The Subacute Angiohypertrophic Myelomalacia Foix-Alajouanine – a Rare
Disease?
Čegan M., Čeganová L., Štěrba J., Dvořáčková J., Ehrmann J.1, Koďousek R.1
CGB laboratoř spol. s r.o., Ostrava 1 Ústav patologické anatomie, LF, Univerzita Palackého, Olomouc |
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Summary:
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.
Key words:
vascular malformations – central nervous systém – subacute angiohypertrophic myelomalacia
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