Akihisa Okumura1, Mika Nakazawa2, Ayuko Igarashi2, Shinpei Abe2, Mitsuru Ikeno2, Eri Nakahara2, Yuichiro Yamashiro3, Toshiaki Shimizu2, Toshiyuki Takahashi4. 1. Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan; Department of Pediatrics, Aichi Medical University, Nagakute, Aichi, Japan. Electronic address: okumura.akihisa.479@mail.aichi-med-u.ac.jp. 2. Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan. 3. Probiotics Research Laboratory, Juntendo University Graduate School of Medicine, Tokyo, Japan. 4. Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Abstract
OBJECTIVE: To describe the clinical and neuroimaging features of a young female patient with acute disseminated encephalomyelitis associated with anti-aquaporin-4 antibodies. METHODS: The patient had mild encephalopathy 14 days after influenza vaccination. Cerebrospinal fluid analysis revealed an increased cell count and a marked increase in myelin basic protein. Magnetic resonance imaging (MRI) demonstrated multiple lesions in the juxtacortical white matter. The patient was diagnosed with acute disseminated encephalomyelitis and treated with methylprednisolone pulse therapy. She recovered in 1 month. However, right retrobulbar optic neuritis appeared 2 months after discharge, and serum anti-aquaporin 4 antibodies were measured with a cell-based assay. RESULTS: Anti-aquaporin 4 antibodies were present in the patient's serum. She was treated with a prolonged course of oral prednisolone. The patient was negative for serum anti-aquaporin 4 antibodies 8 months after the second clinical event, and prednisolone was discontinued 13 months after the second clinical event. Serum anti-aquaporin 4 antibodies remained negative 4 months after the discontinuation of prednisolone. There was no evidence of relapse at 9 months after discontinuation of steroids. CONCLUSIONS: This case will expand the spectrum of anti-aquaporin-4 antibody-related central nervous system disorders. The measurement of anti-aquaporin 4 antibody may be considered in patients with a clinical diagnosis of acute disseminated encephalomyelitis and a second clinical event within a short interval.
OBJECTIVE: To describe the clinical and neuroimaging features of a young female patient with acute disseminated encephalomyelitis associated with anti-aquaporin-4 antibodies. METHODS: The patient had mild encephalopathy 14 days after influenza vaccination. Cerebrospinal fluid analysis revealed an increased cell count and a marked increase in myelin basic protein. Magnetic resonance imaging (MRI) demonstrated multiple lesions in the juxtacortical white matter. The patient was diagnosed with acute disseminated encephalomyelitis and treated with methylprednisolone pulse therapy. She recovered in 1 month. However, right retrobulbar optic neuritis appeared 2 months after discharge, and serum anti-aquaporin 4 antibodies were measured with a cell-based assay. RESULTS: Anti-aquaporin 4 antibodies were present in the patient's serum. She was treated with a prolonged course of oral prednisolone. The patient was negative for serum anti-aquaporin 4 antibodies 8 months after the second clinical event, and prednisolone was discontinued 13 months after the second clinical event. Serum anti-aquaporin 4 antibodies remained negative 4 months after the discontinuation of prednisolone. There was no evidence of relapse at 9 months after discontinuation of steroids. CONCLUSIONS: This case will expand the spectrum of anti-aquaporin-4 antibody-related central nervous system disorders. The measurement of anti-aquaporin 4 antibody may be considered in patients with a clinical diagnosis of acute disseminated encephalomyelitis and a second clinical event within a short interval.
Authors: Woojun Kim; Jee Eun Lee; Su Hyun Kim; So Young Huh; Jae Won Hyun; In Hye Jeong; Min Su Park; Joong Yang Cho; Sang Hyun Lee; Kwang Soo Lee; Ho Jin Kim Journal: J Clin Neurol Date: 2016-01-28 Impact factor: 3.077