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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.
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