gms | German Medical Science

56. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e. V. (DGNC)
3èmes journées françaises de Neurochirurgie (SFNC)

Deutsche Gesellschaft für Neurochirurgie e. V.
Société Française de Neurochirurgie

07. bis 11.05.2005, Strasbourg

Recurrent subdural bleeding due to cerebral amyloid angiopathy in brain-restricted ALλ-amyloid deposition with vascular metaplastic bone formation

Subakutes Subduralhämatom durch eine zerebrale Amyloidangiopathie bei auf das Gehirn begrenzter Ablagerung von ALλ-Amyloid mit vaskulärer metaplastischer Knochenbildung

Meeting Abstract

  • corresponding author S. A. Kuhn - Klinik für Neurochirurgie, Klinikum der Friedrich-Schiller-Universität, Jena
  • K. Ebmeier - Klinik für Neurochirurgie, Klinikum der Friedrich-Schiller-Universität, Jena
  • M. Brodhun - Institut für Pathologie, Klinikum der Friedrich-Schiller-Universität, Jena
  • R. P. Linke - Max-Planck-Institut für Biochemie, Molekulare Struktur Biologie, Martinsried
  • A. Hochstetter - Klinik für Neurochirurgie, Klinikum der Friedrich-Schiller-Universität, Jena
  • R. Kalff - Klinik für Neurochirurgie, Klinikum der Friedrich-Schiller-Universität, Jena

Deutsche Gesellschaft für Neurochirurgie. Société Française de Neurochirurgie. 56. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3èmes journées françaises de Neurochirurgie (SFNC). Strasbourg, 07.-11.05.2005. Düsseldorf, Köln: German Medical Science; 2005. DocP212

Die elektronische Version dieses Artikels ist vollständig und ist verfügbar unter: http://www.egms.de/de/meetings/dgnc2005/05dgnc0480.shtml

Veröffentlicht: 4. Mai 2005

© 2005 Kuhn et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielf&aauml;ltigt, verbreitet und &oauml;ffentlich zug&aauml;nglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Gliederung

Text

Objective

Cerebral amyloid angiopathy (CAA) is characterized by the deposition of amyloid in leptomeningeal and cerebral blood vessels leading to hemorrhage. The proteins deposited as amyloid in CAA are different, that is Aβ-protein in CAA is seen mostly in Alzheimer’s disease and familial Dutch apoplexia, but also in CAA sui generis, cystatin C is seen in hereditary Islandic apoplexia and transthyretin in familial amyloid angiopathy. CAA can be sporadic or inherited, the latter is induced by point mutations which increase the amyloidogenic potential of the amyloid-precursor protein. This report describes a very rare disease since the protein is different from tnose already published.

Results

A 55-year old woman without family history of dementia and neurological disease perceived increasing memory loss more than nine years ago. Worsened mental and neurological status with dementia, global aphasia, affect incontinence, severe apraxia, ataxia, bilaterally negative Achilles-reflexes and positive signs of Babinsky, as well as incomplete orientation were observed. The patient became somnolent and had a left-sided hemiparesis. Cranial computer tomography revealed a subacute subdural hematoma over the right hemisphere with mass effect and unusual intracranial calcifications which made surgery necessary. Immediate decompressive surgery indicated that bleeding originating from calcified blood vessels. In addition, brain atrophy was noticed. The patient recovered postoperatively well and was discharged to a rehabilitation facility. Blood vessels of the cortical biopsy revealed massive local amyloid deposition. Surrounding brain tissue was focally degenerated. In addition, calcification and bone formation were noticed while skin biopsy did not show any amyloid. Immunohistochemical classification diagnosed the presence of ALλ amyloid while such other amyloid classes as AA, ALκ, AHγ, Aβ2M, ATTR, AFib, AApoAI, AApoAII, ALys, Aβ, and ACys were excluded.

Conclusions

An intracerebral monoclonal gammopathy with Ig-λ-light chain synthesis is the cause of intravascular ALλ amyloid. This amyloid is brain-restricted since systemic amyloidosis is absent. A case like thishas not been published before.