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Results for “OSTEOSCLEROSIS”

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At least 1,315 records · Page 73Linked to original sources
PubMed1998

Rapidly deteriorating polyneuropathy associated with osteosclerotic myeloma responsive to intravenous immunoglobulin and radiotherapy.

Osteosclerotic myeloma is a plasma-cell dyscrasia characterized by osteosclerotic bone lesions, which may be associated with progressive demyelinating polyneuropathy. We describe a 49-year-old patient with rapidly deteriorating polyneuropathy associated with osteosclerotic myeloma, who responded favorably to a combination of intravenous immunoglobulin and radiotherapy.

Combined Modality Therapy↗
PubMedDate not supplied

Sclerosteosis involving the temporal bone: clinical and radiologic aspects.

Sclerosteosis is one of the rare, potentially lethal, autosomal recessive, progressive, craniotubular sclerosing bone dysplasias. Syndactyly of the second and third or other fingers is evident at birth. Hyperostosis and sclerosis are most prominent in the skull and tubular bones, and are frequently associated with excessive height and weight. The typical facial deformity is apparent by the age of 5 years. The changes involving the temporal bone include a marked increase in overall dimensions, extreme sclerosis, and narrowing and constriction of the external ear canal, middle ear cleft, internal acoustic meatus, and falloppian canal. Impairment of hearing, as a rule bilateral, is a frequent presenting symptom which may manifest in early childhood. Initially it is an expression of interference with sound conduction; later it may become associated with a loss of sound perception. Impairment of facial nerve function is another salient feature which occasionally is present at birth. As a rule, it manifests initially as a unilateral, recurrent paresis, eventually progressing to a bilateral permanent partial loss of facial nerve function. Since impairments of hearing and facial nerve function are two of the salient features, present at birth or in early childhood, the responsibility for recognizing the disease often falls upon the otolaryngologist. The clinical and radiologic features permit not only early recognition of the disorder but also differentiation from similar bony dysplasias. Hyperosteosis and sclerosis of the skull lead to thickening and distortion of the calvaria, cranial base, and foramen magnum resulting in reduction of the intracranial volume, interference with the cerebral blood flow, resorption of cerebrospinal fluid, and gradual increase of intracranial pressure. Severe headaches resulting from this mechanism often develop in early adulthood, and several patients have died suddenly from impaction of the medulla oblongata in the foramen magnum. Decompression of the transverse sigmoid sinus and jugular bulb may be lifesaving, combined with a posterior, and if necessary, an anterior, craniectomy for decompression. Early decompression of the internal acoustic meatus and falloppian canal may help in the preservation of cochlear and facial nerve function.

Adult↗
PubMedDate not supplied

Sclerosteosis involving the temporal bone: histopathologic aspects.

Sclerosteosis is a rare, potentially lethal, autosomal recessive, progressive craniotubular sclerosing bone dysplasia with characteristic facial and skeletal features. The temporal bone changes include a marked increase in overall size, extensive sclerosis, narrowing of the external auditory canal, and severe constriction of the internal auditory meatus, fallopian canal, eustachian tube, and middle ear cleft. Attenuation of the bony canals of the 9th, 10th, and 11th cranial nerves, reduction in size of the internal carotid artery, and severe obliteration of the sigmoid sinus and jugular bulb also occur. Loss of hearing, generally bilateral, is a frequent symptom. It often manifests in early childhood and initially is expressed as sound conduction impairment. Later, a sensorineural hearing loss and loss of vestibular nerve function often develop. Impairment of facial nerve function is another feature occasionally present at birth. In the beginning, a unilateral intermittent facial weakness may occur which eventually progresses to a bilateral permanent facial paresis. The histologic examination of the temporal bones from a patient with sclerosteosis explains the mechanisms involved in the progressive impairment of sound conduction and loss of cochlear, vestibular, and facial nerve function. There is a decrease of the arterial blood supply to the brain and an obstruction of the venous drainage from it. The histopathology reveals the obstacles to decompression of the middle ear cleft, ossicular chain, internal auditory and facial canals, and the risks, and in many instances the contraindications, to such procedures. On the other hand, decompression of the sigmoid sinus and jugular bulb should be considered as an additional life-saving procedure in conjunction with the prophylactic craniotomy recommended in all adult patients.

Adult↗
PubMed1996

Acquired anterior open bite and facial arthromyalgia: possible aetiology.

The development of an anterior open bite in four patients with facial arthromyalgia is described. There were radiological signs of condylar erosion in three of these cases. It is proposed that the pre-senile destructive arthropathy of the temporomandibular joint, seen in a small proportion of patients with facial arthromyalgia, results from lysis of articular cartilage and bone, secondary to an inflammatory process initiated by the local release of neuropeptides. Occlusal changes, including anterior open bite, may occur if the destructive arthropathy is severe and outstrips dentoalveolar compensation.

Adolescent↗
PubMed1995

Analysis of proximal fragment sclerosis and surgical outcome of scaphoid non-union by magnetic resonance imaging.

32 patients with scaphoid non-union were examined to clarify the relationship between pre-operative radiographic sclerosis in the proximal fragment and signal intensity on magnetic resonance imaging (MRI). In addition, the correlation between changes in signal intensity in the proximal fragment and surgical outcome after bone grafting and internal fixation was investigated. Proximal fragment sclerosis was observed in seven patients. Comparison of the T1 and T2-weighted images with those of the 25 patients without proximal fragment sclerosis revealed no differences. Therefore, there was no relationship between sclerotic changes on plain radiography and the degree of avascularity of the proximal fragment assessed by MRI. None of the patients who had low signal intensity on both T1 and T2-weighted images achieved union within 5 months after surgery. Thus the detection of signal intensity on both T1 and T2-weighted images can be useful to predict the prognosis after the surgery.

Adolescent↗
PubMed1995

The carpal boss. A 20-year review of operative management.

Between 1969 and 1989, 116 patients were evaluated and treated surgically for symptomatic carpal boss. Their mean age was 32 years and male and female patients were equally affected. 28 patients gave a history of previous injury. Surgical treatment consisted of excision of the localized bony abnormality and the associated degenerative arthritic process to the level of normal articular surfaces and normal adjacent cancellous bone. The mean follow-up period for the patients in this study was 42 months. Complete symptomatic relief was observed in 94% of the patients undergoing surgical treatment. Recurrence or persistence of symptoms developed in seven surgical patients. Six had a second operation with more extensive removal of sclerotic bone and degenerate cartilage, and all patients had relief of symptoms.

Adolescent↗
PubMed1996

Melorheostosis of the hand.

We report a case of melorheostosis of the hand which showed a sclerotomal pattern of involvement, with swelling and pain. Surgical debulking resulted in correction of the deformity and relief of pain.

Adult↗