11/30. Autosomal dominant osteopetrosis type II with "malignant" presentation: further support for heterogeneity?The osteopetroses are a heterogeneous group of disorders characterised by generalised bony sclerosis. The autosomal dominant form usually has a "benign" prognosis, in contrast to the "malignant" course of the autosomal recessive variety. In this paper we describe a kindred in which the phenotypic spectrum varied from an asymptomatic condition in adults to a severely affected infant, presenting with anaemia, hepatosplenomegaly, hydrocephalus and blindness. The findings in this family are reported and discussed to elucidate further the possible genetic heterogeneity in autosomal dominant osteopetrosis.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
12/30. neuromyelitis optica: two new cases and review of the literature.The clinical features of two recent cases of neuromyelitis optica are reviewed, along with 43 cases from the literature. Severe bilateral visual impairment, thoracic myelitis, prodromal symptoms suggesting a viral syndrome, and moderate pleocytosis of the cerebrospinal fluid (CSF) were characteristic. Respiratory failure developed in 22% of the cases. Seventy percent of patients improved neurologically, 14% had a poor neurological outcome, and 16% died in the acute stages. Predictors of a poor outcome were older age, marked CSF pleocytosis, and severe myelitis. Forty-two percent of patients had a recurrence of demyelinating disease after initial recovery, suggesting a diagnosis of multiple sclerosis. Fifty-eight percent of patients had a self-limited monophasic illness, consistent with a post-infectious encephalomyelitis. No clear predictors of patients at risk for recurrence were identified. CSF oligoclonal bands were absent in three patients with information available.- - - - - - - - - - ranking = 13.492691071149keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
13/30. amaurosis fugax in teenagers. A migraine variant.Sudden, transient loss of vision in one eye (amaurosis fugax) is associated frequently with atherosclerosis of the internal carotid artery in adults and may herald a stroke. Thus, cerebral angiography is often performed. amaurosis fugax in children is uncommon and an underlying cause is rarely demonstrated. Recurrent episodes of amaurosis fugax occurred in five adolescents. A characteristic evolution and pattern of visual loss, consistent with choroidal ischemia as the underlying mechanism, was described by four of them. Although none of the episodes were accompanied by headache, four patients had a history of common migraine at other times or a family history of migraine. These episodes of visual loss may represent a migraine variant, and cerebral angiography is not indicated in adolescents with such a history.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
14/30. Bilateral retrobulbar optic nerve infarctions after blood loss and hypotension. A clinicopathologic case study.A 59-year-old woman with anemia became totally blind after repeated gastrointestinal bleeding and acute hypotension. Neuropathologic examination was normal apart from bilateral infarctions centered on the orbital portion of the optic nerves. This is the only "pure" histopathologic study of visual loss after hemorrhage and hypotension in the recent literature, the single previous case being complicated by arteriosclerosis and vasculitis. The authors suggest that visual loss after hypotension is of three types. Profound hypotension in patients with neither anemia nor arteriosclerosis generally causes water-shed infarctions in the parietal and occipital lobes. Brief hypotension combined with arteriosclerosis favors juxtalaminar optic nerve infarction indistinguishable from spontaneous anterior ischemic optic neuropathy. In anemic patients without arteriosclerotic risk factors, hypotension is likely to cause infarction in the orbital optic nerve, where pial end vessels are subject to compression from hypoxic edema.- - - - - - - - - - ranking = 3keywords = sclerosis (Clic here for more details about this article) |
15/30. Retinal arteriolar occlusion in multiple sclerosis.A 25-year-old white man presented with acute multiple sclerosis manifested by right blindness, difficulty urinating, and paresthesias and weakness of both legs. Retinal examination revealed a distal occlusion of the descending branch of the superior temporal arteriole leading to an area of retinal ischemia of the right eye. The optic disc was edematous, and there were focal areas of periphlebitis. All retinal signs resolved in three weeks, and the only abnormality that persisted was a pale right optic disc. The finding of small arteriolar disease is unusual and may represent another possible pathogenetic mechanism in multiple sclerosis.- - - - - - - - - - ranking = 80.956146426895keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
16/30. Postprandial transient painful amaurosis fugax.A case of recurrent postprandial amaurosis fugax (AF) associated with periorbital pain is described. Clinical and angiographic examination revealed moderate atherosclerosis of the cerebral vessels and narrow-angle glaucoma. Cerebral postprandial hypoperfusion combined with increased intraocular pressure probably precipitated the painful monocular blindness. The attacks of AF subsided partially after treatment of the glaucoma.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
17/30. Absent common carotid artery associated with amaurosis fugax: a case report.A patient with amaurosis fugax and extensive atherosclerosis of the internal carotid artery combined with an absent common carotid artery that required extra-anatomic reconstruction is presented.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
18/30. Schilder's (1912) disease. Total cerebral blindness due to acute demyelination.A 51-year-old woman became blind from involvement of both occipital lobes with a confluent lesion demonstrated by computed tomographic scan. biopsy showed demyelination consistent with Schilder's 1912 variant of diffuse sclerosis. No cytoplasmic inclusions were found on electron microscopy.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
19/30. Temporal arteritis-like presentation of carotid atherosclerosis.A 68 year-old woman presented with a two-week history of amaurosis fugax, ipsilateral fronto-temporal headache and jaw claudication suggesting carotid giant cell arteritis. However, this syndrome proved to be due to atherosclerosis causing complete occlusion of the external carotid artery at its origin and narrowing of the internal carotid artery. Combined external and internal carotid endarterectomy relieved the symptoms. The symptom complex of temporal arteritis may be rarely mimicked by carotid atherosclerotic occlusive disease.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
20/30. Central retinal artery spasm.Central retinal artery spasm, a seldom witnessed ocular sign, has been recognized in one eye of a patient complaining of transient monocular blindness. Removal of an ulcerative atheromatous plaque of the carotid artery on the same side as the eye with central retinal artery spasm resulted in complete disappearance of symptoms. No other signs of ocular pathology were noted in addition to the central retinal artery spasm. Central retinal artery spasm occurring in both eyes simultaneously may be the mechanism explaining the visual loss of amaurosis fugax. Physiologic mechanisms associating brief episodes of monocular blindness and carotid artery obstructive disease with atherosclerosis are discussed. The possibility of central retinal artery spasm initiating occlusion of the same vessel is mentioned.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
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