• Je něco špatně v tomto záznamu ?

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

G. Tsivgoulis, G. Dervenoulas, P. Kokotis, C. Zompola, JS. Tzartos, SJ. Tzartos, KI. Voumvourakis,

. 2014 ; 346 (1-2) : 328-30. [pub] 20140917

Jazyk angličtina Země Nizozemsko

Typ dokumentu kazuistiky, časopisecké články, práce podpořená grantem

Perzistentní odkaz   https://www.medvik.cz/link/bmc15031838

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.

Citace poskytuje Crossref.org

000      
00000naa a2200000 a 4500
001      
bmc15031838
003      
CZ-PrNML
005      
20151013115755.0
007      
ta
008      
151005s2014 ne f 000 0|eng||
009      
AR
024    7_
$a 10.1016/j.jns.2014.09.013 $2 doi
035    __
$a (PubMed)25248951
040    __
$a ABA008 $b cze $d ABA008 $e AACR2
041    0_
$a eng
044    __
$a ne
100    1_
$a Tsivgoulis, Georgios $u Second Department of Neurology, University of Athens, School of Medicine, "Attikon" University Hospital, Athens, Greece; International Clinical Research Center, St. Anne's University Hospital in Brno, Czech Republic. Electronic address: tsivgoulisgiorg@yahoo.gr.
245    10
$a Double seronegative myasthenia gravis with low density lipoprotein-4 (LRP4) antibodies presenting with isolated ocular symptoms / $c G. Tsivgoulis, G. Dervenoulas, P. Kokotis, C. Zompola, JS. Tzartos, SJ. Tzartos, KI. Voumvourakis,
520    9_
$a 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.
650    _2
$a dospělí $7 D000328
650    _2
$a senioři nad 80 let $7 D000369
650    _2
$a blefaroptóza $x farmakoterapie $x imunologie $7 D001763
650    _2
$a cholinesterasové inhibitory $x terapeutické užití $7 D002800
650    _2
$a diplopie $x farmakoterapie $x imunologie $7 D004172
650    _2
$a kombinovaná farmakoterapie $7 D004359
650    _2
$a ženské pohlaví $7 D005260
650    _2
$a glukokortikoidy $x terapeutické užití $7 D005938
650    _2
$a lidé $7 D006801
650    _2
$a proteiny související s LDL-receptory $x imunologie $7 D026502
650    _2
$a mužské pohlaví $7 D008297
650    _2
$a lidé středního věku $7 D008875
650    _2
$a myasthenia gravis $x farmakoterapie $x imunologie $7 D009157
650    _2
$a prednison $x terapeutické užití $7 D011241
650    _2
$a pyridostigmin-bromid $x terapeutické užití $7 D011729
650    _2
$a výsledek terapie $7 D016896
655    _2
$a kazuistiky $7 D002363
655    _2
$a časopisecké články $7 D016428
655    _2
$a práce podpořená grantem $7 D013485
700    1_
$a Dervenoulas, Georgios $u Second Department of Neurology, University of Athens, School of Medicine, "Attikon" University Hospital, Athens, Greece.
700    1_
$a Kokotis, Panagiotis $u Second Department of Neurology, University of Athens, School of Medicine, "Attikon" University Hospital, Athens, Greece; First Department of Neurology, University of Athens, School of Medicine, "Eginition" University Hospital, Athens, Greece.
700    1_
$a Zompola, Christina $u Second Department of Neurology, University of Athens, School of Medicine, "Attikon" University Hospital, Athens, Greece.
700    1_
$a Tzartos, John S $u Hellenic Pasteur Institute, Athens, Greece.
700    1_
$a Tzartos, Socrates J $u Hellenic Pasteur Institute, Athens, Greece; Tzartos NeuroDiagnostics, Athens, Greece.
700    1_
$a Voumvourakis, Konstantinos I $u Second Department of Neurology, University of Athens, School of Medicine, "Attikon" University Hospital, Athens, Greece.
773    0_
$w MED00003004 $t Journal of the neurological sciences $x 1878-5883 $g Roč. 346, č. 1-2 (2014), s. 328-30
856    41
$u https://pubmed.ncbi.nlm.nih.gov/25248951 $y Pubmed
910    __
$a ABA008 $b sig $c sign $y a $z 0
990    __
$a 20151005 $b ABA008
991    __
$a 20151013115945 $b ABA008
999    __
$a ok $b bmc $g 1092714 $s 914964
BAS    __
$a 3
BAS    __
$a PreBMC
BMC    __
$a 2014 $b 346 $c 1-2 $d 328-30 $e 20140917 $i 1878-5883 $m Journal of the neurological sciences $n J Neurol Sci $x MED00003004
LZP    __
$a Pubmed-20151005

Najít záznam

Citační ukazatele

Nahrávání dat ...

Možnosti archivace

Nahrávání dat ...