Edinsel Demiyelinizan Sendrom: Tek Merkez Deneyimi
Küçük Resim Yok
Tarih
2019
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Amaç: Edinsel demiyelinizan sendromlar, santral sinir sisteminin immün aracılı demiyelinizan bozukluğudur. Klinik spektrum; akut dissemine ensefalomiyelit, optik nörit, transvers miyelit, klinik izole sendrom ve nöromiyelitis optikayı kapsar. Bu çalışmanın amacı, multiple skleroz dışı demiyelinizan hastalık tanısı ile izlenen hastaların klinik özelliklerini ve tedavi deneyimlerimizi sunmaktır. Yöntem: Ocak 2013 ile Ocak 2018 tarihleri arasında Ege Üniversitesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Çocuk Nörolojisi Bilim Dalında takip edilen 30 edinsel demiyelinizan hastalık tanılı hastanın verileri retrospektif olarak incelendi. Demografik veriler, başvuru yakınmaları, klinik bulgular, laboratuvar tetkikleri, nörogörüntüleme sonuçları, oftalmolojik bakı ve uygulanan tedaviler kaydedildi. Bulgular: Hastaların yaşı 3-15 yaş arasında değişmekte olup, yaş ortalaması 8,76±3,59 idi. Akut dissemine ensefalomiyelit en yaygın tanı idi. Başvuru yakınmaları sıklık sırasına göre ataksi, baş ağrısı, ensefalopati, pleji (mono+parapleji), bulanık görme ve görme kaybıydı. Akut enfeksiyon öyküsü, transvers miyelit, akut dissemine ensefalomiyelit ve optik nörit olgularında görüldü. Tüm hastalarda oligoklonal bant ve antiaquaporin-4 antikoru negatif saptandı. Yedi hastada anti-miyelin oligodendrosit antikoru pozitif bulundu. Bu hastalardan birinde hem anti-aquaporin 4 hem de anti-miyelin oligodendrosit antikorları pozitif bulundu. Nörogörüntülemede serebral beyaz cevher, spinal kord ve optik sinir tutulumuyla uyumlu bulgular mevcuttu. Tüm hastalara atak sırasında yüksek doz metilprednizolon tedavisi başlandı ve idame oral steroid ile devam edildi. Relapsları olan hastalarda uzun dönem tedavide azatioprin ve aylık IVIG tedavileri uygulandı. Sonuç: Çocukluk çağı edinsel demiyelinizan hastalıkları multiple sklerozun ilk atağı olabileceğinden hastaların takibi ve ayırıcı tanı için başvuruda ayrıntılı serolojik testlerin, BOS incelemelerinin yapılması önemlidir.
Objective: Acquired demyelinating syndromes are immune-mediated demyelinating disorders of the central nervous system. Clinical spectrum includes acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, clinically isolated syndrome, and neuromyelitis optica. Aim of this study is to present the clinical features of patients followed up with the diagnosis of demyelinating disease other than multiple sclerosis and also our therapeutic experience. Method: Data of 30 patients with acquired demyelinating disease followed up in Ege University Department of Child Neurology between January 2013 and January 2018 were evaluated retrospectively. Demographic data, admission complaints, clinical and neuroimaging findings, laboratory results, ophthalmologic findings and treatments used were recorded. Results: the age of the patients ranged between 3, and 15 years and mean age was 8.76±3.59 years. the most common diagnosis was acute disseminated encephalomyelitis. the most common complaints were ataxia, headache, encephalopathy, plegia (mono+paraplegia), blurred vision and visual loss in order of decreasing frequency. Oligoclonal banding, and anti-aquaporin 4 antibody negativities were detected in all patients. Anti-myelin oligodendrocyte antibody-positivity was found in seven patients. in one of these patients tests were positive for both anti-aquaporin 4 and anti-myelin oligodendrocyte antibodies. Neuroimaging revealed findings consistent with cerebral white matter, spinal cord and optic nerve involvement. All patients were started on intravenous pulse methylprednisolone therapy during the acute episode Conclusion: Since childhood acquired demyelinating diseases may be the first episode of multiple sclerosis, it is important to perform detailed serological tests and CSF examinations for follow-up of patients, and differential diagnosis.
Objective: Acquired demyelinating syndromes are immune-mediated demyelinating disorders of the central nervous system. Clinical spectrum includes acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, clinically isolated syndrome, and neuromyelitis optica. Aim of this study is to present the clinical features of patients followed up with the diagnosis of demyelinating disease other than multiple sclerosis and also our therapeutic experience. Method: Data of 30 patients with acquired demyelinating disease followed up in Ege University Department of Child Neurology between January 2013 and January 2018 were evaluated retrospectively. Demographic data, admission complaints, clinical and neuroimaging findings, laboratory results, ophthalmologic findings and treatments used were recorded. Results: the age of the patients ranged between 3, and 15 years and mean age was 8.76±3.59 years. the most common diagnosis was acute disseminated encephalomyelitis. the most common complaints were ataxia, headache, encephalopathy, plegia (mono+paraplegia), blurred vision and visual loss in order of decreasing frequency. Oligoclonal banding, and anti-aquaporin 4 antibody negativities were detected in all patients. Anti-myelin oligodendrocyte antibody-positivity was found in seven patients. in one of these patients tests were positive for both anti-aquaporin 4 and anti-myelin oligodendrocyte antibodies. Neuroimaging revealed findings consistent with cerebral white matter, spinal cord and optic nerve involvement. All patients were started on intravenous pulse methylprednisolone therapy during the acute episode Conclusion: Since childhood acquired demyelinating diseases may be the first episode of multiple sclerosis, it is important to perform detailed serological tests and CSF examinations for follow-up of patients, and differential diagnosis.
Açıklama
Anahtar Kelimeler
Pediatri
Kaynak
İzmir Dr. Behçet Uz Çocuk Hastanesi Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
9
Sayı
2
