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Kikuchi-Fujimoto disease presenting as brainstem encephalitis with secondary blepharospasm.

ABSTRACT: Central nervous system involvement in Kikuchi-Fujimoto disease is a very rare clinical manifestation. We report a 15-year-old girl who presented to us with fever, drowsiness, neck swellings, and involuntary closure of both eyelids of 2 days duration. Magnetic resonance imaging brain showed T2-weighted and fluid-attenuated inversion recovery hyperintensities in dorsal midbrain and pons. Cervical lymph node fine-needle aspiration cytology was suggestive of Kikuchi-Fujimoto disease. Blepharospasm secondary to infectious etiology is rare. Positron emission computed tomography brain showed increased focal uptake in anterior cingulate gyrus which can be the site of origin of blepharospasm. The patient was managed with steroids and trihexyphenidyl with significant recovery. Kikuchi-Fujimoto disease is a rare disease which has to be considered as one of the differential diagnosis in a case of acute encephalopathy with cervical lymphadenopathy.

SUBMITTER: Jasti DB

PROVIDER: S-EPMC4750320 | biostudies-literature | 2016 Jan-Mar

REPOSITORIES: biostudies-literature

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Kikuchi-Fujimoto disease presenting as brainstem encephalitis with secondary blepharospasm.

Jasti Dushyanth Babu DB Naveen Prasad S V SV Naveen Thota T Vengamma Bhuma B

Journal of neurosciences in rural practice 20160101 1

Central nervous system involvement in Kikuchi-Fujimoto disease is a very rare clinical manifestation. We report a 15-year-old girl who presented to us with fever, drowsiness, neck swellings, and involuntary closure of both eyelids of 2 days duration. Magnetic resonance imaging brain showed T2-weighted and fluid-attenuated inversion recovery hyperintensities in dorsal midbrain and pons. Cervical lymph node fine-needle aspiration cytology was suggestive of Kikuchi-Fujimoto disease. Blepharospasm s ...[more]

PMID: 26933369

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Kikuchi-Fujimoto disease associated with systemic lupus erythematosus.

Project description:A cervical lymph node biopsy from a 38-year-old woman initially revealed necrotising lymphadenitis. Her case is presented herein. An exhaustive examination that included renal biopsy did not suggest systemic lupus erythematosus (SLE). She was diagnosed with Kikuchi-Fujimoto Disease (KFD) and was treated with prednisone. One year later, a renal biopsy performed for renal failure revealed Class IV SLE. It was proposed that lymphadenitis in this KFD patient should be considered as SLE so that the SLE would be properly treated. In our patient, this hypothesis was partially correct, because even though SLE could not be verified at initial presentation, it evolved into full SLE after a year interval.

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Splenic uptake on FDG PET/CT correlates with Kikuchi-Fujimoto disease severity.

Project description:Kikuchi-Fujimoto disease (KFD) is usually self-limiting, but prolonged systemic symptoms often result in frequent hospital visits, long admission durations, or missed workdays. We investigated the role of fluorine-18 fluoro-2-deoxy-D-glucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) in assessing KFD severity. We reviewed the records of 31 adult patients with pathologically confirmed KFD who underwent 18F-FDG PET/CT between November 2007 and April 2018 at a tertiary-care referral hospital. Disease severity was assessed using criteria based on clinical manifestations of advanced KFD. Systemic activated lymph nodes and severity of splenic activation were determined using semi-quantitative and volumetric PET/CT parameters. The median of the mean splenic standardized uptake value (SUVmean) was higher in patients with severe KFD than those with mild KFD (2.38 ± 1.18 vs. 1.79 ± 0.99, p = 0.058). Patients with severe KFD had more systemically activated volume and glycolytic activity than those with mild KFD (total lesion glycolysis: 473.5 ± 504.4 vs. 201.6 ± 363.5, p = 0.024). Multivariate logistic regression showed that myalgia (odds ratio [OR] 0.035; 95% confidence interval [CI] 0.001-0.792; p = 0.035), total lymph node SUVmax (cutoff 9.27; OR 24.734; 95% CI 1.323-462.407; p = 0.032), and spleen SUVmean (cutoff 1.79; OR 37.770; 95% CI 1.769-806.583; p = 0.020) were significantly associated with severe KFD. 18F-FDG PET/CT could be useful in assessing KFD severity.

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Sub internal limiting membrane hemorrhage followed by bilateral optic disc hemorrhage in Kikuchi-Fujimoto disease: a case report.

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Kikuchi-Fujimoto disease in a tertiary care teaching hospital in Coastal South India: A 8-year retrospective study.

Project description:Background: Kikuchi-Fujimoto disease (KFD) is a rare, benign condition of unknown etiology, presenting as cervical lymphadenitis. Lymphadenopathy is usually tender and maybe associated with systemic symptoms. Despite the extensive literature on this disease, it continues to be misdiagnosed owing to its misleading clinical presentation.MethodsA retrospective hospital-based descriptive cross-sectional study was conducted in tertiary care hospitals from 2011 to 2019. All patients with confirmed KFD diagnosis were included and after ethics committee approval the clinical details and histopathological data was retrieved from the medical records department and analyzed.ResultsA total of 67 cases were included. The mean age was 26.9±11.3 years with a female: male ratio of 1.9:1. There were 50 patients with tender cervical lymphadenopathy which was the most common clinical presentation. The mean length and width of palpable lymph nodes were 2.3±1.0 cm and 2.2±0.7 cm respectively. Histology revealed proliferative stage in majority of patients ( n=40, 59.7%). Lymphadenopathy resolved in 83.6% within 2 months. There were 42 patients who had complete recovery with symptomatic treatment within a period of 9 months.ConclusionsKFD is prevalent in young, female patients of Asian descent and often presents as tender cervical lymphadenopathy. Early diagnosis with excisional lymph node biopsy is crucial to avoid unnecessary investigations and treatment. Treatment is symptomatic unless complicated, where steroid therapy is considered. KFD has an excellent prognosis with almost no risk of fatality.

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Positron emission tomography/computed tomography hypermetabolism of Kikuchi-Fujimoto disease mimicking malignant lymphoma: a case report and literature review.

Project description:Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a benign, self-limiting inflammatory disorder of unknown etiology and pathogenesis. This report presents a rare case involving a man with 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) hypermetabolism caused by KFD mimicking malignant lymphoma. The PET/CT maximum intensity projection showed multiple hypermetabolic lymphadenopathies and homogeneous FDG uptake in the bone marrow and spleen. Malignant lymphoma was initially suspected. The patient then underwent excision biopsy of one enlarged right cervical lymph node that was selected because it showed the highest FDG uptake in PET/CT, and examination of this biopsy specimen confirmed the diagnosis of KFD. PET/CT is useful for assessing the general condition of patients and can help to select lymph nodes for excision biopsy based on the highest FDG uptake. However, KFD can predispose to localized FDG uptake and limit the specificity of PET/CT by mimicking malignancy. Thus, positive results of PET/CT should be interpreted with caution.

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TB or not to be? Kikuchi-Fujimoto disease: a rare but important differential for TB.

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