; Middle Aged; Potassium Channels, Voltage-Gated - immunology; Seizures - etiology
• ISSN: 1471-2377; 1471-2377
• DOI: 10.1186/s12883-015-0266-8

Castleman's disease is a rare lymphoproliferative disorder which occurs in localized and multicentric forms and can mimic lymphoma. Despite its well-known association with certain autoimmune diseases, including paraneoplastic pemphigus and myasthenia gravis, Castleman's disease has not previously been associated with limbic encephalitis. We report the case of a 47-year old Caucasian man who presented with subacute onset of constitutional symptoms, diffuse lymphadenopathy, and stereotyped spells involving olfactory aura, nausea, disorientation, and unresponsiveness. He was found to have focal dyscognitive seizures of temporal lobe origin, cerebrospinal fluid with lymphocytic pleocytosis, hyponatremia, and serum positive for voltage-gated potassium channel antibodies, consistent with limbic encephalitis. An extensive infectious workup was unrevealing, but lymph node biopsy revealed multicentric Castleman's disease. His symptoms improved with antiepileptic drugs and immunotherapy. This case highlights the clinical diversity of voltage-gated potassium channel autoimmunity and expands the association of Castleman's disease and autoimmune syndromes to include limbic encephalitis. Clinicians should be aware that paraneoplastic disorders of the central nervous system can be related to underlying hematologic disorders such as Castleman's disease.