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 and posterior 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.