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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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keywords = diabetes insipidus, insipidus, diabetes
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2/76. Langerhans cell histiocytosis of the vulva: an ultrastructural study.

    langerhans cells histiocytosis (LCH) is a proliferative disorder of langerhans cells. The lesions are normally characterized by infiltration of eosinophils, neutrophils, lymphocytes, plasma cells, and langerhans cells. The specific cells of LCH contain Birbeck granules, express the phenotype of langerhans cells but with markers fixed at an early stage of activation, and are functionally defective in antigen-presenting ability. The disease most often affects children; when it occurs in older patients, anal and groin involvement is quite common and vulvar lesions can be found in older females. The authors report a case of a 64-year-old woman with LCH of the vulva and diabetes insipidus. An immunohistochemical and ultrastructural study of the vulvar lesions showed an infiltrate in which antigenically and morphologically mature langerhans cells, monocytoid cells, and cells with an intermediate phenotype between monocytes and langerhans cells were concurrently observed. Although the clinical and histological aspects of LCH are well established, the pathogenetic mechanism of lesions is not yet known. The finding of an infiltrate composed by langerhans cells and many putative precursors of these cells suggests the hypothesis of an in situ differentiation of langerhans cells from immature monocytoid precursors.
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keywords = diabetes insipidus, insipidus, diabetes
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3/76. diabetes insipidus in Langerhans' cell histiocytosis: report of a case.

    The incidence of diabetes insipidus secondary to Langerhans' cell histiocytosis (LCH) varies among different reports, ranging from 9.5 to 50%, but it has never been reported in literature in taiwan. Therefore, we presented a case suffering from polyuria, polydipsia, body weight loss for more than one year and seborreic dermatitis-like skin lesions over the scalp and trunk for more than two years. Her body weight and body length were both less than 3 percentile. Fluid restriction and vasopressin test were performed to differentiate nephrogenic from neurogenic diabetes insipidus. skin biopsy revealed picture of LCH and LCH with complete central diabetes insipidus was diagnosed. Brain MRI and other laboratory examinations were all within normal limits. She received nasal DDAVP treatment and chemotherapy with TPOG-H 94 protocol. After 3 months treatment, her skin lesions disappeared and daily urine amount returned to normal range.
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keywords = diabetes insipidus, insipidus, diabetes
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4/76. brain stem infiltration by mixed Langerhans cell histiocytosis and Chester-Erdheim disease: more than just an isolated case?

    Langerhans cell histiocytosis is classically considered as totally different from Chester-Erdheim's disease which consists in the infiltration of various parenchymas by macrophagic CD68-positive histiocytes. We report the case of a 46-year-old woman with a long history of diabetes insipidus who presented typical lesions of Langerhans cell histiocytosis on vulvar and skin biopsies as well as bony cellular infiltrates characteristic of Chester-Erdheim's disease. A few months later she presented cerebellar disorders and died after an 18-month course. At autopsy the pons was enlarged, due to numerous cellular infiltrates which were also scattered in the middle cerebellar pedoncles, dentate nuclei, midbrain and hypothalamus. There were S100-protein positive langerhans cells intermingled with numerous ovoid CD68-positive histiocytes. There are a few reported cases of Chester-Erdheim's disease presenting foci of langerhans cells histiocytosis in other parenchymas. In addition, there are 10 reported cases with diabetes insipidus and bilateral infiltration of the brain stem and cerebellum, considered as presenting either one type of histiocytosis or the other. Our case demonstrates that both histiocytoses may coexist in the brain and thus correspond in fact to the same pathology in certain particular cases.
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keywords = diabetes insipidus, insipidus, diabetes
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5/76. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis.

    Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination.
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keywords = diabetes insipidus, insipidus, diabetes
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6/76. Hypothalamic Langerhans cell histiocytosis with no eosinophils.

    A 26-year-old man presented with diabetes insipidus, sexual dysfunction and memory impairment. Nuclear magnetic resonance imaging and pathologic examination revealed Langerhans cell histiocytosis of the hypothalamus, which contained no eosinophils. The lesional cells were positive for S-100 protein and CD1a antigen, and negative for CD68 antigen. The possible pathogenesis, absence of eosinophils and prognosis are discussed.
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keywords = diabetes insipidus, insipidus, diabetes
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7/76. Neuropsychologic deficits in children with Langerhans cell histiocytosis.

    BACKGROUND: Manifestations of Langerhans cell histiocytosis (LCH) in children range from only a rash, to bony lesions accompanied by pain, to major organ disease. When the central nervous system (CNS) is affected, the LCH patient may exhibit signs and symptoms of hypothalamic and pituitary dysfunction (most often resulting in diabetes insipidus or other endocrinopathies) or more global neurologic and neuropsychologic sequelae. Surprisingly, researchers have only recently begun to examine the neuropsychologic manifestations of the disease, but early findings suggest that they may, in fact, be significant in a small percentage of children with LCH. PROCEDURE: We evaluated two CNS-positive patients with LCH and long-term intermittent treatments, using extensive neuropsychologic assessments, including intellectual functioning, memory, visual-motor functioning, attention and concentration, sensory and motor performance, and gross academic achievement. Objective measures of behavior were obtained through parental report. Neuroradiologic imaging was obtained concurrently with the neuropsychologic evaluations. RESULTS: The neuropsychologic assessments indicated significant deficits in a number of the measured areas of functioning. Global cognitive deficiencies in full-scale IQ were identified, as were deficits in memory, attention/concentration, and perceptual-organizational capabilities. Similarities were noted in the patterns of deficits obtained with both patients, despite differences in the pathophysiology of their disease. Behavioral functioning in both children had suffered, presumably in relation to the neuropsychologic deficits. There were radiologic findings of gross cerebellar white matter damage in one patient, in addition to focal (e.g., hypothalamic) lesions in the other. CONCLUSIONS: LCH has an adverse impact on cognitive functions in some children with evidence of CNS involvement, and further study into the etiology, incidence, and means of remedial intervention is needed.
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ranking = 1
keywords = diabetes insipidus, insipidus, diabetes
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8/76. Langerhans' cell histiocytosis: report of an atypical case.

    A patient is described with Langerhans' cell histiocytosis and polyneuropathy diagnosed 12 years after the development of diabetes insipidus after head trauma.
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keywords = diabetes insipidus, insipidus, diabetes
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9/76. histiocytosis X and compulsive water drinking: report of a case.

    We describe a patient with histiocytosis X and compulsive water drinking. The association of diabetes insipidus with histiocytosis X is well recognized, and this patient was initially considered to have diabetes insipidus. It was only after further testing that the proper diagnosis was made.
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keywords = diabetes insipidus, insipidus, diabetes
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10/76. Langerhans cell histiocytosis of the thyroid gland. A case report.

    BACKGROUND: The variant of Langerhans histiocytosis commonly encountered in adults is the benign, localized form (eosinophilic granuloma). The more-aggressive or diffuse type (Letterer-Siwe disease) is rare in adults. CASE: A 28-year-old woman presented with enlargement of the thyroid gland three years after she had been diagnosed with and placed on treatment for diabetes insipidus. thyroidectomy was performed following an initial fine needle aspiration cytology report of either papillary thyroid carcinoma or Langerhans cell histiocytosis. The latter diagnosis was confirmed on histopathology and immunohistochemical staining for S-100 protein. Intracellular Birbeck granules were also demonstrated by electron microscopy. The disease progressed over a 10-week period to involve the kidneys, resulting in renal dysfunction. CONCLUSION: Synchronous or metachronous involvement of the hypothalamus and thyroid gland by Langerhans histiocytosis could not be excluded in the present case, with subsequent progression to involve other organs. This was an unusual presentation of the disease in an adult.
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ranking = 1
keywords = diabetes insipidus, insipidus, diabetes
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