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J Neurol Neurosurg Psychiatry 1999;66:357-364 ( March )

Basilar impression complicating osteogenesis imperfecta type IV: the clinical and neuroradiological findings in four cases

M Hayes,a G Parker,b J Ell,c D Sillenced

a Department of Neurology, Concord Hospital, Concord NSW, Australia, b Department of Radiology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia, c Department of Neurology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia, d University Department of Paediatrics and Child Health, Royal Alexandra Hospital for Children, Parramatta, NSW, Australia

Correspondence to: 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 and in revised form 3 June 1998; Accepted 8 July 1998

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.

Keywords: basilar impression; osteogenesis imperfecta


© 1999 by Journal of Neurology, Neurosurgery, and Psychiatry



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