1/91. Encephalopathy associated with haemophagocytic lymphohistiocytosis following rotavirus infection.A 2-year-old Japanese boy with a haemophagocytic lymphohistiocytosis (HLH) associated encephalopathy which developed after rotavirus infection is described. The neurological symptoms consisted of coma, seizures and spastic quadriplegia. On therapy with steroids, etoposide and cyclosporin A, the patient recovered without any neurological deficits. The interferon-gamma levels in serum and CSF were elevated at onset of the disease but had returned to normal at the time of clinical remission. brain MRI revealed diffuse white matter abnormalities and parenchymal volume loss. Proton magnetic resonance spectroscopy revealed elevated lactate in the abnormal lesions observed on MRI, indicating that macrophages not exhibiting aerobic metabolism had infiltrated the CNS. At the time of clinical remission, the white matter abnormalities and brain lactate had disappeared. These findings suggested that the neurological symptoms resulted from the overproduction of cytokines by activated T-cells and macrophages. The pathophysiology of a HLH associated encephalopathy was considered to be a local immune response within the CNS, because interferon-gamma can induce the expression of major histocompatibility complex class I and II antigens on glial cells in the CNS. CONCLUSION: Haemophagocytic lymphohistiocytosis associated encephalopathy should be considered early in the differential diagnosis of cases with acute onset neuropathy.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
2/91. 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.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
3/91. 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.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
4/91. Histiocytic lesion mimicking intrinsic brainstem neoplasm. Case report.This 10-year-old girl presented with a 1-month history of progressive bulbar palsy and a solitary enhancing mass originating within the floor of the fourth ventricle. Results of initial imaging studies and presentation were suggestive of neoplasia. Subtotal resection was performed and pathological examination revealed the mass to be a histiocytic lesion, with no evidence of a glioma. The patient had no other stigmata of histiocytosis and was treated with steroid medications, resulting in prolonged resolution of the lesion. This case demonstrates that for discrete brainstem lesions the differential diagnosis includes entities other than glioma for which treatment is available. biopsy sampling should be considered when technically feasible.- - - - - - - - - - ranking = 0.16666666666667keywords = histiocytosis (Clic here for more details about this article) |
5/91. Atypical form of non-Langerhans histiocytosis with disseminated brain and leptomeningeal lesions.An 18 year old girl presented with acute visual loss. T2 weighted brain MRI showed areas of hyperintensities in the thalamic nuclei, internal capsule, lentiform nuclei, the subarachnoidal spaces, and a retrobulbar infiltration. Analysis of CSF showed numerous foamy histiocytes without malignant cells, raised protein, and depressed glucose concentration. biopsy of the right thalamus demonstrated aggregates of histiocytes with immunohistological and ultrastructural characteristics of non-Langerhans cell histiocytosis. The patient improved with chemotherapy and corticosteroids. After 3 months of treatment, CSF analysis showed no more histiocytes. Cytological examination of CSF can be helpful for the management of patients with extensive histiocytic infiltration.- - - - - - - - - - ranking = 0.83333333333333keywords = histiocytosis (Clic here for more details about this article) |
6/91. September 2000: 15 year old girl with intracranial lesion.The September COM: We reported the case of a 15 year old white girl with solitary brain cortical lesion clinically mimicking meningioma. Microscopic examination revealed histiocytic lesion with foamy and giant cells predominately of Touton type. Subsequent clinical examination did not reveal any similar lesion in the skin or in other location. Expression of S-100 protein in histiocytes is unusual finding, but has been reported recently in juvenile xanthogranulomas (JXG). The only specific ultra-structural finding differing JXG from histiocytic neoplasm of Langerhans' cells histiocytosis type seems to be Birbeck granules.- - - - - - - - - - ranking = 0.16666666666667keywords = histiocytosis (Clic here for more details about this article) |
7/91. 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.- - - - - - - - - - ranking = 0.16666666666667keywords = histiocytosis (Clic here for more details about this article) |
8/91. A report of intracranial Rosai-Dorfman disease with literature review.Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy), is a rare benign histiocytic proliferative disorder. Over 650 cases have been reported since 1969. To the best of our knowledge, there have been only 31 cases with central nervous system involvement reported in the literature. Intracranial disease usually presents clinically and radiologically as a "meningioma". It can be misdiagnosed as a nonspecific inflammatory process because of the atypical histologic features of Rosai-Dorfman disease occurring in a non-nodal location. Familiarity with such atypical histologic features and appropriate use of immunohistochemical stains is required for a definitive diagnosis of central nervous system Rosai-Dorfman disease. We report such an intracranial lesion with other extranodal sites of involvement with a 5-year follow up and a review of previously reported cases. Ann Diagn Pathol 5:96-102, 2001.- - - - - - - - - - ranking = 0.16666666666667keywords = histiocytosis (Clic here for more details about this article) |
9/91. Rosai-Dorfman disease presenting with isolated bilateral orbital masses: report of two cases.Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare benign idiopathic proliferative disease of phagocytic histiocytes. Approximately 80% of patients present with painless massive cervical lymphadenopathy. Isolated extranodal involvement is relatively uncommon. Two cases of Rosai-Dorfman disease are reported: one with isolated bilateral orbital involvement and one with marked cervical lymphadenopathy and multiple dural-based and intraventricular masses.- - - - - - - - - - ranking = 0.16666666666667keywords = histiocytosis (Clic here for more details about this article) |
10/91. Relapsing intracranial Rosai-Dorfman disease.Two patients presenting with recurrent visual impairment due to relapsing intracranial Rosai-Dorfman disease are described. In both patients a preoperative diagnosis of meningioma was made. Histological examination disclosed the characteristic picture of S100 and CD68 positive histiocytosis with prominent lymphophagocytosis. In both patients complete tumour removal by surgery was impossible with residual tissue being the origin of relapsing disease. Low dose radiation led to partial recovery of vision and resolution of the intracranial mass. review of the literature on intracranial Rosai-Dorfman disease leads to the suggestion that postoperative radiotherapy may be advisable in all cases.- - - - - - - - - - ranking = 0.16666666666667keywords = histiocytosis (Clic here for more details about this article) |
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