Involvement of central nervous system occasionally occurs as a form of aseptic meningitis in Kikuchi-Fujimoto disease (KFD). However, acute cerebellar symptoms are very rare in KFD. We describe a 42 year-old woman presenting kinetic tremor and gait ataxia preceding cervical lymphadenopathy. The diagnosis of KFD was made based on pathology. Lymphocyte-dominant pleocytosis was observed in cerebrospinal fluid. Brain and spinal magnetic resonance imaging showed no structural abnormalities. Acute cerebellar symptoms and cervical lymphadenopathy disappeared spontaneously within 2 months. This case of KFD involved unusual acute cerebellar symptoms. Selective involvement of the cerebellar system by viral or immunologic response may be attributed to acute cerebellar symptoms in KFD.
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