1/15. Extraventricular neurocytoma with ganglionic differentiation associated with complex partial seizures.We report an unusual case of extraventricular ("cerebral") neurocytoma with ganglion cells located in the right temporal lobe in a 9-year-old girl with complex partial seizures and precocious puberty. CT showed a calcified mass with central cystic zones. MR imaging showed a markedly hyperintense predominately solid tumor on both T1- and T2-weighted images, without appreciable contrast enhancement. Cerebral neurocytomas are histologically benign and radical surgery is curative; they should be included in the differential diagnosis of temporal lobe tumors in children.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/15. Exuberant transverse ligament degeneration causing high cervical myelopathy.Two patients with cervical myelopathy and C1-C2 retro-odontoid masses were examined. Preoperative magnetic resonance imaging studies suggested soft tissue pannus, as might be seen in rheumatoid arthritis; however, the results of serologic testing for rheumatoid factor were negative in both patients. Intraoperative findings and pathologic examination revealed degenerative fibrocartilage without inflammation or neoplasia. Similar lesions reported in the literature have been described as retro-odontoid disk hernia, damaged transverse ligaments, transverse ligament degeneration, synovial cysts, ganglion cysts, and degenerative articular cysts. These lesions may share a common pathophysiologic origin and represent a single disease process, namely exuberant degeneration of the transverse ligament.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
3/15. Neonatal intestinal obstruction simulating meconium ileus in infants with long-segment intestinal aganglionosis: radiographic findings that prompt the need for rectal biopsy.BACKGROUND: The initial clinical presentation and radiographic finding of microcolon in children with long-segment intestinal aganglionosis involving the entire colon, ileum and sometimes the jejunum can mimic meconium ileus. This makes the diagnosis difficult for the radiologist and surgeon. OBJECTIVE: To document and describe the clinical and radiographic findings in children with long-segment intestinal aganglionosis who are initially thought to have meconium ileus. MATERIALS AND methods: We reviewed the cases of six neonates with long-segment intestinal aganglionosis presenting as meconium ileus at our institutions between 1978 and 2002. We examined the clinical presentation and the radiographic, surgical, and pathologic findings. In addition, 17 cases from the literature were identified and are included in the discussion. RESULTS: A total of 23 cases were reviewed. Right lower quadrant intraluminal calcifications were noted on abdominal radiographs in all six neonates of our series and were described in 13 of the 17 neonates reported in the literature. Similarly, a microcolon was present in five of the six neonates of our series and in 14 of 16 historical neonates (one not reported). CONCLUSION: In a neonate with small-bowel obstruction and a microcolon, the presence of right lower quadrant intraluminal calcifications should raise the suspicion of long-segment intestinal aganglionosis even if the operative findings are typical of meconium ileus and a biopsy should be performed.- - - - - - - - - - ranking = 1.6keywords = ganglion (Clic here for more details about this article) |
4/15. Autosomal dominant hypoparathyroidism with intracranial calcification outside the basal ganglia.We describe a family with autosomal dominant hypoparathyroidism. The 3 affected individuals had no detectable serum parathyroid hormone on radioimmunoassay. The propositus presented with seizures and on CT scan had bilateral basal ganglion calcification and calcification in the frontal lobes. His similarly affected mother had even more intracerebral calcification. The latter manifestation has not been described previously in autosomal dominant hypoparathyroidism.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
5/15. Basal ganglion calcification in hyperphenylalaninemia due to deficiency of dihydropteridine reductase.The disease course and therapy of a nine-and-a-half-year-old boy with hyperphenylalaninemia due to a dihydropteridine reductase deficiency are reported. Clinically, there is a marked mental retardation and complex basal ganglion symptoms. The cranial computed tomographic investigation shows bilateral, symmetrical, comma-shaped calcifications in the globus pallidus and the putamen of the lentiform nucleus. The cause of these basal ganglion calcifications remains unclear. Lowering of serum and CSF folic acid levels could not be detected, in contrast to cases with the same enzyme defect described previously.- - - - - - - - - - ranking = 1.2keywords = ganglion (Clic here for more details about this article) |
6/15. Calcified ganglioneuroma of the bladder.Ganglioneuromas involving the genitourinary tract are extremely rare but are potentially serious tumors. We describe a patient, known to have von Recklinghausen's disease, who presented with a calcified ganglioneuroma of the bladder demonstrated by intravenous urography, sonography, and computed tomography.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
7/15. Cerebral subcortical calcification and hypoparathyroidism--a case report and review of the literature.Hypoparathyroidism is known to exhibit various neurological manifestations, but most of the neurological disorders seen in this condition are motor ones, and involuntary movement is rarely encountered. Basal ganglion calcification is a common feature in hypoparathyroidism. But calcification of the cerebral cortex is, to our knowledge, extremely rare with only nine previously documented cases reported. We report a case of both involuntary movement and extensive cortical calcification with some reference to the literature. Furthermore, we discuss the relationship between neurological disorders and intracranial calcification.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
8/15. frontal lobe calcification in hypoparathyroid states.Basal ganglion calcification is common in hypoparathyroid states. However, cerebral cortical calcification is rarely seen. Of the six cases previously recorded, five were in patients with idiopathic hypoparathyroidism and only one in a patient with pseudohypoparathyroidism. The present report is of a second case of pseudohypoparathyroidism with cerebral cortical calcification. It is stressed that cortical calcification occurs predominantly in the frontal lobes.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
9/15. Intraluminal calcifications in the small bowel of newborn infants with total colonic aganglionosis.Intraluminal calcifications were found in the small bowel of 4 newborns with total colonic aganglionosis. Abdominal radiography demonstrated circular aggregations of small punctate calcific densities in the right lower quadrant and evidence of bowel obstruction. There was a microcolon in each case. The calcifications, which resemble those seen in small intestinal atresia and stenosis, are probably related to fetal intestinal stasis, and may be differentiated from those due to meconium peritonitis.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
10/15. Intracranial calcification in oculodento-osseous dysplasia.Oculodento-osseous dysplasia (ODOD) is a rare genetic disorder characterized by microphthalmia, hypoplasia of the dental enamel, dysplastic and sclerotic changes in the skeleton and various digital malformations. The occurrence of basal ganglion calcification in this condition is of considerable interest, particularly as it has some biochemical features in common with hypoparathyroidism and pseudohypoparathyroidism. The 2 patients presented in this article did not show any features of an extrapyramidal disorder, but both experienced progressive spasticity in the limbs. The neurological disturbance was not due to compression of the neuraxis by bony overgrowth, but to an associated neurological lesion of undetermined nature. The biochemical abnormalities are discussed but as yet no explanation for the association between intracranial calcification and ODOD can be offered.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
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