The subacute angiohypertrophic myelomalacia Foix-Alajouanine--a rare disease?
Cegan, M.; Ceganová, L.; Stĕrba, J.; Dvorácková, J.; Ehrmann, J.; Kod'ousek, R.
Ceskoslovenska Patologie 42(2): 76-80
2006
ISSN/ISBN: 1210-7875 PMID: 16715632 Document Number: 598561
The subacute angiohypertrophic myelomalacia Foix-Alajouanine was first described by French authors in 1926 and belongs to the wider category of CNS vascular malformations. Both individual casuistics and larger sets of patients with this disease can be found in literature scarcely. We are presenting three necroptic cases of subacute angiohypertrophic myelomalacia (Foix-Alajouanine) that were mistakenly diagnosed clinically, once as metastatic lesion of spinal canal, once as amyotrophic lateral sclerosis, and once as inflammatory lesion of spinal cord. The histologic finding is dominated by myelomalacia of varying extent with varicosely dilated, constricted or even occluded arterialized both extra- and intramedullary veins, sporadically coupled with secondary thrombi. Clinical diagnostics is difficult and requires spinal angiography. According to literature data, it is presumable that this disease is not as rare as it may seem at first sign.