In patients with T2DM, treatment with resveratrol regulates energy expenditure through increased skeletal muscle SIRT1 and AMPK expression. These findings indicate that resveratrol may have beneficial exercise-mimetic effects in patients with T2DM.
Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disorder associated with GGC repeats of >60 to 500 copies in the 5′‐untranslated region of NOTCH2NLC. The clinical and genetic characterization of NIID outside of East Asia remains unknown. We identified twelve patients who underwent genetic testing using long‐read sequencing or repeat primed polymerase chain reaction. All were positive for a GGC repeat expansion; the median repeat length was 107 (range 92‐138). Ten were Chinese and two of Malay ethnicity. Age at onset ranged from 50 to 69 years. Eight (66.7%) patients had dementia, while four (33.3%) patients were oligosymptomatic, without typical NIID symptoms of dementia, Parkinsonism, or muscle weakness. GGA interruptions within the GGC expansion were present in four patients; the number of GGA interruptions was highest (6.71%) in the patient with the earliest age at onset (50 years). Median plasma neurofilament light level was 47.3 pg/mL in seven patients (range 26‐380 pg/mL). The highest level (380 pg/mL) was found in one patient who experienced an encephalitic episode. Overall, we describe a cohort of genetically confirmed NIID patients from Southeast Asia and provide further information that the presence of GGA interruptions within GGC repeat expansions may serve as a potential genetic modifier in NIID.
The pineal gland, also known as the conarium, is one of the six circumventricular organs of the central nervous system.1 It has a location in the midline within the anterior quadrigeminal cistern, and lies above the superior colliculi, at the posterior recesses of the third ventricle. The pineal gland has unique histology among central nervous system tissues. It consists of prominent lobules surrounded by connective tissue septa. The pineocytes within the lobules form sheets within a fibrillary background, punctuated by distinctive pineocytic rosettes with central fibrillary cores. A population of astrocytes forms a loose network among the pineocytes. The pineocytes demonstrate immunoperoxidase expression of synaptophysin and neurofilament protein, while the supporting astrocytes are positive for glial fibrillary acid protein and S100 protein. Ultrastructural analyses of pineocytes have revealed morphological evidence of neurosensory differentiation by the presence of vesicle-crowned rodlets, fibrous filaments and paired twisted filaments, and also of neuroendocrine differentiation with dense core granules.
3After puberty, mineralized concretions known as corpora arenacea (or brain sand) become present and accrue with age. This is accompanied by gliosis and cystic change which gradually replace the cellular pineocytic population.
1Since the discovery of melatonin more than half a century ago, great strides have been made in characterization of the structure and function of the pineal gland.4 However to date, the cytological features of the normal pineal gland, as seen in intraoperative squash preparations has not been well illustrated in the literature.Intraoperative smear cytology using the technique of squash preparations is very commonly practiced in surgical neuropathology. This method provides superior morphological preservation in the neurosurgical setting where biopsies are often limited in size and the tissue is particularly vulnerable to freezing artefact.Unfortunately, the smear cytology features of the pineal gland are not familiar to many surgical pathologists, perhaps due to the relative rarity of lesions involving the pineal area, and the small size of the gland, rendering it readily overrun and destroyed by aggressive neoplasms.As lesions of the pineal region are being increasingly targeted by stereotactic biopsies and submitted for intraoperative diagnosis, knowledge of the cytological features of the normal pineal gland as encountered in the intraoperative setting is of importance. An ability to distinguish normal pineal tissue from lesional tissue is pivotal in intraoperative management; the finding of normal tissue prompts further surgical sampling to identify the actual lesion under the same anesthetic procedure.In this report, we describe the features of the native pineal gland on intraoperative squash preparation, as fortuitously encountered in a biopsy of a pineal lesion in a young female.The intraoperative smears were moderately cellular with cohesive clumps of cells within a fibrillary ba...
BackgroundKidney involvement in non-Hodgkin lymphoma is well recognized and glomerulonephritis, when present, has been commonly reported to be associated with a membranoproliferative pattern.Case presentationWe report a case of a 58-year-old lady with a recurrence of non-Hodgkin MALT B-cell lymphoma, presenting with acute kidney injury, nephrotic range proteinuria and a cellular urinalysis. She underwent a renal biopsy that showed a severe diffuse proliferative and exudative lupus-like glomerulonephritis, which is likely paraneoplastic in nature. We discuss the differential diagnosis and possible pathogenesis of glomerular injury in lymphoma-related proliferative glomerulonephritis.ConclusionDifferentiating between true lupus nephritis and a paraneoplastic glomerulonephritis is important, as it would have significant implications on treatment and clinical course.
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