Non-hereditary multiple telangiectasias of the central nervous system. Report of two clinicopathological cases.
We describe 2 clinicopathological cases of non-hereditary multiple telangiectasias of the nervous system. In the first case, the general course of the disease was characterized by spells over a period of 7 years and the major lesions successively involved cranial nerves, spinal cord and brain. Neuropathological examination showed both ischemic and hemorrhagic changes. Systematized degenerative changes were found and were similar to those observed in spinocerebellar heredodegenerations (Friedreich's ataxia). In the second case, the 3 years of evolution were characterized by spells of the encephalitic type only. A review of the literature indicates the rarity of this kind of disease, the usual localization of capillary malformations and the clinical polymorphism (epilepsy, strokes, multifocal syndromes masquerading as multiple sclerosis). The pathogenesis of pathological changes is discussed.
Milandre
L
L
Department of Neurology, CHU Timone, Marseille France.
Pellissier
J F
JF
Boudouresques
G
G
Bonnefoi
B
B
Ali Cherif
A
A
Khalil
R
R
D000368:Aged; D002490:Central Nervous System / Q000473:pathology; D002493:Central Nervous System Diseases / Q000473:pathology*; D005260:Female; D005621:Friedreich Ataxia / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D013684:Telangiectasis / Q000473:pathology*