Multiple-system atrophy (MSA) is a progressive neurodegenerative disorder. It is characterized by dysfunction of multiple systems including autonomic, extrapyramidal, pyramidal and cerebellar. Pontine hot-cross bun (HCB) sign in MSA is a well-documented and highly specific entity. To the best of our knowledge, medullary hot-cross bun (HCB) sign has never been described in literature. Herein, we report a case of MSA-C with medullary HCB sign. Detection of medullary HCB sign might be a surrogate marker in the diagnosis of MSA-C, particularly in the absence of classical pontine HCB sign; however, further research will help to validate this sign.
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http://dx.doi.org/10.1016/j.jmir.2015.10.001 | DOI Listing |
BMJ Open Respir Res
January 2025
Fundació de Recerca Clínic Barcelona - Institut d'Investigacions Biomèdiques August Pi i Sunyer (FRCB-IDIBAPS), Barcelona, Spain.
Introduction: Reducing unplanned hospital admissions in chronic patients at risk is a key area for action due to the high healthcare and societal burden of the phenomenon. The inconclusive results of preventive strategies in patients with chronic obstructive respiratory disorders and comorbidities are explainable by multifactorial but actionable factors.The current protocol (January 2024-December 2025) relies on the hypothesis that intertwined actions in four dimensions: (1) management change, (2) personalisation of the interventions based on early detection/treatment of acute episodes and enhanced management of comorbidities, (3) mature digital support and (4) comprehensive assessment, can effectively overcome most of the limitations shown by previous preventive strategies.
View Article and Find Full Text PDFAJNR Am J Neuroradiol
December 2024
From the Department of Radiology and Center for Imaging Science (C.Y.L., Y.S., B.S., M.S., S.T.K., E.Y.K.), Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea; Department of Digital Health (S.H.), Samsung Advanced Institute of Health Sciences and Technology (SAIHST), Sungkyunkwan University, Seoul, Republic of Korea; Medical AI Research Center, Research Institute for Future Medicine (S.H.), Samsung Medical Center, Seoul, Republic of Korea; Department of Neurology (J.Y.), Neuroscience Center (J.Y.), Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
Background And Purpose: The hot cross bun (HCB) sign is a hallmark feature of multiple system atrophy with predominant cerebellar ataxia (MSA-C), typically observed in advanced stages of the disease; however, it can also present in other conditions such as spinocerebellar ataxia (SCA), making the differentiation challenging. The middle cerebellar peduncle (MCP) sign may be observed in various medical conditions and in healthy individuals. We hypothesized that the inferior cerebellar peduncle (ICP), known to be affected in MSA-C, may exhibit hyperintensity on fluid-attenuated inversion recovery (FLAIR) imaging, potentially aiding in differentiating MSA-C from SCA.
View Article and Find Full Text PDFBackground Multiple system atrophy (MSA) is a rare, adult-onset neurodegenerative disorder marked by autonomic failure, parkinsonism, and cerebellar ataxia, with subtypes classified as parkinsonian (MSA-P), cerebellar (MSA-C), and autonomic (MSA-A). This study aims to identify MRI biomarkers for MSA and their correlation with disease severity. Methodology A total of 30 patients with probable MSA (20 MSA-C, 10 MSA-P) aged 45-65 years were studied.
View Article and Find Full Text PDFBMC Neurol
July 2024
C-Care International Hospital Kampala, Plot 4686 Barnabas Road, Kampala, Uganda.
Background: John Cunningham virus related granule cell neuronopathy (JCV-GCN) is a rare manifestation of the reactivation of infection of the cerebellar granule cells by the JCV, mostly in immunocompromised individuals. The "hot cross bun" (HCB) sign is a cruciform hyperintensity seen in the midpons on T2-weighted and fluid attenuated inversion recovery (FLAIR) sequences on magnetic resonance imaging (MRI) of the brain. An index sub-Saharan Africa report of a case of JCV-GCN with HCB sign follows.
View Article and Find Full Text PDFPLoS One
December 2022
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Background: The standardized T1-weighted/T2-weighted (sT1w/T2w) ratio for the middle cerebellar peduncle (MCP) has been reported to be sensitive for detecting degenerative changes in the cerebellar subtype of multiple system atrophy (MSA-C), even in the early stages. We aimed to investigate the diagnostic value of the MCP sT1w/T2w ratio for differentiating between MSA-C and spinocerebellar ataxia (SCA).
Methods: We included 32 MSA-C, 8 SCA type 3 (SCA3), 16 SCA type 6 (SCA6) patients, and 17 controls, and the MCP sT1w/T2w ratio was analyzed using a region-of-interest approach.
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