MRI of a 26-year-old woman with occipital headaches shows peg-like cerebellar tonsils extending 7 mm below the foramen magnum with crowding at the craniocervical junction and a normal-sized posterior fossa. The fourth ventricle and vermis are normally positioned. What is the most likely diagnosis?
- A Chiari II malformation
- B Intracranial hypotension
- C Dandy-Walker malformation
- D Chiari I malformation ✓
Explanation
Tonsillar descent exceeding 5 mm below the foramen magnum in an adult, with a normal posterior fossa, normally positioned fourth ventricle and no vermian or brainstem malformation, defines Chiari I malformation. Chiari II features a small posterior fossa with caudal displacement of the vermis, brainstem and fourth ventricle and is associated with myelomeningocele. Dandy-Walker malformation shows vermian hypoplasia with a cystic fourth ventricle, which is absent here.
Reference: Osborn's Brain, 2nd ed.
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