No abstract

Sinus histocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. Although extranodal involvement has been reported in diverse sites, central nervous system (CNS) manifestation, particularly in the absence of nodal disease is uncommon. We report 11 cases of RDD primary to the CNS without evidence of other sites of involvement. The cases included 7 males and 4 females ranging in age from 22 to 63 years (mean: 41 y). The patients presented with headaches, seizures, numbness, or paraplegia. Eight cases involved the cranial cavity and three cases, the spinal canal. Lesions were most often extra-axial and dura based. Only one presented in the CNS parenchyma. Histologically, the lesions consisted of variable numbers of pale-staining histocytes with emperipolesis often overshadowed by extensive lymphoplasmacytic infiltrates and fibrosis in the background. Special stains for organisms were negative. By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein and CD68 and negative for CD1a. Treatment consisted of surgical biopsy or excision. Follow-up, available for 10 cases with intervals ranging from 5 days to 42 months (mean: 15 mo), disclosed one patient dying of operative complications 5 days after biopsy and nine patients with no evidence of disease progression RDD should be considered in the differential diagnosis of inflammatory lesions of the CNS. Our study suggests that this entity may have been misdiagnosed in the past as plasma cell granuloma or inflammatory pseudotumor.

Neurocysticercosis (NCC) is a common central nervous system (CNS) infection caused by Taenia solium metacestodes. Despite the well-documented importance of the granulomatous response in the pathogenesis of this infection, there is limited information about the types of cells and cytokines involved. In fact, there has been limited characterization of human brain granulomas with any infectious agent. In the present study a detailed histological and immunohistochemical analysis of the immune response was performed on eight craniotomy specimens where a granuloma surrounded each T. solium metacestode. The results indicated that in all the specimens there was a dying parasite surrounded by a mature granuloma with associated fibrosis, angiogenesis, and an inflammatory infiltrate. The most abundant cell types were plasma cells, B and T lymphocytes, macrophages, and mast cells. Th1 cytokines were prevalent and included gamma interferon, interleukin-18 (IL-18), and the immunosuppressive, fibrosis-promoting cytokine transforming growth factor ␤. The Th2 cytokines IL-4, IL-13, and IL-10 were also present. These observations indicate that a chronic immune response is elicited in the CNS environment with multiple cell types that together secrete inflammatory and anti-inflammatory cytokines. In addition, both collagen type I and type III deposits were evident and could contribute to irreversible nervous tissue damage in NCC patients.

No abstract

To determine the cause of death (as a result of neurologic or nonneurologic complications or accidents) in patients with multiple sclerosis (MS), we reviewed the autopsies of 50 subjects with MS from the Office of the Chief Medical Examiner of Maryland (OCME) between 1982 and 2004. The series included 32 females and 18 males (mean age, 45.8 years; range, 25-69 years) and the causes of death were classified into 3 categories: (A) neurologic complication directly related to MS; (B) nonneurologic complications or other medical causes; and (C) accidents, etc. Of the 50 cases, in 43 there was a history of MS, but in 7 subjects there was not, and the diagnosis was established by neuropathologic examination. In Group A, 21 (42%) cases, deaths were directly related to a neurologic complication; in Group B, 14 (28%) cases were related to the following nonneurologic and medical causes: ASCVD 9 (18%), metabolic disorder 1 (2%), pulmonary embolism 3 (6%), and bronchopneumonia 1 (2%); and in Group C, 15 (30%) cases, deaths were due to trauma, 9 (18%); intoxication, 5 (10%); and thermal injury, 1 (2%). Thus, among the 50 subjects, in 26, deaths occurred naturally; and in 24, from accidents, homicides, suicides, or undetermined causes. Pathologically, the majority of cases showed either chronic inactive (66.7%) or chronic active (15.6%) demyelinating lesions, mainly in the cerebral hemispheres. In some cases, it appears that demyelinating lesions, involving brain regions that regulate cardiorespiratory activity, could be considered as the immediate cause of death, but a large proportion appears to be due to other causes such as accidents and trauma. Thus, it seems likely that taking specific precautions could prevent some deaths in MS.