Anti-aquaporin 4 antibody-positive acute disseminated encephalomyelitis.

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.

• Authors: Akihisa Okumura, Mika Nakazawa, Ayuko Igarashi, Shinpei Abe, Mitsuru Ikeno, Eri Nakahara, Yuichiro Yamashiro, Toshiaki Shimizu, Toshiyuki Takahashi
• Journal: Brain & development (Brain Dev) Vol. 37 Issue 3 Pg. 339-43 (Mar 2015) ISSN: 1872-7131 [Electronic] Netherlands
• PMID: 24837901 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Chemical References

• Anti-Inflammatory Agents
• Aquaporin 4
• Autoantibodies
• Prednisolone

Topics

• Anti-Inflammatory Agents (administration & dosage, pharmacology)
• Aquaporin 4 (immunology)
• Autoantibodies (blood)
• Child, Preschool
• Encephalomyelitis, Acute Disseminated (drug therapy, immunology)
• Female
• Humans
• Prednisolone (administration & dosage, pharmacology)