Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations

Association with neuromyelitis optica

J. H. Min, H. J. Kim, Byung Jo Kim, K. W. Lee, I. N. Sunwoo, S. M. Kim, B. J. Kim, S. H. Kim, M. S. Park, P. Waters, A. Vincent, J. J. Sung, K. H. Lee

Research output: Contribution to journalArticle

40 Citations (Scopus)

Abstract

Background and objectives: Optic neuritis or longitudinally extensive myelitis in Sjogren syndrome (SS) suggests a neuromyelitis optica spectrum disorder (NMOSD). However, brain abnormalities of SS remain to be elucidated for the association with neuromyelitis optica (NMO). Methods: Twelve primary SS patients (all women, 42 ± 13.2 years) who had recurrent central nervous system (CNS) manifestations with brain involvement were retrospectively identified. Brain MRI, and neurologic and serologic findings were analyzed with the measurement of anti-aquaporin-4 antibody (AQP4-Ab). Results: All patients showed brain lesions characteristic of NMO as follows: 1) the involved sites adjacent to the third and fourth ventricles and in the posterior limb of the internal capsule, 2) unique configurations, such as the longitudinal course from the internal capsule to the midbrain, large cerebral or cerebellar lesions over 3 cm, and cavity-like formations. AQP4-Ab was positive in six of eight patients tested, and all the seropositive patients showed lesions with increased diffusion, suggestive of vasogenic edema. Four patients met the revised criteria of NMO, and nine had features of NMOSDs. Of the remaining three patients showing only brain involvement, one had AQP4-Ab. Conclusions: This study demonstrates that SS patients with recurrent CNS involvement have brain abnormalities characteristic of NMO and AQP4-Ab in Korea. The presence of AQP4-Ab in one SS patient with only brain involvement may suggest that the coexistence of NMO should be explored in SS patients with recurrent CNS manifestations, even without optic neuritis or myelitis.

Original languageEnglish
Pages (from-to)1069-1076
Number of pages8
JournalMultiple Sclerosis
Volume15
Issue number9
DOIs
Publication statusPublished - 2009 Aug 28

Fingerprint

Neuromyelitis Optica
Sjogren's Syndrome
Central Nervous System
Brain
Myelitis
Internal Capsule
Optic Neuritis
Aquaporin 4
Fourth Ventricle
Third Ventricle
Korea
Mesencephalon
Neurologic Manifestations
Edema
Extremities

Keywords

  • Aquaporin 4
  • Beuromyelitis optica
  • Brain MRI
  • Multiple sclerosis
  • Sjogren's syndrome
  • Vasogenic brain edema

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations : Association with neuromyelitis optica. / Min, J. H.; Kim, H. J.; Kim, Byung Jo; Lee, K. W.; Sunwoo, I. N.; Kim, S. M.; Kim, B. J.; Kim, S. H.; Park, M. S.; Waters, P.; Vincent, A.; Sung, J. J.; Lee, K. H.

In: Multiple Sclerosis, Vol. 15, No. 9, 28.08.2009, p. 1069-1076.

Research output: Contribution to journalArticle

Min, JH, Kim, HJ, Kim, BJ, Lee, KW, Sunwoo, IN, Kim, SM, Kim, BJ, Kim, SH, Park, MS, Waters, P, Vincent, A, Sung, JJ & Lee, KH 2009, 'Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations: Association with neuromyelitis optica', Multiple Sclerosis, vol. 15, no. 9, pp. 1069-1076. https://doi.org/10.1177/1352458509106228
Min, J. H. ; Kim, H. J. ; Kim, Byung Jo ; Lee, K. W. ; Sunwoo, I. N. ; Kim, S. M. ; Kim, B. J. ; Kim, S. H. ; Park, M. S. ; Waters, P. ; Vincent, A. ; Sung, J. J. ; Lee, K. H. / Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations : Association with neuromyelitis optica. In: Multiple Sclerosis. 2009 ; Vol. 15, No. 9. pp. 1069-1076.
@article{215df7ec6b3342dfb7639576767f1afd,
title = "Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations: Association with neuromyelitis optica",
abstract = "Background and objectives: Optic neuritis or longitudinally extensive myelitis in Sjogren syndrome (SS) suggests a neuromyelitis optica spectrum disorder (NMOSD). However, brain abnormalities of SS remain to be elucidated for the association with neuromyelitis optica (NMO). Methods: Twelve primary SS patients (all women, 42 ± 13.2 years) who had recurrent central nervous system (CNS) manifestations with brain involvement were retrospectively identified. Brain MRI, and neurologic and serologic findings were analyzed with the measurement of anti-aquaporin-4 antibody (AQP4-Ab). Results: All patients showed brain lesions characteristic of NMO as follows: 1) the involved sites adjacent to the third and fourth ventricles and in the posterior limb of the internal capsule, 2) unique configurations, such as the longitudinal course from the internal capsule to the midbrain, large cerebral or cerebellar lesions over 3 cm, and cavity-like formations. AQP4-Ab was positive in six of eight patients tested, and all the seropositive patients showed lesions with increased diffusion, suggestive of vasogenic edema. Four patients met the revised criteria of NMO, and nine had features of NMOSDs. Of the remaining three patients showing only brain involvement, one had AQP4-Ab. Conclusions: This study demonstrates that SS patients with recurrent CNS involvement have brain abnormalities characteristic of NMO and AQP4-Ab in Korea. The presence of AQP4-Ab in one SS patient with only brain involvement may suggest that the coexistence of NMO should be explored in SS patients with recurrent CNS manifestations, even without optic neuritis or myelitis.",
keywords = "Aquaporin 4, Beuromyelitis optica, Brain MRI, Multiple sclerosis, Sjogren's syndrome, Vasogenic brain edema",
author = "Min, {J. H.} and Kim, {H. J.} and Kim, {Byung Jo} and Lee, {K. W.} and Sunwoo, {I. N.} and Kim, {S. M.} and Kim, {B. J.} and Kim, {S. H.} and Park, {M. S.} and P. Waters and A. Vincent and Sung, {J. J.} and Lee, {K. H.}",
year = "2009",
month = "8",
day = "28",
doi = "10.1177/1352458509106228",
language = "English",
volume = "15",
pages = "1069--1076",
journal = "Multiple Sclerosis",
issn = "1352-4585",
publisher = "SAGE Publications Ltd",
number = "9",

}

TY - JOUR

T1 - Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations

T2 - Association with neuromyelitis optica

AU - Min, J. H.

AU - Kim, H. J.

AU - Kim, Byung Jo

AU - Lee, K. W.

AU - Sunwoo, I. N.

AU - Kim, S. M.

AU - Kim, B. J.

AU - Kim, S. H.

AU - Park, M. S.

AU - Waters, P.

AU - Vincent, A.

AU - Sung, J. J.

AU - Lee, K. H.

PY - 2009/8/28

Y1 - 2009/8/28

N2 - Background and objectives: Optic neuritis or longitudinally extensive myelitis in Sjogren syndrome (SS) suggests a neuromyelitis optica spectrum disorder (NMOSD). However, brain abnormalities of SS remain to be elucidated for the association with neuromyelitis optica (NMO). Methods: Twelve primary SS patients (all women, 42 ± 13.2 years) who had recurrent central nervous system (CNS) manifestations with brain involvement were retrospectively identified. Brain MRI, and neurologic and serologic findings were analyzed with the measurement of anti-aquaporin-4 antibody (AQP4-Ab). Results: All patients showed brain lesions characteristic of NMO as follows: 1) the involved sites adjacent to the third and fourth ventricles and in the posterior limb of the internal capsule, 2) unique configurations, such as the longitudinal course from the internal capsule to the midbrain, large cerebral or cerebellar lesions over 3 cm, and cavity-like formations. AQP4-Ab was positive in six of eight patients tested, and all the seropositive patients showed lesions with increased diffusion, suggestive of vasogenic edema. Four patients met the revised criteria of NMO, and nine had features of NMOSDs. Of the remaining three patients showing only brain involvement, one had AQP4-Ab. Conclusions: This study demonstrates that SS patients with recurrent CNS involvement have brain abnormalities characteristic of NMO and AQP4-Ab in Korea. The presence of AQP4-Ab in one SS patient with only brain involvement may suggest that the coexistence of NMO should be explored in SS patients with recurrent CNS manifestations, even without optic neuritis or myelitis.

AB - Background and objectives: Optic neuritis or longitudinally extensive myelitis in Sjogren syndrome (SS) suggests a neuromyelitis optica spectrum disorder (NMOSD). However, brain abnormalities of SS remain to be elucidated for the association with neuromyelitis optica (NMO). Methods: Twelve primary SS patients (all women, 42 ± 13.2 years) who had recurrent central nervous system (CNS) manifestations with brain involvement were retrospectively identified. Brain MRI, and neurologic and serologic findings were analyzed with the measurement of anti-aquaporin-4 antibody (AQP4-Ab). Results: All patients showed brain lesions characteristic of NMO as follows: 1) the involved sites adjacent to the third and fourth ventricles and in the posterior limb of the internal capsule, 2) unique configurations, such as the longitudinal course from the internal capsule to the midbrain, large cerebral or cerebellar lesions over 3 cm, and cavity-like formations. AQP4-Ab was positive in six of eight patients tested, and all the seropositive patients showed lesions with increased diffusion, suggestive of vasogenic edema. Four patients met the revised criteria of NMO, and nine had features of NMOSDs. Of the remaining three patients showing only brain involvement, one had AQP4-Ab. Conclusions: This study demonstrates that SS patients with recurrent CNS involvement have brain abnormalities characteristic of NMO and AQP4-Ab in Korea. The presence of AQP4-Ab in one SS patient with only brain involvement may suggest that the coexistence of NMO should be explored in SS patients with recurrent CNS manifestations, even without optic neuritis or myelitis.

KW - Aquaporin 4

KW - Beuromyelitis optica

KW - Brain MRI

KW - Multiple sclerosis

KW - Sjogren's syndrome

KW - Vasogenic brain edema

UR - http://www.scopus.com/inward/record.url?scp=69149090153&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=69149090153&partnerID=8YFLogxK

U2 - 10.1177/1352458509106228

DO - 10.1177/1352458509106228

M3 - Article

VL - 15

SP - 1069

EP - 1076

JO - Multiple Sclerosis

JF - Multiple Sclerosis

SN - 1352-4585

IS - 9

ER -