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• Vol.53 No.2
• CLINICA NEUROL, 53: 119−124, 2013
• Vol.53 No.2 contents

Case Report

A case of chronic active Epstein-Barr virus infection associated with recurrent cerebellar ataxia and skin eruptions

Katsuya Araki, M.D.¹⁾, Tatsusada Okuno, M.D., Ph.D.¹⁾, Josephe Archie Honorat, M.D.¹⁾, Makoto Kinoshita, M.D., Ph.D.¹⁾, Masanori P. Takahashi, M.D., Ph.D.¹⁾, Masao Mizuki, M.D., Ph.D.²⁾, Kazuo Kitagawa, M.D., Ph.D.¹⁾ and Hideki Mochizuki, M.D.,Ph.D.¹⁾

¹⁾Department of Neurology, Osaka University Hospital
²⁾Department of Hematology and Oncology, Osaka University Hospital

A 62-year-old woman presented with subacute cerebellar ataxia, lymph node swelling and skin eruption. Laboratory tests revealed elevated titers of anti-VCA-IgG antibody and anti-EADR-IgG antibody, with Epstein-Barr virus (EBV) DNA detected from the blood and CSF by PCR. Since these data were highlighted with the diagnosis of chronic active EBV infection (CAEBV) and her ataxia improved concomitantly with the remission of other infectious mononucleosislike symptoms, we supposed her ataxia is associated with CAEBV. Five years later, at the age of 67, her ataxia relapsed concurrently with skin eruptions, whereas MRI demonstrated progression of cerebellar atrophy. After high-dose intravenous methylprednisolone treatment, the clinical symptoms resolved. Initial infection of EBV in childhood often causes autoimmune acute cerebellitis but cerebellar ataxia has rarely been described in CAEBV. Furthermore, immunohistochemical analysis revealed a reactivity of the patient's serum and CSF on rat cerebellum, suggesting an autoimmune pathomechanism for the ataxia.
Full Text of this Article in Japanese PDF (8922K)

(CLINICA NEUROL, 53: 119−124, 2013)
key words: Epstein-Barr virus (EBV), chronic active EBV infection (CAEBV), cerebellar ataxia

(Received: 26-Mar-12)