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Table of Contents    
Year : 2019  |  Volume : 67  |  Issue : 4  |  Page : 1131-1133

Kikuchi Disease: A Rare Cause of Aseptic Meningitis

1 Department of Neurosciences, Sir H.N. Reliance Foundation Hospital, Mumbai, Maharashtra, India
2 Department of Internal Medicine, Sir H.N. Reliance Foundation Hospital, Mumbai, Maharashtra, India
3 Department of Laboratory Medicine, Sir H.N. Reliance Foundation Hospital, Mumbai, Maharashtra, India

Date of Web Publication10-Sep-2019

Correspondence Address:
Dr. Dhiren R Patel
Department of Neurosciences, Sir H.N. Reliance Foundation Hospital, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.266242

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How to cite this article:
Patel DR, Shah AB, Shah HR, Thorat KB. Kikuchi Disease: A Rare Cause of Aseptic Meningitis. Neurol India 2019;67:1131-3

How to cite this URL:
Patel DR, Shah AB, Shah HR, Thorat KB. Kikuchi Disease: A Rare Cause of Aseptic Meningitis. Neurol India [serial online] 2019 [cited 2021 Jan 20];67:1131-3. Available from:


Kikuchi Fujimoto disease (KFD) is a rare entity usually manifesting as a febrile illness with cervical lymphadenopathy in young women. We report a case of KFD in a middle-aged lady who presented atypically with features suggestive of meningitis at the onset and was found to have mediastinal and abdominal lymphadenopathy on further workup.

A 57-year-old otherwise healthy female presented with febrile illness of 1-week duration associated with headache and vomiting with abdominal discomfort. On examination, she was febrile but hemodynamically stable. Systemic examination was unremarkable with no signs of meningeal irritation. Routine hematological and biochemical workup was normal except for a low platelet count – 68000/mm 3, raised erythrocyte sedimentation rate (ESR) – 43 mm/h, and C-reactive protein – 3.59 mg/dL. Fever workup for establishing etiologies such as dengue, malaria, and typhoid was normal. Blood and urine cultures were sterile. Considering her symptoms, possibility of meningoencephalitis was considered, and hence, brain imaging followed by cerebrospinal fluid (CSF) examination was performed. Magnetic resonance imaging (MRI) of the brain showed extensive supratentorial leptomeningeal enhancement without any basal exudates [Figure 1]. CSF analysis revealed increased cell count – 30/mm 3 with predominant lymphocytes (100%). CSF proteins were raised – 80.47 mg/dL with normal CSF glucose – 66 mg/dL (serum 121 mg/dL) and normal ADA levels. CSF culture was sterile and showed no malignant cells or Cryptococcus. Polymerase chain reaction (PCR) for tuberculosis and herpes simplex virus (HSV) was negative.
Figure 1: MRI Brain T2 FLAIR Images: extensive supratentorial leptomeningeal enhancement along the pial surfaces of both cerebral hemispheres including both lateral convexities, right sylvian fissure, and medial parasagittal aspect of the right hemisphere

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She was managed with broad-spectrum intravenous antibiotics (cephalosporins) and intravenous steroids for symptomatic relief. However, vomiting and abdominal discomfort persisted prompting abdominal imaging. Ultrasonography was unremarkable, hence computed tomography (CT) of the abdomen with contrast was performed. It showed an enhancing non-necrotic lesion measuring approximately 2 cm × 1.5 cm, located in ileocolic mesenteric fold representing an enlarged lymph node [Figure 2]. This warranted further analysis of the lesion. Whole body positron emission tomography (WB-PET) contrast-enhanced CT was done, which confirmed metabolically active non-necrotic mediastinal, bilateral hilar, and right ileocolic adenopathy [Figure 3]. Etiological workups for lymphadenopathy [Epstein Barr virus (EBV), varicella zoster virus (VZV), cytomegalovirus (CMV), Toxoplasma gondii, HIV, hepatitis B and C, herpes simplex virus (HSV) 1 and 2] were negative. Hence, CT-guided biopsy of the right ileocolic node was performed, assessment which showed discrete foci of necrosis containing abundant karyorrhetic debris, along with few crescentic and epithelioid histiocytes and thick-walled blood vessels without any neutrophilic infiltration or granuloma or lymphoma cells [Figure 4]. These histological findings were consistent with necrotizing type of KFD.
Figure 2: CT abdomen with contrast showing an enhanced enlarged non-necrotic ileocolic lymph node measuring approximately 2 × 1.5 cm

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Figure 3: FDG PET-CT showing metabolically active non-necrotic mediastinal, bilateral hilar, and right ileocolic adenopathy

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Figure 4: Right ileocolic lymph node core biopsy: lymph node parenchyma with thick capsule and discrete foci of necrosis containing abundant karyorrhectic nuclear debris, few crescentic, and epithelioid histiocytes and thick-walled blood vessels. No neutrophilic infiltration or granuloma was seen. Lymphoma cells were not detected

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Patient's condition improved with steroids in due course and she was later discharged on a tapering course of oral steroids. Follow-up MRI brain done 10 days later showed complete resolution of the leptomeningeal enhancement.

KFD is a rare but well-known benign entity first described in Japan by Fujimoto and Kikuchi in 1972.[1],[2] It has been reported in most parts of the world with most cases reported in Japanese and Asian populations.[2],[3],[4] It typically affects younger population under 40 years of age but has been reported in patients between 6 and 80 years old.[1],[4] Etiology of KFD is unknown with many investigators suggesting it to be infective, autoimmune, or genetic.[1],[2],[5],[6] It commonly presents with fever and cervical lymphadenopathy, but uncommon presentations such as nausea, vomiting, night sweats, rash, arthritis, noncervical regional lymphadenopathy, and hepatosplenomegaly can also typically occur.[1],[2],[4] Central nervous system (CNS) involvement is rare but few cases have been reported with aseptic meningitis, acute cerebellar ataxia, brachial neuritis, brainstem encephalitis,[2] neuromyelitis optica spectrum disorder,[1] and peripheral neuropathy. Some uncommon lab findings seen in KFD are thrombocytopenia, pancytopenia, and ESR.[3],[4]

The diagnosis of KFD is confirmed after histopathological examination of a lymph node biopsy sample, which shows patchy or confluent paracortical foci of non-neutrophilic necrosis with a histiocytic cellular infiltrate along with phagocytic debris and crescent nuclei.[2],[3],[4] Diagnosis can be confused with close mimics [Table 1]. The treatment of KFD is steroids with symptomatic treatment. Other treatments that have been tried are immunoglobulins,[2] hydroxychloroquine,[7] minocycline, and other antibiotics.[2]
Table 1: Differential diagnosis

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Jasti D, Prasad SV, Naveen T, Vengamma B. Kikuchi-Fujimoto disease presenting as brainstem encephalitis with secondary blepharospasm. J Neurosci Rural Pract 2016;7:157-60.  Back to cited text no. 1
[PUBMED]  [Full text]  
Lin DY, Villegas MS, Tan PL, Wang S, Shek LP. Severe Kikuchi's disease responsive to immune modulation. Singapore Med J 2010;51:e18-e21.  Back to cited text no. 2
Ranabhat S, Tiwari M, Kshetri J, Maharjan S, Osti B. An uncommon presentation of Kikuchi Fujimoto disease: A case report with literature review. BMC Res Notes 2015;8:478.  Back to cited text no. 3
Fitzsimmons P, Akpan A, Michael B. Kikuchi–Fujimoto disease as a rare cause of fever of unknown origin in a septuagenarian. Age Ageing 2008;37:233-4.  Back to cited text no. 4
Kaku M, Shin S, Goldstein M, Pleet J, Fabian M. Neuromyelitis optica spectrum disorder in a patient with Kikuchi-Fujimoto disease. Neurol Neuroimmunol Neuroinflamm 2016;3:e221.  Back to cited text no. 5
Amir ARA, Amr SS, Sheikh SS. Kikuchi–Fujimoto's disease: Report of familial occurrence in two human leucocyte antigen-identical non-twin sisters. J Intern Med 2002;252:79-83.  Back to cited text no. 6
Rezai K, Kuchipudi S, Chundi V, Ariga R, Loew J, Sha B. Kikuchi-Fujimoto Disease: Hydroxychloroquine as a Treatment. Clin Infect Dis 2004;39:e124-6.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]


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