Kikuchi-Fujimoto's disease presenting as acute cerebellar ataxia

Article type: Research Article

Authors: Yelsangikar, Kalpana Ramesh | Srikanteswara, Praveen-kumar

Affiliations: Department of Pediatrics, Bangalore Medical College and Research Institute, Bangalore, India | Department of Neurology, Bangalore Medical College and Research Institute, Bangalore, India

Note: [] Correspondence: Dr. Praveen-kumar Srikanteswara, Associate Professor of Neurology, Bowring and Lady Curzon Hospitals, Bangalore Medical College and Research Institute, Bangalore 560001, Karnataka, India. Tel.: +91 9448685155; E-mail: dmpraveen@gmail.com

Abstract: Kikuchi-Fujimoto disease (KFD) is a benign and self-limiting condition primarily affecting the cervical lymph nodes. Central nervous system involvement with acute cerebellar symptoms is a rare presentation in KFD. We report a 15-year-old girl presenting with intentional tremor, dysarthria and gait ataxia following episodes of recurrent fever and cervical lymphadenopathy. The diagnosis of KFD was made based on histopathology. Brain magnetic resonance imaging showed lesions in mid-pons and bilateral middle cerebellar peduncles. The patient's symptoms spontaneously resolved without any sequelae. A diagnosis of KFD should be considered when enlarged cervical lymphnodes are observed in patients with acute cerebellar symptoms. Selective inflammation of cerebellum and related structures by viral or immunological responses may be attributed to this rare presentation in KFD.

Keywords: Hemophagocytosis, cervical lymphadenopathy, cerebellar ataxia

DOI: 10.3233/JPN-140667

Journal: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 221-224, 2014