1/10. Multiple meningiomas, craniofacial hyperostosis and retinal abnormalities in proteus syndrome.Because clinical evidence suggests that proteus syndrome may be caused by a somatic mutation during early development, resulting in mosaicism, the possible types of abnormalities and their clinical distributions are highly variable. Here, we report on an unusual patient with proteus syndrome. Manifestations included multiple meningiomas, polymicrogyria, and periventricular heterotopias. Both eyes had epibulbar cystic lesions. The retina showed diffuse disorganization with nodular gliosis, retinal pigmentary abnormalities, chronic papilledema, and optic atrophy. Other abnormalities included progressive cranial, mandibular, maxillary, and auditory canal hyperostoses, epidermal nevi, and mental deficiency. The limbs were proportionate, and the hands and feet were normal.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
2/10. Spinal hyperostosis--a rare skeletal manifestation of psoriasis vulgaris.A 28-year-old woman with uncomplicated psoriasis vulgaris presented with spinal hyperostosis and osteitis. The absence of peripheral arthritis, sacroiliitis, and diffuse idiopathic skeletal hyperostosis (DISH) raised the possibility that the spinal lesion was an extracutaneous manifestation of psoriasis. We review the association between uncomplicated psoriasis vulgaris and skeletal involvement.- - - - - - - - - - ranking = 0.9825445355322keywords = spinal (Clic here for more details about this article) |
3/10. Stenosis of the cervical canal in craniodiaphyseal dysplasia.Craniodiaphyseal dysplasia (CDD) is a rare sclerosing bone disorder, the severity of which depends on its phenotypic expression. hyperostosis can cause progressive foraminal stenosis leading to palsy of cranial nerves, epilepsy and mental retardation. We report the only case of CDD in an adult, with stenosis of the cervical canal leading to quadriparesis as a late complication of hyperostosis, and describe the problems associated with its treatment. Although the syndrome is rare, its pathophysiological and therapeutic considerations may be applicable to the management of stenosis of the spinal canal in other hyperostotic bone disorders.- - - - - - - - - - ranking = 26.860417745583keywords = spinal canal, canal, spinal (Clic here for more details about this article) |
4/10. Diffuse idiopathic skeletal hyperostosis causes artificially elevated lumbar bone mineral density measured by dual X-ray absorptiometry.A 76-yr-old man with bilateral total hip arthroplasties was referred for a baseline bone mineral density (BMD) measurement. The L1-L4 lumbar bone density revealed a density above the upper expected value for a young individual (i.e., T-score > 2.5) with large intervertebral variation, while the forearm study revealed an osteoporotic measurement. Lumbar spine radiographs demonstrated abundant, flowing ossification of the anterior spinal ligament, predominantly at L3, consistent with diffuse idiopathic skeletal hyperostosis, which accounted for the increased BMD.- - - - - - - - - - ranking = 0.4912722677661keywords = spinal (Clic here for more details about this article) |
5/10. Case report: bilateral vocal cord paralysis caused by cervical spinal osteophytes.Thyroid and cervical surgery, tracheal intubation, trauma and neurodegenerative and neuromuscular diseases may cause bilateral vocal cord paralysis. There are only a few reported cases of bilateral cord paralysis associated with cervical hyperostosis in the English literature. We report the MR and CT findings of a case of bilateral vocal cord paralysis caused by cervical spinal osteophytes compressing the recurrent laryngeal nerves.- - - - - - - - - - ranking = 2.4563613388305keywords = spinal (Clic here for more details about this article) |
6/10. Abnormal bony protuberance of anterior atlas causing dysphagia. A rare congenital anomaly.STUDY DESIGN: A Case report. OBJECTIVE: To report an exceedingly rare case of dysphagia caused by abnormal bony protuberance of anterior atlas. SETTING: Kocaeli University faculty of medicine, Departments of neurosurgery and Otorhinolaryngology. METHOD: Radiological examinations revealed a large anterior abnormal bony tuberance of atlas in an 11-year-old boy who complained of dysphagia of 5 years donation. RESULT: The anterior bony tuberance of the atlas was resected by a transoral approach. Histopathological examination of the surgical specimens showed normal bone tissue. Resection resulted in complete resolution of the dysphagia. CONCLUSIONS: Dysphagia can be caused by disorders of the cervical spine. These disorders are usually seen in elderly adults. In the pediatric population, spinal abnormality is an exceedingly rare cause of dysphagia. Abnormal inductive signals from the adjacent notocord and ventral neural tube may play role in the pathogenesis of this abnormal bony protuberance.- - - - - - - - - - ranking = 0.4912722677661keywords = spinal (Clic here for more details about this article) |
7/10. Entrapment neuropathy of the optic nerve due to hyperostosis associated with congenital anemia.The authors report on the case of a 14-year-old boy who presented with bilateral visual impairment due to optic canal stenosis caused by hyperplasia of the bone marrow arising from anemia. The patient had hereditary hemolytic anemia with unstable hemoglobin of the Christchurch type. This congenital form of anemia caused hyperplasia of the bone marrow as well as hyperostosis of the entire calvarial bone, which in turn led to optic canal stenosis. The patient underwent surgical decompression of the optic canal, resulting in significant improvement in visual acuity. Pathological findings in the calvarial bone indicated hypertrophic bone marrow with no other specific features such as neoplastic pattern or fibrous dysplasia. With the exception of objective hearing impairment, no other significant cranial neuropathy has been detected thus far. On reviewing the published literature, this case was found to be the first in which hyperostosis due to congenital anemia resulted in symptomatic entrapment neuropathy of the optic nerve. The authors concluded that surgical decompression effectively improves visual acuity.- - - - - - - - - - ranking = 3keywords = canal (Clic here for more details about this article) |
8/10. Bilateral visual loss in craniodiaphysial dysplasia.PURPOSE: To report a rare case of craniodiaphysial dysplasia (CDD) that resulted in a profound loss of vision in both eyes. DESIGN: Observational case report. methods: A 2-year-old girl presented with midfacial anomaly. Marked thickening and sclerosis in the calvaria and facial bones were detected on the plain x-rays, which were compatible with CDD. Two years later, she visited our clinic because of visual loss in both eyes. RESULTS: The visual acuity was light perception in both eyes. The optic disk swelling with temporal pallor was observed in her both eyes. Orbital computed tomography scan revealed near-total obliteration of the optic canal in both eyes. CONCLUSIONS: CDD is a severe bone disorder characterized by massive generalized hyperostosis and sclerosis, especially involving the facial bones. Bony encroachment on the cranial foramina causes optic nerve compression, and this may lead to progressive visual impairment and ultimately to blindness.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
9/10. Extensive extraspinal hyperostoses after long-term oral retinoid treatment in a patient with pityriasis rubra pilaris.We describe a patient with severe pityriasis rubra pilaris in whom extensive extraspinal hyperostoses developed after 13 years of oral retinoid treatment. The most prominent abnormality was a bridging exostosis between the left acetabulum and collum. X-ray examinations of the spine during retinoid therapy showed no abnormalities. During oral retinoid treatment, it is important to ask the patient on a regular basis about any skeletal pains or mobility restriction. Normal spinal x-ray results are no guarantee that a patient is free of hyperostoses. Discontinuation of acitretin therapy resulted in a severe exacerbation of the patient's pityriasis rubra pilaris after 2 weeks. The clinical response to administration of azathioprine was clearly inferior to that of acitretin. However, low-dose oral methotrexate therapy appeared to be a good alternative in this patient, with a clinical result comparable to acitretin and no side effects after 6 months of therapy.- - - - - - - - - - ranking = 2.9476336065966keywords = spinal (Clic here for more details about this article) |
10/10. Cervical myelopathy caused by pseudoarthrosis between the atlas and axis associated with diffuse idiopathic skeletal hyperostosis.STUDY DESIGN. This is a report of a patient with severe cervical myelopathy due to pseudoarthrosis between the posterior tubercle of the atlas and the spinous process of the axis, associated with diffuse idiopathic skeletal hyperostosis. Radiographs of 170 patients with neck pain were reviewed to identify lesions involving abnormal contact between the atlas and axis. OBJECTIVE. Based on an analysis of 170 radiographs, the prevalence of the reported condition was estimated. SUMMARY OF BACKGROUND DATA. A number of histologic studies on pseudoarthrosis involving the lumbar spine have been reported. In contrast, lesions between the posterior tubercle of the atlas and the spinous process of the axis have not been reported in association with cervical myelopathy. methods. Clinical and pathologic features of a patient with pseudoarthrosis between the posterior tubercle of the atlas and the spinous process of the axis were investigated. Radiographs of 170 Japanese patients over 40 years old were examined, and abnormal contact between the atlas and axis was classified into two groups based on the degree of spinal hyperostosis. RESULTS. Of 170 patients, 53 showed abnormal contact between the atlas and axis. The prevalence of abnormalities in the pronounced hyperostosis group (Forestier's stages II and III) was much higher than in the group with normal or slight hyperostosis. Two men had radiographic patterns showing osteophytes projecting into the spinal canal and associated with marked cervical myelopathy. CONCLUSION. In the group with pronounced hyperostosis, pseudoarthrosis or a variant can cause serious problems in the upper cervical spine that should not be overlooked.- - - - - - - - - - ranking = 22.351690013349keywords = spinal canal, canal, spinal (Clic here for more details about this article) |
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