1/269. Type 1 GM1 gangliosidosis with basal ganglia calcification: a case report.This report concerns a 10-month-old boy, admitted to the veterans General Hospital-Kaohsiung with generalized tonic convulsion and aspiration pneumonia. He was found to have had developmental regression, progressive hypotonia and hepatosplenomegaly since four months of age. physical examination revealed a large head circumference (97th percentile), frontal bossing, depressed nasal bridge, hepatosplenomegaly, broad hands and short fingers. neurologic examination showed poor control of eye movement, profound hypotonia, muscle weakness, brisk deep tendon reflexes and Babinski's sign. Hypoplasia of the vertebral bodies with anterior beaking, wedge-shaped metacarpals, spatulated ribs and a J-shaped sella turcica were displayed on bone radiographs. Cranial computerized tomography scans showed diffuse brain atrophy, dilated ventricles and calcification of the bilateral basal ganglia. Vacuolated lymphocytes were noted in a peripheral blood smear. Type 1 GM1 gangliosidosis was diagnosed based on a deficiency of beta-galactosidase activity. To our knowledge, basal ganglia calcification in type 1 GM1 gangliosidosis has never been reported in the literature. We suggest that type 1 GM1 gangliosidosis be considered in the differential diagnosis of patients with an early onset of neurologic decline, organomegaly and basal ganglia calcification.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/269. 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 = 0.58188432331944keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/269. 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 = 1.1637686466389keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/269. Extensive brain calcification in two children with bilateral Coats' disease.We report two children with bilateral Coats' disease associated with cerebral calcifications in the basal ganglia and deep white matter, asymptomatic at the time of their discovery. cerebellar ataxia developed secondarily in one of them. Both children were born small for date and had febrile convulsive seizures. Three similar patients have been previously reported, two of them in the same sibship; the third reported patient died of aplastic anemia. Bilateral Coats' disease in children should prompt systematic CT scan in search of cerebral calcifications. If present, neurological and genetic prognosis should be cautious.- - - - - - - - - - ranking = 4keywords = brain (Clic here for more details about this article) |
5/269. Epileptogenic mineralization: pathological variants with good prognosis.Four examples of grossly mineralized lesions of the brain, causing seizures, are described; they include 2 hamartomas, a choristoma (novel associations), and an idiopathic "brain stone." Each was detectable with routine roentgenograms, and the seizures were ameliorated by surgical therapy.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
6/269. Calcification of presumed ovarian carcinoma brain metastases following radiotherapy.Multiple brain metastases from ovarian carcinoma are rare. CT findings are reported in a case treated with whole brain radiation therapy and chemotherapy. Radiological complete-calcified brain metastases following treatment and long morphological changes occurred. Delayed neurological findings in relation to treatment are considered. Long survival with unmodified calcified lesions may suggest a stabilization of CNS disease.- - - - - - - - - - ranking = 7keywords = brain (Clic here for more details about this article) |
7/269. Neuronal cytoskeletal changes are an early consequence of repetitive head injury.While neuropathological studies have established the pathology of dementia pugilistica to be similar to that of Alzheimer's disease, there is little information about the early histological changes caused by the repetitive trauma that eventually produces dementia pugilistica. We have examined the brains of four young men and a frontal lobectomy specimen from a fifth, age range 23-28 years, all of whom suffered mild chronic head injury. There were two boxers, a footballer, a mentally subnormal man with a long history of head banging, and an epileptic patient who repeatedly hit his head during seizures. The four autopsy cases were widely sampled; the lobectomy specimen was serially sliced after fixation. Routine stains were performed; inmmunostaining included beta-amyloid precursor protein, amyloid beta-protein (Abeta), tau and apolipoprotein E (apoE). Pathological findings in all five cases were of neocortical neurofibrillary tangles (NFTs) and neuropil threads, with groups of tangles consistently situated around blood vessels in the worst affected regions. No Abeta immunoreactivity was detected. The amount of neuronal apoE expression varied widely between the cases with no clear relation to the NFTs. The apoE genotype was determined in only two cases (both epsilon3/epsilon3). It appears that repetitive head injury in young adults is initially associated with neocortical NFT formation in the absence of Abeta deposition. The distribution of the tau pathology suggests that the pathogenesis of cytoskeletal abnormalities may involve damage to blood vessels or perivascular elements.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
8/269. 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 = 2.3275372932777keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/269. Hypoxic-ischemic encephalopathy with cystic brain stem necroses and thalamic calcifications in a preterm twin.A severe and rare ischemic brain lesion in a preterm twin boy is reported. The boy was born after two weeks of anhydramnios and amnionic infection at 24 weeks of gestation. Following a difficult Caesarean section and prolonged umbilical cord compression he developed prenatal acidosis with an umbilical cord pH of 6.96. At the age of 7 h, heart rate variability narrowed due to severely disturbed brain stem function and the patient developed clinical signs of hypoxic-ischemic encephalopathy. Sonography demonstrated extensive symmetrical brain stem and basal ganglia lesions. After a prolonged comatose and apneic state, death occurred at the age of 25 days. autopsy confirmed columnar bilateral cavitation of basal ganglia, diencephalon, brain stem and spinal gray matter, as well as focal calcifications in the palladium, thalamus, and brain stem. The findings highly resemble those observed after experimental or clinical cardiac arrest.- - - - - - - - - - ranking = 9keywords = brain (Clic here for more details about this article) |
10/269. 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 = 7.5818843233194keywords = brain, central nervous system, nervous system (Clic here for more details about this article) |
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