Double seronegative myasthenia gravis with low density lipoprotein-4 (LRP4) antibodies presenting with isolated ocular symptoms

Georgios Tsivgoulis, Georgios Dervenoulas, Panagiotis Kokotis, Christina Zompola, John S. Tzartos, Socrates J. Tzartos, Konstantinos I. Voumvourakis

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

The detection of low density lipoprotein-4 (LRP4) antibodies in double seronegative (dSN) myasthenia gravis (MG) patients has provided new insights in the diagnosis and treatment of MG. However, there are limited data regarding the clinical presentation and treatment response in dSN MG patients with LRP4-antibodies. We present a case series of three Caucasian dSN MG patients with positive LRP4-antibodies sharing a common ethnic background that presented with isolated ocular symptoms (MGFA I). The demographic and clinical characteristics, the diagnostic work-up as well as the treatment response during a follow-up period of 12-24 months are described in detail. All patients were treated successfully with acetylcholinesterase inhibitors (AcheI) and prednisone with two exhibiting full remission of their symptoms, while the remaining exhibited mild residual diplopia. Notably, we documented no signs of generalized disease progression, while no patient required immunosuppressive treatment. In conclusion, the distinct clinical phenotype of our patients highlights the clinical relevance of screening for LRP4-antibodies in patients presenting with isolated ocular MG independent of age and gender, since it may lead to the timely diagnosis of MG and prompt initiation of effective therapy with ACheI and corticosteroids.

Original languageEnglish (US)
Pages (from-to)328-330
Number of pages3
JournalJournal of the Neurological Sciences
Volume346
Issue number1-2
DOIs
StatePublished - Nov 15 2014

Fingerprint

Myasthenia Gravis
LDL Lipoproteins
Antibodies
Therapeutics
Diplopia
Cholinesterase Inhibitors
Immunosuppressive Agents
Prednisone
Disease Progression
Adrenal Cortex Hormones
Demography
Phenotype

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology

Cite this

Double seronegative myasthenia gravis with low density lipoprotein-4 (LRP4) antibodies presenting with isolated ocular symptoms. / Tsivgoulis, Georgios; Dervenoulas, Georgios; Kokotis, Panagiotis; Zompola, Christina; Tzartos, John S.; Tzartos, Socrates J.; Voumvourakis, Konstantinos I.

In: Journal of the Neurological Sciences, Vol. 346, No. 1-2, 15.11.2014, p. 328-330.

Research output: Contribution to journalArticle

Tsivgoulis, Georgios ; Dervenoulas, Georgios ; Kokotis, Panagiotis ; Zompola, Christina ; Tzartos, John S. ; Tzartos, Socrates J. ; Voumvourakis, Konstantinos I. / Double seronegative myasthenia gravis with low density lipoprotein-4 (LRP4) antibodies presenting with isolated ocular symptoms. In: Journal of the Neurological Sciences. 2014 ; Vol. 346, No. 1-2. pp. 328-330.
@article{0b79c21743e94ffe96d5c05593400ea6,
title = "Double seronegative myasthenia gravis with low density lipoprotein-4 (LRP4) antibodies presenting with isolated ocular symptoms",
abstract = "The detection of low density lipoprotein-4 (LRP4) antibodies in double seronegative (dSN) myasthenia gravis (MG) patients has provided new insights in the diagnosis and treatment of MG. However, there are limited data regarding the clinical presentation and treatment response in dSN MG patients with LRP4-antibodies. We present a case series of three Caucasian dSN MG patients with positive LRP4-antibodies sharing a common ethnic background that presented with isolated ocular symptoms (MGFA I). The demographic and clinical characteristics, the diagnostic work-up as well as the treatment response during a follow-up period of 12-24 months are described in detail. All patients were treated successfully with acetylcholinesterase inhibitors (AcheI) and prednisone with two exhibiting full remission of their symptoms, while the remaining exhibited mild residual diplopia. Notably, we documented no signs of generalized disease progression, while no patient required immunosuppressive treatment. In conclusion, the distinct clinical phenotype of our patients highlights the clinical relevance of screening for LRP4-antibodies in patients presenting with isolated ocular MG independent of age and gender, since it may lead to the timely diagnosis of MG and prompt initiation of effective therapy with ACheI and corticosteroids.",
author = "Georgios Tsivgoulis and Georgios Dervenoulas and Panagiotis Kokotis and Christina Zompola and Tzartos, {John S.} and Tzartos, {Socrates J.} and Voumvourakis, {Konstantinos I.}",
year = "2014",
month = "11",
day = "15",
doi = "10.1016/j.jns.2014.09.013",
language = "English (US)",
volume = "346",
pages = "328--330",
journal = "Journal of the Neurological Sciences",
issn = "0022-510X",
publisher = "Elsevier",
number = "1-2",

}

TY - JOUR

T1 - Double seronegative myasthenia gravis with low density lipoprotein-4 (LRP4) antibodies presenting with isolated ocular symptoms

AU - Tsivgoulis, Georgios

AU - Dervenoulas, Georgios

AU - Kokotis, Panagiotis

AU - Zompola, Christina

AU - Tzartos, John S.

AU - Tzartos, Socrates J.

AU - Voumvourakis, Konstantinos I.

PY - 2014/11/15

Y1 - 2014/11/15

N2 - The detection of low density lipoprotein-4 (LRP4) antibodies in double seronegative (dSN) myasthenia gravis (MG) patients has provided new insights in the diagnosis and treatment of MG. However, there are limited data regarding the clinical presentation and treatment response in dSN MG patients with LRP4-antibodies. We present a case series of three Caucasian dSN MG patients with positive LRP4-antibodies sharing a common ethnic background that presented with isolated ocular symptoms (MGFA I). The demographic and clinical characteristics, the diagnostic work-up as well as the treatment response during a follow-up period of 12-24 months are described in detail. All patients were treated successfully with acetylcholinesterase inhibitors (AcheI) and prednisone with two exhibiting full remission of their symptoms, while the remaining exhibited mild residual diplopia. Notably, we documented no signs of generalized disease progression, while no patient required immunosuppressive treatment. In conclusion, the distinct clinical phenotype of our patients highlights the clinical relevance of screening for LRP4-antibodies in patients presenting with isolated ocular MG independent of age and gender, since it may lead to the timely diagnosis of MG and prompt initiation of effective therapy with ACheI and corticosteroids.

AB - The detection of low density lipoprotein-4 (LRP4) antibodies in double seronegative (dSN) myasthenia gravis (MG) patients has provided new insights in the diagnosis and treatment of MG. However, there are limited data regarding the clinical presentation and treatment response in dSN MG patients with LRP4-antibodies. We present a case series of three Caucasian dSN MG patients with positive LRP4-antibodies sharing a common ethnic background that presented with isolated ocular symptoms (MGFA I). The demographic and clinical characteristics, the diagnostic work-up as well as the treatment response during a follow-up period of 12-24 months are described in detail. All patients were treated successfully with acetylcholinesterase inhibitors (AcheI) and prednisone with two exhibiting full remission of their symptoms, while the remaining exhibited mild residual diplopia. Notably, we documented no signs of generalized disease progression, while no patient required immunosuppressive treatment. In conclusion, the distinct clinical phenotype of our patients highlights the clinical relevance of screening for LRP4-antibodies in patients presenting with isolated ocular MG independent of age and gender, since it may lead to the timely diagnosis of MG and prompt initiation of effective therapy with ACheI and corticosteroids.

UR - http://www.scopus.com/inward/record.url?scp=84911003051&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84911003051&partnerID=8YFLogxK

U2 - 10.1016/j.jns.2014.09.013

DO - 10.1016/j.jns.2014.09.013

M3 - Article

C2 - 25248951

AN - SCOPUS:84911003051

VL - 346

SP - 328

EP - 330

JO - Journal of the Neurological Sciences

JF - Journal of the Neurological Sciences

SN - 0022-510X

IS - 1-2

ER -