Cerebral amyloid angiopathy-related inflammation (CAA-ri): A case report.

Rationale: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a treatable condition characterized by an acute or subacute onset, with its primary pathological hallmark being the deposition of amyloid, predominantly β-amyloid (Aβ), within intracranial microvessels. Despite its potential for treatment, CAA-ri is a rare disorder that is frequently underrecognized by clinicians in practice. This article provides a comprehensive overview of the clinical manifestations and therapeutic approaches associated with CAA-ri, aiming to enhance awareness among healthcare professionals.

An older man with progressive short-term memory loss and confusion.

A 72-year-old man with progressive memory loss and confusion presented to a neurology clinic for evaluation. He initially had difficulty remembering names and misplaced objects; however, his memory deficits had progressed, and more recently he had numerous car accidents and difficulty managing his own medications and finances. Cognitive testing revealed significant memory deficits reflecting moderate-stage dementia, and his brain MRI showed several cortical microbleeds and an area of siderosis consistent with the diagnosis of cerebral amyloid angiopathy (CAA).

PRIMARY SYSTEMIC AMYLOIDOSIS: A BRIEF OVERVIEW.

Primary systemic amyloidosis, or light chain (AL) amyloidosis, is a rare lymphoproliferative disorder in which aberrant light-chain immunoglobulins secreted into the bloodstream aggregate into fibrils and deposit into tissues, causing widespread organ damage and, if not treated, death. This review provides a comprehensive summary of the pathophysiology and manifestations of AL amyloidosis; standard-of-care diagnostic approach; typical treatment regimens; and areas of active investigation.

From experimental studies to computational approaches: recent trends in designing novel therapeutics for amyloidogenesis.

Amyloidosis is a condition marked by misfolded proteins that build up in tissues and eventually destroy organs. It has been connected to a number of fatal illnesses, including non-neuropathic and neurodegenerative conditions, which in turn have a significant influence on the worldwide health sector. The inability to identify the underlying etiology of amyloidosis has hampered efforts to find a treatment for the condition.

Mevalonate kinase deficiency: an updated clinical overview and revision of the SHARE recommendations.

Mevalonate kinase deficiency (MKD), a rare auto-inflammatory disorder, arises from mutations in the gene, disrupting isoprenoid biosynthesis, and affecting cellular processes. This comprehensive review provides an updated perspective on MKD, including its aetiology, pathogenesis, diagnostic modalities, and therapeutic strategies. Based on recent research and clinical advances, our objective is to bridge the knowledge gaps in the 2015 SHARE guidelines.