1/7. Basilar impression complicating osteogenesis imperfecta type IV: the clinical and neuroradiological findings in four cases.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.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
2/7. Treatment of craniocervical spine lesion with osteogenesis imperfecta: a case report.STUDY DESIGN: A case report of craniocervical spine lesions including basilar impression, atlantoaxial dislocation, and syringomyelia, with osteogenesis imperfecta is presented, and the literature is reviewed. OBJECTIVE: To discuss the problems involved in the surgical management of craniocervical spine lesion with osteogenesis imperfecta. SUMMARY OF BACKGROUND DATA: osteogenesis imperfecta is known to have various spine lesions as complications. However, few reports have described craniocervical lesions associated with osteogenesis imperfecta. methods: A 14-year-old girl with osteogenesis imperfecta, Silence classification IVB, experienced difficulty walking, with marked motor disturbance and muscle weakness in the extremities. Deep tendon reflexes were exaggerated bilaterally in the upper and lower extremities, and positive Babinski reflex and ankle clonus were observed bilaterally. Basilar impression, atlantoaxial dislocation, and syringomyelia were shown by plain radiography, tomography, three-dimensional computed tomography, and magnetic resonance imaging. RESULTS: In the reported patient, posterior fossa decompression and atlantoaxial posterior fusion could not be performed because the foramen magnum and upper cervical spine invaginated to the base of the skull. Therefore, occipitocervical spine fusion using titanium loop and wires was performed at the reduced position of the atlantoaxial dislocation, resulting in improvement of neurologic deficits. CONCLUSIONS: For patients with atlantoaxial dislocation, syringomyelia, and basilar impression without clinical symptoms or signs of brain stem compression, occipitocervical spine fusion alone at the reduction of the atlantoaxial dislocation may be indicated because these procedures improve neurologic deficits and prevent postoperative development of basilar impression and enlargement of syringomyelia.- - - - - - - - - - ranking = 0.31139904971284keywords = upper (Clic here for more details about this article) |
3/7. Syringobulbia: a surgical appraisal.Syringobulbia is a term which has been clinically applied to brain stem symptoms or signs in patients with syringomyelia. Syringobulbia clefts are found on investigation or at necropsy caused by cutting outwards of the CSF under pressure from the fourth ventricle into the medulla. These should be differentiated from the ascending syringobulbia which may occur from upward impulsive fluid movements in a previously established syringomyelia. Clinical analysis of 54 patients suggests that bulbar features are most often found with neither of the above mechanisms but are due to the effects of pressure differences acting downward upon the hind-brain with consequent distortion of the cerebellum and brainstem, traction on cranial nerves or indentation of the brain-stem by vascular loops. The commonest symptoms in the 54 patients were headache (35), vertigo (27), dysphonia or dysarthria (21), trigeminal paraesthesiae (27), dysphagia (24), diplopia (16), tinnitus (11), palatal palsy (11) and hypoglossal involvement (11). Careful attention to hydrocephalus is advisable before craniovertebral surgery, but the decompression of the hindbrain and the correction of craniospinal pressure dissociation remains the mainstay of surgical treatment. The results of careful surgery are good, 45 of the 54 cases reported improvement. Most of the reported deterioration occurred in a few patients who did conspicuously badly.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
4/7. osteogenesis imperfecta associated with basilar impression and cerebral atrophy: a case report.osteogenesis imperfecta is a disease of bone formation subdivided into two types, congenita and tarda. It is associated with bony fragility, blue sclerae and abnormality of tooth dentin. Rarely the tarda form is associated with basilar invagination or infolding of the foramen magnum and upper cervical segments into the posterior fossa. This results in hydrocephalus and a spectrum of neurologic dysfunction known as the foramen magnum compression syndrome. Many radiologic methods have been used to evaluate basilar invagination including plain film and CT. We describe a patient with osteogenesis imperfecta tarda examined with CT, with a unique finding of diffuse cerebral atrophy associated with basilar invagination.- - - - - - - - - - ranking = 0.15569952485642keywords = upper (Clic here for more details about this article) |
5/7. MR imaging of familial basilar impression.Basilar impression was found in three members of one family. The mother showed an asymptomatic deformity, her eldest son complained of headache, drop-attacks, nystagmus, unilateral ophthalmoplegia, and ataxia; the middle son presented with headache, nystagmus, and hemiparesis. Magnetic resonance (MR) imaging demonstrated convexobasia of various degrees with elevation of the upper spine and malformation of the occipital bone. The medulla oblongata and the pons were flattened and dislocated backward in two cases. Chiari malformation was present in one case and mild hydrocephalus in another. A comparison of MR with CT imaging demonstrates some advantages of the former method in the assessment of the neural structures directly involved in basilar impression.- - - - - - - - - - ranking = 2.1556995248564keywords = headache, upper (Clic here for more details about this article) |
6/7. Basilar impression in an achondroplastic dwarf: causative role in tetraparesis.The neurological and radiological findings in a case of chondrodystrophic dwarfism are described. The progressive tetraparesis proved to be based on a high-level medullary compression resulting from basilar impression and upper cervical stenosis. Surgical decompression led to the disappearance of the tetraparetic phenomena. Various aspects of chondrodystrophic dwarfism and basilar impression are discussed in relation to the neurological symptomatology, and the question of whether, in general, more attention should be given to the cranio-cervical region, is raised.- - - - - - - - - - ranking = 0.15569952485642keywords = upper (Clic here for more details about this article) |
7/7. A case of Arnold-Chiari Type I malformation presenting with dysrhythmic breathing during sleep.A 43-year-old woman presented with dull headache, left tinnitus and dizziness. Neurological examination revealed down-beat gaze nystagmus, left tinnitus, positive Romberg sign, poor standing on the left foot, poor tandem gait, left spastic gait and positive pathological reflexes in the bilateral upper and lower extremities. Plain X-ray of the skull and cervical vertebrae demonstrated basilar impression and atlantoaxial fusion. magnetic resonance imaging of the brain and cervical spine showed cerebellar tonsil descent and syringomyelia located in the left side of the spine at the II-III vertebral level which communicated with the fourth ventricle. The patient was diagnosed as having cervical syringomyelia. Arnold-Chiari type I malformation and basilar impression. Preoperative polysomnography showed dysrhythmic breathing and bradypnea during sleep. Abnormal breathing improved after suboccipital decompression craniotomy and upper cervical laminectomy. It was suggested that dysrhythmic breathing was caused by a disorder of the medullary respiratory center. Herniation of the cerebellar tonsil and syringomyelia might have compressed the medulla.- - - - - - - - - - ranking = 1.3113990497128keywords = headache, upper (Clic here for more details about this article) |