An atypical neurologic complication of Kikuchi-Fujimoto Disease

Shabana, Mohamed; Warnack, Worthy

doi:10.1212/nxi.0000000000000707

Cited by 15 publications

(19 citation statements)

References 5 publications

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“…The clinical manifestations of KFD involving the CNS are complex and diverse, including meningitis, encephalitis, subdural effusion, optic neuritis, cerebellar ataxia, hemiplegia, and other signs (10,23,(34)(35)(36)(37)(38). KFD concomitant with neurological symptoms is rare and prone to missed diagnosis and misdiagnosis.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Histiocytic Necrotizing Lymphadenitis (Kikuchi–Fujimoto Disease) Concurrent With Aseptic Meningitis

et al. 2021

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Kikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign, self-limiting disease characterized by local lymphadenopathy. Central nervous system involvement in KFD is extremely rare and remains a diagnostic challenge. Only 41 cases of aseptic meningitis associated with KFD have been reported worldwide, with just four cases (including our case) of KFD with meningitis as the first symptom. We report a case of KFD accompanied by aseptic meningitis with severely high intracranial pressure (400 mmH2O), increased white blood cell count (56 × 106/L), and moderately elevated protein level (0.52 g/L). This case is unique in the delayed appearance of lymphadenopathy. After 1 month of treatment with steroids, fever, headache, and lymphadenopathy gradually disappeared, and the result of cerebrospinal fluid examination gradually became normal. In conclusion, based on our case findings and our literature review on KFD with aseptic meningitis, a diagnosis of KFD should be considered when delayed appearance of lymphadenopathy is observed in patients with aseptic meningitis.

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Section: Discussionmentioning

confidence: 99%

Case Report: Histiocytic Necrotizing Lymphadenitis (Kikuchi–Fujimoto Disease) Concurrent With Aseptic Meningitis

et al. 2021

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“…5,6 Symptoms of central nervous system involvement in KFD are rare and include encephalitis, meningitis, and cerebellar ataxia. 5,6,12 Individual cases of patients in whom KFD was manifested by optic neuritis were also reported in the literature. 9 In most cases, the course of the disease is mild.…”

Section: Symptomsmentioning

confidence: 97%

Kikuchi-Fujimoto disease: State of the art

2021

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Introduction: Kikuchi-Fujimoto disease (KFD), also called histiocytic necrotizing lymphadenitis, is a rare disease that occurs with swollen lymph nodes and associated fever. This disease occurs in both children and adults. Aim: The aim of our work was to review the literature and to remind family doctors, otolaryngologists, hematologists and rheumatologists about this rare disease that should be included in the differential diagnosis of long-term cervical lymphadenopathy. Material and methods: Current information on Kikuchi-Fujimoto disease was sought and presented based on literature that was published in reputable magazines in the period 2007–2020 in English. We searched for articles in the Medline, PubMed, and Scopus databases. Results and discussion: KFD occurs in both children and adults. This disease is found all over the world, most often in the Asian population. The etiology of Kikuchi-Fujimoto disease is not entirely known, however, two causal theories are suspected, which are discussed in detail in our article. The course of the disease is mild and usually disappears on its own. A biopsy of an involved lymph node presented as the standard for diagnosis. KFD treatment was causal – nonsteroidal anti-inflammatory drugs and/or glucocorticosteroids were used. Conclusions: There are few reports in the literature about Kikuchi-Fujimoto disease. KFD is associated with cervical lymphadenopathy and associated fever. If the diagnosis of the above-mentioned symptoms is not obvious, then in the differential diagnosis rarer diseases, such as KFD, should be included.

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“…The diagnosis was KFD, based on the pathognomonic features of paracortical foci composed of various types of histiocytes, including crescentic type, in the background of abundant apoptotic karyorrhectic debris. In a follow-up visit after six weeks, the patient had no significant systemic symptoms; the enlarged and swollen lymph nodes had significantly decreased in size, ESR and platelet count had become normal [ 16 ].…”

Section: Reviewmentioning

confidence: 99%

“…Low-grade fever and localized lymphadenopathy of KFD might be mistaken for tuberculosis [ 16 ]. Consequently, it serves as the most prevalent preliminary diagnosis for any long-term non-tender lymphadenopathy.…”

Section: Reviewmentioning

confidence: 99%

Kikuchi-Fujimoto Disease: A Rare Cause of Cervical Lymphadenopathy

Ahmed¹,

Quadir²,

Hakobyan³

et al. 2021

Cureus

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Kikuchi-Fujimoto disease (KFD) is a rare benign disease, clinically characterized by fever and tender cervical lymphadenopathy affecting the posterior cervical lymph nodes. This disease is usually accompanied by night sweats, rashes, and headaches. It generally affects young individuals, especially females, of Oriental-Asian origin. The etiology of KFD remains uncertain, but associations have been noted with viral diseases including Epstein-Barr virus (EBV), herpes simplex virus (HSV), and varicella-zoster virus (VZV), as well as autoimmune disorders including systemic lupus erythematosus (SLE) and Sjogren's syndrome. This review points out the etiology of KFD with cervical lymphadenopathy alongside its clinical presentation, histological highlights, lab investigations, complications, and treatment. Accurate diagnosis of this disease depends on lymph node excisional biopsy. Three histological patterns of KFD are recognized: proliferative, necrotizing, and xanthomatous. Distinction from lymphadenopathy-associated alternate disorders (e.g., SLE, malignancy, tuberculosis, or another infectious lymphadenitis) is essential to ensure appropriate therapy. This self-limited condition entails nonsteroidal anti-inflammatory drugs (NSAIDs) for pain relief with consideration of corticosteroids and hydroxychloroquine in severe cases.

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An atypical neurologic complication of Kikuchi-Fujimoto Disease

Cited by 15 publications

References 5 publications

Case Report: Histiocytic Necrotizing Lymphadenitis (Kikuchi–Fujimoto Disease) Concurrent With Aseptic Meningitis

Case Report: Histiocytic Necrotizing Lymphadenitis (Kikuchi–Fujimoto Disease) Concurrent With Aseptic Meningitis

Kikuchi-Fujimoto disease: State of the art

Kikuchi-Fujimoto Disease: A Rare Cause of Cervical Lymphadenopathy

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