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Transthyretin-type cerebral amyloid angiopathy in type I familial amyloid polyneuropathy

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Summary

To clarify the pathogenesis of cerebrovascular amyloid deposits, histological and immunocytochemical studies were performed on the central nervous system (CNS) in ten casès with type I familial amyloid polyneuropathy (FAP). They commonly suffered from peripheral somatic and autonomic nerve disorders without any CNS dysfunctions. However, all cases showed CNS amyloid deposits, mainly on the leptomeningeal vessels and pia-arachnoid membranes, with arteries and arterioles in the subarachnoidal space being the predominant site of cerebral amyloid accumulation. Using immunocytochemical staining methods with antibodies to amyloid β-protein, human cystatin C and transthyretin (prealbumin), all of these amyloid deposits were specifically immunolabeled by the anti-human transthyretin antibody. However, there were no transthyretin-related amyloid deposits in the brain parenchyma. It is concluded that CNS transthyretin-immunoreactive amyloid deposition with cerebral amyloid angiopathy (CAA) is a common pathological finding in this disease. Moreover, the patients with type I FAP are known to have an amyloid protein precursor (a variant of transthyretin) in serum. This transthyretin type of CAA, therefore, seems to be an example of cerebrovascular amyloid deposits derived from a serum precursor.

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Authors and Affiliations

  1. Department of Neurology, Kenwakai Hospital, 1936 Kanae, 395, Iida, Japan

    M. Ushiyama

  2. Department of Medicine (Neurology), Shinshu University School of Medicine, 390, Matsumoto, Japan

    S. Ikeda & N. Yanagisawa

Authors
  1. M. Ushiyama
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  2. S. Ikeda
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  3. N. Yanagisawa
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Supported by a grant-in-aid for Scientific Research from the Ministry of Education, Science and Culture and grants from the Intractable Disease Division, Ministry of Health and Welfare, Primary Amyloidois Research Committee, and Kanae Foundation of New Medicine, Japan

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Ushiyama, M., Ikeda, S. & Yanagisawa, N. Transthyretin-type cerebral amyloid angiopathy in type I familial amyloid polyneuropathy. Acta Neuropathol 81, 524–528 (1991). https://doi.org/10.1007/BF00310133

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  • DOI: https://doi.org/10.1007/BF00310133

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