1/99. Periarticular calcification in systemic lupus erythematosus.OBJECTIVE: To describe the radiologic manifestations of periarticular calcification in patients with systemic lupus erythematosus (SLE) and to investigate clinical variables associated with its occurrence. methods: hand radiographs and clinical records of 52 patients who had 4 or more features of the 1982 revised criteria for classifying SLE and who had no other collagen vascular diseases were analyzed retrospectively. RESULTS: Periarticular calcifications were found in 7 patients (13.5%) near the distal and proximal interphalangeal (DIP and PIP) joints and metacarpophalangeal (MCP) joints. No significant association with calcification was noted for the following variables: age at disease onset, duration of the disease, sex, the maximum value of the serum calcium, organic phosphate, and uric acid, Raynaud's phenomenon, lupus nephritis, femoral avascular necrosis, central nervous system lupus, proteinuria, or the use of drugs such as corticosteroids, synthetic vitamin d, and nonsteroidal antiinflammatory drugs. However, a significant association was noted with the use of furosemide (p < 0.01 by chi-square). In 5 patients periarticular calcification was observed during or just after hyperuricemia had developed while taking diuretics. CONCLUSION: Periarticular calcification in patients with SLE was seen in the DIP, PIP, and MCP joints, and appeared to be associated with the use of diuretics. If patients with SLE are prescribed a diuretic regimen, crystal associated arthritis should be considered as a possibility when diagnosing oligoarthritis.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/99. Intracranial calcification mimicking the sturge-weber syndrome: a consequence of cerebral folic acid deficiency?Cerebral cortical calcification identical to that of the sturge-weber syndrome was observed in two children. In one child the calcification appeared after intrathecal administration of methotrexate and skull irradiation because of leukemia involving the central nervous system. In the other child, who had coeliac disease and epilepsy, the calcification appeared after treatment with anticonvulsants. This treatment was also contributing to the development of profound megaloblastic anemia. The unspecificity of the Sturge-Weber calcification is stressed and the hypothesis is put forward that the calcification may be secondary to folic acid deficiency interfering with the matabolism in the central nervous system.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
3/99. A strange complex of diffuse congenital cardiovascular disease and cardiomyopathy, with localised myocardial calcification.This unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years. Necropsy showed a grossly abnormal arrangement of ventricular myocardial fascicles and bands, with absence of the papillary muscles causing tricuspid and mitral regurgitation. The various malformations are considered to be a gross example of a diffuse congenital cardiovascular disease complex, to the best of our knowledge previously undescribed.- - - - - - - - - - ranking = 0.43563210836601keywords = malformation (Clic here for more details about this article) |
4/99. Fibro-osseous lesions of the central nervous system: report of four cases and literature review.Fibro-osseous lesions, also reported as calcifying pseudoneoplasms of the neural axis, are uncommon lesions of the CNS. We report four additional cases: two extraaxial and two intraaxial, in patients ages 33, 47, 49, and 59 years at presentation. Fibro-osseous lesions involving the CNS demonstrate variable proportions of fibrous stroma, bone, palisading spindle to epithelioid to multinucleated cells in association with a highly distinctive, perhaps pathognomonic, chondromyxoid-like matrix often distributed in a nodular pattern. This histopathologically distinctive lesion can be seen in many regions of the neuraxis, often with a dural association, and most commonly along the vertebral column. It appears to be a slow-growing lesion and, with wide excision, the prognosis is excellent. The etiology remains unclear, but the preponderance of data favors a reactive rather than neoplastic process. If this putative pseudotumor is not recognized histopathologically, a neoplastic or infectious differential might result in inappropriate investigations and potentially harmful therapies.- - - - - - - - - - ranking = 4keywords = nervous system (Clic here for more details about this article) |
5/99. Idiopathic tumoral calcinosis associated with congenital malformation of the hand. Case report.The authors describe a case of tumoral calcinosis of the hand in a three-year-old child, involving the digits and carpus, and associated with ectrodactyly. The clinical features, diagnostic imaging procedures, particularly radiography and MRI, and therapeutic approach, in most cases radical excision, of this rare soft tissue tumour are discussed.- - - - - - - - - - ranking = 1.742528433464keywords = malformation (Clic here for more details about this article) |
6/99. radiation-induced brain calcification: paradoxical high signal intensity in T1-weighted MR images.BACKGROUND: Irradiation to the central nervous system (CNS) in childhood is known to induce cerebral calcification after a latent period. Calcification has been generally found to show nil or a reduction in signal intensity in magnetic resonance (MR) images. However, we have studied three patients with radiation-induced brain calcification, who manifested increased signal intensity on T1-weighted MR images. METHOD: Three girls had each been diagnosed as having a suprasellar germ cell tumour and were treated with conventional fractionated radiotherapy in their childhood. In one case, chemotherapy was given prior to the CNS irradiation. FINDINGS: All three patients survived their disease, and a follow-up CT scan revealed calcification in the brain, which has shown an increased signal intensity in the T1-weighted images of MR. INTERPRETATION: Cerebral calcification may be presented as a high signal intensity in the T1-weighted MR images. This may be explained by a surface-relaxation effect by the calcium salt particle, precipitated in the brain due to radiation-induced mineralising microangiopathy.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
7/99. Greenberg dysplasia: first reported case with additional non-skeletal malformations and without consanguinity.In 1988 Greenberg et al. reported an association of fetal hydrops with irregular calcification and moth-eaten skeletal dysplasia. Here, we report on the first case of this disorder accompanied by additional malformations (omphalocele, intestinal malrotation, disturbed fingernails and toes, hypolobated lungs) in a German couple without consanguinity (karyotype 46,XY). Sonograpically, the fetus was characterised by tetraphokomelia, severe generalised hydrops, pulmonal hypoplasia and hepato-splenomegaly. Greenberg dysplasia should be considered in differential diagnosis in cases with severe fetal hydrops and phokomelia on antenatal sonography.- - - - - - - - - - ranking = 2.17816054183keywords = malformation (Clic here for more details about this article) |
8/99. Adams-Oliver syndrome: further evidence for autosomal recessive inheritance.We report a 7-year-old girl with Adams-Oliver syndrome who presented with extremely rare central nervous system anomalies including microcephaly, epilepsy, mental retardation and intracranial calcifications in addition to the classical scalp and limb defects.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
9/99. Spinal toxoplasmic arachnoiditis associated with osteoid formation: a rare presentation of toxoplasmosis.STUDY DESIGN: An extremely rare presentation of an isolated spinal toxoplasmic arachnoiditis is described. OBJECTIVE: To draw attention to the fact that spinal arachnoid membranes may be a potential reservoir for toxoplasma gondii. SUMMARY OF BACKGROUND DATA: central nervous system toxoplasmosis is a common manifestation in patients who are immunodeficient. Reports on the spinal toxoplasmosis are rare and focused on spinal cord involvement. methods: An adult patient presented with symptoms of spastic paraparesis that had begun 13 years before admission. Thoracic spinal magnetic resonance imaging showed small lesions in posterior subarachnoid space at Th7-Th8. A Th7-Th8 laminectomy was performed. Intradural-extramedullary lesions were excised. RESULTS: Clinical, immunologic, and pathologic examinations showed adhesive spinal arachnoiditis associated with osteoid formation caused by past toxoplasmic infection. There was no impairment of the immunologic defense system. CONCLUSION: Where no causative factor is found in serious spinal adhesive arachnoiditis, the possibility of spinal toxoplasmosis should also be investigated.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
10/99. focal nodular hyperplasia with calcification and ossification.We describe a 33-year-old woman with Crohn's disease, who presented with recurrent episodes of small bowel obstruction. A solitary liver lesion was discovered incidentally by abdominal ultrasound. Pathological examination of the resected specimen revealed features typical of focal nodular hyperplasia together with uncommon findings including calcification, ossification and fibrous obliteration of blood-filled "cysts", changes more commonly associated with regression in hepatic haemangiomas. This report strengthens the favoured hypothesis that a vascular malformation underlies the pathogenesis of focal nodular hyperplasia.- - - - - - - - - - ranking = 0.43563210836601keywords = malformation (Clic here for more details about this article) |
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