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January 13, 2015; 84 (2) ARTICLE


Diagnostic utility of aquaporin-4 in the



analysis of active demyelinating lesions
Bogdan F.G. Popescu, Yong Guo, Mark E. Jentoft, Joseph E. Parisi, Vanda A. Lennon, Sean J. Pittock,
Brian G. Weinshenker, Dean M. Wingerchuk, Caterina Giannini, Imke Metz, Wolfgang Brück,
Elizabeth A. Shuster, Jonathan Carter, Clara D. Boyd, Stacey Lynn Clardy, Bruce A. Cohen,
Claudia F. Lucchinetti

First published December 10, 2014, DOI: https://doi.org/10.1212/WNL.0000000000001126

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 Article

 Abstract
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This article requires a subscription to view the full text. If  GLOSSARY
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 RESULTS
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purchased.
 DISCUSSION


 AUTHOR
Abstract 
CONTRIBUTIONS
 STUDY

Objective: To assess, in a surgical biopsy cohort of
FUNDING
active demyelinating lesions, the diagnostic utility of
 DISCLOSURE

aquaporin-4 (AQP4) immunohistochemistry in


identifying neuromyelitis optica (NMO) or NMO ACKNOWLEDGMENT
spectrum disorder (NMOSD) and describe pathologic  Footnotes


features that should prompt AQP4  REFERENCES



immunohistochemical analysis and AQP4– 

 Figures & Data

immunoglobulin G (IgG) serologic testing. 

 Info &
Disclosures

Methods: This was a neuropathologic cohort study of

20 surgical biopsies (19 patients; 11 cord/9 brain),
performed because of diagnostic uncertainty,
interpreted as active demyelinating disease and ASSOCIATION OF CSF
containing 2 or more of the following additional GLUCOCEREBROSIDASE
ACTIVITY WITH THE RISK
features: tissue vacuolation, granulocytic infiltrates, or
OF INCIDENT DEMENTIA
astrocyte injury.
IN PATIENTS WITH
PARKINSON DISEASE
Results: AQP4 immunoreactivity was lost in 18 Dr. Joseph
biopsies and increased in 2. Immunopathologic features Friedman and
Dr. Avner
of the AQP4 loss cohort were myelin vacuolation (18),
Thaler
dystrophic astrocytes and granulocytes (17), vascular
hyalinization (16), macrophages containing glial ▶ WATCH

fibrillary acid protein (GFAP)–positive debris (14), and

Creutzfeldt-Peters cells (0). All 14 cases with available
serum tested positive for AQP4-IgG after biopsy.
RELATED
Diagnosis at last follow-up was NMO/NMOSD (15) ARTICLES
and longitudinally extensive transverse myelitis (1 each
AQP4 in
relapsing and single). Immunopathologic features of the biopsied
AQP4 increased cohort were macrophages containing demyelinating
lesions as a
GFAP-positive debris and granulocytes (2), myelin diagnostic clue
to NMOSD and
vacuolation (1), dystrophic astrocytes (1), Creutzfeldt- MS
Peters cells (1), and vascular hyalinization (1). Diagnosis Final answer?

at last follow-up was multiple sclerosis (MS) and both

tested AQP4-IgG seronegative after biopsy. TOPICS
DISCUSSED
Conclusions: AQP4 immunohistochemistry with
subsequent AQP4-IgG testing has diagnostic utility in All
Demyelinating
identifying cases of NMO/NMOSD. This study
disease (CNS)
highlights the importance of considering NMOSD in
Devic's
the di!erential diagnosis of tumefactive brain or spinal
syndrome
cord lesions. AQP4-IgG testing may avert biopsy and
avoid ine!ective therapies if these patients are
erroneously treated for MS. ALERT ME

GLOSSARY Alert me when

eletters are
AQP4 = aquaporin-4; GFAP = glial fibrillary acid published

protein; IgG = immunoglobulin G;

LETM = longitudinally extensive transverse myelitis;
LFB = Luxol fast blue; MAG = myelin-associated
glycoprotein; MOG = myelin oligodendrocyte RECOMMENDED
glycoprotein; MS = multiple sclerosis; ARTICLES
NMO = neuromyelitis optica;
ARTICLES
NMOSD = neuromyelitis optica spectrum disorders;
Neuromyelitis
ON = optic neuritis; PLP = proteolipid protein; optica unique
PPWM = periplaque white matter; TM = transverse area postrema
lesions
myelitis Nausea,
vomiting, and
Footnotes pathogenic
implications
B.F.Gh. Popescu,
V.A. Lennon, J.E.
Go to Neurology.org for full disclosures. Funding
Parisi et al.
information and disclosures deemed relevant by the Neurology, March
authors, if any, are provided at the end of the article. 02, 2011

ARTICLES
Pathologic and
Editorial, page 110
immunologic
profiles of a
Received April 10, 2014. limited form of
neuromyelitis
Accepted in final form August 1, 2014. optica with
myelitis
K. Yanagawa, I.
© 2014 American Academy of Neurology
Kawachi, Y.
Toyoshima et al.
View Full Text Neurology,
November 16,
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Justine J. Wang,
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EDITORIAL
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MS
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Letters: Rapid online correspondence

Biopsy for NMOSD ?
Jagannadha Avasarala, Consultant Neurologist and
Neuroimmunologist, Greenville Health System
Greenville, SC
Submitted March 02, 2015

 COMMENT
YOU MAY ABSTRACTS
ALSO BE Development of New or Enlarging MRI
INTERESTED
Lesions Outside of Clinical Attacks in
IN MOG-Antibody-Associated Disease
Stephanie Syc-Mazurek, John Chen, Padraig
Morris, et al.
December 05, 2022

CLINICAL/SCIENTIFIC NOTE
Frequency of New or Enlarging Lesions
on MRI Outside of Clinical Attacks in
Patients With MOG-Antibody–
Associated Disease
Stephanie B. Syc-Mazurek, John J. Chen,
Pearse Morris, et al.
September 29, 2022

RESEARCH ARTICLE
Comparison of MRI Lesion Evolution
in Di!erent Central Nervous System
Demyelinating Disorders
Elia Sechi, Karl N. Krecke, Steven A. Messina,
et al.
July 14, 2021

ARTICLE
Diagnostic algorithm for relapsing
acquired demyelinating syndromes in
children
Yael Hacohen, Kshitij Mankad, W.K. Chong, et
al.
June 14, 2017

ARTICLE
Incidence of interattack asymptomatic
brain lesions in NMO spectrum
disorder
Min Young Lee, Kok Pin Yong, Jae-Won Hyun,
et al.
September 14, 2020

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