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1/76. Visual loss with Langerhans cell histiocytosis: multifocal central nervous system involvement.

    A 42-year-old woman with a 6-year history of diabetes insipidus and progressive hypersomnolence presented with visual loss. neuroimaging showed infiltration in the hypothalamus, the optic nerve, and the chiasm, as well as multiple lesions in other areas of the brain parenchyma. biopsy showed Langerhans cell histiocytosis. This is an unusual presentation of Langerhans cell histiocytosis, involving the visual pathways without manifestations outside of the central nervous system. The differential diagnosis and the magnetic resonance imaging findings will be discussed.
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ranking = 1
keywords = diabetes insipidus, insipidus, diabetes
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2/76. Endoscopic sinus surgery in the management of mucormycosis.

    This is a report of the use of endoscopic sinus surgery in the management of three patients diagnosed with rhino-orbital or rhino-orbito-cerebral mucormycosis. A retrospective review was performed of the clinical examinations and imaging studies of three patients who underwent endoscopic sinus surgery as part of their therapy for mucormycosis. In addition to endoscopic surgery, all patients had aggressive control of underlying risk factors (diabetes mellitus, immunosuppression) and prolonged intravenous amphotericin b therapy. All three patients survived and avoided orbital exenteration. In selected patients with rhino-orbito-cerebral mucormycosis, endoscopic techniques can play a valuable role in diagnosis and management.
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ranking = 0.023551559800277
keywords = diabetes
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3/76. Sinus histiocytosis with massive lymphadenopathy: a case of multiple dural involvement.

    An exceptional case of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) arising from the meninges in a 60-year-old Japanese man is presented. Computerized tomographic scans and magnetic resonance images demonstrated well-circumscribed tumorous lesions that were homogeneously enhanced with contrast medium. Systemic examination revealed no abnormalities except for a cervical lymphadenopathy and diabetes mellitus. Microscopic examination of the resected specimens showed proliferated histiocytosis and infiltration of plasma cells and lymphocytes. The histology was characterized by the presence of histiocytes demonstrating lymphophagocytosis and immunoreactivity for S-100 protein staining. Immunohistochemical studies and electron microscopy were useful in confirming the diagnosis. The clinical and histopathological features of this disease are discussed.
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ranking = 0.023551559800277
keywords = diabetes
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4/76. Rhinocerebral mucormycosis in patients with burns: case report and review of the literature.

    mucormycosis is an opportunistic infection most commonly occurring in patients with impaired host defenses or diabetes mellitus. In patients with burns the rhinocerebral form is rare, and mucormycotic infections more commonly involve the cutaneous burn wound. Both forms are associated with a high mortality rate that increases with delays in treatment. The initial management of these types of infections includes vigorous glucose control, correction of acidosis, and the administration of systemic antifungal agents such as amphotericin b. The rhinocerebral form of mucormycosis is extremely virulent and may warrant the use of interstitial and intraventricular antifungal therapy. Despite these measures, the mainstay of treatment for both forms of mucormycosis is the extensive surgical debridement of all infected and necrotic tissue.
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ranking = 0.023551559800277
keywords = diabetes
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5/76. Myoclonic encephalopathy and diabetes mellitus in a boy.

    We describe an 18-month-old boy with insulin-dependent diabetes mellitus who developed idiopathic myoclonic encephalopathy (dancing eye syndrome) at 26 months of age. The neurological symptomatology (multifocal myoclonus, opsoclonus, ataxia, behavioural disturbance) developed within 10 to 14 days after presentation. Biological, neuroradiological, and scintigraphic examination excluded CNS infectious diseases, intoxication, or tumours. At onset of diabetes mellitus, anti-glutamic-acid decarboxylase (GAD) antibodies were observed, and markedly increased in titre when myoclonic encephalopathy occurred. Corticosteroid treatment resulted in a decrease in anti-GAD autoantibody titres and the disappearance of neurological disturbances. As GAD is expressed both in pancreatic beta-cells and cerebellar purkinje cells, it is possible that a common autoimmune disorder in this patient may account for both the diabetes and myoclonic encephalopathy.
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ranking = 0.16486091860194
keywords = diabetes
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6/76. Five cases with central diabetes insipidus and hypogonadism as first presentation of neurosarcoidosis.

    OBJECTIVES: We retrospectively reviewed 5 patients with neurosarcoidosis, who all presented with central diabetes insipidus and hypogonadism. DESIGN: This was a single-centre, retrospective analysis of 5 cases with a minimum follow-up of 2 years. methods: Case analysis included clinical, biochemical, and endocrinological evaluation and frequent CT/MRI scans of involved organs as primary evaluation and in response to immunosuppressive therapy. RESULT: Neurosarcoidosis was diagnosed in all patients. Two patients had no proven extracerebral manifestation and had a stable disease over 3 and 5 years. One patient showed deterioration with corticosteroids alone but partial remission after additional cyclophosphamide. Pituitary dysfunction remained unchanged in all patients, despite total clinical and radiological remission in two patients. However, one of these patients died of acute granulomatous meningoencephalitis after two years of follow-up. CONCLUSION: Although the presenting symptoms of neurosarcoidosis may vary, the occurrence of central diabetes insipidus associated with typical radiological features is suggestive of neurosarcoidosis. However, there is an increasing number of case reports on lymphocytic hypophysitis. Without the bioptic diagnosis, the differentiation between potentially lethal isolated neurosarcoidosis and lymphocytic hypophysitis is difficult. These cases demonstrate the difficulties in diagnosing neurosarcoidosis and reflect experiences with follow-up parameters.
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ranking = 6
keywords = diabetes insipidus, insipidus, diabetes
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7/76. Acute subdural hematoma and intracerebral hemorrhage. Rare complications of rhinocerebral mucormycosis.

    Rhinocerebral mucormycosis is a short-term and often rapidly lethal fungal disease. It is generally seen in uncontrolled cases of diabetes with ketoacidosis. This case exhibits many of the features of a typical fulminating rhinocerebral mucormycosis. However, the fatal complications of acute subdural hematoma and massive intracerebral hemorrhage due to rupture of aneurysm, as demonstrated by angiography, are unique clinical manifestations of patients with rhinocerebral mucormycosis.
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ranking = 0.023551559800277
keywords = diabetes
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8/76. mucormycosis in a transplant recipient.

    mucormycosis classically occurs in patients who have uncontrolled diabetes who develop rhinocerebral disease. A fatal case of rhinocerebral infection caused by rhizopus arrhizus in a 53-year-old man who had received a renal homograft three years previously is reported. Only five similar cases have been documented, all since 1970. Although direct smears of the purulent nasal exudate revealed the presence of numerous Gram-negative bacilli, later identified as haemophilus influenzae, the diagnosis of mucormycosis was made by demonstrating the typical broad, nonseptate branched hyphae in the necrotic tissue obtained by surgical debridement of the paranasal sinuses. culture of this material revealed growth of mold-like fungus which, upon direct microscopic examination, showed sporangiophores bearing spherical sporangia arising directly from a cluster of root-like structures of rhizoids. Despite the immediate institution of therapy with amphotericin b postoperatively, the patient died 48 hours later. Subsequently, the rhizopus isolated was shown to be resistant to both amphotericin b and 5-fluorocytosine. The present case and two others stress the importance of an aggressive diagnostic approach to patients suspected of having mucormycosis, because the usual microbiologic technics are frequently, inexplicably, unsuccessful, and possibly even misleading in this disease.
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ranking = 0.023551559800277
keywords = diabetes
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9/76. 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 = 0.023551559800277
keywords = diabetes
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10/76. Central diabetes insipidus as the first manifestation of neurosarcoidosis in a 10-year-old girl.

    Central diabetes insipidus with thickening of the pituitary stalk has been demonstrated in adults with Langerhans cell histiocytosis (LCH), tuberculosis or sarcoidosis, and in children with LCH. We present a 10.5-year-old girl with central diabetes insipidus. magnetic resonance imaging (MRI) revealed thickening of the central part of the pituitary stalk and multiple hyperintense lesions in the frontal white matter on T(2)-weighted images. Laboratory findings were normal except for an elevated serum angiotensin-converting enzyme (ACE) level. The MRI findings together with the elevated serum ACE level highly suggest the diagnosis of sarcoidosis. We conclude that central diabetes insipidus can be the first clinical manifestation of sarcoidosis in children.
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ranking = 7
keywords = diabetes insipidus, insipidus, diabetes
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