[Occipitalization of the atlas (Wackenheim III), basilar impression, chiari malformation (I) and paracondyloid process].
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Trigeminal involvement in syringomyelia or in a malformation of the occipito-vertebral junction may rarely be the presenting feature of the disorder. Two cases are reported: in the first, recurrent pruritus of the side of the nose due to sensory involvement of CN V was the presenting feature of syringomyelia; in the second, a malformation of the occipito-vertebral junction presented with refractory and painful trismus by irritation of the motor part of the trigeminal nerve.
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A case of neurofibromatosis with varied clinical symptoms due to parental consanguinity is reported. The patient presented the following manifestations: mental retardation, curved tibias, kyphoscoliosis, basillary impression with pyramidal syndrome and parkinsonism, subluxation of the lenses, bilateral blindness, and hypogonadism. Subluxation of the lenses and hypogonadism deserve special mention because of the rarity of their presentation in this disease. The hypogonadism was of the hypogonadotrophic type without evidence of pituitary or gonadal tumor. The possible causes of endocrine dysfunction in neurofibromatosis are discussed. The more likely hypothesis explains endocrine dysfunction on the basis of an elongation of the pituitary stem; if such abnormality was caused by gliosis of the optic chiasma, an explanation would be apparent for the bilateral blindness presented by our patient since infancy. The alterations of the nervous, endocrine, and osteoarticular systems, and the ocular manifestations that can be present in von Recklinghausen's disease are also reviewed.
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Clinical and X-ray examination of the occipital foramen in healthy individuals and in persons with various congenital and acquired lesions in this region showed, on the one hand, a wide variety of shapes of the foramen in normal conditions and, on the other, the direct dependence of neurologic disorders on the degree of changes in the position of the occipital foramen, its constriction and deformity of its edges. Marked bone changes call for emergency decompression of the compressed or dislocated bulbospinal parts of the central nervous system, the nerves and vessels.
A patient with basilar impression presented with a progressive myelopathy due to odontoid invagination. It was thought that a posterior decompression would be hazardous; therefore, the inferior clivus, odontoid process, and anterior arch of the atlas were removed transorally. We have found that, even with symptoms of long duration, marked improvement can be expected when the operation is targeted to the actual abnormality. In such cases, analysis of craniocervical tomograms will show the direction of medullary compression and thus indicate the correct surgical approach.
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The many kinds of complication that have been observed following operations of basilar impression and/or malformation of Arnold-Chiari are analysed. Two surgical techniques were adopted in the treatment of the patients. In the first 64 cases, the operation consisted in the craniectomy of the posterior fossa and high cervical laminectomy, while the dura-mater remained opened and saturated to the lateral musculature. Concerning the other 75 cases, it was made a plastic of the dura-mater of the posterior fossa and, in most of the cases, the patients were intubated with no retroflexion of the head and operated in sitting position without anterior flexion of the head. In the second group of patients, it was observed lesser occurrence of complications and death.
Within a period of ten years we operated on 126 cases of basilar impression and/or Arnold-Chiari malformation. Two surgical techniques were employed for decompression of the posterior fossa. In the group I (64 cases) the operation consisted of craniectomy and the dura mater was left open and was fixed to the lateral musculature. The technique of the group II (62 cases) consisted in endotracheal intubation without flexion of the head, position of the head without anterior flexion during the operation and plastic closure of the dura. The dural graft was employed to create more space at the craniocervical joint, to avoid cerebrospinal fluid fistula and to restore the integrity of the dura as a protection for the nervous structures of the posterior fossa. The frequency of postoperative complications and mortality observed in group II was definitely less than in the group I.
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After a review of the literature and an analysis of the clinical and radiographic features of basilar impression (b.i.) the authors describe 27 personal cases. In 6 b.i. was present in its pure form and in 21 it was associated with other anomalies at the cranio-vertebral junction. Because of the frequent misdiagnosis with multiple sclerosis or other chronic neurologic diseases and because of the possible improvement of symptoms following decompressive occipital craniectomy and cervical laminectomy, the importance of a correct and complete radiographic study of the cranio-vertebral junction in these cases is stressed.
A clinico-roentgenological examination of the transitional craniovertebral division has revealed a high incidence and a great diversity of isolated underdevelopment of the arches of the upper cervical vertebra--from their fissure-like splitting to complete absence. Usually these forms create no preconditions for disturbances of any functions, do not manifest clinically, and can be regarded as variants of normal structure of the craniovertebral division.
It has been found that anomalies of the craniovertebral region are characterized by polymorph degenerative changes in the bones. This regularity appeared to be especially typical for Arnold-Chiari's anomaly, the fact, that gives one ground to expand the scope of the notion and to specify some variants of that anomaly. Complicated anomalies manifest themselves in the form of three clinical syndromes, that is, the cerebellobulbar, the syringomyelitic and the pyramid ones. In cases of acute progressing of those syndromes surgical intervention is indicated, while in chronically developing syndromes restorative and rehabilitation treatment can be given.
We report a case of cerebellar infarctions which occurred in the territories of the bilateral posterior inferior cerebellar arteries. This case was complicated with cranio-cervical anomalies composed of assimilation of the atlas, atlanto-axial dislocation, and basilar impression. The 40-year-old male patient had no detectable risk factors predisposing to atherosclerotic arterial occlusion or cardiogenic embolism, and there were no angiographic findings of atherosclerosis. It was, therefore, postulated that the cerebellar infarctions were secondary to those cranio-cervical anomalies. The developing mechanism is discussed.