Ano de Publicação: 2021
AUTORIA
Marcelo Houat de Brito, MD, Marcos Castello Barbosa de Oliveira, MD, Yuri Reis Casal, MD, Andre Neder Ramires Abdo, MD, Leandro Tavares Lucato, MD, PhD, and Mateus Mistieri Simabukuro, MD
RESUMO
A 41-year-old man was admitted to the neurology ward due to progressive vertigo and unsteadiness
for the previous 2 months. Neurologic examination was remarkable for a global
cerebellar syndrome. Investigation with brain MRI led to the hypothesis of a histiocytosis due to
infiltrative lesions of the pons, cerebellar peduncles, and pituitary. Therefore, investigation
progressed with chest/abdomen/pelvis CT, bone scintigraphy, and a tibial biopsy that confirmed
the diagnosis of Erdheim-Chester disease (figures 1 and 2, video).
Erdheim-Chester disease is a rare disorder characterized by the infiltration of non-Langerhans
histiocytes in multiple tissues, mainly bone, but with CNS involvement in around 40% of
cases.1,2