Primary cerebellar agenesis-chiari IV malformation

Total yada subtotal serebellar agenezi ilk kez 1831'de M.Combettes tarafından tariflenen çok nadir bir durumdur. Serebellum lezyonlarında hareketde ciddi bozukluk gözlenir. Parsiyel serebellum lezyonlarında belirgin bir iyileşme olduğundan dolayı serebellumun normal hareket ile ilgili olmadığı düşünülür. Literatürdeki total serebellar agenezi va-kaları genellikle otopsilerde bildirilmiştir. Burada sunulan vaka, literatürde bizim bildiğimiz yaşayan altıncı vakadır.

Anahtar Kelimeler:

Arnold Chiari malformasyonu, Nöral tüp defektleri, Merkezi sinir sistemi hastalıkları, Sinir sistemi malformasyonları, Çocuk, Manyetik rezonans görüntüleme

Primer serebellar agenezi-chiari IV malformasyonu

Total or subtotal cerebellar agenesis that was described firstly by M. Combettes in 1831 is very rare. Severe dysfunction in movement is observed in the cerebellar lesions. Since there is a significant recovery in movement partial cerebellum lesions, it has been suggested that normal movement is not absolute dependent to cerebellum. But this issue is still controversial. The cases of complete cerebellar agenesis in the literature are usually reported in the autopsy. The study presented here is the sixth living case in the literature according to our knowledge.

Keywords:

Arnold Chiari Malformation, Neural Tube Defects, Central Nervous System Diseases, Nervous System Malformations, Child, Magnetic Resonance Imaging,

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