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Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and...
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Updated: Apr 24, 2026

Detecting Amyloid-β Accumulation via Immunofluorescent Staining in a Mouse Model of Alzheimer's Disease
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[AL amyloidosis].

A Jaccard1, E Desport2, D Mohty3

  • 1Centre national de référence de l'amylose AL et des autres maladies de dépôts d'immunoglobulines monoclonales, hôpital Dupuytren, CHU de Limoges, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France; Service d'hématologie et thérapie cellulaire, hôpital Dupuytren, CHU de Limoges, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France.

La Revue De Medecine Interne
|September 8, 2014
PubMed
Summary
This summary is machine-generated.

AL amyloidosis, a conformational disease, arises from misfolded light chains. Early diagnosis and treatment, often involving chemotherapy, are crucial for managing this common condition affecting multiple organs.

Keywords:
AL amyloidosisAmylose ALCardiopathie restrictiveChaînes légères libres d’immunoglobulinesImmunoglobulin free light chainsNephrotic syndromeRestrictive cardiomyopathySyndrome néphrotique

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Area of Science:

  • Medicine
  • Pathology
  • Oncology

Background:

  • AL amyloidosis is a common conformational disease caused by misfolded monoclonal immunoglobulin light chains.
  • It affects approximately 500 new cases annually in France, originating from an indolent plasma cell clone.

Purpose of the Study:

  • To provide an overview of AL amyloidosis, including its diagnosis, clinical manifestations, and current treatment strategies.
  • To emphasize the importance of early diagnosis for improving patient outcomes.

Main Methods:

  • Diagnosis relies on non-invasive biopsies like abdominal fat aspiration or minor salivary gland biopsy, with examination of involved tissues if necessary.
  • Clinical presentation varies widely due to potential organ involvement, excluding the brain.

Main Results:

  • The kidney is the most frequently affected organ, while cardiac involvement (restrictive cardiomyopathy) presents the most severe clinical picture.
  • Current effective treatments include alkylating agents with high-dose dexamethasone, and combinations with proteasome inhibitors, achieving high response rates.

Conclusions:

  • Early diagnosis, particularly before advanced cardiac complications, is critical for better outcomes in AL amyloidosis.
  • Close monitoring of clonal and organ response is essential for guiding therapy adjustments and duration.
  • Novel therapeutic approaches targeting amyloid deposit removal are under investigation.