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

Central nervous system cavernomatosis

Cavernomatose des ZNS

Meeting Abstract

  • corresponding author E. E. Gehrke - Abt. Stereotaktische Neurochirurgie, Albert-Ludwigs-Universität Freiburg
  • J. Vesper - Abt. Stereotaktische Neurochirurgie, Albert-Ludwigs-Universität Freiburg
  • M. Tippel - Abt. Stereotaktische Neurochirurgie, Albert-Ludwigs-Universität Freiburg
  • J. Weber - Abt. Neuroradiologie, Albert-Ludwigs-Universität Freiburg
  • N. Ghanem - Radiologische Klinik, Albert-Ludwigs-Universität Freiburg
  • C. Ostertag - Abt. Stereotaktische Neurochirurgie, Albert-Ludwigs-Universität Freiburg

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. DocP221

Die elektronische Version dieses Artikels ist vollständig und ist verfügbar unter: http://www.egms.de/de/meetings/dgnc2005/05dgnc0489.shtml

Veröffentlicht: 4. Mai 2005

© 2005 Gehrke 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

Cavernous haemangiomas (CH) consist of venous vascular malformations which occur in 0.4% of the population. CH represent 5-13% of all vascular malformations. Usually CH patients present with seizures, rarely with haemorrhage. CH can be sporadic or familial, single or multiple (usually not more than 3 lesions). Multiple cavernous angiomas are more frequent in the familial type.

Methods

The case of a 67-year-old man is reported here with more than 100 cavernous malformations in the brain including the brainstem and none in the spinal cord. Examination revealed multiple seborrhoeic keratoses and angiomas on the upper part of the body as well as multiple angiomas in the face. MR images showed unilateral acoustic neuroma on the right side.

Results

This patient presented a 40y history of complex focal epilepsy. In 1987 a cranial computertomography (CCT) was performed and 5 supratentorial, partly calcified lesions were detected. At that time, the diagnosis granulomatosis disease of unknown origin was considered. Since 2004 the patient had increasing problems with walking because of dizziness and an uncertain feeling. The CCT from August 2004 showed an increase in the incidence and size of these partly calcified lesions. In September 2004 one lesion was biopsied using stereotaxy (histological diagnosis: vascular lesion). The MRI showed more than 100 cavernous haemangiomas, located in the supra- and infratentorial space as well as a unilateral acoustic neuroma on the right side. Whole body MRI and MRI of the spinal cord showed multiple haemangiomas of the cervical and thoracic vertebral bodies and excluded other vascular malformations. Clinically the patient presented with complex focal epilepsy, gait disturbance, progressive dementia, unilateral tinnitus and hypacusis (right side).

Conclusions

Central nervous system cavernomatosis with more than 100 lesions is uncommon. To our knowledge only one case has been reported so far with more than 100 central nervous system cavernous malformations, including several intramedullar spinal cord lesions. We reported an interesting and uncommon case with multiple CH, especially in combination with multiple haemangiomas of the cervical and thoracic vertebral bodies, multiple skin lesions and a unilateral acoustic neuroma.