Anti-aquaporin 4 antibody-positive acute disseminated encephalomyelitis.
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.
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.
Akihisa
A
Okumura
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
Mika
M
Nakazawa
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
Ayuko
A
Igarashi
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
Shinpei
S
Abe
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
Mitsuru
M
Ikeno
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
Eri
E
Nakahara
Probiotics Research Laboratory, Juntendo University Graduate School of Medicine, Tokyo, Japan.
Yuichiro
Y
Yamashiro
Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
Toshiaki
T
Shimizu
Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Toshiyuki
T
Takahashi
D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000494:pharmacology; D051401:Aquaporin 4 / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D002675:Child, Preschool; D004673:Encephalomyelitis, Acute Disseminated / Q000188:drug therapy / Q000276:immunology*; D005260:Female; D006801:Humans; D011239:Prednisolone / Q000008:administration & dosage / Q000494:pharmacology
D000893:Anti-Inflammatory Agents; D051401:Aquaporin 4; D001323:Autoantibodies; D011239:Prednisolone
Acute disseminated encephalomyelitis; Anti-aquaporin 4 antibody; Optic neuritis; Steroids; Vaccination