Brainstem Encephalitis as an Atypical Neurologic Complication Following Kikuchi-Fujimoto Disease

Area of Science:

• Neurology
• Immunology
• Infectious Diseases

Background:

• Kikuchi-Fujimoto disease (KFD) is a rare, benign, lymphohistiocytic disorder.
• Neurological manifestations of KFD are uncommon, but can include aseptic meningitis and encephalitis.

Observation:

• A patient presented with oscillopsia and intractable hiccups, initially suspected as brainstem stroke.
• Brain MRI revealed a T2-hyperintense lesion in the medulla oblongata's area postrema extending to the cervical cord.
• Extensive workup for infectious and demyelinating central nervous system disorders was negative.

Findings:

• The patient developed brainstem encephalitis 3-4 months post-KFD recovery.
• The encephalitis resolved spontaneously and rapidly prior to immunosuppressive therapy.
• Literature review identified similar rare cases of KFD-associated encephalitis.

Implications:

• This case suggests a potential delayed neurological complication of Kikuchi-Fujimoto disease.
• The spontaneous recovery observed may offer insights into the pathophysiology and management of KFD-related encephalitis.
• Further research is warranted to understand the link between KFD and central nervous system inflammation.