Abstract Details

Title Kikuchi's Disease: An Unrecognized Cause of Aseptic Meningitis

Topic Neurotoxicology

Presentation(s) P06 - (-)

Poster/Presentation
Number 215

Objective

Background BACKGROUND: Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease is a benign self-limited clinicopathologic entity described in young, usually East Asian, adults and characterized by regional lymphadenopathy, fever, elevated sedimentation rate and liver enzymes, and the presence of proliferation of lymphoreticular cells and necrosis in lymph node biopsies. KD is often misdiagnosed as lymphoma or infectious diseases. CNS involvement is uncommon or not well recognized in KD and neurologists are not familiar with this disease.

Design/Methods DESIGN/METHODS: Case report and literature review.

Results RESULTS: A 43-year-old woman from a Caribbean Island with history of smoldering IgG lambda multiple myeloma developed severe headache, fever, seizures, lymphadenopathy, bilateral papilledema and depressed level of consciousness. Brain MRI showed FLAIR sulcal hyperintensity, leptomeningeal enhancement and bilateral subdural hygromas. Lumbar puncture revealed an opening pressure of 52 cmH2O and CSF pleocytosis (570 WBCs with 79% lymphocytes and CSF protein of 528 mg/dl) with no clonality. Biopsy of an enlarged cervical lymph node showed necrotizing histiocytic lymphadenitis; immunophenotyping showed no light chain restriction (excluding B-cell clonality) and no T-cell phenotypic aberrancy (reducing likelihood of T-cell malignancy). Multiple infectious disease studies of her CSF were negative. Serologic studies indicated presence of antibodies to ribonucleoprotein, SS-A and low c4 level but no clear clinical features of lupus or Sjogren's syndrome were present. Patient was treated with methylprednisolone 1g daily for 3 days and then prednisone 80mg tapering to 10mg over 2 months. Her symptoms and signs gradually improved over about two months and her MRI findings resolved.

Conclusions CONCLUSIONS: Kikuchi's disease should be considered in the differential diagnosis of adults presenting with lymphadenopathy and aseptic meningitis. The etiology and optimal management of the CNS manifestations of KD has yet to be identified.

Authors/Disclosures
Payam Soltanzadeh, MD, FAAN (UCLA Reed Neurological Research Center) PRESENTER	The institution of Dr. Soltanzadeh has received research support from Ra Pharma (UCB). The institution of Dr. Soltanzadeh has received research support from Avidity Biosciences.
	No disclosure on file
	No disclosure on file
Michael D. Geschwind, MD, PhD, FAAN (UCSF)	Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Brainstorm Cell Therapeutics, Inc.. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Walter Grubb. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gerson Lehrman Group. Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Reata Pharmaceuticals, Inc..
	No disclosure on file
	No disclosure on file
Carolyn Cronin, MD, PhD, FAAN (Vanderbilt University Medical Center)	Dr. Cronin has nothing to disclose.

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