Clin Neuropathol
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Case Reports
Plaque-like demyelination in acute disseminated encephalomyelitis (ADEM) - an autopsy case report.
The pattern of demyelination in the scant autopsy literature on acute disseminated encephalomyelitis (ADEM) is described as perivenous sleeves. We report an unusual neuropathological presentation of ADEM, also known as postinfectious or perivenous encephalomyelitis. ⋯ The morphology of individual demyelinating< lesions is indistinguishable from the lesions in a new onset case of multiple sclerosis (MS) and adds to the autopsy literature on overlapping neuropathological findings in ADEM and multiple sclerosis.
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The study describes a very rare case of primary extranodal marginal zone Bcell lymphoma of the central nervous system (MZL CNS) with an unusual clinical and radiological presentation mimicking subarachnoid bleeding and subdural hematoma (SDH) after head injury. The patient presented symptoms which had commenced 3 weeks earlier: a gradually-progressing headache associated with periodic right-sided cramp of the face muscles and numbness of the right upper limb. During urgent craniotomy for drainage of the presumed SDH, a tumor mass histopathologically and immunohistochemically matching marginal zone B-cell lymphoma was found. ⋯ The patient underwent successful radiotherapy, and achieved complete response. At present, no evidence of either systemic disease or lymph node enlargement has been found. The recognition of an indolent type of lymphoma in a rare anatomical localization is very important due to the proper management of the patient.
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IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory condition which often shows a dramatic response to steroid therapy. IgG4-RD can present either as a single lesion or as a systemic multi-organ disorder. Common histological findings include a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and phlebitis. ⋯ We add a case of probable IgG4-related pachymeningitis in a 42-year-old woman who presented with headache and left transverse sinus obstruction. Follow-up after 2-months of high dose steroids shows dramatic clinical and imaging improvement. The differential diagnosis for IgG4-related pachymeningitis, including lymphoplasmacyte-rich meningioma, idiopathic hypertrophic pachymeningitis, and lymphoproliferative disease is discussed.
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This study was an immunohistological study of IgG4-positive cell infiltration in 6 cases of hypertrophic pachymeningitis excluding secondary hypertrophic pachymeningitis caused by infectious diseases such as aspergillosis. The cases included 5 males and 1 female, ranging in age from 36 to 82 years (mean, 55 years). A biopsy was performed in all of the cases for diagnostic purposes, revealing fibrous dural hyperplasia with nonspecific inflammatory cell infiltration histologically. ⋯ There was no evidence of any other lesions associated with IgG4-related sclerosing disease, other than in the dura. It is not rare for IgG4-positive cells to appear in the dura in cases of hypertrophic pachymeningitis; however, no IgG4-related systemic disease is present in these cases. Hypertrophic pachymeningitis with IgG4-positive cells may have some kind of relation to other systemic autoimmune diseases.
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Glioblastoma (GBM) is the most common primary malignant human brain tumor with a poor prognosis. The diagnosis of GBM is based on histological features, however, few studies have evaluated their prognostic relevance in light of the latest WHO classification of 2007. ⋯ Our findings confirm the strong prognostic value of age, treatment, performance score, and localization for glioblastoma patients. Amongst the histopathological features only large necrosis was an independent prognostic factor.