Dementia & Neuropsychologia ISSN: 1980-5764 [email protected] Associação Neurologia Cognitiva e do Comportamento Brasil Ribeiro Soares Neto, Herval; Palmeira Cavalcante, Wagner Cid; Nunes Martins Filho, Sebastião; Smid, Jerusa; Nitrini, Ricardo Capgras syndrome associated with limbic encephalitis in a patient with diffuse large B-cell lymphoma Dementia & Neuropsychologia, vol. 10, núm. 1, enero-marzo, 2016, pp. 63-69 Associação Neurologia Cognitiva e do Comportamento São Paulo, Brasil Available in: http://www.redalyc.org/articulo.oa?id=339544714012 How to cite Complete issue Scientific Information System More information about this article Network of Scientific Journals from Latin America, the Caribbean, Spain and Portugal Journal's homepage in redalyc.org Non-profit academic project, developed under the open access initiative Dement Neuropsychol 2016 March;10(1):63-69 Case Report Capgras syndrome associated with limbic encephalitis in a patient with diffuse large B-cell lymphoma Herval Ribeiro Soares Neto1, Wagner Cid Palmeira Cavalcante1, Sebastião Nunes Martins Filho2, Jerusa Smid1, Ricardo Nitrini1 ABSTRACT. We report the case of a patient with insidious onset and slowly progressive cognitive impairment, behavioral symptoms, temporal lobe seizures and delusional thoughts typical of delusional misidentification syndromes. Clinical presentation along with extensive diagnostic work-up revealed limbic encephalitis secondary to diffuse large B-cell lymphoma. The patient underwent immunotherapy with high-dose corticosteroid but no significant improvement was observed. No specific treatment for lymphoma was performed because the patient died of septic shock following a nosocomial respiratory infection. Delusional misidentification syndromes are an unusual and unique form of cognitive impairment in which a patient consistently misidentifies persons, places, objects, or events. Capgras syndrome is the most common subtype of this disorder, being defined by the recurrent and transient belief that someone close has been substituted by an imposter. These entities are generally associated with neurodegenerative diseases and psychiatric disturbances. Rare reports of associations between misidentification syndromes and autoimmune diseases such as multiple sclerosis have been published, but no papers address a correlation with limbic encephalitis or lymphoma. Key words: Capgras syndrome, limbic encephalitis, lymphoma. SÍNDROME DE CAPGRAS ASSOCIADA A ENCEFALITE LÍMBICA EM UM PACIENTE COM LINFOMA DIFUSO DE GRANDES CÉLULAS B RESUMO. Nós relatamos o caso de um paciente com início insidioso e lentamente progressivo de declínio cognitivo, sintomas comportamentais, crises convulsivas do lobo temporal e pensamentos delirantes típicos dos delírios monotemáticos de identificação. A apresentação clínica associada aos exames complementares levaram ao diagnóstico de encefalite límbica relacionada a linfoma difuso de grandes células B. O paciente foi submetido a imunoterapia com altas doses de corticoide sem melhora clínica. Não foi realizado tratamento específico para o linfoma em função do paciente ter evoluído para óbito por choque séptico secundário a infecção respiratória nosocomial. Os delírios monotemáticos de identificação representam uma forma única e incomum de comprometimento cognitivo nas quais o paciente consistentemente identifica de forma errônea pessoas, lugares, objetos ou eventos. A síndrome de Capgras é o subtipo mais comum dessas desordens, sendo definida como a crença recorrente e transitória que um impostor substituiu uma pessoa familiar. Essas entidades são geralmente relacionadas com doenças neurodegenerativas e distúrbios psiquiátricos. Raros relatos da associação entre os delírios monotemáticos de identificação e doenças autoimunes como esclerose múltipla foram publicados, porém não há artigos abordando a correlação com encefalite límbica ou linfoma. Palavras-chave: síndrome de Capgras, encefalite límbica, linfoma. INTRODUCTION another.1 The most frequent DMS is Capgras elusional misidentification syndromes Syndrome (CS), which is defined by the recur- D(DMS) are an uncommon group of dis- rent and transient belief that a person, usually orders characterized by delusional beliefs someone closely related, has been substituted that the people, objects, or places around the by an imposter with very similar features as patient change or are made to change with one the original person.2 Lesions found in DMS This study was conducted at the Department of Neurology, University of São Paulo, São Paulo SP, Brazil. 1Department of Neurology, University of São Paulo, São Paulo SP, Brazil. 2Department of Pathology, University of São Paulo, São Paulo SP, Brazil. Herval Ribeiro Soares Neto. Rua Oscar Freire, 2239 / apto 409 – 05409-011 São Paulo SP – Brazil. E-mail: [email protected] Disclosure: The authors report no conflits of interest. Received October 23, 2015. Accepted in final form December 26, 2015. Soares Neto et al. Capgras syndrome in large B cell lymphoma 63 Dement Neuropsychol 2016 March;10(1):63-69 are usually bifrontal or right hemispheric, resulting in and valproate without any significant improvement. A disconnection between the frontal lobes and the right neurosurgeon diagnosed normal pressure hydrocepha- temporolimbic regions, which are necessary for reconcil- lus and performed a ventriculoperitoneal shunt with no ing information about self-identification of the person apparent symptomatic relief. and their associated emotions.3 These syndromes are At a later stage, he presented to the Neurology generally associated with neurodegenerative illnesses Department of the University of São Paulo with the and psychiatric disorders,4 and rarely with other entities main complaint of worsening delusional thoughts. The such as autoimmune diseases.5 patient stated that his wife had been substituted by a Limbic encephalitis (LE) is an uncommon inflamma- perfect copy of herself, that the house he was living in tory and autoimmune neuropsychiatric condition affect- only resembled his original property, and the city’s most ing the medial temporal lobe of the brain characterized famous avenue had been duplicated in two identical by subacute cognitive symptoms, short-term memory copies. loss, seizures, and affective changes.6 This condition Past medical history was relevant for Pulmonary was initially described as a paraneoplastic syndrome of Abscess, Hypertension, Depression, Diabetes and former the central nervous system.6 Tumors generally associ- tobacco and alcohol abuse. He had an unremarkable fam- ated with LE are small cell lung cancer, breast cancer, ily history. Prescribed medications were Carbamazepine, testicular tumors, teratoma, Hodgkin’s lymphoma and Olanzapine, Sertraline and Clonazepam. thymoma.7 Other types of cancer such as non-Hodgkin Neurological examination was remarkable for a cog- lymphomas are rarely reported to be associated with this nitive impairment characterized by dysexecutive syn- paraneoplastic syndrome.8 drome (inappropriate digit span, low fluency verbal test, We describe the case of a patient who presented with impairment of abstract thinking and problem solving, the clinical picture of a DMS which, following a thorough inadequate clock drawing test), psychomotor slowing and investigation, was found to have LE associated with Dif- inappropriate behavior. The Mini-Mental State Exami- fuse Large B Cell Lymphoma.9 To our knowledge, there nation test score was 26 out of 30 (dropped 1 point in are no reports in the medical literature associating DMS spatial orientation and 3 in recall). Glabellar and palmo- with LE or non-Hodgkin lymphoma. mental primitive reflexes were markedly present as well as inhibitory paratonia, demonstrating frontal release CASE REPORT signs. A 64-year-old right handed male with a university Based on initial impressions, the clinical presentation degree in arts presented to the neurology service with was attributed to LE (short-term memory impairment, the chief medical complaint of behavioral changes temporal lobe seizures and behavioral abnormalities) of slowly progressing over the course of the past seven protracted course associated with a delusional misiden- years. Initially the patient experienced an insidious tification syndrome (Capgras Syndrome concomitant to onset of decline in work productivity and gradually Reduplicative Paramnesia). stopped working within the space of a year. Mean- The patient was then admitted to the neurology ward while, his family also noticed a short-term memory for extensive investigation. General laboratory tests deficit primarily evidenced by difficulty remembering were unremarkable, serology for common infectious messages he was supposed to give to his wife, as well diseases were negative (HIV, Syphilis, Lyme, Cytomega- as not being able to name some specific objects. In the lovirus, Herpes Simplex), Antinuclear Antibodies (ANA) ensuing years the symptoms kept worsening while new were positive in high titers (1:2560) but the remaining behavioral and motor changes such as apathy, irrita- rheumatologic panel was unrevealing (AntiSm, Anti-Ro, bility, aggressiveness, delusional thoughts, psycho- Anti-La, Anti-dsDNA). Brain Magnetic Resonance Imag- motor and gait slowing were contributing to progressive ing (MRI) revealed high signal intensity on T2/ FLAIR in cognitive impairment. There were also reports of parox- the left temporal pole and left mesial temporal region. ysmal events characterized by self-limited episodes of Cerebral spinal