1/136. 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 = 1keywords = white (Clic here for more details about this article) |
2/136. A distinct difference in clinical expression of two siblings with Aicardi-Goutieres syndrome.Two sibs with an encephalopathy, including intracerebral calcification and a white matter disease, are reported. In the younger sister, the cerebrospinal fluid showed chronic pleocytosis and clinically she strictly fits to the diagnosis of Aicardi-Goutieres syndrome. Both sisters were affected by a spastic tetraplegia, truncal hypotonia and dystonic posturing, but the clinical course and the neuroradiological findings were milder in the older sister and she showed no cerebrospinal fluid pleocytosis. The present cases and recent reports of intrafamilial variability of Aicardi-Goutieres syndrome may raise interesting aspects as to the limits and criteria of this syndrome.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
3/136. hemangioma calcificans. Case report of an intraparenchymatous calcified vascular hematoma with epileptogenic potential.A middle-aged woman, with a previous history of medically suppressed absence attacks, presented with mild changes in mental status and a skull film demonstrating several areas of mottled, granular, intracranial calcifications. These lesions, although readily visible on computerized tomography, appeared avascular during the course of cerebral angiography. At the time of surgery the masses, which were densely calcified and generally circular, demonstrated numerous areas of superficial, white, verrucous excrescences. Microscopic, pathological evaluation confirmed the diagnosis of hemangioma calcificans. The literature describing this rare entity is briefly reviewed.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
4/136. Abdominal pregnancy in a 67-year-old woman undetected for 37 years. A case report.BACKGROUND: Abdominal pregnancy is an exceedingly rare occurrence, but even more unusual is prolonged retention of an advanced abdominal pregnancy with lithopedion formation. We present the case of prolonged retention of an advanced abdominal pregnancy in an elderly women. CASE: A 67-year-old, white woman presented to the emergency department with abdominal pain. An acute abdominal series revealed a fetal skeleton extending from the patient's pelvis to her lower costal margins. Pelvic examination revealed a normal postmenopausal uterus, and human chorionic gonadotropin was negative. On further questioning the patient reported that she had become pregnant 37 years earlier and was diagnosed as having a "missed" pregnancy. She refused intervention at that time but suffered no untoward consequences. She reported having had later a healthy intrauterine pregnancy, delivered vaginally at term. No attempt was made to remove the prior missed abdominal pregnancy. The acute pain episode resolved, and there was no surgical intervention. CONCLUSION: Abdominal pregnancies can have a complex course, and management decisions can be difficult. This case presents an unusual outcome of an advanced abdominal pregnancy and illustrates a unique approach to management.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
5/136. Aicardi-Goutieres syndrome: a genetic microangiopathy?Aicardi-Goutieres syndrome (AGS) (McKusick 225750) is an autosomal recessive disease with onset in the 1st year of life, resulting in progressive microcephaly, calcification of cerebral white matter, thalamus and basal ganglia, generalized cerebral demyelination and a chronic low-grade CSF lymphocytosis, without evidence of infection. We report the autopsy of a patient who died with this disorder at the age of 17 years. Findings were severe microencephaly, diffuse but inhomogeneous cerebral white matter loss with associated astrocytosis, calcific deposits in the white matter, thalami and basal ganglia. neocortex and cerebellar cortex were affected by wedge-shaped microinfarctions. Small vessels showed calcification in the media, adventitia and perivascular spaces. These findings are similar to some previous publications that in retrospect may have been AGS, but this is the first reported cerebral microangiopathy in which the diagnosis AGS was made during lifetime. This report provides evidence that microangiopathy plays a significant role in the pathogenesis of AGS.- - - - - - - - - - ranking = 3keywords = white (Clic here for more details about this article) |
6/136. Calcifying epithelial odontogenic tumor. A histologic, histochemical, fluorescent, and ultrastructural study.A previously unreported calcifying epithelial odontogenic tumor (CEOT; Pindborg tumor) occurring in a Negro patient is presented. Only 12 per cent of these neoplasms have been found in nonwhite patients. Ultrastructural and histochemical studies indicated that the amorphous, acellular, eosinophilic substance present within the tumor was not amyloid. The presence of only sparse calcifications is an unusual histologic variant. No relationship between neoplasm and reduced enamel epithelium could be demonstrated.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
7/136. Sclerochoroidal calcification associated with gitelman syndrome.PURPOSE: To investigate sclerochoroidal calcification in a patient with Gitelman syndrome. METHOD: Case report. Bilateral fundus abnormalities observed in a 58-year-old woman were documented with fluorescein angiography and tomodensitometry. RESULTS: Symmetric yellow-white subretinal lesions were observed in the superotemporal midperiphery of the fundus of each eye. Tomodensitometry examination was consistent with calcium deposition. The medical history included gitelman syndrome. Sclerochoroidal calcification probably resulted from the severe hypomagnesemia. CONCLUSION: gitelman syndrome may be a cause of sclerochoroidal calcification.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
8/136. Unusual signs for dural arteriovenous fistulas with diffuse basal ganglia and cerebral calcification.We present a case of multiple dural arteriovenous fistulas (AVFs) in a 60-year-old man with the chief complaint of worsening headache, altered mental status and progressively unsteady gait over the course of one year. Computerized tomography revealed diffuse, symmetric calcification in the bilateral basal ganglia and bilateral periventricular and subcortical white matter. magnetic resonance imaging revealed multiple, enhanced, punctate and linear vessels. These images were due to reflux into the parenchymal veins in the dural AVF of the superior sagittal sinus within the basal ganglia and deep white matter of both cerebral hemispheres. cerebral angiography disclosed multiple dural AVFs. The exact mechanism of basal ganglia and subcortical calcification is proposed to be an arterial steal phenomenon or persistent venous congestion, with calcification occurring in a chronic hypoperfused state or with dystrophic changes in the walls of congested veins.- - - - - - - - - - ranking = 2keywords = white (Clic here for more details about this article) |
9/136. Inflammatory pseudotumor of the spleen: a case report.We report on an inflammatory pseudotumor of the spleen. A 72-year-old woman visited our hospital complaining of nausea. physical examination and laboratory investigations were unremarkable. ultrasonography, computed tomography, magnetic resonance imaging and angiography showed a hypovascular splenic mass measuring about 5 cm in diameter with a calcification in the center of the lesion. splenectomy was performed. The removed spleen, weighing 145 g, contained a tan-white, circumscribed mass, measuring 6.2 x 5.5 x 5.3 cm. Histologically, the splenic mass was composed of an admixture of inflammatory cellular elements, predominantly plasma cells and lymphocytes with hyalinization, fibrosis, lymph follicles and multinuclear giant cells, suggestive of a inflammatory pseudotumor. The patient is currently alive and asymptomatic, 24 months after surgery. Inflammatory pseudotumors of the spleen are extremely rare and only 39 cases have been reported in the medical literature.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
10/136. pseudoxanthoma elasticum and calcinosis cutis.A 42-year-old white woman presented with clinical and histologic manifestations of both calcinosis cutis and pseudoxanthoma elasticum: discrete milia-like calcifications at the anterior aspect of the neck, a funduscopic examination with classic eye findings, peripheral vascular disease, and a mottled appearance of the skin at the axillae, groin, and lateral aspects of the neck. A younger sibling had similar skin lesions and deteriorating visual acuity. The patient was normocalcemic and normophosphatemic. This case may represent the coincidental occurrence of two rare entities in the same person or may be suggestive of a pattern of dystrophic calcification associated with pseudoxanthoma elasticum.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
| | Next -> |