1/4. Determination of extent and activity with radionuclide imaging in erdheim-chester disease.erdheim-chester disease usually involves the diaphyseal and metaphyseal regions of tubular bones and various visceral organs. A 56-year-old woman presented with the histologically confirmed diagnosis of erdheim-chester disease. A Tc-99m MDP bone scan revealed the entire extent of the skeletal disease and showed unusual involvement of the epiphyses and axial skeleton. In addition to MRI, a Ga-67 citrate scan including SPECT showed extensive soft-tissue infiltration of different organs. Both Tc-99m MDP and Ga-67 scintigraphy are useful tools in determining the distribution of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/4. Cardiopulmonary complications in multicentric reticulohistiocytosis. Report of a case.Multicentric reticulohistiocytosis (MR) is a rare disease. Only recently was its systemic nature appreciated. It affects the skin, mucous membranes, joints, muscles, tendon sheaths, synovial membranes, bones, liver, kidney, lymph nodes, heart, and lungs. Our patient, a 50-year-old woman, had life-threatening cardiopulmonary complications of MR. The connection between the skin lesions, the arthritis, and the pathologic changes in the heart and lungs is still obscure.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/4. Cervical angina caused by atlantoaxial instability.Cervical angina is defined as a paroxysmal precordialgia that resembles true cardiac angina caused by cervical spondylosis. Cervical angina most commonly results from compression of the C7 ventral root. We present here a case of cervical angina caused by atlantoaxial instability. This case had marked atlantoaxial instability but no flexibility of the middle to lower levels of the cervical spine. Although there was mild C7 root compression on the radiologic findings, the chest pain was induced by neck motion, and the precordialgia disappeared after posterior atlantoaxial fusion without C7 root decompression. Therefore, we diagnosed this case as cervical angina caused by spinal cord compression at the C1-C2 level. It was speculated that a perturbation of the sympathetic nervous system or a hypofunction of the pain suppression pathway in the posterior horn of the spinal cord caused the pectoralgia. Although cervical angina is a rare disease, physicians should be aware of it; if there are no abnormal findings on cardiac examinations for angina pectoris, they should examine the cervical spine. Cervical angina due to atlantoaxial instability is one of the differential diagnoses of precordialgia.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/4. Tumoral calcinosis with unusual dental radiographic findings.Tumor calcinosis is a rare disease of unknown cause, manifesting itself as abnormal calcifications of cystic masses in the fibrous tissues adjacent to, but not involving, the joint spaces. A family with multiple siblings affected by tumoral calcinosis and found to have unusual dental radiographic findings consistent with varying degrees of expression of dentinal dysplasia is described.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |