331/3657. Latent coeliac disease in a child with epilepsy, cerebral calcifications, drug-induced systemic lupus erythematosus and intestinal folic acid malabsorption associated with impairment of folic acid transport across the blood-brain barrier. A 15-year-old boy with epilepsy and cerebral calcifications, treated with valproic acid, ethyl phenylbarbiturate and ethosuximide, was referred for drug induced systemic lupus erythematosus. Anti-gliadin (AGA) and anti-endomysium (EMA) antibody tests were both positive (EMA titre 1:50). Endoscopic duodenal biopsy showed intense chronic inflammation without villous atrophy or crypt hyperplasia. The child was discharged with a gluten-containing diet. The follow-up showed an increase in EMA titre (1:200) and the persistence of AGA. After 15 months, a second endoscopic intestinal biopsy showed flat mucosa and villous atrophy. Three serum folic acid determinations showed 1.8, 2.4, 2.0 ng/ml (reference range 2.5-16.9 ng/ml) prior to the two intestinal biopsies, but returned to normal levels (11.8 ng/ml) after a gluten-free diet and oral supplementation together. Two years later, the frequency of epileptic seizures was unchanged despite ongoing anti-epileptic treatment and a gluten-free diet. As cerebral calcification and epilepsy are reminiscent of the findings in congenital folate malabsorption, oral loading tests with 5 mg folic acid were carried out and showed impaired intestinal absorption and a defect in the transport across the blood-brain barrier. Low CSF folate levels (13.9 and 12.6 ng/ml, reference range 15-40 ng/ml) and an alteration in the CSF/serum folate ratio (1.43 and 1.16, normal ratio 3:1) were also found as well as increased levels of cystathionine both in CSF (40 micromol/l, reference range 18-28 micromol/l) and in serum (32 micromol/l, reference value <0.10 micromol/l). CONCLUSION: Impairment of intestinal folic acid absorption with a defect in folic acid transport across the blood-brain barrier has been demonstrated in a case of epilepsy and cerebral calcifications associated with coeliac disease. ( info) |
332/3657. Case report: Calcifying fibrous pseudotumour of the adrenal gland. Calcifying fibrous pseudotumour is a distinct pathological entity usually occurring in the soft tissue of the extremities, trunk, axilla, pleura, mediastinum and peritoneum. This report describes the hitherto unreported occurrence of this tumour of the adrenal gland in a 10-year-old girl whose imaging findings closely resembled a neuroblastoma. This entity is a potential pitfall in diagnosing adrenal neuroblastoma. ( info) |
333/3657. Recurrent calcified spinal meningioma detected by plain radiograph. STUDY DESIGN: A case report of a patient presenting with a rare calcified spinal meningioma that recurred and became symptomatic 33 years after initial surgery was performed. OBJECTIVES: To demonstrate a calcified recurrent spinal meningioma visible on a plain radiograph. The present case is discussed in conjunction with a review of previous cases of calcified spinal meningioma. SUMMARY OF BACKGROUND DATA: meningioma is a common spinal tumor that has a favorable prognosis if resected completely. Microscopic calcification is frequently found in spinal meningiomas because of psammoma bodies. However, calcified meningiomas in the spinal canal that can be detected on a plain radiograph are uncommon. To the authors' knowledge, there have been no previous reports of recurrent spinal meningioma with calcification visible on a plain radiograph. methods: A patient experienced difficulty in walking and was diagnosed as having a spinal meningioma. Laminectomies and a total tumor excision were performed. Histologically, the tumor was diagnosed as a psammomatous meningioma. The tumor recurred and became symptomatic 33 years after the initial operation. The tumor was detected on a plain radiograph, appearing as a calcified tumor at the same thoracic level as the site of the initial operation. The recurrent tumor was completely removed, and a histologic examination showed huge psammoma bodies with calcification, but without ossification. RESULTS: Preoperative paraplegia improved after the surgery, and she was able to walk with a crutch. The patient was discharged from the hospital. CONCLUSIONS: The recurrent psammomatous meningioma contained clusters of calcified psammoma bodies, resulting in a hard tumor that was visible on a plain radiograph. ( info) |
| A 60-year-old man was diagnosed as adult T-cell leukemia with severe hypercalcemia because of production of parathyroid hormone-related protein. After admission, the patient had respiratory insufficiency with an infiltrative shadow in his lungs suggestive of pneumonia. However, neither improvement in respiratory function nor disappearance of the abnormal chest shadow was observed with administration of various antibiotics. An autopsy demonstrated the chest shadow had been caused by metastatic calcification associated with hypercalcemia due to production of parathyroid hormone-related protein. The possibility of metastatic calcification should be considered in patients with adult T-cell leukemia and hypercalcemia who have an abnormal chest shadow. ( info) |
335/3657. Documented acquired asteroid hyalosis in a case of early diagnosed diabetes mellitus. BACKGROUND: There have been many reports in the literature of the possible linkage of asteroid hyalosis (AH) to diabetes mellitus (DM). The controversy regarding an association between AH and DM has been one of the longest disputes in the ophthalmic literature. Here we present a case in which AH developed in a patient followed for 9 years after being diagnosed with DM. CASE REPORT: The patient had been examined on nine occasions (since his initial visit in June 1989) and asteroid hyalosis was not discovered until July 1996, when he came in with newly diagnosed diabetes mellitus. This suggests there may well be an association of asteroid hyalosis and diabetes mellitus. DISCUSSION: The patient in this case had a number of risk factors for the development of this ocular condition. He had a long history of systemic arterial hypertension, which has been reported to be linked to the formation of AH. He also had a chronic case of cystoid macular edema, which indicated a vascular compromise to the retinal vessels in the posterior pole, and this leakage may be responsible for serous constituents leaking into the vitreous, which may have caused AH. CONCLUSIONS: This may be the first time in the reported literature that AH was found to occur in a previously normal-appearing vitreous, which was documented over a 9-year period. We would suggest that asteroid hyalosis may be secondary to some form of vasculopathy in many incidences and that diabetes mellitus is one of the conditions that may be associated with the formation of AH. ( info) |
336/3657. Metastatic calcification of the nasal septum presenting as an intraoral mass: a case report with a review of the literature. Metastatic calcification is a pathologic condition characterized by deposition of calcified product in otherwise normal tissues as a result of hyperphosphatemia with or without concurrent hypercalcemia. Metastatic calcification presenting clinically as an oral lesion is extremely rare. To date, only 7 cases of metastatic calcification involving the oral soft tissues have been described. This report describes a case of metastatic calcification of the nasal septum presenting as a mass of the anterior maxillary vestibule in a patient with end-stage renal disease. The case reported is only the second example with nasal septum involvement. A brief review of the clinical and histopathologic features of previously reported intraoral cases is also presented. ( info) |
337/3657. Calcification of the alar ligament of the cervical spine: imaging findings and clinical course. Ligamentous calcification of the cervical spine has been reported in the yellow ligament, anterior and posterior longitudinal ligaments and interspinous ligament. Calcification in the upper cervical spine is rare, although some cases with calcification of the transverse ligament of the atlas have been reported. Two patients with calcification of the alar ligament with an unusual clinical presentation and course are described. Examination by tomography and computed tomography (CT) showed calcification of the alar ligament and the transverse ligament of the atlas. CT documented decreased calcification as symptoms resolved. There may be a role for CT in the search for calcifications in the upper cervical spine in patients presenting with neck pain and pharyngodynia if radiographs are normal. ( info) |
338/3657. Ictal deafness--a report of three cases. Though negative phenomena like motor inhibition, blindness or aphasia are described as an ictal manifestation of seizures, ictal deafness has not been reported so far. We observed transient ictal deafness in three cases of partial seizures. One of them had seizure spread to the temporal lobe to produce a complex partial seizure. Two of them have CT-detected lesions in the left temporal/parietal area. The other one had left temporal focus on EEG with a normal imaging study. The mechanism of such ictal negative phenomena is unclear. An epileptic focus around the primary auditory cortex, dampening its receptive ability may manifest as cortical deafness. ( info) |
339/3657. Periarticular calcinosis associated with anti-Jo-1 antibodies sine myositis. Expanding the clinical spectrum of the antisynthetase syndrome. We describe a 58-year-old woman who developed interstitial lung disease (ILD), polyarthritis, and anti-Jo-1 antibodies, with no clinical evidence of myositis. Despite successful treatment with corticosteroid and azathioprine for her arthritis and pulmonary condition, she developed deforming arthropathy of the hands, with periarticular calcinosis. The association of anti-Jo-1 antibodies, ILD, and periarticular calcinosis with subluxing arthropathy sine myositis is rare, with few cases reported. This report expands the clinical spectrum of the antisynthetase syndrome, which is broader than previously reported. ( info) |
340/3657. Synovial chondromatosis in the temporomandibular joint complicated by displacement and calcification of the articular disk: report of two cases. Two cases of synovial chondromatosis of the temporomandibular joint (TMJ) are presented, including correlation of CT and MR imaging characteristics with surgical and pathologic findings. The usefulness of CT and MR imaging in the diagnosis of TMJ disorders is discussed. ( info) |