Emilė Tilindytė-Muliuolienė1,Rūta Samaitienė-Aleknienė2
1Vilnius university Faculty of Medicine, Vilnius, Lithuania
2Vilnius university Faculty of Medicine; Vilnius university Santaros clinics, Children’s hospital, Department of Neurology, Vilnius, Lithuania
Abstract
Background. Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory-autoimmune disorder of the central nervous system (CNS) that has been identified and actively researched in the last decade. It is characterized by episodes of immune-mediated demyelination of the brain, spinal cord, and optic nerves. The recognition of MOGAD significantly changed the approach to the diagnosis and treatment of demyelinating diseases of the central nervous system (CNS) and optic neuritis (ON).
Case report. This article describes the case of an 11-year-old patient with ON caused by MOG antibodies. The diagnosis of MOGAD was confirmed based on clinical history, head and neck magnetic resonance imaging (MRI), and serological tests. For the first time the disease manifested as acute disseminated encephalomyelitis (ADEM), similar symptoms were observed during the first relapse. MOGAL optic neuritis occurred during the second relapse, two more relapses manifested later, both of them as ON. Disease relapses were treated with methylprednisolone initial pulse-therapy and prednisolone tapering. Maintenance treatment was continued with intravenous immunoglobulin, mycophenolate mofetil and rituximab. Although the effect of treatment was positive and regression of symptoms was observed, the course of the disease remained relapsing.
Conclusion. ON is one of the most common manifestations of MOGAD, and is characterized by relapses and cumulative visual impairment, therefore, when differentiating the causes of ON, it is important to test for MOG antibodies, as the tactics of supportive treatment may depend on it.
Keywords: autoimmune encephalomyelitis, optic neuritis, anti-MOG associated encephalomyelitis, MOG-IgG test, demyelinating disease of the central nervous system.
Optinis neuritas sergant mielino oligodendrocito glikoproteinų antikūnų asocijuota liga: klinikinis atvejis
Emilė Tilindytė-Muliuolienė1,Rūta Samaitienė-Aleknienė2
1Vilniaus universiteto Medicinos fakultetas, Vilnius, Lietuva
2Vilniaus universiteto Medicinos fakultetas; Vaikų ligoninė viešosios įstaigos Vilniaus universiteto ligoninės Santaros klinikų filialas, Vaikų neurologijos skyrius, Vilnius, Lietuva
Santrauka
Įvadas. Pastarąjį dešimtmetį įvardinta ir aktyviai tyrinėjama su mielino oligodendrocito glikoproteinų (MOG) antikūnais susijusi liga MOGAL (angl. myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) – tai uždegiminis-autoimuninis centrinės nervų sistemos (CNS) sutrikimas. Jai būdingi imuninės sistemos sąlygoti galvos ir nugaros smegenų, regos nervų demielinizacijos epizodai. Atradus MOGAL reikšmingai pasikeitė demielinizuojančių centrinės nervų sistemos (CNS) ligų bei optinio neurito (ON) diferencinės diagnostikos, ir, svarbiausia, gydymo taktika.
Atvejis. Šiame straipsnyje aprašomas 11 metų pacientės ON, sąlygoto MOG antikūnų, atvejis. MOGAL diagnozė buvo patvirtinta remiantis klinika, galvos ir kaklo magnetinio rezonanso tomografijos (MRT) bei serologiniais tyrimais. Pirmąjį kartą liga manifestavo kaip ūminis diseminuotas encefalomielitas (ŪDEM), panaši simptomatika stebėta ir pirmojo atkryčio metu. MOGAL optiniu neuritu pasireiškė antrojo ligos atkryčio metu, vėliau stebėti dar du ligos recidyvai, abu pasireiškė ON. Ligos atkryčiai gydyti metilprednizolono pulsterapija bei prednizolonu, jo kursą palaipsniui nutraukiant. Ilgalaikis gydymas tęstas intraveniniu imunoglobulinu, mikofenolato mofetiliu ir rituksimabu. Nors buvo stebėtas teigiamas gydymo efektas – simptomatikos regresavimas, ligos eiga išliko recidyvuojanti.
Išvados. ON – viena dažniausių MOGAL išraiška, kuriai būdingi atkryčiai bei kumuliacinė regos pažaida, todėl diferencijuojant ON priežastis svarbu atlikti MOG antikūnų tyrimą, kadangi nuo to gali priklausyti palaikomojo gydymo taktika.
Raktažodžiai: autoimuninis encefalomielitas, optinis neuritas, Anti-MOG asocijuotas encefalomielitas, MOG-IgG tyrimas, demielinizuojanti centrinės nervų sistemos liga.
https://doi.org/10.53453/ms.2024.5.18
Optic neuritis caused by myelin oligodendrocyte glycoprotein
antibody-associated disease: clinical case report
Emilė Tilindytė-Muliuolienė
1
,Rūta Samaitienė-Aleknienė
2
1
Vilnius university Faculty of Medicine, Vilnius, Lithuania
2
Vilnius university Faculty of Medicine; Vilnius university Santaros clinics, Children’s hospital, Department of
Neurology, Vilnius, Lithuania
Abstract
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Journal of Medical Sciences. 5 May, 2024 - Volume 12 | Issue 3. Electronic - ISSN: 2345-0592
Medical Sciences 2024 Vol. 12 (3), p. 165-177, https://doi.org/10.53453/ms.2024.5.18
165
Optinis neuritas sergant mielino oligodendrocito glikoproteinų
antikūnų asocijuota liga: klinikinis atvejis
Emilė Tilindytė-Muliuolienė
1
,Rūta Samaitienė-Aleknienė
2
1
Vilniaus universiteto Medicinos fakultetas, Vilnius, Lietuva
2
Vilniaus universiteto Medicinos fakultetas; Vaikų ligoninė viešosios įstaigos Vilniaus universiteto ligoninės
Santaros klinikų filialas, Vaikų neurologijos skyrius, Vilnius, Lietuva
Santrauka
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2 pav. Optinis neuritas kairėje. Kairėje: DWI (angl. diffusion-weighted imaging) sekoje stebimas aukšto
signalo kairysis optinis nervas. Dešinėje: T2 FLAIR sekoje hiperintensinio signalo židiniai prechiazmatiškai,
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Journal of Medical Sciences. 5 May, 2024 - Volume 12 | Issue 3. Electronic - ISSN: 2345-0592
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3 pav. Optinis neuritas dešinėje. DWI (angl. diffusion-weighted imaging) (dešinėje), ADC (angl. apparent
diffusion coefficient) (kairėje) - stebimas aukšto signalo dešinysis optinis nervas DWI sekoje, atitinkamai žemo
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4 pav. Optinis neuritas dešinėje. T2 FLAIR sekoje stebimas sustorėjęs dešinysis optinis nervas.
3. Literatūros apžvalga
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Journal of Medical Sciences. 5 May, 2024 - Volume 12 | Issue 3. Electronic - ISSN: 2345-0592
177