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Biomedical subjects

J Provenzale

Publications and source records attributed to J Provenzale.

5 recordsLinked to original sources

Lupus-related myelopathy: report of three cases and review of the literature.

Transverse myelopathy is an uncommon complication of systemic lupus erythematosus (SLE). Three patients with SLE are reported who developed transverse myelopathy, including the neuropathological findings in one patient on whom necropsy was performed. Paraparesis was present in all three cases, but definite sensory changes were present in only one patient. In two patients, the CSF findings were remarkable for elevated protein and depressed glucose concentrations. Microscopic examination of the brain demonstrated small, scattered foci of recent necrosis consistent with microinfarctions. Striking abnormalities were found in the spinal cord at all levels, including multiple foci of vacuolar spongy degeneration in the peripheral white matter, as well as ballooning of myelin sheaths, swollen axons, myelin pallor, and loss of glial nuclei. The pathological findings in previously reported cases of SLE-related transverse myelopathy are reviewed, and the possible pathogenesis of the findings in our case are discussed.

Adolescent

Tuberous sclerosis.

Tuberous sclerosis is an inherited disorder characterized by a triad of signs--mental retardation, seizures and adenoma sebaceum. The hamartomas that commonly affect multiple organ systems can be seen on plain film radiography. Computed tomography and ultrasonography are useful for assessing whether lesions are present in the abdomen, kidneys and brain. The hamartomas tend to bleed, causing symptoms and prompting the need for evaluation.

Brain

Desmoplastic fibroma.

This benign bone tumor is locally invasive and has a high rate of recurrence. Three-quarters of cases present before age 30. The characteristic plain film finding is a centrally located lytic lesion, usually in the metaphysis or metadiaphyseal region of a long bone. Treatment is surgical.

Bone Neoplasms