A 64-year-old woman suffering from progressive amyloid A (AA) amyloidosis of the gastrointestinal (GI) tract, associated with active rheumatoid arthritis, was transferred to our hospital due to hypovolemic shock. Although intensive care, including treatment with prednisolone and methotrexate, improved the hypovolemic shock, paralytic ileus became dominant instead of the marked diarrhea, suggesting the terminal stage of AA amyloidosis of the GI tract. Thus, we administered tocilizumab, a humanized anti-interleukin 6 receptor antibody (8 mg/kg, repeated every 4 weeks). Two weeks after the first injection of tocilizumab, serum AA rapidly returned to their normal ranges in accordance with the amelioration of paralytic ileus and systemic joint pain. Surprisingly, after three courses of tocilizumab treatment, colon biopsy revealed no amyloid deposition. Tocilizumab is a promising agent to treat secondary AA amyloidosis by strongly suppressing serum AA levels.
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http://dx.doi.org/10.1007/s10067-010-1422-6 | DOI Listing |
J Peripher Nerv Syst
March 2025
Referral Center for Familial Amyloid Polyneuropathy and Other Rare Peripheral Neuropathies (CERAMIC) and Department of Neurology, Bicêtre University Hospital, AP-HP, Le Kremlin Bicêtre, France.
Objective: To assess the effectiveness of labial minor salivary gland biopsy (LSGB) alone or in combination with punch skin biopsy (SB) for the detection of amyloid deposits in hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN).
Methods: In this single-center retrospective study, Congo red staining of minimal invasive LSGB (4 mm) and SB (3 mm) was assessed in ATTRv-PN patients consecutively evaluated between 2012 and 2023.
Results: Histopathological data of 171 ATTRv-PN, including 49 early-onset p.
J Prev Alzheimers Dis
January 2025
Department of Neuropsychiatry, Seoul National University Hospital, Seoul, 03080, Republic of Korea; Department of Psychiatry, Seoul National University College of Medicine, Seoul, 03080, Republic of Korea; Institute of Human Behavioral Medicine, Medical Research Center Seoul National University, Seoul, 03080, Republic of Korea; Interdisciplinary Program of Cognitive Science, Seoul National University College of Humanities, Seoul, 08826, Republic of Korea. Electronic address:
Importance: The neuropathological links underlying the association between changes in liver function and AD have not yet been clearly elucidated.
Objective: We aimed to examine the relationship between liver function markers and longitudinal changes in Alzheimer's disease (AD) core pathologies.
Design: Data from the Korean Brain Aging Study for the Early Diagnosis and Prediction of Alzheimer's Disease, a longitudinal cohort study initiated in 2014, were utilized.
J Prev Alzheimers Dis
January 2025
CenExel iResearch, Atlanta, GA, USA.
Background: Soluble species of multimeric amyloid-beta including globular amyloid-beta oligomers (AβOs) and linear amyloid-beta protofibrils are toxic to neurons. Sabirnetug (ACU193) is a humanized monoclonal antibody, raised against globular species of soluble AβO, that has over 650-fold greater binding affinity for AβOs over monomers and appears to have relatively little binding to amyloid plaque.
Objectives: To assess safety, pharmacokinetics, and exploratory measures including target engagement, biomarker effects, and clinical efficacy of sabirnetug in participants with early symptomatic Alzheimer's disease (AD; defined as mild cognitive impairment and mild dementia due to AD).
J Neuroimmune Pharmacol
January 2025
Pharmacy Department, Baotou Central Hospital, Baotou, 014040, Inner Mongolia, China.
Microglial polarization and ferroptosis are important pathological features in Alzheimer's disease (AD). Ghrelin, a brain-gut hormone, has potential neuroprotective effects in AD. This study aimed to explore the potential mechanisms by which ghrelin regulates the progression of AD, as well as the crosstalk between microglial polarization and ferroptosis.
View Article and Find Full Text PDFJACC Cardiovasc Imaging
January 2025
National Amyloidosis Centre, University College London, Royal Free Campus, Rowland Hill Street, London, United Kingdom.
Cardiac amyloidosis represents a unique disease process characterized by amyloid fibril deposition within the myocardial extracellular space. Advances in multimodality cardiac imaging enable accurate diagnosis and facilitate prompt initiation of disease-modifying therapies. Furthermore, rapid advances in multimodality imaging have enriched understanding of the underlying pathogenesis, enhanced prognostication, and resulted in the development of imaging-based markers that reflect the amyloid burden, which is of increasing importance when assessing the response to treatment.
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