Basilar impression complicating osteogenesis imperfecta type IV: the clinical and neuroradiological findings in four cases
- aDepartment of Neurology, Concord Hospital, Concord NSW, Australia, bDepartment of Radiology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia, cDepartment of Neurology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia, dUniversity Department of Paediatrics and Child Health, Royal Alexandra Hospital for Children, Parramatta, NSW, Australia
- Professor D Sillence, Department of Clinical Genetics, Royal Alexandra Hospital for Children, PO Box 3515, Parramatta, NSW 2124, Australia. Telephone 0061 2 9845 3273; fax 0061 2 9845 3204.
- Received 2 March 1998
- Revised 3 June 1998
- Accepted 8 July 1998
Abstract
OBJECTIVES To describe the clinical and neuroradiological features of basilar impression in patients with osteogenesis imperfecta type IV.
METHODS Four patients with basilar impression were ascertained in a population study of osteogenesis imperfecta. All four had detailed clinical and neuroradiological examination with both CT and MRI of the craniocervical junction andposterior fossa structures.
RESULTS All four showed significant compression of the posterior fossa structures and surgical decompression was performed with relief of symptoms.
CONCLUSION Symptoms of cough headache and trigeminal neuralgia occurring in patients with osteogenesis imperfecta are indications for detailed clinical and neuroradiological investigation to document basilar impression.
