Immunohistochemical confirmation of Creutzfeldt-Jakob disease with a long clinical course with amyloid plaque core antibodies

• Published in: The American journal of pathology Vol. 131; no. 3; pp. 435 - 443
• Main Authors: Kitamoto, T, Tateishi, J
• Format: Journal Article
• Language: English
• Published: Bethesda, MD ASIP 01-06-1988; American Society for Investigative Pathology
• Subjects: Amyloid - immunology; Antibodies - analysis; Biological and medical sciences; Creutzfeldt-Jakob Syndrome - diagnosis; Creutzfeldt-Jakob Syndrome - immunology; Creutzfeldt-Jakob Syndrome - physiopathology; Human viral diseases; Humans; Immune Sera - immunology; Immunochemistry; Infectious diseases; Medical sciences; Nervous System Diseases - immunology; Slow Virus Diseases - immunology; Viral diseases; Viral diseases of the nervous system; Infection; Human; Immunohistochemistry; Pathology; Nervous system diseases; Antibody; Viral disease; Amyloid; Creutzfeldt Jakob disease
• ISSN: 0002-9440; 1525-2191

Amyloid plaques have been found in the brains of some patients with Creutzfeldt-Jakob disease (CJD) and all patients with Gerstmann-Sträussler syndrome (GSS). We examined paraffin sections from 45 patients with CJD or GSS and from 51 patients with other neurologic diseases, using an antiserum against GSS amyloid plaque cores. The GSS amyloid plaque core antiserum revealed not only birefringent amyloid plaques but also small plaques that cannot be detected by the staining with Congo red dye. Positive immunolabeling was demonstrated in 59% of 34 Japanese patients with CJD, in 100% of 11 patients with GSS, and in none with other neurologic diseases. All cases of CJD of short duration (less than 11 months) were evaluated as being negative, and 95% of 21 long survivors (over 12 months) were positive. This immunohistochemical approach revealed that amyloid plaque is a hallmark of CJD with a long clinical course.