Yoğun Bakımda Enteral ve Parenteral Beslenme
Özet
Kortikobazal dejenerasyon (KBD), motor semptomlar ve bilişsel etkilenim ile seyreden nadir ve ilerleyici bir nörodejeneratif hastalıktır. Hastalık, tau protein birikimiyle karakterize olup, kortikal ve bazal ganglia yapılarında belirgin dejenerasyonla ilişkilidir. Klinik olarak en sık kortikobazal sendrom (KBS) şeklinde ortaya çıkar ve asimetrik uzuv sertliği, distoni, miyoklonus, apraksi ve yabancı uzuv fenomeni gibi belirtilerle karakterizedir. Tanı, klinik kriterlere ve ileri görüntüleme yöntemlerine dayanırken, hastalık biyobelirteçleri giderek önem kazanmaktadır. Kortikobazal dejenerasyonun etiyolojisinde genetik faktörlerin yanı sıra nöroenflamasyon ve mitokondriyal disfonksiyonun da rol oynadığı düşünülmektedir. Günümüzde hastalığı modifiye edici bir tedavi bulunmamakta olup, yönetim semptomatik ve multidisipliner yaklaşımlarla sağlanmaktadır. Bu bölümde, kortikobazal dejenerasyonun epidemiyolojisi, patofizyolojisi, klinik özellikleri, tanı kriterleri, biyobelirteçleri ve güncel tedavi yaklaşımları ele alınmaktadır.
Corticobasal degeneration (CBD) is a rare and progressive neurodegenerative disease characterized by motor symptoms and cognitive impairment. The disease is associated with tau protein accumulation and significant degeneration in cortical and basal ganglia structures. Clinically, it most commonly presents as corticobasal syndrome (CBS), which is characterized by asymmetric limb rigidity, dystonia, myoclonus, apraxia, and alien limb phenomenon. Diagnosis relies on clinical criteria and advanced imaging techniques, with biomarkers playing an increasingly important role. The etiology of CBD is thought to involve not only genetic factors but also neuroinflammation and mitochondrial dysfunction. Currently, no disease-modifying treatment exists, and management is primarily symptomatic, requiring a multidisciplinary approach. This chapter discusses the epidemiology, pathophysiology, clinical features, diagnostic criteria, biomarkers, and current therapeutic approaches for corticobasal degeneration.
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