Pediatrik Multipl Sklerozun Klinik Özellikleri
Özet
Multipl skleroz (MS), santral sinir sisteminin kronik, inflamatuar, demiyelinizan bir hastalığıdır. Pediatrik başlangıçlı MS (PBMS), klinik, radyolojik ve laboratuvar bulguları açısından yetişkin popülasyona göre çeşitli farklılıklar göstermektedir. PBMS vakalarının neredeyse tamamında ataklar ve ataklar arasında iyileşme ve stabil seyir ile karakterize “relapsing-remitting” tip MS gözlenir. Akut relapslar yetişkin başlangıçlı RRMS'ye kıyasla hastalığın erken evrelerinde daha sıktır ancak çoğu çocuk hasta bu erken relapslardan tam olarak iyileşir ve fiziksel dizabilite nadirdir. Çocuklarda engellilik gelişimi erişkin hastalara göre daha yavaş olsa da hastalar uzun hastalık süreleri nedeniyle erişkinlere göre daha erken yaşta ciddi engellilik seviyelerine ulaşabilirler. En sık görülen başlangıç belirtileri, optik nörit, motor disfonksiyon, duyusal semptomlar, ataksi ve beyin sapı semptomlarıdır. Bilişsel bozukluklar, yorgunluk ve psikiyatrik komorbiditeler sık görülür. Beyin sapı tutulumu, ataktan kötü iyileşme ve daha yüksek atak sıklığı, kötü prognostik göstergeler olarak kabul edilir ve kümülatif engellilik olasılığının yüksek olduğunu işaret eder.
Multiple sclerosis (MS) is a chronic, inflammatory, demyelinating disease of the central nervous system. Pediatric-onset MS (POMS) exhibits several differences in clinical, radiological, and laboratory findings compared to the adult MS. In almost all POMS cases, the relapsing-remitting (RRMS) type of MS is observed, characterized by attacks and periods of recovery and stability between attacks. Acute relapses are more frequent in the early stages of the disease compared to adult-onset RRMS, but most pediatric patients recover fully from these early relapses, and physical disability is rare. Although disability progression is slower in children compared to adults, patients may reach severe disability levels earlier in life due to longer disease duration. The most common initial symptoms include optic neuritis, motor dysfunction, sensory symptoms, ataxia, and brainstem symptoms. Cognitive impairment, fatigue, and psychiatric comorbidities are frequently observed. Brainstem involvement, poor recovery from attacks, and a higher frequency of attacks are considered poor prognostic indicators and suggest a higher likelihood of cumulative disability.
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