Three cases of hindbrain herniation in adults with comments on some diagnostic difficulties

Abstract

In discussing the clinical features and surgical treatment of adult patients with Arnold Chiari deformity type I the authors focus on the differential diagnosis from demyelinating disease and present three illustrative cases. Out of 118 patients initially diagnosed as having demyelinating disease and admitted to our hospital between December 1991 and April 1993, three were subsequently found to have the Arnold Chiari deformity on MRI. The delay until correct diagnosis amounted to 10, 16 and 32 years, respectively. Our study stresses the diagnostic importance of MRI of the craniocervical area in cases of unexplained neurological symptoms and external signs of dysplasia. Accurate examination, early diagnosis and selection of proper surgical management can alleviate severe symptoms in many instances and prevent neurological complications.

• Arnold Chiari Deformity Demyelinating Disease Hindbrain Herniation Multiple Sclerosis