Santral sinir sisteminin multiple skleroz dışı inflamatuar demiyelinizan hastalıklarında görüntüleme bulguları
Küçük Resim Yok
Tarih
2021
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Ege Üniversitesi, Tıp Fakültesi
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Giriş ve Amaç: Merkezi sinir sisteminin inflamatuar demiyelinizan hastalıkları, klinik seyir, görüntülemede lezyon dağılımı ve laboratuvar bulguları temelinde ayırt edilmesi kolay olmayan geniş bir hastalık spektrumunu temsil etmektedir. Bu spektrum esas olarak multipl skleroz (MS), nöromiyelitis optika spektrum bozuklukları (NMOSB), MOG-IgG ilişkili hastalık (MOGAD) ve akut dissemine ensefalomiyeliti (ADEM) içerir. Bu hastalıklar arasında tedavi protokolleri farklı olduğundan, demiyelinizan hastalıkların tanısı çok önemli olup manyetik rezonans görüntüleme (MRG) tanıda önemli bir rol oynamaktadır. Bu çalışmada, lezyonların yerleşim yeri, morfolojik özellikleri, kontrast tutulum varlığı ve kontrast tutulum paterni gibi görüntüleme bulguları ortaya konularak bu hastalıkların birbirinden ayrımında yararlı olabilecek ayırıcı tanı özellikleri araştırılmıştır. Gereç ve Yöntem: Çalışma retrospektif olarak yapılmıştır. Çalışmamıza MS dışı major demiyelinizan hastalık (Nöromiyelitis optika spektrum bozuklukları, MOGAD ve ADEM) tanısı alan 43 olgu dahil edilmiştir. Olguların 13’ ü (%30.2) AQP4-IgG pozitif NMO, 14’ ü (%32.6) seronegatif NMO, 5’ i (%11.6) MOGAD tanılı, 11’ i (%25.6) ADEM tanılı idi. Olguların görüntüleme bulguları PACS veri tabanımızdan retrospektif olarak değerlendirilmiş olup gruplar arası karşılaştırma yapılmıştır. Bulgular: Gruplar arası bulgularda istatistiksel anlamlı farklılık olmamakla birlikte, bazı bulguların çalışmadaki gruplar arası dağılımında farklılıklar izlendi. Çalışmada seropozitif NMO hastalarında lezyonlar tipik olarak AQP4’ ün eksprese edildiği aquaduktus kanalı çevresinde, beyin sapı, periventriküler alan /korpus kallosum ve derin beyaz cevherde bulundu. Ayrıca area postrema tutulumu da en sık seropozitif NMO grubunda görüldü. Seronegatif NMO hastalarında da seropozitif NMO grubuyla benzer lokalizasyonlarda lezyonlar bulundu. Seropozitif NMO grubundan farklı olarak bu grupta jukstakortikal ve subkortikal alanlarda, ayrıca infratentorial bölgede tutulum daha sık görüldü. Seropozitif ve seronegatif NMO hasta gruplarında spinal bölgede servikal ve torasik ağırlıklı, tipik olarak uzun segment, santral yerleşimli ve geniş (aksiyel imajda kordun> %50’ si) tutulum gösteren miyelit bulguları sık görüldü. Seropozitif ve seronegatif NMO hasta gruplarında optik sinir tutulumu tipik olarak uzun segment, en sık intraorbital ve intrakanaliküler segmentlerde ve daha çok unilateral tutulum izlendi. MOGAD hastalarında spinal ve optik sinir tutulumu kranial tutuluma göre daha yüksek oranda görülmektedir. Çalışmaya dahil MOGAD hastalarında servikal ve torakal bölgede, uzun segment, santral yerleşimli ve geniş (aksiyel imajda kordun> %50’ si) tutulum gösteren miyelit bulguları izlendi. Bu çalışmada hasta sayısı az olduğundan MOGAD’ ın optik sinir bulguları değerlendirilemedi. Bu hasta grubunda beyinde periventriküler alanlar, beyin sapı, jukstakortikal ve subkortikal alanlarda istatistiksel anlamlı olmasa da daha sık tutulum görüldü. Ayrıca pediatrik MOGAD hastalarında tipik olarak daha büyük (>2cm) ve yaygın lezyonlar bulundu. Çalışmaya dahil ADEM hastalarında derin beyaz cevher, jukstakortikal ve subkortikal beyaz cevherde, tipik olarak çok sayıda ve diğer demiyelinizan hastalıklara göre daha büyük boyutlu (bazılarında tümefaktif) lezyonlar bulundu. Spinal tutulum olarak genelde servikal ve torakal bölgede, uzun segment, büyük, ödemli ve ekspansil lezyonlar daha sık görüldü. Sonuç: Sonuç olarak, MRG bulguları ile NMOS, MOGAD ve ADEM hastalarının MS’ den ayrımı mümkündür. Ancak kendi aralarında bazı bulgular ayırıcı tanıya yardımcı olsa da kesin ayrım için sadece görüntüleme yetersiz kalmaktadır. Görüntüleme, klinik ve laboratuar bulgular ile doğru ayırıcı tanıya gidilmelidir.
Introduction and Objective: Inflammatory demyelinating diseases of the central nervous system represent a wide spectrum of disorders that are not easy to distinguish on the basis of clinical course, lesion distribution on imaging, and laboratory findings. This spectrum mainly includes multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), MOG associated disease (MOGAD), and acute disseminated encephalomyelitis (ADEM). Differential diagnosis of demyelinating diseases is crucial since treatment protocols differ between these diseases and magnetic resonance imaging (MRI) plays an important role in the diagnosis. In this study, imaging findings such as location of lesions, morphological features, presence of contrast enhancement and contrast enhancement pattern were revealed and imaging features that may be useful in distinguishing these diseases from each other were investigated. Materials and Methods: This study was conducted retrospectively. Our study included 43 cases diagnosed with non-MS major demyelinating disease (NMOSD, MOGAD and ADEM). Of the cases, 13 (30.2%) were diagnosed with AQP4-IgG positive NMO, 14 (32.6%) with seronegative NMO, 5 (11.6%) with MOG associated disease and 11 (25.6%) with ADEM, respectively. The imaging findings of the cases were evaluated retrospectively from our PACS database and comparisons were made between the groups. Findings: Although there was no statistically significant difference in the findings between the groups, differences were observed in the intergroup distribution of some findings in the study. In the study, lesions in seropositive NMO patients were typically found around the cerebral aqueduct, in the brainstem, periventricular area/corpus callosum, and deep white matter, where AQP4 is expressed. Furthermore, area postrema involvement was most commonly seen in the seropositive NMO group. In seronegative NMO patients, lesions were found in similar localizations with the seropositive NMO group. Unlike the seropositive NMO group, the juxtacortical and subcortical areas, as well as the infratentorial region were more commonly involved. In both seropositive and seronegative NMO patients, typically long segment, centrally located, wide (>50% of the cord in the axial image) and mainly cervical and thoracic myelitis findings were commonly observed in the spinal cord. In seropositive and seronegative NMO patients, optic nerve involvement was typically observed in the long segment, most commonly in the intraorbital and intracanalicular segments, and mostly unilaterally. Spinal and optic nerve involvement is seen at a higher rate than cranial involvement in MOGAD patients. In the MOGAD patients included in the study, signs of myelitis were observed in the cervical and thoracic region, with long segment involvement, wide (>50% of the cord in the axial image) and centrally located. In this study, the optic nerve findings of MOGAD could not be evaluated due to the small number of patients. In this group, brainstem, periventricular areas as well as juxtacortical and subcortical areas of the brain were more frequently involved, although not statistically significant. Additionally, larger (>2cm) and widespread lesions were typically found in pediatric MOGAD patients. The ADEM patients included in the study had lesions in the deep white matter, juxtacortical, and subcortical white matter, typically multiple and larger in size (some tumefactive) than other demyelinating diseases. As spinal involvement, predominantly cervical and thoracic, long segment, large, edematous and expansile lesions were more frequently seen. Conclusion: In conclusion, it is possible to distinguish NMOS, MOGAD and ADEM patients from MS with MRI findings. However, although some findings among themselves help in the differential diagnosis, imaging alone is insufficient to make a definitive distinction. Correct differential diagnosis should be made with imaging, clinical and laboratory findings.
Introduction and Objective: Inflammatory demyelinating diseases of the central nervous system represent a wide spectrum of disorders that are not easy to distinguish on the basis of clinical course, lesion distribution on imaging, and laboratory findings. This spectrum mainly includes multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), MOG associated disease (MOGAD), and acute disseminated encephalomyelitis (ADEM). Differential diagnosis of demyelinating diseases is crucial since treatment protocols differ between these diseases and magnetic resonance imaging (MRI) plays an important role in the diagnosis. In this study, imaging findings such as location of lesions, morphological features, presence of contrast enhancement and contrast enhancement pattern were revealed and imaging features that may be useful in distinguishing these diseases from each other were investigated. Materials and Methods: This study was conducted retrospectively. Our study included 43 cases diagnosed with non-MS major demyelinating disease (NMOSD, MOGAD and ADEM). Of the cases, 13 (30.2%) were diagnosed with AQP4-IgG positive NMO, 14 (32.6%) with seronegative NMO, 5 (11.6%) with MOG associated disease and 11 (25.6%) with ADEM, respectively. The imaging findings of the cases were evaluated retrospectively from our PACS database and comparisons were made between the groups. Findings: Although there was no statistically significant difference in the findings between the groups, differences were observed in the intergroup distribution of some findings in the study. In the study, lesions in seropositive NMO patients were typically found around the cerebral aqueduct, in the brainstem, periventricular area/corpus callosum, and deep white matter, where AQP4 is expressed. Furthermore, area postrema involvement was most commonly seen in the seropositive NMO group. In seronegative NMO patients, lesions were found in similar localizations with the seropositive NMO group. Unlike the seropositive NMO group, the juxtacortical and subcortical areas, as well as the infratentorial region were more commonly involved. In both seropositive and seronegative NMO patients, typically long segment, centrally located, wide (>50% of the cord in the axial image) and mainly cervical and thoracic myelitis findings were commonly observed in the spinal cord. In seropositive and seronegative NMO patients, optic nerve involvement was typically observed in the long segment, most commonly in the intraorbital and intracanalicular segments, and mostly unilaterally. Spinal and optic nerve involvement is seen at a higher rate than cranial involvement in MOGAD patients. In the MOGAD patients included in the study, signs of myelitis were observed in the cervical and thoracic region, with long segment involvement, wide (>50% of the cord in the axial image) and centrally located. In this study, the optic nerve findings of MOGAD could not be evaluated due to the small number of patients. In this group, brainstem, periventricular areas as well as juxtacortical and subcortical areas of the brain were more frequently involved, although not statistically significant. Additionally, larger (>2cm) and widespread lesions were typically found in pediatric MOGAD patients. The ADEM patients included in the study had lesions in the deep white matter, juxtacortical, and subcortical white matter, typically multiple and larger in size (some tumefactive) than other demyelinating diseases. As spinal involvement, predominantly cervical and thoracic, long segment, large, edematous and expansile lesions were more frequently seen. Conclusion: In conclusion, it is possible to distinguish NMOS, MOGAD and ADEM patients from MS with MRI findings. However, although some findings among themselves help in the differential diagnosis, imaging alone is insufficient to make a definitive distinction. Correct differential diagnosis should be made with imaging, clinical and laboratory findings.
Açıklama
Anahtar Kelimeler
Nöromiyelitis Optika Spektrum Bozukluğu Hastalıkları, MOG İlişkili Hastalık (MOGAD), Akut Dissemine Ensefalomiyelit (ADEM), Neuromyelitis Optica Spectrum Diseases (NMOSD), MOG Associated Disease (MOGAD), Acute Disseminated Encephalomyelitis (ADEM)
