Choroba związana z przeciwciałami białka mieliny oligodendrocytów (mogad)
Objawy
Choroba związana z przeciwciałami białka mieliny oligodendrocytów (MOGAD) to autoimmunologiczne schorzenie ośrodkowego układu nerwowego, charakteryzujące się zapaleniem nerwu wzrokowego, poprzecznym zapaleniem rdzenia kręgowego oraz ostrym rozsianym zapaleniem mózgu i rdzenia (ADEM). MOGAD różni się klinicznie i immunologicznie od stwardnienia rozsianego (SM) oraz neuromyelitis optica spectrum disorder (NMOSD) związanego z przeciwciałami przeciwko akwaporynie-4 (AQP4). Zapalenie nerwu wzrokowego w MOGAD często przebiega obustronnie, z bólem oka nasilającym się przy ruchu gałki ocznej, a u 6-14% pacjentów pozostaje ostrość wzroku ≤20/200. Poprzeczne zapalenie rdzenia kręgowego obejmuje zmiany rozległe (≥3 segmenty kręgowe) i prowadzi do ciężkiej dysfunkcji neurologicznej, z 1/3 pacjentów niezdolnych do samodzielnego chodzenia w fazie ostrej. U dzieci dominuje fenotyp ADEM (68% przypadków), z objawami takimi jak dezorientacja, drgawki i zaburzenia świadomości, natomiast u dorosłych częściej obserwuje się zapalenie nerwu wzrokowego i zapalenie rdzenia kręgowego. Przebieg choroby może być monofazowy (40-50% pacjentów) lub nawracający, z nawrotami częstszymi u pacjentów z utrzymującymi się przeciwciałami MOG-IgG.
- Choroba związana z przeciwciałami białka mieliny oligodendrocytów (MOGAD) – wprowadzenie
- Główne objawy MOGAD
- Objawy oczne (zapalenie nerwu wzrokowego)
- Objawy rdzeniowe (poprzeczne zapalenie rdzenia)
- Objawy mózgowe (ADEM i inne)
- Przebieg i progresja choroby
- Szczególne grupy pacjentów
- Rzadkie manifestacje MOGAD
- Aspekty poznawcze MOGAD
- Podsumowanie
Choroba związana z przeciwciałami białka mieliny oligodendrocytów (MOGAD) – wprowadzenie
Choroba związana z przeciwciałami białka mieliny oligodendrocytów (MOGAD – Myelin oligodendrocyte glycoprotein antibody-associated disease) to rzadkie schorzenie autoimmunologiczne, w którym układ odpornościowy atakuje białka znajdujące się w osłonce mielinowej ochraniającej nerwy (mielina). Choroba wywołuje stan zapalny w ośrodkowym układzie nerwowym (OUN), powodując objawy dotyczące oczu, mózgu i rdzenia kręgowego, takie jak utrata wzroku, zaburzenia świadomości i osłabienie mięśni.123
MOGAD jest obecnie uznawana za odrębną jednostkę chorobową, różniącą się od innych demielinizacyjnych chorób OUN, takich jak stwardnienie rozsiane (SM) czy choroba z widocznym spektrum objawów związanych z przeciwciałami przeciwko akwaporynie-4 (NMOSD). Choroba ma predylekcję do występowania u dzieci, choć może również dotykać dorosłych.234
Główne objawy MOGAD
Objawy MOGAD mogą obejmować utratę wzroku, osłabienie mięśni, sztywność lub paraliż, dezorientację, drgawki i bóle głowy. Pacjenci mogą usłyszeć od lekarza, że ich objawy są określane jako zapalenie nerwu wzrokowego, poprzeczne zapalenie rdzenia kręgowego oraz ostre rozsiane zapalenie mózgu i rdzenia (ADEM).156
Objawy oczne (zapalenie nerwu wzrokowego)
Zapalenie nerwu wzrokowego jest najczęstszą manifestacją kliniczną MOGAD na początku choroby i jest jeszcze bardziej dominujące podczas nawrotów. Objawy mogą dotyczyć jednego lub obu oczu i obejmują:178
- Niewyraźne widzenie
- Podwójne widzenie
- Utratę wzroku
- Ból oka, który pogarsza się podczas ruchu gałki ocznej
- Utratę widzenia kolorów
Zapalenie nerwu wzrokowego w MOGAD różni się od SM, ponieważ częściej występuje obustronnie i prowadzi do poważniejszych ubytków wzroku, jeśli nie jest leczone. Jest to niemal zawsze związane z bólem oka, który nasila się podczas ruchu gałki ocznej, często poprzedzając skargi na utratę wzroku.101112
Powrót do zdrowia w przypadku MOGAD jest zazwyczaj dobry, z tylko 6-14% pacjentów z MOGAD mających pozostałą ostrość wzroku 20/200 lub gorszą w porównaniu z jedną trzecią w przypadku NMOSD związanego z przeciwciałami AQP4, chociaż pozostała bladość tarczy nerwu wzrokowego jest częsta w MOGAD.713
Objawy rdzeniowe (poprzeczne zapalenie rdzenia)
Poprzeczne zapalenie rdzenia kręgowego definiuje się jako stan zapalny przechodzący przez miąższ rdzenia kręgowego, prowadzący do dysfunkcji neurologicznej, która rozwija się w ciągu godzin lub dni i osiąga swoje maksimum (maksymalny deficyt) w ciągu 21 dni. Objawy obejmują:14715
- Osłabienie mięśni mogące prowadzić do paraparesis lub quadriparesis
- Drętwienie lub utratę czucia
- Problemy z kontrolą pęcherza lub jelit, lub trudności z opróżnianiem pęcherza
- Sztywność mięśni (spastyczność)
- Ostry, przeszywający ból lub uczucie mrowienia w okolicy szyi, pleców lub brzucha
Nasilenie zapalenia rdzenia kręgowego jest większe niż w przypadku SM, a do jednej trzeciej pacjentów nie będzie zdolnych do samodzielnego chodzenia w punkcie maksymalnego nasilenia. Zajęcie rdzenia kręgowego w MOGAD może wystąpić jako izolowany atak lub w połączeniu z zajęciem innych regionów OUN (np. ADEM, zapalenie nerwu wzrokowego).1517
U około trzech czwartych pacjentów zmiany towarzyszące zapaleniu rdzenia kręgowego na obrazach MRI ważonych T2 w projekcji strzałkowej mają długość trzech lub więcej segmentów kręgowych, co określa się jako podłużnie rozległe poprzeczne zapalenie rdzenia kręgowego (LETM); są one zwykle zlokalizowane centralnie w rdzeniu kręgowym.1518
Objawy mózgowe (ADEM i inne)
ADEM jest najczęstszą początkową manifestacją MOGAD u dzieci, występującą u około 68% wszystkich pediatrycznych przypadków z dodatnim wynikiem na obecność przeciwciał MOG. Objawy dotyczące mózgu obejmują:141519
- Dezorientację i zmiany stanu świadomości
- Utratę równowagi i koordynacji
- Bóle głowy
- Zmiany zachowania
- Utratę przytomności
- Drgawki
Zapalenie kory mózgowej jest obecnie uznawane za charakterystyczną cechę MOGAD. Manifestacje kliniczne obejmują drgawki, afazję, epizody podobne do udaru, ból głowy i gorączkę. U niektórych pacjentów z MOGAD drgawki mogą być objawem początkowym; występuje to najczęściej u dzieci z zapaleniem kory mózgowej lub ADEM.152122
Drgawki występują częściej w przypadku MOGAD niż w przypadku NMOSD związanego z przeciwciałami AQP4. Zajęcie pnia mózgu jest kolejnym częstym znaleziskiem w MOGAD, występującym u około 30% pacjentów z MOGAD.2116
Przebieg i progresja choroby
Objawy MOGAD pojawiają się i ustępują. Pacjenci mogą doświadczać okresów bez żadnych objawów, po których następuje nawrót objawów (relaps). MOGAD różni się od SM i NMOSD związanego z przeciwciałami AQP4 tym, że duża część pacjentów (40-50%) ma pojedynczy atak bez nawrotu, co określa się jako przebieg monofazowy.142123
Ataki zwykle rozwijają się w ciągu dni i mogą mieć przebieg ciężki i wyniszczający. Niepełnosprawność zwykle pogarsza się z każdym atakiem. Powrót do zdrowia po ataku może trwać tygodnie do miesięcy.232425
Prezentacja zależna od wieku
Prezentacja kliniczna MOGAD różni się w zależności od wieku pacjenta:142627
- U dzieci MOGAD najczęściej atakuje mózg (ADEM), co powoduje objawy takie jak dezorientacja i utrata koordynacji. Czasami towarzyszy temu zapalenie nerwu wzrokowego.
- U dorosłych MOGAD atakuje rdzeń kręgowy i oczy, powodując objawy takie jak osłabienie lub drętwienie oraz niewyraźne widzenie.
Młodsze dzieci (≤11 lat) mają tendencję do prezentacji fenotypu ADEM, podczas gdy starsi pacjenci (>11 lat) częściej prezentują zapalenie nerwu wzrokowego. Nasilenie ataków i stopień powrotu do zdrowia również zależą od wieku, przy czym młodsze dzieci prezentują najgorsze nasilenie kliniczno-radiologiczne, ale z szybszym i pełniejszym powrotem do zdrowia.2730
Przebieg monofazowy a nawracający
MOGAD może mieć przebieg monofazowy lub nawracający:233132
- Przebieg monofazowy: Około 50% pacjentów z MOGAD doświadcza tylko jednego ataku bez nawrotu. Jest to nieco częstsze niż przebieg nawracający.
- Przebieg nawracający: Druga połowa pacjentów doświadcza wielu ataków, nazywanych nawracającym MOGAD.
Wczesne nawroty w pierwszym roku po wystąpieniu objawów były związane z dalszymi nawrotami w długim okresie, chociaż u dzieci bardzo wczesne nawroty w ciągu pierwszych 90 dni nie prognozowały przewlekłej nawracającej choroby. Nawroty są najczęstsze u pacjentów, którzy prezentowali objawy zapalenia nerwu wzrokowego i występowały w miesiącach do 1-2 lat po początkowym ataku.3134
Trwale dodatnie miana przeciwciał MOG-IgG zostały powiązane z wyższym ryzykiem nawrotów, ale nawroty mogą również wystąpić u osób, które stają się seronegatywne, a niektóre przypadki pozostają monofazowe pomimo dodatnich przeciwciał MOG-IgG.3135
Rokowanie i możliwe powikłania
Uszkodzenie mieliny może trwale wpłynąć na funkcjonowanie organizmu. Pacjenci mogą doświadczyć następujących powikłań:36
- Ślepota
- Dysfunkcja pęcherza lub jelit
- Paraliż (ramiona i nogi)
- Przewlekły ból
- Przewlekłe zmęczenie
- Zaburzenia poznawcze (trudności w uczeniu się, zapamiętywaniu i myśleniu)
Ogólnie rzecz biorąc, rokowanie dla osób z MOGAD jest zazwyczaj korzystne. Większość pacjentów doświadcza łagodniejszego przebiegu choroby w porównaniu z NMOSD, z mniej poważną niepełnosprawnością. U dzieci między 75% a 96% zgłaszano całkowity powrót do zdrowia. Spośród pacjentów, którzy prezentują MOG-ON, występuje całkowity powrót ostrości wzroku u 56%-73% pacjentów, co jest lepsze niż u dorosłych pacjentów.3330
Pacjenci z zapaleniem nerwu wzrokowego mają wyższe ryzyko nawrotu w porównaniu do tych z poprzecznym zapaleniem rdzenia kręgowego lub ADEM. Według badań opartych na przypadkach, osoby prezentujące poprzeczne zapalenie rdzenia kręgowego są najbardziej narażone na długotrwałą niepełnosprawność. Niektóre przypadki donoszą również o pozostałej niepełnosprawności rozwijającej się u 50-80% pacjentów z MOGAD.3839
Radzenie sobie z MOGAD może być trudne. Kiedy pojawiają się objawy, mogą one odciągać pacjenta od aktywności, które lubi, i wywołać poczucie bezradności. Z tego powodu wiele osób doświadcza depresji związanej z MOGAD.36
Szczególne grupy pacjentów
MOGAD u dzieci
MOGAD u dzieci jest bardziej heterogeniczną chorobą niż u dorosłych, a różnice w pediatrycznej formie choroby są bardziej wyraźne u młodszych pacjentów. Najczęstszą manifestacją MOGAD u dzieci jest ostre rozsiane zapalenie mózgu i rdzenia (ADEM), które stanowi około 40%-50% wszystkich przypadków pediatrycznych MOGAD.2630
Typowe objawy MOGAD u dzieci obejmują:40
- Problemy z oczami, takie jak podwójne widzenie, ból podczas poruszania oczami i utrata wzroku
- Trudności w przetwarzaniu informacji, organizowaniu, planowaniu i podejmowaniu decyzji
- Osłabienie, mrowienie i drętwienie
- Problemy z równowagą i koordynacją
- Drżenia (drżenie)
- Sztywność mięśni i skurcze, które mogą być bolesne
- Problemy z kontrolą jelit i pęcherza
- Zaburzenia mowy
Przebieg choroby MOG-ON u dzieci jest łagodniejszy w porównaniu z przebiegiem u dorosłych, a dzieci częściej doświadczają całkowitego powrotu do zdrowia. Długoterminowe ryzyko nawrotu wynosi około 35%, ale wskaźniki nawrotów u dzieci są niższe niż u dorosłych. Wczesne nawroty w ciągu 12 miesięcy od początkowego wystąpienia zwiększają ryzyko długotrwałej nawracającej choroby.3042
MOGAD a ciąża
Wyniki badań sugerują zmniejszenie nawrotów MOGAD w czasie ciąży i potencjalny umiarkowany wpływ w ciągu pierwszych 3 miesięcy po porodzie. Rezultaty dla matek z MOGAD i ich potomstwa wydają się mieścić w zakresie raportowanym dla osób bez choroby.43
Rzadkie manifestacje MOGAD
Poza bardziej powszechnymi obrazami klinicznymi, MOGAD może również prezentować się z mniej powszechnymi fenotypami klinicznymi:3244
- Zapalenie kory mózgowej: Występuje u około 6,8% przypadków, głównymi objawami są drgawki, ból głowy i gorączka.
- Nakładający się zespół MOGAD i zapalenia mózgu z przeciwciałami anty-NMDA (MNOS): Występuje u około 5,5% przypadków, przy czym najczęstszym objawem klinicznym są drgawki (92,3%).
- Fenotyp podobny do leukodystrofii mózgowej: Obecny u około 3,0% przypadków, z nawrotami u 50% pacjentów.
- Izolowane drgawki bez żadnych znalezisk w MRI: Występuje u około 1,3% przypadków, z drgawkami o pochodzeniu ogniskowym jako jedynym objawem klinicznym.
- Aseptyczne zapalenie opon mózgowych: Występuje u około 1,3% przypadków, prezentuje się z przedłużającą się gorączką.
Łącznie, około 16,9% dzieci z MOGAD ma mniej powszechne fenotypy. Te rzadsze obrazy kliniczne pediatrycznego MOGAD są podatne na błędną diagnozę i zasługują na większą uwagę.44
Aspekty poznawcze MOGAD
Choć charakterystyki kliniczne MOGAD są coraz lepiej analizowane w ostatnich latach, niewiele badań zbadało profil poznawczy pacjentów z MOGAD. Niedawne badanie sugeruje, że pacjenci z MOGAD wykazują obniżoną szybkość przetwarzania wzrokowo-motorycznego i płynność semantyczną w takim stopniu, w jakim obciążenie chorobowe obejmuje zmiany mózgowe.4748
Około 1 na 10 pacjentów wykazywało deficyty w dwóch lub więcej miarach poznawczych (11%). Częstość pacjentów z MOGAD z deficytami w testach neuropsychologicznych zmniejszyła się w okresie obserwacji 1-rocznej i 2-letniej.4950
Podsumowanie
MOGAD to stosunkowo niedawno odkryta choroba autoimmunologiczna, która powoduje różnorodne objawy neurologiczne, w tym zapalenie nerwu wzrokowego, poprzeczne zapalenie rdzenia kręgowego i ADEM. Choroba może mieć przebieg monofazowy lub nawracający, z około połową pacjentów doświadczających tylko jednego ataku, a pozostałymi doświadczającymi wielu ataków w ciągu życia.2131
Prezentacja kliniczna różni się w zależności od wieku, przy czym dzieci częściej doświadczają ADEM, a dorośli częściej prezentują zapalenie nerwu wzrokowego i poprzeczne zapalenie rdzenia kręgowego. Ogólnie rzecz biorąc, rokowanie jest zazwyczaj korzystne, a większość pacjentów doświadcza dobrego powrotu do zdrowia po odpowiednim leczeniu, chociaż niektórzy pacjenci mogą cierpieć na długotrwałe powikłania, takie jak utrata wzroku, dysfunkcja pęcherza/jelit i zaburzenia poznawcze.143133
Wczesna i dokładna diagnoza MOGAD jest kluczowa dla właściwego leczenia i zapobiegania nawracającej chorobie. Lepsze zrozumienie tej choroby i jej naturalnego przebiegu pomoże w optymalizacji strategii leczenia i poprawie wyników dla pacjentów z MOGAD.5152
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Materiały źródłowe
- #1 MOGAD: What It Is, Diagnosis, Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/myelin-oligodendrocyte-glycoprotein-antibody-disease-mogad
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune condition where your immune system attacks proteins in the protective cover around your nerves (myelin). It causes symptoms that affect your eyes, brain and spinal cord, like vision loss, confusion and muscle weakness. […] Common symptoms of MOGAD include vision loss, muscle weakness and loss of coordination. You may hear your provider refer to these symptoms as optic neuritis, transverse myelitis and acute disseminated encephalomyelitis (ADEM). […] Symptoms of MOGAD affect different parts of your body, including your eyes, spinal cord and brain. […] Eye symptoms of MOGAD are known as optic neuritis. They can affect one or both eyes and include: Blurry vision, Double vision, Vision loss, Eye pain, Color vision loss.
- #2 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD): Clinical features and diagnosis – UpToDatehttps://www.uptodate.com/contents/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-mogad-clinical-features-and-diagnosis
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory disorder of the central nervous system characterized by attacks of immune-mediated demyelination predominantly targeting the optic nerves, brain, and spinal cord. The disease has a predilection for children. […] Characteristic attacks â While none of the clinical features of MOGAD are disease-specific, some are highly characteristic. These include acute attacks of: Unilateral or bilateral optic neuritis resulting in severe visual loss; Acute disseminated encephalomyelitis (ADEM), resulting in altered mental status, focal neurologic features, and features of transverse myelitis; Transverse myelitis, often causing limb weakness, sensory loss, and bowel, bladder, or sexual dysfunction. MOGAD may have a monophasic or relapsing course.
- #3 Myelin Oligodendrocyte Antibody Disease Fact Sheet | Cleveland Clinichttps://my.clevelandclinic.org/departments/neurological/depts/multiple-sclerosis/ms-approaches/myelin-oligodendrocyte-glycoprotein-antibody-disease
Myelin oligodendrocyte glycoprotein (MOG) antibody associated disorders (MOGAD) are a spectrum of idiopathic, inflammatory, demyelinating diseases affecting the central nervous system (CNS). […] Several distinct clinical phenotypes have been identified in MOGAD including optic neuritis, myelitis, brainstem syndrome, acute dissemination encephalomyelitis, and cortical encephalitis. MOGAD may have a monophasic or relapsing course and prognosis highly depends on the clinical phenotype and unlike MS progression is not typically seen. […] Optic neuritis is the most common presentation of MOGAD at disease onset occurring in approximately 60% of patients. […] The typical presentation of myelitis in MOGAD patients is longitudinally extensive transverse myelitis (LETM) with involvement of greater than 3 vertebral segments and commonly involved areas include cervical and thoracic spinal cord.
- #3 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD): Clinical features and diagnosis – UpToDatehttps://www.uptodate.com/contents/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-mogad-clinical-features-and-diagnosis/print
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory disorder of the central nervous system characterized by attacks of immune-mediated demyelination predominantly targeting the optic nerves, brain, and spinal cord. The disease has a predilection for children. […] Characteristic attacks â While none of the clinical features of MOGAD are disease-specific, some are highly characteristic. These include acute attacks of: Unilateral or bilateral optic neuritis resulting in severe visual loss; Acute disseminated encephalomyelitis (ADEM), resulting in altered mental status, focal neurologic features, and features of transverse myelitis; Transverse myelitis, often causing limb weakness, sensory loss, and bowel, bladder, or sexual dysfunction. MOGAD may have a monophasic or relapsing course.
- #4 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-mogad?lang=us
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) represents a group of inflammatory demyelinating disorders united by the presence of IgG antibodies to myelin oligodendrocyte glycoprotein (MOG). MOGAD represents a distinct clinical entity that clinically overlaps but is nonetheless separate from acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS). […] MOGAD is primarily encountered in children and young adults with an estimated incidence of between 1.6 and 3.4 per 1,000,000 person-years and a median age onset between 20-30 years. […] Additionally, children with MOGAD are more likely to present with an ADEM-like clinical picture, whereas adults are more likely to present with an NMOSD-like syndrome.
- #5 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/mogad/symptoms-causes/syc-20560476
Myelin oligodendrocyte glycoprotein antibody-associated disease, also known as MOGAD, is a rare inflammatory disease that affects the central nervous system. […] Symptoms of MOGAD may include vision loss, muscle weakness, stiffness or paralysis, confusion, seizures, and headaches. […] MOGAD causes painful swelling, known as inflammation. Symptoms are caused by attacks from: […] Inflammation of the optic nerve. Called optic neuritis, this condition may lead to vision loss in one or both eyes and eye pain that gets worse with eye movement. […] Inflammation of the spinal cord. Called transverse myelitis, this condition may lead to arm or leg weakness, muscle stiffness, or paralysis. […] Inflammation of the brain and spinal cord. Called acute disseminated encephalomyelitis, also known as ADEM, this condition may lead to vision loss, weakness, unsteady walk and confusion.
- #6https://www.kuh.ku.edu.tr/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-mogad
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare inflammatory disease that affects the central nervous system. Symptoms of MOGAD may include vision loss, muscle weakness, stiffness or paralysis, confusion, seizures, and headaches. These symptoms can be sometimes confused with other diseases such as multiple sclerosis. MOGAD causes painful swelling, known as inflammation. Symptoms are caused by attacks from: […] Inflammation of the optic nerve. Called optic neuritis, this condition may lead to vision loss in one or both eyes and eye pain that gets worse with eye movement. Inflammation of the spinal cord. Called transverse myelitis, this condition may lead to arm or leg weakness, muscle stiffness, or paralysis. It also may cause sensory loss and changes in bowel, bladder or sexual function. Inflammation of the brain and spinal cord. Called acute disseminated encephalomyelitis, also known as ADEM, this condition may lead to vision loss, weakness, unsteady walk and confusion. ADEM is more common in children with MOGAD. Other symptoms of MOGAD may include: Seizures. Headaches. Fever. Some people who have MOGAD only experience one attack of symptoms. This is called monophasic MOGAD and is slightly more common. However, some people experience multiple attacks, called relapsing MOGAD. Attacks usually develop over days and can be severe and debilitating. Disability usually gets worse with each attack. Attack recovery can take weeks to months.
- #7 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD): Clinical features and diagnosis – UpToDatehttps://www.uptodate.com/contents/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-mogad-clinical-features-and-diagnosis/print
Attacks usually develop over days and may plateau with variable recovery over weeks to months. […] Optic neuritis is the most common clinical manifestation of MOGAD at onset and is even more predominant during relapses. […] The recovery from MOGAD is typically good, with just 6 to 14 percent of MOGAD patients having a residual visual acuity 20/200 or worse in comparison with a third in AQP4-IgG NMOSD, although residual optic disc pallor is common in MOGAD. […] Transverse myelitis is defined as inflammation across the spinal cord parenchyma leading to neurologic dysfunction that develops over hours or days and reaches its nadir (maximal deficit) within 21 days. […] Spinal cord involvement in MOGAD may occur as an isolated attack or in conjunction with involvement in other regions of the CNS (eg, ADEM, optic neuritis).
- #8 MS Minute: Myelin Oligodendrocyte Glycoprotein AntibodyâAssociated Diseasehttps://practicalneurology.com/articles/2024-nov/ms-minute-myelin-oligodendrocyte-glycoprotein-antibody-associated-disease
MOGAD, an inflammatory demyelinating disease of the central nervous system, has some overlapping clinical features with NMOSD and MS but is a distinct entity with unique pathologic, clinical, and imaging features. […] Myelin oligodendrocyte glycoprotein (MOG) antibodyassociated disease (MOGAD) is an inflammatory demyelinating disease of the central nervous system (CNS) associated with MOG immunoglobulin G (MOG-IgG). […] Optic neuritis is the most common clinical manifestation of MOGAD in adults and in some pediatric series. […] Myelitis is the second most common manifestation of MOGAD in adults. […] Brainstem or cerebellar syndromes may also occur in about one-third of individuals with MOGAD, typically in combination with involvement of other regions. […] Cerebral cortical encephalitis is a recently described MOGAD phenotype that seems to be more frequent in children.
- #9 MOGAD Disorder and Multiple Sclerosis | National MS SocietyNational Multiple Sclerosis Society LogoNational Multiple Sclerosis Society LogoOpen searchExpand SectionExpand SectionExpand SectionExpand SectionExpand Sectionhttps://www.nationalmssociety.org/understanding-ms/what-is-ms/related-conditions/mogad
Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is an autoimmune disorder that shares some symptoms with multiple sclerosis and may be misdiagnosed as MS. It is associated with the presence of antibodies directed against myelin oligodendrocyte glycoprotein (MOG). MOG is found in the myelin that insulates the nerves of the central nervous system, which consists of the brain, spinal cord and optic nerves. The antibody attack on MOG disrupts the transmission of nerve signals in the body and causes a variety of symptoms. […] Changes in vision (optic neuritis) and symptoms caused by spinal cord inflammation (transverse myelitis) are common in MOGAD. Symptoms caused by optic neuritis include: Loss or blurring of vision in one or both eyes, Loss of color vision, Eye pain. Symptoms caused by transverse myelitis include: Weakness, numbness or loss of sensation, Paralysis, Loss of bowel or bladder control or difficulty emptying the bladder, Spasticity (increased muscle tone or stiffness in the arms or legs), Shooting pain or tingling in the neck, back or abdomen. Children with MOGAD may develop acute disseminated encephalomyelitis (ADEM). This is rare in adults.
- #10 Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: A Case Reporthttps://pmc.ncbi.nlm.nih.gov/articles/PMC10996974/
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a newly discovered autoimmune demyelinating disorder. The clinical manifestations of MOGAD are divergent but often characterized by inflammatory central nervous system (CNS) deficits such as optic neuritis, encephalitis, or transverse myelitis that predominantly affect the pediatric population. Despite the distinct features often associated with MOGAD, the disease exhibits a diverse range of clinical manifestations, making timely diagnosis and treatment challenging. In particular, distinguishing MOGAD from multiple sclerosis (MS) is important for adequate treatment and the prevention of relapsing disease. […] The most common clinical manifestation of MOGAD is MOGAD-associated encephalitis, with optic neuritis being the second. Of note, optic neuritis in MOGAD differs from MS as it tends to be bilateral and leads to severe visual deficits if left untreated.
- #11 Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: A Case Reporthttps://pmc.ncbi.nlm.nih.gov/articles/PMC10996974/
Many clinical features of MOGAD are characteristic but not disease-specific, posing challenges for accurate and timely diagnosis. Delays in diagnosis in patients with persistent MOG-IgG seropositivity potentiate an increased risk of recurrence, necessitating prompt diagnosis for a better prognosis. Common clinical manifestations include unilateral or bilateral optic neuritis, with up to 50% of patients experiencing optic neuritis bilaterally. Optic neuritis often presents with varying vision loss and is pathognomonically described as eye pain that worsens with eye movement, sometimes preceding complaints of vision loss. […] However, intracranial hypertension is a rare presentation of MOGAD and has been sparsely reported. Increased cerebrospinal fluid (CSF) in the inflammatory context is more commonly associated with manifestations such as acute disseminated encephalomyelitis or meningoencephalitis.
- #12 MOG antibody-associated optic neuritis | Eyehttps://www.nature.com/articles/s41433-024-03108-y
Optic neuritis (ON) is the most common initial manifestation of MOGAD in adults (~30-60%), followed by transverse myelitis (~10-25%), and is the second most common manifestation in children, after acute disseminated encephalomyelitis (ADEM; ~45% in children 11 years of age vs. 5% in adults). […] MOG-ON presents with typical symptoms of acute ON such as pain and loss of visual acuity. However, certain features, such as bilateral involvement, optic disc swelling and optic perineuritis are characteristic and favour the diagnosis of MOG-ON over other demyelinating aetiologies such as NMOSD and MS. […] The presence of severe optic disc swelling and peripapillary haemorrhage in the setting of ON is highly suggestive of MOG-ON. […] Eye pain may be common in MOG-ON, reported in 73-92% of cases internationally.
- #13 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD): Clinical features and diagnosis – UpToDatehttps://www.uptodate.com/contents/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-mogad-clinical-features-and-diagnosis
Attacks usually develop over days and may plateau with variable recovery over weeks to months. […] Other central nervous system (CNS) involvement may occur, including the clinical syndrome of neuromyelitis optica spectrum disorder (NMOSD) without aquaporin-4-immunoglobulin G (AQP4-IgG) detected. […] Optic neuritis is the most common clinical manifestation of MOGAD at onset and is even more predominant during relapses. […] Optic neuritis presents with varying degrees of vision loss and is almost always associated with eye pain that worsens with movement of the eye. […] The recovery from MOGAD is typically good, with just 6 to 14 percent of MOGAD patients having a residual visual acuity 20/200 or worse in comparison with a third in AQP4-IgG NMOSD, although residual optic disc pallor is common in MOGAD.
- #14 MOGAD: What It Is, Diagnosis, Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/myelin-oligodendrocyte-glycoprotein-antibody-disease-mogad
Spinal cord symptoms (transverse myelitis) include: Muscle weakness, Numbness, Bowel or bladder control issues, Difficulty emptying your bladder, Stiff muscles (spasticity), A sharp, shooting pain or a tingling sensation in your neck, back or abdominal region. […] Symptoms that affect your brain (also known as ADEM) include: Confusion, Loss of balance and coordination, Headache, Behavioral changes, Loss of consciousness, Seizures. […] MOGAD symptoms come and go. You may experience periods without any symptoms before symptoms appear again (relapse). […] Among children, MOGAD most often targets the brain (ADEM), which causes symptoms like confusion and a loss of coordination. It will sometimes accompany optic neuritis. […] In adults, MOGAD targets your spinal cord and eyes to cause symptoms like weakness or numbness and blurry vision.
- #15 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD): Clinical features and diagnosis – UpToDatehttps://www.uptodate.com/contents/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-mogad-clinical-features-and-diagnosis/print
The severity of the myelitis is more than that seen with MS, and up to one-third will be nonambulatory at nadir. […] In approximately three-quarters of patients, the lesions accompanying a myelitis on sagittal T2-weighted magnetic resonance imaging (MRI) are three or more vertebral segments in length, which is termed longitudinally extensive transverse myelitis (LETM); these are usually located centrally in the spinal cord. […] ADEM is the most common initial presentation of MOGAD in children, noted in 68 percent of all MOG-positive pediatric cases. […] While ADEM is usually monophasic, a subset of patients develop relapsing disease with multiple separate ADEM attacks known as multiphasic ADEM; historically, many of these patients may have had MOGAD. […] Neurologic deficits with MOGAD may be monofocal or polyfocal and develop over hours to days.
- #16 Myelin Oligodendrocyte Antibody Disease Fact Sheet | Cleveland Clinichttps://my.clevelandclinic.org/departments/neurological/depts/multiple-sclerosis/ms-approaches/myelin-oligodendrocyte-glycoprotein-antibody-disease
Symptoms will vary based on the affected levels and include limb weakness and sensory changes, bowel or bladder dysfunction, and spasticity. […] In the pediatric population, ADEM is considered the most common presenting phenotype in MOGAD occurring in approximately 68% of patients. […] Brainstem involvement is another frequent finding in MOGAD occurring in approximately 30% of MOGAD patients. […] Although rare, meningoencephalitis and encephalitic presentations of MOGAD can also occur. […] Another rare phenotype in MOGAD is steroid-responsive aseptic meningitis typically seen with or without leptomeningeal enhancement. […] Generally, most patients are expected to have favorable outcome with expanded disability status scale (EDSS) 3 and more favorable compared to NMOSD. […] Overall, prediction of risk of relapse in MOGAD remains challenging and there is conflicting data surrounding relationship between MOGAD seropositivity over time and risk of future relapses.
- #17 Myelin Oligodendrocyte Glycoprotein-Antibody Associated Disease: An Updated Review of the Clinical Spectrum, Pathogenetic Mechanisms and Therapeutic Managementhttps://www.mdpi.com/2073-4468/13/2/43
Optic neuritis is the most common initial manifestation of MOGAD in adults (50%). Approximately 80% of MOGAD patients will develop optic neuritis at some point over the course of the disease. It is characterized by varying degrees of vision loss, and is nearly always accompanied by eye pain that worsens with eye movement, which often precedes vision loss. […] Around 30% of adults with MOGAD will present with transverse myelitis. Symptoms include weakness that can result in paraparesis or quadriparesis, and sensory loss below the level of the lesion with a sensory level across the trunk and prominent bladder (urinary retention requiring catheterization) and bowel involvement. […] ADEM is defined as a clinical syndrome marked by an initial polyfocal episode within the CNS, presumed to result from demyelination. ADEM typically features encephalopathy, which is not attributable to fever, systemic illness, or postictal phenomena.
- #18 MRI features of myelin oligodendrocyte glycoprotein antibody disease: a descriptive studyâhow it differs from neuromyelitis optica spectrum disorders and multiple sclerosis | Egyptian Journal of Radiology and Nuclear Medicine | Full Texthttps://ejrnm.springeropen.com/articles/10.1186/s43055-023-01066-1
Typical spinal MRI findings in our cohort of MOGAD patients include centrally located, T2 hyperintense, longitudinally extensive lesions involving both grey and white matter, with involvement of more than 50% of the axial section of the cord, and cord swelling. […] In our cohort, seven patients (78%) had longitudinally extensive transverse myelitis (LETM), while six patients (67%) had short-segment spinal cord lesions (SSTM). […] Patients with MOGAD who underwent orbital MRI revealed oedematous, enlarged, and tortuous optic nerves, with bilateral long or short segments of abnormal T2 hyperintense signal that typically involved anterior segments of the optic nerves while sparing the optic chiasm and retro-chiasmatic pathways. […] The average length of the optic nerve lesion was 19.1 mm. Five out of eight patients (63%) had longitudinally extensive lesions. […] We could identify numerous characteristic radiological features that could help distinguish MOGAD from NMOSD and MS.
- #19 The diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in childrenhttps://www.explorationpub.com/Journals/ent/Article/100469
The diagnosis basically depends on the detection of anti-MOG antibodies in the presence of clinical findings consistent with MOGAD. […] Core clinical presentations suggested by the most recent International MOGAD Panel proposed criteria include ON, myelopathy, ADEM, cerebral monofocal or polyfocal deficits, brainstem or cerebellar deficits, and cortical encephalitis. […] ADEM is the most common presenting phenotype of MOGAD in children (40%), whereas it accounts for only 3% of adult patients with MOGAD. […] Although it is mostly a monophasic disease, some children may have further attacks of ADEM (M-ADEM) or ON (ADEM-ON) in the following months or years. […] ON can be limited to the optic nerve (isolated ON); or it can be the first episode of other CNS demyelinating disorders such as ADEM, MS, NMOSD, or a part of the clinical picture of these disorders; or an initial manifestation of various connective tissue or neurometabolic disorders such as systemic lupus erithematosus or mitochondrial disorders.
- #20 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) | Altru Health Systemhttps://www.altru.org/health-library/conditions/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-mogad
Myelin oligodendrocyte glycoprotein antibody-associated disease, also known as MOGAD, is a rare inflammatory disease that affects the central nervous system. […] Symptoms of MOGAD may include vision loss, muscle weakness, stiffness or paralysis, confusion, seizures, and headaches. […] MOGAD causes painful swelling, known as inflammation. Symptoms are caused by attacks from: […] Inflammation of the optic nerve. Called optic neuritis, this condition may lead to vision loss in one or both eyes and eye pain that gets worse with eye movement. […] Inflammation of the spinal cord. Called transverse myelitis, this condition may lead to arm or leg weakness, muscle stiffness, or paralysis. […] Inflammation of the brain and spinal cord. Called acute disseminated encephalomyelitis, also known as ADEM, this condition may lead to vision loss, weakness, unsteady walk and confusion.
- #21 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD): Clinical features and diagnosis – UpToDatehttps://www.uptodate.com/contents/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-mogad-clinical-features-and-diagnosis/print
Cerebral cortical encephalitis is now recognized as a characteristic feature of MOGAD. […] In some patients with MOGAD, seizures may be the presenting feature; this occurs most often in children with cerebral cortical encephalitis or ADEM. […] Seizures occur more frequently with MOGAD than AQP4-IgG NMOSD. […] MOGAD differs from MS and AQP4-IgG NMOSD in that a large proportion of patients (40 to 50 percent) have a single attack without recurrence, what is termed a monophasic course. Identifying patients destined to have a monophasic course is crucial to avoid using attack-prevention immunosuppressant medications in those destined never to relapse. […] The exact frequency of a monophasic course is not yet known with this newly identified disease, as longer follow-up is needed to better determine the relapse risk.
- #22 MOG Antibody Disease (MOGAD) | Signs & Symptoms | SRNAhttps://wearesrna.org/living-with-myelitis/disease-information/mog-antibody-disease/signs-symptoms/
Brainstem or cerebellar deficits (inflammation in the back or bottom of the brain), which often includes double vision or issues with balance and coordination […] Cerebral cortical encephalitis (inflammation in the outer layer of the brain), which often causes seizures, changes in thinking or behavior, stroke-like episodes, headache, or fever.
- #23 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/mogad/symptoms-causes/syc-20560476
Other symptoms of MOGAD may include: Seizures. Headaches. Fever. […] Some people who have MOGAD only experience one attack of symptoms. This is called monophasic MOGAD and is slightly more common. However, some people experience multiple attacks, called relapsing MOGAD. […] Attacks usually develop over days and can be severe and debilitating. Disability usually gets worse with each attack. Attack recovery can take weeks to months.
- #24 Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) // Middlesex Healthhttps://middlesexhealth.org/learning-center/diseases-and-conditions/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-mogad
Other symptoms of MOGAD may include seizures, headaches, and fever. […] Some people who have MOGAD only experience one attack of symptoms. This is called monophasic MOGAD and is slightly more common. However, some people experience multiple attacks, called relapsing MOGAD. Attacks usually develop over days and can be severe and debilitating. Disability usually gets worse with each attack. Attack recovery can take weeks to months.
- #25 Mayo Clinic Health Library – Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) | Swiss Medical Networkhttps://www.swissmedical.net/en/healtcare-library/con-20557675
Inflammation of the brain and spinal cord. Called acute disseminated encephalomyelitis, also known as ADEM, this condition may lead to vision loss, weakness, unsteady walk and confusion. ADEM is more common in children with MOGAD. […] Other symptoms of MOGAD may include seizures, headaches, and fever. […] Some people who have MOGAD only experience one attack of symptoms. This is called monophasic MOGAD and is slightly more common. However, some people experience multiple attacks, called relapsing MOGAD. Attacks usually develop over days and can be severe and debilitating. Disability usually gets worse with each attack. Attack recovery can take weeks to months.
- #26 The diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in childrenhttps://www.explorationpub.com/Journals/ent/Article/100469
Over the last two decades, immunoglobulin G (IgG) antibodies against myelin oligodendrocyte glycoprotein (MOG), previously thought to be a biomarker of multiple sclerosis (MS), have been shown to cause a distinct disease called MOG antibody-associated disease (MOGAD). MOGAD accounts for approximately one-third of all demyelinating syndromes in children and is the second most common central nervous system (CNS) demyelinating disease after MS. […] Clinical presentations of MOGAD vary by age; the highest rates are seen in acute disseminated encephalomyelitis in younger children and optic neuritis, myelitis, or brainstem symptoms in older children. […] Although it was previously thought to be a milder demyelinating disorder and to have a monophasic course in the majority of patients, recent studies have shown that relapses occur in about half of the patients and sequelae develop in a significant proportion of them, especially in those with persistently high antibody titers, leukodystrophy-like magnetic resonance imaging (MRI) lesions, and spinal cord involvement.
- #27https://link.springer.com/article/10.1007/s00247-024-06023-2
The MOGAD phenotype is age dependent, which might reflect variability in MOG expression at different age groups. Young children (â¤1 year) tend to present with ADEM phenotype, while older patients (>1 year) present more commonly with optic neuritis. […] The severity of attacks and degree of recovery are also age dependent, with younger children presenting with worst clinical-radiological severity, but with faster and more complete recovery. […] MOG antibody seropositivity is reported in 37% of children presenting with optic neuritis. Clinically they may complain of severe new-onset headache, followed by painful eye movements and severe visual deficits at nadir. […] MOG antibody seropositivity is reported in 13% of children presenting with transverse myelitis at initial attack. Presentation is often severe with paraparesis and sphincter dysfunction, being reported in 45/54 (83%) mixed pediatric and adult MOGAD cohorts.
- #28 Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: Current Insights into the Disease Pathophysiology, Diagnosis and Managementhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7795410/
Myelin oligodendrocyte glycoprotein (MOG)-associated disease (MOGAD) is a rare, antibody-mediated inflammatory demyelinating disorder of the central nervous system (CNS) with various phenotypes starting from optic neuritis, via transverse myelitis to acute demyelinating encephalomyelitis (ADEM) and cortical encephalitis. […] Optic neuritis seems to be the most frequent presenting phenotype in adults and ADEM in children. In adults, the disease course is multiphasic and subsequent relapses increase disability. In children ADEM usually presents as a one-time incident. […] In adults with the positive anti-MOG test, ADEM presentation is less frequent, varies from a few up to 18% of cases. Ongoing myelin development and compaction likely have an important influence on ADEM’s clinical picture of MOG antibody disease. As mentioned, the presentation of this condition in children is mostly ADEM or ADEM-like (multiphasic disseminated encephalomyelitis (MDEM), ADEM-optic neuritis) and evolves with age to optico-spinal syndrome (optic neuritis, myelitis and brainstem encephalitis) in adults.
- #29 Myelin Oligodendrocyte Glycoprotein-Antibody Associated Disease: An Updated Review of the Clinical Spectrum, Pathogenetic Mechanisms and Therapeutic Managementhttps://www.mdpi.com/2073-4468/13/2/43
Clinical syndromes associated with antibodies against myelin oligodendrocyte glycoprotein (MOG) are now recognized as a distinct neurological disease entity, and are gaining increasing attention. […] MOGAD may manifest with a variety of clinical syndromes, including acute disseminated encephalomyelitis (ADEM), autoimmune encephalitis, optic neuritis (ON) and transverse myelitis (TM). MOGAD can be either monophasic or relapsing. […] Limited epidemiological data on MOGAD exist, primarily due to the recent discovery of myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) in 2007, with widespread testing becoming available only years to a decade later. […] MOGAD associated syndromes are summarized in Table 1. Similarly, to frequency, the disease clinical phenotype largely depends on the age of the patient. Children are more prone to develop an encephalopathic syndrome (50%), which manifests mostly as ADEM with or without optic neuritis. On the other hand, adults most frequently present with optic neuritis (50%) or transverse myelitis (30%).
- #30 Pediatric Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) – EyeWikihttps://eyewiki.org/Pediatric_Myelin_Oligodendrocyte_Glycoprotein_Antibody-Associated_Disease_(MOGAD)
Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) is a demyelinating disease of the central nervous system associated with seropositivity of the MOG antibody. Pediatric MOGAD is more heterogenous than the adult form of the disease, with the differing features of pediatric disease accentuated in younger patients. The most common presentation of MOGAD in children is acute disseminated encephalomyelitis (ADEM), which makes up around 40%-50% of all presenting cases of pediatric MOGAD. This high rate of ADEM in pediatric cases is predominantly seen in children 11 years of age. However, the MOGAD-ON disease course in children is milder compared with the course in adults, and children are more likely to experience complete recovery. Pediatric patients have good recovery, with studies suggesting less long-term disability compared with adults. In pediatric patients, between 75% and 96% were reported to have a complete recovery. Of those patients who present with MOG-ON, there is complete recovery of visual acuity in 56%-73% of patients, which is better than that of adult patients. Long-term risk of relapse is around 35% but relapse rates in children are lower than that in adults. Early relapses within 12 months of initial onset increase the risk of long-term relapsing disease.
- #31 MS Minute: Myelin Oligodendrocyte Glycoprotein AntibodyâAssociated Diseasehttps://practicalneurology.com/articles/2024-nov/ms-minute-myelin-oligodendrocyte-glycoprotein-antibody-associated-disease
The core clinical phenotypes of MOGAD may occur in isolation or as part of a multifocal presentation (eg, simultaneous optic neuritis and myelitis). […] Regardless of the severity of acute symptoms, most individuals experience good recovery of neurologic deficits. However, a small percentage of individuals can have poor long-term outcomes, with residual vision loss, sphincter dysfunction, or cognitive dysfunction. […] Approximately 50% of people with MOGAD will have a relapsing course, and in the other 50%, the disorder will remain monophasic. […] Early relapses in the first year after onset have been associated with further relapses in the long term, although in children, very early relapses within the first 90 days did not predict chronic relapsing disease. […] Persistently positive MOG-IgG titers have been associated with a higher risk of relapses, but relapses also may occur in individuals who become seronegative, and some cases remain monophasic despite positive MOG-IgG.
- #32 The Evolution of Myelin Oligodendrocyte Glycoprotein AntibodyâAssociated Diseasehttps://practicalneurology.com/articles/2023-mar/the-evolution-of-myelin-oligodendrocyte-glycoprotein-antibody-associated-disease
MOGAD is a common cause of isolated TM, especially in young adults. […] In addition to the much more common clinical syndromes mentioned previously, MOGAD also can present with less common clinical phenotypes. […] Prognosis after the incident event and risk of long-term relapse are a function of age. […] The risk of relapse in MOGAD is variable, but likely up to 50% of people with MOGAD will not have a relapse. […] Many individuals with MOGAD may benefit from long-term immunotherapy, and further insight into the precise pathophysiology of MOGAD will be critical in helping identify the most effective treatments to reduce the risk of future neurologic disability.
- #33 Myelin oligodendrocyte glycoprotein antibody-associated disease â Overview of Information and Clinical Researchhttps://clinicaltrials.eu/disease/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease/
It is important to note that each treatment option comes with potential side effects. Long-term use of certain medications may lead to complications such as infections, lymphoma, skin cancers, headaches, and kidney failure. […] Living with MOGAD involves managing symptoms and reducing the likelihood of relapses. While up to 50% of individuals may experience a monophasic course, meaning they have only one attack, others may have multiple relapses. […] Early diagnosis of MOGAD is crucial for effective management. Unlike MS, where residual T2 lesions are common, MOGAD may present with a normal MRI despite multiple relapses. […] The prognosis for individuals with MOGAD is generally favorable. Most patients experience a milder disease course compared to NMOSD, with less severe disability. […] Despite the absence of a cure, rehabilitation programs and support networks play a vital role in enhancing the quality of life for patients. The importance of early diagnosis cannot be overstated, as it allows for timely intervention and better management of the disease.
- #34 Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: Current Insights into the Disease Pathophysiology, Diagnosis and Managementhttps://www.mdpi.com/1422-0067/22/1/100
Cortical inflammation associated with the MOGAD manifests mostly with epileptic seizures, sometimes also with disturbances of consciousness and behavior. […] In general, patients suffer from the above presented isolated syndrome, but sometimes a combination occurs: TM + ON, brainstem + cortical encephalitis, brainstem encephalitis + ON, ADEM + ON. […] MOGAD appeared to have an earlier age of onset compared to NMO. Finally, despite a frequently relapsing disease course, patients with MOGAD had an overall milder disease course compared to NMO patients. […] Further studies with follow-up have shown that in adults the disease usually has multiple attacks. Relapses were most frequent in patients who presented ON symptoms and occurred in the months up to 1â2 years after the onset attack.
- #35 Myelin Oligodendrocyte Glycoprotein-Antibody Associated Disease: An Updated Review of the Clinical Spectrum, Pathogenetic Mechanisms and Therapeutic Managementhttps://www.mdpi.com/2073-4468/13/2/43
Studies have shown that MOGAD disease course is heterogenous. In the past, it was believed that MOGAD is a monophasic disease. However, we now know that MOGAD can be relapsing in approximately 35% of the cases. […] High MOG-antibody titers on presentation and MOG-antibody persistence favor a relapsing course and worse outcomes, whereas low titers and seroconversion to negativity indicate increased likelihood of a monophasic and milder disease course.
- #36 MOGAD: What It Is, Diagnosis, Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/myelin-oligodendrocyte-glycoprotein-antibody-disease-mogad
Damage to myelin can permanently affect how your body functions. You may experience the following complications: Blindness, Bladder or bowel dysfunction, Paralysis (arms and legs), Chronic pain, Chronic fatigue, Cognitive impairment (difficulty learning, remembering and thinking). […] It can be difficult to live with and manage MOGAD on your own. When symptoms happen, it can pull you away from activities you enjoy and leave you feeling helpless. Because of this, many people experience depression with MOGAD.
- #37 Myelin-oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) – Child Neurology Foundationhttps://www.childneurologyfoundation.org/disorder/myelin-oligodendrocyte-glycoprotein-antibody-disease-mogad/
Transverse myelitis is inflammation of the spinal cord. Symptoms may include changes in sensation, changes in motor function, and problems with bladder and/or bowel control. Symptoms depend on the part of the spinal cord that is affected. Recurrence is rare in people who presented with transverse myelitis as the only symptom. […] MOGAD may only happen once, or it may be relapsing. Relapses are most common in the first six months after the first attack. Relapses are also more common if MOG antibodies are positive. Adults are more likely to have relapses than children. […] Most children with ADEM will have a complete recovery. However, sometimes the lesions on the MRI will not completely resolve. Children who have more extensive involvement on MRI have poor recovery. […] Most children with optic neuritis will have good recovery of their vision. Adults are less likely to have a good recovery from optic neuritis. […] Transverse myelitis typically fully resolves. However, people may continue to have problems with bladder and bowel control.
- #38 Myelin Oligodendrocyte Antibody Disease Fact Sheet | Cleveland Clinichttps://my.clevelandclinic.org/departments/neurological/depts/multiple-sclerosis/ms-approaches/myelin-oligodendrocyte-glycoprotein-antibody-disease
Age of onset can be an indicator in predicting risk of relapse. Earlier and later age of onset (18 years and 50 years respectively) can both be associated with a more monophasic course compared to a relapsing course. […] Patients with optic neuritis have a higher risk of relapse as opposed to those with transverse myelitis or ADEM.
- #39 MOG Antibody Disease Information – MyMyelitishttps://mymyelitis.com/mog-antibody-disease/information/
The impact of attacks on disability seems to vary, with studies reporting no differences between one and multiple attacks. However, other studies report worsening disability when more attacks occur. […] According to case-based studies, those presenting with Transverse Myelitis are most likely to be left with long-term disability. Some cases also report residual disability developing in 50-80% of MOGAD patients.
- #40 Myelin Oligodendrocyte Glycoprotein Antibody Diseasehttps://www.cincinnatichildrens.org/health/m/mogad
MOGAD Symptoms […] Common symptoms for MOGAD in children include: Eye problems, such as double vision, pain when moving the eyes and vision loss. Difficulty processing information, organizing, planning and decision-making. Weakness, tingling and numbness. Problems with balance and coordination. Tremors (shaking). Muscle stiffness and spasms, which can be painful. Problems with bowel and bladder control. Slurred speech. […] Other symptoms of MOGAD include: Trouble with memory and paying attention. Seizures. Fatigue. […] Some children may only have one MOG attack and recover well with acute therapy. Some may have residual symptoms after an attack. The risk of having another MOG attack after a first event is around 40-50%. With treatment and prevention of relapses, most children do not develop significant disability from MOGAD.
- #41 Pediatric anti-MOG antibody associated disorder (MOGAD) – Children’s Health Neurologyhttps://www.childrens.com/specialties-services/conditions/antibody-associated-disorder
Pediatric anti-MOG antibody associated disorder (MOGAD) is an autoimmune disorder of the central nervous system responsible for inflammation in the brain, spinal cord and/or optic nerves. This condition impacts both adults and children but is more common in children. […] The signs and symptoms of anti-MOG associated disease are based on which part of the nervous system is affected. […] Children presenting with ADEM or autoimmune encephalitis may have confusion, a decreased level of alertness, seizures, behavioral changes, numbness, weakness or difficulty walking. […] With optic neuritis children will have vision changes and pain around the eyes. […] Transverse myelitis can cause changes in strength, sensation, walking and bowel/bladder function.
- #42 The diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in childrenhttps://www.explorationpub.com/Journals/ent/Article/100469
In a previous study, 21% of children with any demyelinating disease were found to have TM at the first clinical episode; 72% of these patients remained monophasic. […] Data on recurrence risk and prognosis of patients with MOGAD are highly variable and contradictory, as follow-up durations are highly heterogeneous in studies. […] Although previous studies with relatively short follow-up periods reported a monophasic course in most of the MOGAD patients, recent studies with longer follow-up periods demonstrated a multiphasic course in more than half of the patients. […] In summary, relapses occur in about half of children diagnosed with MOGAD, and permanent sequelae include blindness, loss of ambulation, genitourinary, and cognitive deficits in the long term.
- #43 Advancing Expertise in the Understanding of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: Masterclass Event Summary – European Medical Journalhttps://www.emjreviews.com/neurology/symposium/advancing-expertise-in-the-understanding-of-myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-masterclass-event-summary-s110224/
In MOGAD, attacks are often severe, but usually respond to corticosteroid treatment, especially if given early. […] Red flags indicative of a diagnosis other than MOGAD include progressive neurological impairment in the absence of attacks, lack of efficacy for high-dose corticosteroids in acute attacks, fulfilment of the MS MRI criteria, silent lesions outside of relapse, and lesion contrast enhancement that persists for 6 months. […] There is currently no approved treatment for MOGAD. The speakers explained that management has three main aims: resolving acute attacks, preventing early rebound attacks, and the long-term management of disease. […] Together, such results suggest a reduction of MOGAD relapses during pregnancy, and a potential modest impact during the first 3 months following delivery. Outcomes for mothers with MOGAD and their offspring appear to be within the range reported for those without the disease.
- #44 Less common phenotypes of myelin oligodendrocyte glycoprotein antibody-related diseases in children deserve more attention | Pediatric Researchhttps://www.nature.com/articles/s41390-024-03058-x
16 cases (6.8%) presented as cortical encephalitis, with convulsions, headache, and fever as the main symptoms. […] 13 cases (5.5%) showed the overlapping syndrome of MOGAD and anti-N-methyl-D aspartate receptor encephalitis (MNOS), with seizures (92.3%) being the most common clinical symptom. […] The cerebral leukodystrophy-like phenotype was present in seven cases (3.0%), with a recurrence rate of 50%. […] Isolated seizures without any findings on MRI phenotype was present in three cases (1.3%), with the only clinical symptom being seizures of focal origin. […] Three cases (1.3%) of aseptic meningitis phenotype presented with prolonged fever. […] 40/236 (16.9%) of children with MOGAD had less common phenotypes. Less common clinical phenotypes of pediatric MOGAD are susceptible to misdiagnosis and deserve more attention.
- #45 Myelin Oligodendrocyte Glycoprotein Antibody-Associated Meningoencephalitis | Applied Radiologyhttps://appliedradiology.com/Articles/myelin-oligodendrocyte-glycoprotein-antibody-associated-meningoencephalitis
A child presented to the emergency department with a 6-week history of intermittent fevers, weakness, upper abdominal pain, emesis, and headaches with photophobia. […] The MRI findings were suggestive of encephalitis. However, because the patient was not encephalopathic and had a wide range of nonspecific subacute symptoms, MOG antibody-associated disease (MOGAD) was suspected. […] Myelin oligodendrocyte glycoprotein antibody-associated disease is an autoimmune disorder that results from the antibodies directed against a myelin glycoprotein found within the central nervous system (CNS). […] One of the most common presenting symptoms is visual difficulty from optic neuritis. […] When the cerebral hemispheres are affected, an ADEM-like presentation may dominate, while spinal cord involvement may result in signs and symptoms more typical of transverse myelitis.
- #46 Myelin oligodendrocyte glycoprotein antibody-associated disease presenting as unilateral cerebral cortical encephalitis: a case reporthttps://www.encephalitisjournal.org/journal/view.php?number=68
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune disorder with diverse clinical manifestations including myelitis, meningitis, encephalitis, and optic neuritis. […] MOGAD rarely presents with unilateral cerebral cortical encephalitis (CCE), rendering the diagnosis difficult in these cases. […] Herein, we report a case of unilateral CCE with positive anti-myelin oligodendrocyte glycoprotein (MOG) antibodies. […] A 60-year-old female was admitted to the hospital with an acute onset of headache and fever. Neurological examination revealed left-sided homonymous hemianopsia with intermittent visual hallucinations as flickering red-circular spots in the left visual field. […] This case highlights the clinical phenotypes of MOGAD and the need to assess comorbid autoimmune diseases in patients with MOGAD.
- #47 Cognition in patients with myelin oligodendrocyte glycoprotein antibody-associated disease: a prospective, longitudinal, multicentre study of 113 patients (CogniMOG-Study) | Journal of Neurology, Neurosurgery & Psychiatryhttps://jnnp.bmj.com/content/early/2024/07/30/jnnp-2024-333994
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune disease of the central nervous system (CNS) associated with the presence of myelin oligodendrocyte glycoprotein IgG (MOG-IgG) antibodies. The clinical presentation of MOGAD includes optic neuritis, transverse myelitis, brainstem or cerebellar syndromes and different forms of encephalitis. […] Although clinical characteristics have been increasingly analysed in recent years, very few studies have examined the cognitive profile of patients with MOGAD. […] The aim of this study was to systematically investigate the cognitive performance in a large sample of adult MOGAD patients in a prospective, longitudinal and multicentre study design. […] Based on our findings, MOGAD patients perform below average on MuSIC semantic fluency and MuSIC congruent speed compared with normative data from healthy controls. Impairment in these subtests indicates deficits in verbal fluency, mental set shifting and information processing speed.
- #48 Cognition in patients with myelin oligodendrocyte glycoprotein antibody-associated disease: a prospective, longitudinal, multicentre study of 113 patients (CogniMOG-Study) | Journal of Neurology, Neurosurgery & Psychiatryhttps://jnnp.bmj.com/content/96/3/296
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune disease of the central nervous system (CNS) associated with the presence of myelin oligodendrocyte glycoprotein IgG (MOG-IgG) antibodies. The clinical presentation of MOGAD includes optic neuritis, transverse myelitis, brainstem or cerebellar syndromes and different forms of encephalitis. […] Although clinical characteristics have been increasingly analysed in recent years, very few studies have examined the cognitive profile of patients with MOGAD. […] At baseline, the study sample of MOGAD patients showed impaired standardised performance on MuSIC semantic fluency (mean=0.29, 95% CI (0.47 to 0.12)) and MuSIC congruent speed (mean=0.73, 95% CI (1.23 to 0.23)). […] Around 1 in 10 patients showed deficits in two or more cognitive measures (11%).
- #49 Cognition in patients with myelin oligodendrocyte glycoprotein antibody-associated disease: a prospective, longitudinal, multicentre study of 113 patients (CogniMOG-Study) | Journal of Neurology, Neurosurgery & Psychiatryhttps://jnnp.bmj.com/content/early/2024/07/30/jnnp-2024-333994
In our study sample, the prevalence of cognitive deficits in MOGAD was 11% for impaired performance in at least two and 32% for impaired performance in at least one neuropsychological test. […] Accordingly, it can be assumed that cognitive deficits in MOGAD are still rare but may mainly affect the speed of information processing, with an emphasis on the verbal dimension. […] The frequency of MOGAD patients with deficits in neuropsychological tests decreased during the 1-year and 2-year follow-up period. […] Notably, a disease manifestation with cerebral lesions appears to be an important predictor of reduced visuomotor processing speed and semantic fluency.
- #50 Cognition in patients with myelin oligodendrocyte glycoprotein antibody-associated disease: a prospective, longitudinal, multicentre study of 113 patients (CogniMOG-Study) | Journal of Neurology, Neurosurgery & Psychiatryhttps://jnnp.bmj.com/content/96/3/296
Based on these data, we conclude that MOGAD patients show reduced visuomotor processing speed and semantic fluency to the extent that the disease burden includes cerebral lesions. […] This large multicentre study analysed the cognitive performance of MOGAD patients in a longitudinal setting. One in 10 patients with MOGAD (11%) showed neuropsychological deficits, particularly in visuomotor processing speed and semantic fluency. […] Our study is the first to provide a detailed cognitive profile of patients with MOGAD with the aim to better understand the disease burden in MOGAD, better address rehabilitative needs and identify potential neuropsychological endpoints for future treatment trials.
- #51 The diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in childrenhttps://www.explorationpub.com/Journals/ent/Article/100469
Accurate and early diagnosis of MOGAD is essential for proper management and better outcome. […] The incidence of MOGAD, the only recurrent CNS demyelinating disease that is more common in children than adults, is approximately three times higher than in adults. (0.31 vs. 0.13 per 100,000). […] In children, about one-third of all demyelinating syndromes are MOGAD, and it is the second most common demyelinating disease after MS. […] Diagnosis of MOGAD has been challenging due to controversies in antibody detection methodologies and also to the expansion of the clinical spectrum over the years. […] Although several diagnostic criteria have been developed over time by different groups, the International MOGAD Panel has only very recently published the formal consensus diagnostic criteria and underlined the need for validation.
- #52 MS Minute: Myelin Oligodendrocyte Glycoprotein AntibodyâAssociated Diseasehttps://practicalneurology.com/articles/2024-nov/ms-minute-myelin-oligodendrocyte-glycoprotein-antibody-associated-disease
The first line treatment for acute MOGAD attacks is high-dose intravenous methylprednisolone. […] Given that MOGAD can have a monophasic course, maintenance long-term immunosuppression to prevent new attacks is typically reserved for people with relapsing MOGAD. […] MOGAD is an inflammatory demyelinating CNS disorder with distinct clinical and radiologic features.