Skip to main content
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1982 May;45(5):396–408. doi: 10.1136/jnnp.45.5.396

Congophilic angiopathy of the brain: a clinical and pathological report on two siblings.

R A Griffiths, T F Mortimer, D R Oppenheimer, J M Spalding
PMCID: PMC1083118  PMID: 7086452

Abstract

Clinical and histological accounts are given of a sister and brother, dying aged 61 and 56 years respectively after illnesses lasting 5-6 years marked by a progressive mental and physical disability. The family history suggested transmission by a dominant gene. The histological findings were of a very severe congophilic angiopathy confined to the brain, spinal cord and leptomeninges and giving rise to multiple haemorrhages and softenings. There were, in addition, abundant amyloid-containing "plaques" of various forms, found principally in the hippocampus and cerebellar cortex. The cases are compared with similar cases in the literature, and reasons given for regarding this condition as a separate entity rather than a variant of Alzheimer's disease.

Full text

PDF
396

Images in this article

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Boellaard J. W., Schlote W. Subakute spongiforme Encephalopathie mit multiformer Plaquebildung. "Eigenartige familiär-hereditäre Krankheit des Zentralnervensystems [spino-cerebellare Atrophie mit Demenz, Plaques und plaqueähnlichen Ablagerungen im Klein- und Grossirn" (Gerstmann, Sträussler, Scheinker)]. Acta Neuropathol. 1980;49(3):205–212. doi: 10.1007/BF00707108. [DOI] [PubMed] [Google Scholar]
  2. Boller F., Mizutani T., Roessmann U., Gambetti P. Parkinson disease, dementia, and Alzheimer disease: clinicopathological correlations. Ann Neurol. 1980 Apr;7(4):329–335. doi: 10.1002/ana.410070408. [DOI] [PubMed] [Google Scholar]
  3. CORSELLIS J. A., BRIERLEY J. B. An unusual type of pre-senile dementia (atypical Alzheimer's disease with amyloid vascular change). Brain. 1954 Dec;77(4):571–587. doi: 10.1093/brain/77.4.571. [DOI] [PubMed] [Google Scholar]
  4. Cook R. H., Ward B. E., Austin J. H. Studies in aging of the brain: IV. Familial Alzheimer disease: Relation to transmissible dementia, aneuploidy, and microtubular defects. Neurology. 1979 Oct;29(10):1402–1412. doi: 10.1212/wnl.29.10.1402. [DOI] [PubMed] [Google Scholar]
  5. Gerhard L., Bergener M., Homayun S. Angiopathie bei Alzheimerscher Krankheit. Z Neurol. 1972;201(1):43–61. [PubMed] [Google Scholar]
  6. Glenner G. G. Congophilic microangiopathy in the pathogenesis of Alzheimer's syndrome (presenile dementia). Med Hypotheses. 1979 Nov;5(11):1231–1236. doi: 10.1016/0306-9877(79)90005-7. [DOI] [PubMed] [Google Scholar]
  7. Gudmundsson G., Hallgrímsson J., Jónasson T. A., Bjarnason O. Hereditary cerebral haemorrhage with amyloidosis. Brain. 1972;95(2):387–404. doi: 10.1093/brain/95.2.387. [DOI] [PubMed] [Google Scholar]
  8. Jellinger K. Cerebrovascular amyloidosis with cerebral hemorrhage. J Neurol. 1977 Feb 17;214(3):195–206. doi: 10.1007/BF00316150. [DOI] [PubMed] [Google Scholar]
  9. Lee S. S., Stemmermann G. N. Congophilic angiopathy and cerebral hemorrhage. Arch Pathol Lab Med. 1978 Jun;102(6):317–321. [PubMed] [Google Scholar]
  10. Mandybur T. I., Bates S. R. Fatal massive intracerebral hemorrhage complicating cerebral amyloid angiopathy. Arch Neurol. 1978 Apr;35(4):246–248. doi: 10.1001/archneur.1978.00500280064014. [DOI] [PubMed] [Google Scholar]
  11. Mandybur T. I. Cerebral amyloid angiopathy: possible relationship to rheumatoid vasculitis. Neurology. 1979 Oct;29(10):1336–1340. doi: 10.1212/wnl.29.10.1336. [DOI] [PubMed] [Google Scholar]
  12. Mandybur T. I. The incidence of cerebral amyloid angiopathy in Alzheimer's disease. Neurology. 1975 Feb;25(2):120–126. doi: 10.1212/wnl.25.2.120. [DOI] [PubMed] [Google Scholar]
  13. NEUMANN M. A. Combined amyloid vascular changes and argyrophilic plaques in the central nervous system. J Neuropathol Exp Neurol. 1960 Jul;19:370–382. doi: 10.1097/00005072-196007000-00005. [DOI] [PubMed] [Google Scholar]
  14. Okazaki H., Reagan T. J., Campbell R. J. Clinicopathologic studies of primary cerebral amyloid angiopathy. Mayo Clin Proc. 1979 Jan;54(1):22–31. [PubMed] [Google Scholar]
  15. Okazaki H., Reagan T. J., Campbell R. J. Clinicopathologic studies of primary cerebral amyloid angiopathy. Mayo Clin Proc. 1979 Jan;54(1):22–31. [PubMed] [Google Scholar]
  16. PANTELAKIS S. Un type particuliar d'angiopathie sénile du systeme nerveux central: l'angiopathie congophile; topographie et fréquence. Monatsschr Psychiatr Neurol. 1954 Oct;128(4):219–256. [PubMed] [Google Scholar]
  17. Pro J. D., Smith C. H., Sumi S. M. Presenile Alzheimer disease: amyloid plaques in the cerebellum. Neurology. 1980 Aug;30(8):820–825. doi: 10.1212/wnl.30.8.820. [DOI] [PubMed] [Google Scholar]
  18. Shaw C. M. Primary idiopathic cerebrovascular amyloidosis in a child. Brain. 1979 Mar;102(1):177–192. doi: 10.1093/brain/102.1.177. [DOI] [PubMed] [Google Scholar]
  19. Torack R. M. Congophilic angiopathy complicated by surgery and massive hemorrhage. A light and electron microscopic study. Am J Pathol. 1975 Nov;81(2):349–366. [PMC free article] [PubMed] [Google Scholar]
  20. Torack R. M., Lynch R. G. Cytochemistry of brain amyloid in adult dementia. Acta Neuropathol. 1981;53(3):189–196. doi: 10.1007/BF00688021. [DOI] [PubMed] [Google Scholar]
  21. Ulrich G., Taghavy A., Schmidt H. Zur Nosologie und Atiologie der kongophilen angiopathie (Gefässform der cerebralen Amyloidose. Z Neurol. 1973 Dec 31;206(1):39–59. [PubMed] [Google Scholar]
  22. Wright J. R., Calkins E., Breen W. J., Stolte G., Schultz R. T. Relationship of amyloid to aging. Review of the literature and systematic study of 83 patients derived rom a general hospital population. Medicine (Baltimore) 1969 Jan;48(1):39–60. [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES