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2019

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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FATAL KIKUCHI-FUJIMOTO SYNDROME DUE TO CNS INVOLVEMENT

Catarina Perry da Câmara, Mariana Cardoso Diogo, Jacinto João, Carranca Roberto, Cristina Rios, Conceição Carla, João Reis.

1. Department of Neuroradiology, Hospital Dona Estefania, CHLC

Poster

XII Congresso Nacional da SPNR, 11 - 13 novembro 2016, Lisboa

Introdução: Kikuchi-Fujimoto disease (KFD) is a rare and benign cause of lymphadenopathy, that affects mostly children, with a male predominance. Its etiology is unknown, although environmental factors, in particular viruses, and autoimmune diseases, have been reported. KFD is characterized by localized lymphadenopathy, fever, frequent upper respiratory symptoms, and odynophagia. Onset is typically subacute. Atypical presentations and extranodal involvement are possible, mainly cutaneous manifestation and aseptic meningitis. MRI findings of brain involvement have been described in two cases in literature.
Clinical Case: We present a case of a nine-year-old boy transferred to our pediatric intensive care unit for altered mental status. He had a 3-week history of odynophagia, cervical lymphadenopathy and fever. He had several visits to the pediatric emergency care where he was medicated with non-steroid anti-inflammatory drugs and antibiotic directed at an bacterial tonsillitis. Neck CT was performed at admittance. Despite broad spectrum antibiotic treatment, he progressed to coma and an MRI was performed. He had several lymphadenopathies, diffuse involvement of the supratentorial white matter and cortical and white matter punctiform areas of restricted diffusion. During the MRI examination there was a progressive lowering of the cerebellar tonsils, and the patient died the following morning, despite medical treatment. Postmortem histopathological evaluation was compatible with histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) with CNS involvement.
Conclusion: KFD is an extremely rare cause of adenopathy in children, which even less frequently presents with CNS involvement. This case is of interest for its rarity, but also imaging evolution.

Palavras Chave: Kikuchi-Fujimoto disease