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• Vol.56 No.6
• CLINICA NEUROL, 56: 413−417, 2016
• Vol.56 No.6 contents

Case Report

A case considered gluten ataxia with anti-TG6 IgA antibodies

Kenji Sato, M.D.¹⁾³⁾, Makiko Kobayashi, M.D.²⁾, Yuki Ueta, M.D.³⁾, Nobuyuki Tanaka, M.D.³⁾ and Kazunori Nanri, M.D.³⁾

¹⁾Department of Neurology, Kawasaki Memorial Hospital
²⁾Department of Neurology, Tokyo Medical University
³⁾Department of Neurology, Tokyo Medical University Hachioji Medical Center

An 81-year-old woman presented with a chief complaint of gait disturbance. Brain magnetic resonance imaging (MRI) showed mild cerebellar atrophy and cerebral blood flow scintigraphy revealed reduced blood flow in the cerebellum. The patient was diagnosed with cortical cerebellar atrophy, and was given taltirelin hydrate, but symptoms slowly progressed. Thirteen years after onset, a positive result for anti-transglutaminase 6 (TG6) IgA antibodies was identified, and gluten ataxia was diagnosed. Despite steroid therapy and gluten-free diet therapy, no improvements were seen, and independent walking became difficult for the patient. High-dose intravenous immunoglobulin therapy resulted in improvements in the Posture and Gait subscore of the International Cooperative Ataxia Rating Scale (ICARS) from 15 to 11 points, and the patient regained the ability to walk independently. Gluten ataxia are rarely reported in Japan and anti-TG6 antibodies were considered useful for its diagnosis.
Full Text of this Article in Japanese PDF (490K)

(CLINICA NEUROL, 56: 413−417, 2016)
key words: gluten ataxia, anti-TG6 antibodies, cerebellar ataxia, immunoglobulin therapy

(Received: 27-Sep-15)