Methods: The spinal cord and brain tissues of 13 sporadic ALS
(SALS) patients were investigated using immunohistochemical analysis. Results: TDP-43-positive inclusions in lower motor neurones of SALS patients were immunopositive for Smurf2 and pSmad2/3. www.selleckchem.com/products/bay-57-1293.html Multiple immunofluorescence staining for Smurf2, pSmad2/3, TDP-43 and ubiquitin revealed co-localization of these four proteins within the inclusions in lower motor neurones of SALS patients. Furthermore, the loss of nuclear pSmad2/3 immunoreactivity was observed in cells bearing TDP-43 inclusions. In contrast, TDP-43-positive inclusions in the extramotor neurones in the brain of SALS patients were noticeably negative for Smurf2 and pSmad2/3. In addition, pSmad2/3 immunoreactivity was preserved in the nuclei of inclusion-bearing cells. Conclusions: This regional difference in the expression of Smurf2 and pSmad2/3 within TDP-43-positive inclusions might be one of the pathomechanisms underlying the loss of lower motor neurones and comparatively spared cortical neurones seen in ALS. “
“A case of unusual fibro-osseous lesion resembling osteoblastoma of the pineal region is reported, in a 50-year-old Selleck Rapamycin man. The patient presented with a history
of headache, vomiting and generalized tonic-clonic seizures. CT scan showed a hyperdense lesion in the posterior third ventricle with obstructive hydrocephalus. On histopathology the lesion showed cellular areas with oval to polygonal cells showing clear to eosinophilic cytoplasm along with focal anastomosing network of osetoid-like extracellular material lined by similar cells. The extracellular material was seen densely calcified at places with cement lines and Haversian canal formation. The cells were strongly immunoreactive for epithelial membrane antigen and focally for S-100 protein and negative for glial fibrillary acidic protein. “
“We have reported an autopsy case of primary granulomatous angiitis of the CNS preferentially involving the small veins with a granulomatous cAMP leukoencepalitis-like lesion
in the cerebral white matter of a 48-year-old man. The latter lesion was ischemic necrosis due to circumferential multiple perivenous granulomas in the adjacent Virchow-Robin space. Multifocal progressive involvement of venular adventitia by granulomas, leaving behind mural fibrosis and luminal stenosis, was related clinically to the prolonged stepwise deterioration observed in the patient, and pathologically to diffuse loosening with dilated veins in the deep cerebral white matter and subcortical hemorrhagic infarction in the left parietal lobe through chronic venous stagnation. PCR demonstrated negativity for Mycobacterium tuberculosis and Propionibacterium acnes, and in situ hybridization with EBV-encoded small nuclear RNA probe was also negative.
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