Kikuchi–Fujimoto disease associated with the central nervous system in children

Abstract

The clinical data of patients with Kikuchi–Fujimoto disease (KFD) combined with the involvement of the central system is limited, particularly for children. This study aimed to investigate the clinical features and prognosis of KFD associated with the central nervous system in children. We collected the clinical data of patients diagnosed with KFD combined with the involvement of the central system, hospitalized at Children's Hospital of Chongqing Medical University (CHCMU). Furthermore, we summarized the clinical features and outcomes in children by reviewing the published literature. Together with the two patients from CHMCU enrolled in this study, we identified 19 children diagnosed with KFD combined with the involvement of the central nervous system, including eight males and 11 females. The onset age ranges from 6 to 18 years (the median age was 14 and the interquartile range was 9–16). Sixteen patients were diagnosed with aseptic meningitis. Headache (78.9%, 15/19) was the most common symptom. Cerebrospinal fluid pressure was increased in 3 patients (60.0%, 3/5), cell count was elevated in 14 patients (73.7%, 14/19), glucose was decreased in 4 patients (21.0%, 4/19), and protein was elevated in 12 patients (63.1%, 12/19). A total of 9 patients had cranial imaging changes. Electroencephalogram (EEG) was performed on seven patients and 4 patients presented abnormal EEG (57.1%, 4/7). A total of 12 patients received steroid therapy; two were combined with antiepileptic treatment. In children, KFD complicated with the involvement of the central nervous system was rare and aseptic meningitis was the primary manifestation. Most patients had a good prognosis and a few had residual sequelae of neurological damage.

A Rare Neurological Presentation of Kikuchi-Fujimoto Disease

“…Others report leptomeningeal, brainstem, cerebellar, occipital, or temporal lobe changes. Cerebrospinal fluid analysis may be normal or may show pleocytosis and elevated protein . Lack of familiarity with this rare condition can result in diagnostic delays and inappropriate treatment.…”