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1/69. Rhinocerebral mucormycosis: a case of a rare, but deadly disease.

    Rhinocerebral mucormycosis is a rare fungal infection of the nasal cavity and sinuses that can spread to the orbits and cranium within days. Its presentation can be confused with those of sinusitis, viral infections, diabetic ketoacidosis, or carotid sinus thrombosis, and it is often missed at early presentation. survival is directly linked to early detection and treatment. We present a case of rhinocerebral mucormycosis and discuss the literature on its early signs and symptoms, pathophysiology, and treatment options.
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ranking = 1
keywords = diabetic
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2/69. 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.
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ranking = 9.7829790421562
keywords = microangiopathy
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3/69. Palinopsia and polyopia in the absence of drugs or cerebral disease.

    OBJECTIVE: To report the occurrence of palinopsia and polyopia in patients who neither used drugs nor had diseases of the cerebral hemispheres, a group in which these visual symptoms have not been reported. METHOD: The patient records in the database of an academic neuro-ophthalmology unit were reviewed. RESULTS: Seventeen patients were identified in the database with the diagnosis of palinopsia or polyopia, of whom eight had diseases of the cerebral hemispheres, leaving nine patients for analysis. No patients with a history of drug toxicity were identified. In one patient the symptoms presented during an initial episode of demyelinative optic neuritis in the absence of clinical or laboratory evidence of cerebral lesions. In another patient they developed immediately after laser treatment of diabetic macular edema. A third patient developed the symptoms in association with visual loss from Leber's hereditary optic neuropathy. The other six patients were healthy individuals. CONCLUSION: Palinopsia and related visual symptoms can occur in otherwise healthy individuals and in patients with disease apparently confined to the eye or the optic nerve.
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keywords = diabetic
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4/69. Identification of the asexual state of rhizopus species on histologic tissue sections in a patient with rhinocerebral mucormycosis.

    mucormycosis is an infection caused by a group of fungi in the order mucorales in the phylum Zygomycota. The most well-known form of this disease is rhinocerebral mucormycosis, which usually develops in diabetic or immunocompromised patients. The fungal hyphal elements are easily detected in biopsy specimens by direct or histologic examination. However, the confirmatory identification of the genus or species requires culture of the specimen. This article presents a case of rhinocerebral mucormycosis in which presumptive identification of the genus was made without microbiologic cultures and was based on the extraordinarily rare appearance of fungal sporangia and sporangiospores in histologic tissue sections. Identification of these structures allowed an early and accurate diagnosis of rhinocerebral invasive mucormycosis.
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ranking = 1
keywords = diabetic
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5/69. liver and brain mucormycosis in a diabetic patient type II successfully treated with liposomial amphotericin b.

    A case of liver and brain mucormycosis in a 73-y-old diabetic patient is described. The patient presented with fever and a moderate, tender hepatomegaly and a C/T scan examination of the abdomen and brain showed multiple hepatic and cerebral nodular lesions. The largest of the liver lesions was aspirated and broad hyphae of mucor were demonstrated in the purulent material obtained. The patient was treated successfully (for 40 d) with intravenous liposomal amphotericin b and then with itraconazole for 3 months. To our knowledge, this is the first case of a diabetic patient with both liver and brain mucormycosis who has been treated successfully.
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ranking = 6
keywords = diabetic
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6/69. Rhinocerebral mucormycosis diagnosis by aspiration cytology.

    Rhinocerebral mucormycosis is a rapidly progressing, often fatal fungal infection that occurs commonly in diabetics and immunocompromised individuals. We present 2 cases of rhinocerebral mucormycosis with a paranasal mass. One patient had an intracranial extension. Nasal scrapings and fine-needle aspiration cytology (FNAC) of the paranasal masses showed fungal hyphae morphologically resembling Mucor. Surgical material showed features of mucormycosis. FNAC and scrape smears can give a conclusive diagnosis of mucormycosis, and the patient can be treated with appropriate antifungal therapy and surgical debridement. Preoperative cytology is an effective technique to establish a diagnosis of mucormycosis and obviates the need for a preoperative biopsy.
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ranking = 1
keywords = diabetic
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7/69. 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.
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ranking = 1.630496507026
keywords = microangiopathy
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8/69. susac syndrome: microangiopathy of the retina, cochlea and brain.

    BACKGROUND: susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. methods: Analysis of two male and two female cases of susac syndrome recognized in australia. RESULTS: In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behcet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis. CONCLUSION: susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.
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ranking = 8.1524825351302
keywords = microangiopathy
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9/69. Mucor endophthalmitis.

    PURPOSE: To report on a case of type 2 diabetes, with eyelid gangrene and endophthalmitis as a presenting manifestation of rhino-orbito-cerebral mucormycosis. RESULTS: CECT head showed proptosis, mucosal thickening in the ethmoid sinus and hypodense lesions in the frontal and occipital lobes. Vitreous tap showed right angle branched aseptate hyphae consistent with mucormycosis. CONCLUSIONS: A diabetic patient presenting with sudden loss of vision, eyelid gangrene and endophthalmitis, involvement by an angio-invasive fungus-like mucormycosis is an important consideration.
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ranking = 1
keywords = diabetic
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10/69. Bilateral rhinocerebral phycomycosis.

    A 51-year-old diabetic woman developed bilateral rhinoorbitocerebral phycomycosis. Successful treatment was accomplished by correction of the ketoacidosis, surgical debridement, and amphotericin b therapy. Rhinoorbitocerebral phycomycosis is a fulminant and frequently fatal disease most often seen in debilitated hosts. This survival suggests that aggressive therapy is indicated in patients with extensive fungal involvement.
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ranking = 1
keywords = diabetic
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