Unique presentation of recurrent subdural effusions and subsequent abdominal lymphadenopathy in a patient diagnosed with Kikuchi-Fujimoto disease

Abstract

Kikuchi Fujimoto Disease, originally discovered in 1972, is a rare lymphoproliferative disorder traditionally characterized by cervical lymphadenopathy, fevers, parotid gland enlargement, and several other nonspecific manifestations. Differentials include lymphoma, other viral diseases such as Epstein-Bar Virus, as well as other autoimmune conditions such as Systemic Lupus Erythematosus. Central nervous system involvement is exceptionally rare, with manifestations including meningitis as well as subdural effusions, as presented in this case. This review will summarize a case of a 24-year-old man with recurrent subdural effusions requiring intervention, subsequent relapse with abdominal lymphadenopathy, and possible IgG4 related disease. The background epidemiology, radiology, and potential pathophysiology will be reviewed.

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

“…It is older than the mean ages in KFD pediatric studies and has ranged from 11 to 13.2 years old 19,20 . Meanwhile, in adult patients, the age range was 19–57 with a median age of 28 years old, and most of the onset age was less than 30 years (70%) 13–18 …”

“…KFD with neurological symptoms is rare. There have been 33 reported cases of KFD complicated with central nervous system injury in adults 13–18 . Only a few cases of KFD with central nervous system damage have been reported in children.…”

“…There have been 33 reported cases of KFD complicated with central nervous system injury in adults. [13][14][15][16][17][18] Only a few cases of KFD with central nervous system damage have been reported in children. Studies on adult KFD patients with central nervous system damage have suggested that males may have a gender advantage.…”

“…19,20 Meanwhile, in adult patients, the age range was 19-57 with a median age of 28 years old, and most of the onset age was less than 30 years (70%). [13][14][15][16][17][18] The clinical manifestations of KFD involving the central nervous system are complex and varied. Aseptic meningitis is KFD's most common central nervous system complication, 21 similar to adult patients.…”

Kikuchi-Fujimoto disease: A comprehensive review

“…Involvement of the central nervous system presenting as aseptic meningitis, meningoencephalitis, acute cerebellar symptoms with tremors and ataxia, and optic neuritis has been reported frequently[ 92 , 97 - 102 ]. Aseptic meningitis mostly at the time of the lymphadenopathy is the commonest neurological complication seen in some of these cases and may be associated with very high levels of intracranial pressure, low cerebrospinal fluid (CSF)-serum glucose, and recurrent subdural effusions requiring intervention[ 103 , 104 ]. While recurrent meningitis occurs more often in males, encephalopathy in children may occur after 10 d to 3 mo which is characterized by very high CSF protein levels and extensive magnetic resonance imaging changes requiring early treatment.…”