Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis has a recurrence rate of around 3-7% in reports from most centers but can range as high as 20% in predisposed East-Asian populations [1]. Its pathogenesis still remains controversial [2]. Neurological manifestations of Kikuchi disease are extremely rare and isolated, and include headache, peripheral neuropathy, aseptic meningitis to encephalitis [3,4].

During her current admission, her blood investigations revealed mildly elevated inflammatory markers with raised C-reactive protein (CRP) of 44 mg/L and erythrocyte sedimentation rate (ESR) of 59 mm/h. She was treated initially for infective cervical lymphadenitis with intravenous amoxicillin and clavalanate. Alternative differentials were considered as her fever persistent despite adequate antibiotic coverage. During the fourth day of hospitalization, she was noted to be acutely confused. In view of fluctuating consciousness, she was transferred to intensive care unit (ICU) for further management and subsequently intubated in view of encephalopathy. Antibiotic therapy was escalated to intravenous Ciprofloxacin with addition of Acyclovir. Neuro-imaging did not reveal any abnormalities. Spinal tap was done with opening pressure at 52 cm H2O. Cerebrospinal fluid (CSF) was noted to be turbid with fluid analysis showing significant pleocytosis and raised protein (CSF WBC 60/µL, total protein 0.9 g/L). In view of concerns of possible Kikuchi-related encephalitis, pulse methylprednisolone for 5 days with subsequent tapering doses of steroids were administered. During her stay in ICU, she was noted to be increasingly agitated with sleep-cycle disturbances. An electroencephalogram (EEG) did not reveal any epileptiform activity or electrographic seizure. CSF and blood investigations were not suggestive of N-Methyl-D-Aspartic acid (NMDA) encephalitis. An extended autoimmune screen was negative for any concerns of underlying autoimmune encephalitis. Infective screening was negative for Bartonella, Ebstein-Barr virus, Myco-plasma, Tuber-culosis, HIV, Lepto-spirosis, Meliodosis, Toxoplasmosis and Rickettsia. She underwent lymph node excision biopsy, which showed necrotizing histiocytic lymphadenitis. She was diagnosed to be having a recurrence of KFD with encephalitis. On recovery, she had mild in-coordination with aphasia. There were other neuropsychiatric symptoms of attention-deficit, increased lability and sleep disturbances during her recovery. Her cognition was appropriate, and she was able to return to school within 6 weeks post discharge.

5. Pepe F, Disma S, Teodoro C, Pepe P, Magro G. Kikuchi-Fujimoto disease: A clinicopathologic update. Pathologica. 2016;108:120-9.