Xanthogranulomatous change of craniopharyngioma, consisting of cholesterol clefts, macrophages, chronic inflammatory infiltrates, necrotic debris and hemosiderin deposits, has been traditionally considered a hallmark of the adamantinomatous variant, even in the absence of epithelium. Based on a series of 110 craniopharyngioma patients undergoing primary surgery, we found 37 specimens with a predominating xanthogranulomatous component. Only 3 of these cases (8%) exhibited additional histological features of adamantinomatous craniopharyngioma, while 13 cases (35%) contained non-adamantinomatous epithelium composed of squamous or ciliated cuboidal cells. Subsequent clinical analysis revealed that these 37 xanthogranulomatous lesions differed from 59 classical adamantinomatous craniopharyngiomas with respect to preferential occurrence in adolescents and young adults (mean age 27 years), predominant intrasellar location, smaller tumor size, more severe endocrinological deficits, longer preoperative history, lower frequency of calcification and visual disturbances, better resectability, and a more favorable outcome. On the other hand, xanthogranulomatous and adamantinomatous lesions did not differ with respect to sex, amount of cystic components, or the intraoperative aspect, considered by the neurosurgeon as being typical for craniopharyngioma in all cases. We suggest that xanthogranuloma (cholesterol granuloma) of the sellar region is clinically and pathologically distinct from the classical adamantinomatous craniopharyngioma.
The current World Health Organization classification recognizes three histological types of grade II lowgrade diffuse glioma (diffuse astrocytoma, oligoastrocytoma, and oligodendroglioma). However, the diagnostic criteria, in particular for oligoastrocytoma, are highly subjective. The aim of our study was to establish genetic profiles for diffuse gliomas and to estimate their predictive impact. In this study, we screened 360 World Health Organization grade II gliomas for mutations in the IDH1, IDH2, and TP53 genes and for 1p/19q loss and correlated these with clinical outcome. Most tumors (86%) were characterized genetically by TP53 mutation plus IDH1/2 mutation (32%), 1p/19q loss plus IDH1/2 mutation (37%), or IDH1/2 mutation only (17%). TP53 mutations only or 1p/19q loss only was rare (2 and 3%, respectively). The median survival of patients with TP53 mutation ؎ IDH1/2 mutation was significantly shorter than that of patients with 1p/19q loss ؎ IDH1/2 mutation (51.8 months vs. 58.7 months, respectively; P ؍ 0.0037).Multivariate analysis with adjustment for age and treatment confirmed these results (P ؍ 0.0087) and also revealed that TP53 mutation is a significant prognostic marker for shorter survival (P ؍ 0.0005) and 1p/19q loss for longer survival (P ؍ 0.0002), while IDH1/2 mutations are not prognostic (P ؍ 0.8737). The molecular classification on the basis of IDH1/2 mutation, TP53 mutation, and 1p/19q loss has power similar to histological classification and avoids the ambiguity inherent to the diagnosis of oligoastrocytoma. (Am J Pathol
Most GCA-positive TAs contained VZV in skip areas that correlated with adjacent GCA pathology, supporting the hypothesis that VZV triggers GCA immunopathology. Antiviral treatment may confer additional benefit to patients with GCA treated with corticosteroids, although the optimal antiviral regimen remains to be determined.
Alzheimer's disease (AD) is a neurodegenerative disorder primarily affecting regions of the brain responsible for higher cognitive functions. Immunization against -amyloid (A) in animal models of AD has been shown to be effective on the molecular level but also on the behavioral level. Recently, we reported naturally occurring autoantibodies against A (NAbs-A) being reduced in Alzheimer's disease patients. Here, we further investigated their physiological role: in epitope mapping studies, NAbs-A recognized the mid-/Cterminal end of A and preferentially bound to oligomers but failed to bind to monomers/fibrils. NAbs-A were able to interfere with A peptide toxicity, but NAbs-A did not readily clear senile plaques although early fleecy-like plaques were reduced. Administration of NAbs-A in transgenic mice improved the object location memory significantly, almost reaching performance levels of wild-type control mice. These findings suggest a novel physiological mechanism involving NAbs-A to dispose of proteins or peptides that are prone to forming toxic aggregates.
Aims
Most brain diseases are complex entities. Although animal models or cell culture experiments mimic some disease aspects, human post mortem brain tissue remains essential to advance our understanding of brain diseases using biochemical and molecular techniques. Post mortem artefacts must be properly understood, standardized, and either eliminated or factored into such experiments. Here we examine the influence of several premortem and post mortem factors on pH, and discuss the role of pH as a biochemical marker for brain tissue quality.
Methods
We assessed brain tissue pH in 339 samples from 116 brains provided by 8 different European and 2 Australian brain bank centres. We correlated brain pH with tissue source, post mortem delay, age, gender, freezing method, storage duration, agonal state and brain ischaemia.
Results
Our results revealed that only prolonged agonal state and ischaemic brain damage influenced brain tissue pH next to repeated freeze/thaw cycles.
Conclusions
pH measurement in brain tissue is a good indicator of premortem events in brain tissue and it signals limitations for post mortem investigations.
The aim of the study was to further determine the pathophysiology, clinical course, MRI-features and response to therapy of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), which has recently been proposed as a rare chronic inflammatory central nervous system disorder responsive to immunosuppressive therapy. Three patients with this rare entity underwent serial clinical and bimonthly MRI follow-up over a period of up to 16 months. Extensive laboratory work-up and brain biopsy were performed. Intravenous methylprednisolone or oral dexamethasone was administered as treatment, additionally cyclophosphamide in one patient. Clinically, diplopia, nystagmus, ataxia and facial paresthesia were the cardinal symptoms. Magnetic resonance imaging (MRI) disclosed patchy spot-like gadolinium enhancement in a "salt-and-pepper like appearance" in the pons, midbrain and cerebellum, in two cases with thalamic and in the other with spinal involvement. Brain biopsies demonstrated a predominantly angiocentric but also diffuse infiltration pattern by small mature lymphocytes. Treatment with steroids led to rapid clinical improvement and marked resolution of MRI lesions. As discontinuation of steroids led to clinical relapse, one patient was treated with a further course of steroids and the other with steroids and cyclophosphamide as immunosuppressive therapy. This led to stable remission with only mild clinical residue and normalization of MRI. Extensive laboratory and radiological work-up could not identify any other cause of the disease. Of note, in two cases a marked elevation of IgE in serum was found initially and throughout the course. CLIPPERS seems to be a distinct inflammatory central nervous system disorder. It shows characteristic MRI core features. Extrapontine involvement seems to be frequent. Histologically it is characterised by predominantly angiocentric infiltration by small mature lymphocytes. A pathogenetic relationship between the elevated IgE levels and the perivascular infiltrates can be presumed. It is responsive to immunosuppressive therapy and can require prolonged or maintenance treatment.
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