RESUMO
We report the case of a patient with a history of an atypical lung carcinoid tumor who developed a rapidly progressive memory impairment. The clinical presentation as well as brain MRI, cerebrospinal fluid, and laboratory tests led to the diagnosis of seronegative paraneoplastic autoimmune limbic encephalitis. To the best of our knowledge, this is the first case in literature of such association. This case also highlights an exceptionally favorable outcome, both clinically and radiologically, after immunosuppression and tumor removal.
RESUMO
Autoimmune encephalitis (AE) are a group of rare inflammatory diseases of the central nervous system that can manifest as subacute cognitive impairment. Despite the presence of diagnostic criteria, it can be challenging to identify this disease in certain age groups. In this article, we present the two main clinical phenotypes of AE associated with cognitive impairment, the factors contributing to the long-term cognitive outcome and its management after the acute phase.
Les encéphalites auto-immunes (EAI) sont un groupe de maladies inflammatoires rares du système nerveux central (CNS) pouvant se manifester par une atteinte cognitive d'apparition subaiguë. Malgré la présence des critères diagnostiques, leur identification peut représenter un défi dans certains groupes d'âge. Dans cet article, nous exposons les deux principaux phénotypes cliniques d'EAI s'associant à des troubles cognitifs, les facteurs contribuant au pronostic cognitif et la prise en charge après la phase aiguë.