Neuronal intranuclear inclusion disease (NIID) is a neurological disorder characterized by eosinophilic intranuclear inclusions (INI) in systemic organs and various cell types. High-intensity signals along the corticomedullary junction on diffusion-weighted imaging and presence of cellular p62-INI in skin biopsy are known indicators for NIID. Furthermore, GGC repeat expansion in is a characteristic genetic alteration in patients with NIID. This report presents the clinical and detailed pathological features of a male older adult with NIID. We also confirmed the presence of fluid-attenuated inversion recovery high-intensity signals in the cerebellar paravermal area, showing similar pathological changes in high-intensity signals along the corticomedullary junction on diffusion-weighted imaging.
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http://dx.doi.org/10.5414/NP301499 | DOI Listing |
World J Gastroenterol
January 2025
Department of Radiology, Kindai University, Faculty of Medicine, Osakasayama 589-8511, Osaka, Japan.
Background: Focal nodular hyperplasia (FNH)-like lesions are hyperplastic formations in patients with micronodular cirrhosis and a history of alcohol abuse. Although pathologically similar to hepatocellular carcinoma (HCC) lesions, they are benign. As such, it is important to develop methods to distinguish between FNH-like lesions and HCC.
View Article and Find Full Text PDFMedicine (Baltimore)
November 2024
Department of Neurology, The People's Hospital of Suzhou New District, Suzhou, Jiangsu, China.
Rationale: Neuronal intranuclear inclusion disease (NIID) is a slowly progressing neurodegenerative disease with various manifestations and high heterogeneity. Clinical characteristics, imaging, skin biopsy, and genetic testing are necessary for its diagnosis. Electromyography may also be a useful tool for diagnosing NIID.
View Article and Find Full Text PDFSci Rep
January 2025
Department of Environmental Studies and Geology, Bryn Mawr College, Bryn Mawr, PA, USA.
Geologic records of tropical cyclones (TCs) in low-energy, back-barrier environments are established by identifying marine sediments via their allochthonous biogeochemical signal. These records have the potential to reconstruct TC intensity and frequency through time. However, modern analog studies are needed to understand which biogeochemical indicators of overwash sediments are best preserved and how post-depositional changes may affect their preservation.
View Article and Find Full Text PDFDiabetes Technol Ther
January 2025
Department of Paediatric Diabetes and Endocrinology, John Hunter Children's Hospital, New Lambton Heights, New South Wales, Australia.
To compare glycemic outcomes during and following moderate-intensity exercise (MIE), high-intensity interval exercise (HIE), and resistance exercise (RE) in adolescents with type 1 diabetes (T1D) using a hybrid closed-loop (HCL) insulin pump while measuring additional physiological signals associated with activity. Twenty-eight adolescents (average age 16.3 ± 2.
View Article and Find Full Text PDFJ Korean Neurosurg Soc
January 2025
Department of Neurosurgery, Kyung Hee University Hospital, Kyung Hee University College of Medicine, Seoul, Korea.
Objective: The leptomeningeal ivy sign is a distinctive finding of moyamoya disease (MMD), characterized by a linear high signal intensity along the cortical sulci on contrast-enhanced T1 magnetic resonance imaging (MRI) and fluid-attenuated inversion-recovery MRI. We recently identified a similar linear enhancement along the cortical sulci using gadolinium-enhanced vessel wall MRI (VWMR) in patients with MMD. The aim of this study was to introduce the concept of the "VWMR ivy sign (VIS)".
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