Çocukluk Çağı Santral Sinir Sistemi Sınıflanmamış Otoimmün İnflamatuvar Demiyelinizan Hastalıklarının Çok Merkezli Retrospektif Araştırılması

Abstract

Demyelinating diseases of the central nervous system (CNS) are distinguished by specific diagnostic criteria, and there may be cases with or without clinical symptoms that do not meet any diagnostic criteria, but in which inflammatory demyelinating lesions are observed on magnetic resonance imaging (MRI). These patients may evolve to a specific diagnostic group over time or remain isolated without progression. The study was multicentre and retrospectively designed and included pediatric patients under 18 years of age. Demographic, clinical and radiological data of the patients were obtained from hospital databases. The study included 20 male (59%) and 14 female (41%) patients. The mean age of the patients was 13.27 ± 5.38 [range 1.8-23, median (med): 14.3]. Six of the patients (18%) exhibited no neurological clinical signs or symptoms, and the lesion was detected incidentally on MRI. The follow-up period was 32.14±25.08 (range 6-74, med:21) months. Patients with polysymptomatic clinics (n=17, 61%) exhibited a higher prevalence of indications for attack treatment (p=0.014) and maintenance treatment (p=0.014) in comparison to those with monosymptomatic clinics (n=11, 39%). Those with poly-focal findings on initial MRI (n=14, 41%) exhibited a more multiphasic clinical course (p=0.016) and required more maintenance treatment (p=0.007). Patients with multiphasic clinics (n=7, 25%) exhibited a higher prevalence of poly-focal lesions on MRI (p=0.016) and received more maintenance therapy (p=0.023) compared to patients with monophasic clinics (n=21, 75%). Patients who do not meet any diagnostic criteria but have been found to have demyelinating lesions on MRI and have polifocal MRI findings at the first clinical attack, patients with tumefactive lesions and those with polysymptomatic clinical findings at the first presentation should be considered as having the potential to exhibit a multiphasic clinical course, necessitating further attacks and maintenance treatment, and resulting in the possibility of residual sequelae.