Distribution of Cystatin C Amyloid Deposits in the Icelandic Patients with Hereditary Cystatin C Amyloid Angiopathy
Cystatin C, a basic microprotein inhibitor of lysosomal proteinases is a precursor of the amyloid fibril deposition in patients with hereditary cystatin C amyloid angiopathy (HCCAA) in Iceland. Cystatin C amyloid deposits were demonstrated immunohistochemically in the following areas. Firstly within the CNS in practically all arteries and arterioles throughout the CNS. Massive cystatin C amyloid was also present in interstitial tissue of the basal ganglia and the hippocampus, especially perivascularly. Spinal pia mater showed positive immuno-staining in all cases as did frequently the arachnoid and arachnoid granulations. Secondly, outside the CNS cystatin C amyloid deposits were demonstrated in the walls of small arteries in all lymphnodes investigated independent of localization in the body. Amyloid material was also detected in the inter-lobular connective tissue of the submandibular salivary gland. These findings suggest that the diagnosis of HCCAA can now be supported by two laboratory methods: Estimation of cystatin C in the cerebrospinal fluid and immunohistochemical study of lymphnode biopsies.
KeywordsSubmandibular Salivary Gland Amyloid Deposit Amyloid Fibril Superior Sagittal Sinus Arachnoid Granulation
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