Basilar impression: review of literature and report of a case.
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Within the period of 1971 to 1989 we operated on 230 cases of craniocervical malformations characterised by 189 cases of basilar impression associated with Arnold-chiari malformation, 28 cases of isolated basilar impression and 13 cases of Arnold-Chiari malformation. Autonomic disturbance was observed in 136 (59.1%) cases: sexual disorders like reduced potency and impotency were observed in 86 (60.9%) out of 141 male patients, urinary symptoms in 39 (17%) patients; and other autonomic features like dyspnea, anhidrosis, hyperhidrosis, Claude Bernard-Horner syndrome and constipation in 41 (17.8%) patients.
We report a 30-year-old man presenting with medial longitudinal fasciculus (MLF) syndrome after an afternoon nap. Magnetic resonance imaging revealed a right medial pontine tegmental infarction and right cerebellar infarctions. This patient was complicated with basilar impression detected on cervical X-ray and MRI. Three-dimensional CT angiography disclosed that the odontoid process migrated into the posterior fossa, thrusting the bilateral vertebral arteries postero-laterally. The mechanical stress on the bilateral vertebral arteries may have caused infarctions in the territories of the posterior circulation of this patient with basilar impression.
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Paget's disease of the skull is the main cause of basilar artery syndromes in the adult. They may cause various neurological symptoms, including signs of ischemia of the spinal cord or medulla, or involvement of the cranial nerves and brain stem and, also, distant symptoms due to hydrocephalus, with various mental disorders including transient, recurrent, coma. The authors discuss 30 cases found in the world literature and 6 unreported personal cases, study the clinical symptoms of these cases of basilar artery compression due to Paget's disease, and the methods of neuro-radiological investigation, and emphasize the interest, in severe forms, of early surgical decompression before the stage of severe neurological complications. Regular supervision of patients with Pagets disease is thus essential to detect at an early stage, decompensation of basilar artery insufficiency in Paget's disease. In late forms, calcitonin may be indicated.
Melnick-Needles syndrome or osteodysplasty, a monogenic heritable bone dysplasia, is characterized by a typical facies and characteristic radiological findings. Less than 70 well-documented cases have been reported in literature; most of them were sporadic. We report the first case from Eastern India in an adolescent male, who had cranio-vertebral junction anomalies and presented with spastic quadriparesis at the age of 13 years.
For evaluation of the function of the upper cervical spine, especially assessment of the range of motion, functional X-rays films are useful in addition to the clinical examination. For the diagnosis of segmental instability, passive motion should be induced in order to obtain the full range. If anterior instability of the upper cervical spine is suspected, flexion-extension X-rays in the lateral view are appropriate. If a lesion of the alar ligaments is suspected, then lateral flexion X-rays films should be taken. In the normal situation, the atlas glides in the direction of bending, coupled by forced rotation of the axis. In cases with rotatory instability of the upper cervical spine, functional computed tomography should be performed. Atlantoaxial rotation of more than 52 degrees should be considered pathological as a result of a lesion of the alar ligaments. For examination of the relationship between the spinal cord and bony structures or inflammatory tissue in patients with rheumatoid arthritis, functional MRIs are helpful. Not only the exact diameter of the spinal canal can be measured during flexion and extension, but the degree of basilar impression or cranial migration of the axis can also be assessed. Optimal use of functional X-ray diagnostics, including functional the CT and MRI, is not only helpful for clinical diagnoses, but also for the planning of surgical procedures.
Basilar impression is one of bony deformities occurred in the region of the foramen magnum, in which deformity, rim of the foramen magnum or a part of the vertebra impresses into the posterior cranial fossa. Major symptoms of this deformity are short neck, low hair line, torticollis, webbing of neck, pain and limitation of neck movement and various nerve symptoms, when nervous tissue is impressed by rim of the foramen magnum or dens of the second vertebra. This deformity has a characteristic to be found later as a congenital deformity, because nerve symptoms usually do not occur in earlier than 10 years old. The patient was 7 years and 8 months old at his initial visit, whose chief complaint was rotation of upper central incisors which created chewing disorder. When he became 12 years old, because he was taken palsy, walking disturbance, and neck pain in movement, neurosurgery was planned immediately. Edgewise appliance was, then, removed, and orthodontic treatment had to be discontinued until he left hospital. A case was reported of the problems in orthodontic treatment and characteristic of this deformity through our experience for this cleft lip and palate patient with basilar impression in addition to other reports about this deformity.
Downbeat nystagmus (DBN) is a primary position nystagmus with the fast phase in a downward direction. It is a rare but distinctive disorder of ocular motility and usually localizes the lesion at the posterior fossa. Four patients with DBN were seen in the department. One had a medullary glioma, and another congenital basilar invagination. The other two were initially diagnosed as demyelinating disease. One was subsequently found to have Arnold Chiari Malformation on magnetic resonance imaging (MRI). Review of the literature showed that cerebellar ectopia (Arnold Chiari Malformation) is the commonest cause of DBN. However 1/3 of reported cases have no obvious cause. DBN is of such high localizing value that we recommend MRI of cervicomedullary junction for all patients with DBN to exclude cerebellar ectopia or medullary lesion.
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We have reviewed 29 cases of patients who suffered from basilar impression and had undergone hospital treatment since 1969. 12 of these patients were reexamined. The predominant symptoms were lesions of the long tracts while the ,,classical signs" such as headache and nystagmus occurred less frequently than expected. Only a quarter of our patients showed a marked deterioration during follow-up. The outcome for four patients who underwent operation is reported.
In 3 patients with histiocytosis X the following complications are described: 1. A 16 year old boy suffered from generalized histiocytosis in infancy. He died after acute intracranial hypertension caused by a basilar impression. 2. Recurrent spontaneous pneumothoraces was the first symptom of an initial exclusively pulmonary histiocytosis X. 3. Exophytically growing soft tissue tumors in the late disease state of a progressive histiocytosis X.
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