1/158. Progressive outer retinal necrosis syndrome as an early manifestation of human immunodeficiency virus infection.Progressive outer retinal necrosis syndrome is a recently recognized variant of necrotizing herpetic retinopathy, developing in patients with acquired immune deficiency syndrome (AIDS) or other conditions causing immune compromise. We report a case in which the diagnosis of retinal necrosis syndrome was made before the diagnosis of AIDS was confirmed. A 41-year-old man presented with a 1-month history of blurred vision in his left eye. Ophthalmologic examination revealed extensive retinal necrosis with total retinal detachment in his left eye and multifocal deep retinal lesions scattered in the posterior fundus as well as in the peripheral retina in his right eye. The serologic test for human immunodeficiency virus (hiv) was positive. Despite intravenous acyclovir treatment for 1 week, the lesions in the right eye showed rapid progression. High doses of intravitreal ganciclovir were then given in addition to intravenous acyclovir. After combined treatment for 1 month, the lesions became quiescent and the visual acuity improved to 20/30. Although the patient soon developed full-blown AIDS, the vision in his right eye remained undisturbed. physicians should suspect progressive outer retinal necrosis syndrome in any patient with rapidly progressive necrotizing retinopathy and test the patient for hiv infection. Aggressive combined antiviral agent therapy should be considered to save vision.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
2/158. Infantile and juvenile presentations of Alexander's disease: a report of two cases.We describe 2 new cases of Alexander's disease, the first to be reported in belgium. The first patient, a 4-year-old girl, presented with progressive megalencephaly, mental retardation, spastic tetraparesis, ataxia and epilepsy: post-mortem examination showed widespread myelin loss with Rosenthal fibers (RFs) accumulation throughout the neuraxis. She was the third of heterozygotic twins, the 2 others having developed normally and being alive at age 5 years. The second patient developed at age 10 years and over a decade spastic paraparesis, palatal myoclonus, nystagmus, thoracic hyperkyphosis and thoraco-lumbar scoliosis with radiological findings of bilateral anterior leukoencephalopathy. brain stereotactic biopsy at age 16 years demonstrated numerous RFs. With these 2 cases, we review the literature on the various clinico-pathological conditions reported as Alexander's disease. We discuss the nosology of this entity and the pathogeny of RFs formation and dysmyelination. Clues to the diagnosis of this encephalopathy in the living patient are briefly described.- - - - - - - - - - ranking = 48.993135139687keywords = leukoencephalopathy (Clic here for more details about this article) |
3/158. Intracranial myxoid chondrosarcoma with early intradural growth.Chondrosarcomas are extremely rare intracranial cartilaginous tumors of which the myxoid variant is the least reported in the literature. They develop extradurally and generally infiltrate the dura only in advanced stages or at recurrence. We describe the case of a 55-year-old woman with a posterior cranial fossa myxoid chondrosarcoma which had a primarily intradural extension.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
4/158. posterior leukoencephalopathy syndrome may not be reversible.The association of an acute reversible encephalopathy with transient occipital lobe abnormalities on imaging studies is well known. This condition has been called reversible posterior leukoencephalopathy syndrome. The clinical presentation usually includes seizures, headache, altered mental status, and blindness, often associated with hypertension and immunosuppressants. The authors discuss a two-year-old male with down syndrome who presented 2 months after allogeneic bone marrow transplantation with severe oculogyric crisis, without other complaints. The patient was being treated for hypertension and was receiving cyclosporine for prophylaxis of graft-vs-host disease. A computed tomography scan of the head revealed marked bilateral lucencies mainly involving the white matter of the occipital lobes, with a few foci of punctate hemorrhage. The condition improved when cyclosporine was discontinued, but an area of leukomalacia was identified on follow-up magnetic resonance imaging. To the authors' knowledge, oculogyric crisis as a presentation of reversible posterior leukoencephalopathy has not been previously described. Recognizing this association is important, because patients receiving cyclosporine are often receiving other medications that can potentially cause dystonic eye movements, possibly leading to a delay in diagnosis and treatment, which can result in an irreversible neurologic deficit.- - - - - - - - - - ranking = 295.95881083812keywords = leukoencephalopathy, posterior (Clic here for more details about this article) |
5/158. Pituitary involvement by Wegener's granulomatosis: a report of two cases.We describe two cases of pituitary involvement by Wegener's granulomatosis. At initial presentation, or during subsequent disease "flares," a pattern of pituitary abnormality was suggested. During periods of remission, we found the pituitary returned to a nearly normal appearance. Loss of the normal posterior pituitary T1 hyper-intensity matched a clinical persistence of diabetes insipidus, suggesting there is permanent damage to this structure by the initial disease process.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
6/158. Progressive dysarthria. case reports and a review of the literature.Two patients presenting with progressive dysarthria as the single initial manifestation of a neurodegenerative condition are described. The nature of the dysarthria as well as the additional symptoms that developed in the course of the disorder are very different in these two cases. Nevertheless, neuroimaging findings are strikingly similar and suggest bilateral involvement of posterior inferior frontal lobe structures, mainly in the dominant cerebral hemisphere. The clinical syndrome of these patients can therefore be considered an example of frontotemporal degeneration presenting without dementia or compartmental alteration, at least in the early stages. This broadens the clinical spectrum of frontotemporal degeneration and demonstrates the need for a syndromal subclassification of this nosological entity.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
7/158. Squamous cell carcinoma arising in an intradiploic epidermoid cyst.A 71-year-old woman presented with the symptoms of a posterior cranial fossa mass. CT and MRI revealed a lytic lesion in the occipital bone and a tumour infiltrating the dura mater, venous sinuses and cerebellum. Histopathology demonstrated a moderately differentiated squamous cell carcinoma arising from a primarily intradiploic epidermoid cyst. Despite surgery and radiotherapy, the tumour progressed and the patient died 1 year later.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
8/158. Central neurocytoma of the fourth ventricle. Case report.The authors report on a 17-year-old boy who suffered from slowly progressive and long-standing symptoms of ataxia, neck pain, and headache. Computerized tomography (CT) and magnetic resonance (MR) imaging revealed a tumor arising from the floor of the fourth ventricle that resulted in a moderate hydrocephalus. A partial resection was performed. Histological and immunohistological findings led to the diagnosis of an atypical central neurocytoma of the fourth ventricle. The imaging features on CT scanning, MR imaging, and proton MR spectroscopy studies, the clinical picture, and the prognosis of this very unusual tumor are discussed. Three cases of neurocytomas in the posterior fossa have been described to date; however, in all three cases some atypical aspects were present. In the present case, with the exception of the very unusual location, both imaging findings and clinical history perfectly met the definition of this rare tumor.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
9/158. Unilateral proptosis resulting from giant-cell arteritis.BACKGROUND: Giant-cell arteritis (GCA) is a systemic, inflammatory vasculopathy that affects small- to medium-sized arteries. Arterial wall inflammation results in reduction of blood flow and subsequent ischemia. Arteries of the head and neck are particularly susceptible, including the ophthalmic and posterior ciliary arteries. The eye care provider is in a position to assist with the ultimate diagnosis of GCA. CASE REPORT: A 79-year-old black man was referred to the eye clinic for evaluation of exophthalmos of the left eye. The patient reported increasing proptosis over the previous 6 months; a history of sudden, permanent vision loss of the affected eye (approximately 2 years earlier); and generalized malaise and chronic frontal headache. Examination did reveal an exophthalmic eye of approximately 8-mm difference by Hertel exophthalmometry. Fundus examination revealed optic nerve pallor O.S. CT scan revealed chronic inflammatory changes of orbital tissue, including the extraocular muscles. No compressive lesions were present. Laboratory testing indicated an elevated erythrocyte sedimentation rate. A tentative diagnosis of giant-cell arteritis was made, which was confirmed with temporal artery biopsy. CONCLUSIONS: patients with ocular complications secondary to GCA manifest several different ocular symptoms, including unilateral and bilateral intermittent blur, sudden complete vision loss, double vision, etc. This was an unusual case of GCA because the initially manifested ocular sign was unilateral proptosis. The patient probably had initial ocular complications of GCA 2 years previously, with sudden loss of vision in the left eye. The patient never sought medical attention at that time, and the unilateral exophthalmic eye resulted from chronic inflammatory orbital changes associated with GCA.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
10/158. On the natural history of trigeminal neuralgia.OBJECTIVE: trigeminal neuralgia is usually considered a separate entity from atypical trigeminal neuralgia. The exact relationship among these two and several other syndromes of facial pain remains unknown. There is no long-term prospective study of the natural history of trigeminal neuralgia nor any explanation for the existence of different, albeit somewhat similar, facial pain syndromes. DESCRIPTION OF CONCEPT: On the basis of our clinical experience, we propose a theory that may explain different facial pain syndromes as sequential stages of the same disease process. Typical trigeminal neuralgia caused by microvascular compression of the trigeminal nerve root in the posterior fossa may become transformed over time into atypical trigeminal neuralgia, if left untreated. This transformation involves change in the character of pain and development of sensory impairment. Two representative cases are presented to support this theory. CONCLUSION: If the theory of progressive change in character of pain and degree of sensory impairment in the course of otherwise typical trigeminal neuralgia is correct, trigeminal neuralgia, atypical neuralgia, and trigeminal neuropathic pain may represent different degrees of injury to the trigeminal nerve, therefore comprising a continuous spectrum rather than discrete diagnoses.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
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