[Isolated angiitis of the central nervous system. Report of two cases and review of the literature].
Isolated angiitis of the central nervous system is a rare disease affecting mainly adults of both sex; about 210 cases have been reported. Contrary to other inflammatory arteritis, arthralgia, myalgia, weight loss and fever are exceptional and symptoms are mainly neurologic, but none is specific. The diagnosis is evoked in case of headaches and cognitive impairment, associated or not with multifocal neurologic signs. Evolution is acute, subacute or chronic. Elevated sedimentation rate and cerebrospinal fluid pleiocytosis are present in 2/3 of cases. CT scan and brain MRI generally demonstrate multifocal ischemic lesions involving cortex, white matter, basal ganglia and brainstem. Cerebral arteriography is the key investigation, showing segmental stenoses alternating with fusiform dilatations of blood vessels, which are highly suggestive but not specific. It can be normal and its repetition is then recommended. Certain diagnosis is obtained from cerebromeningeal biopsy, showing a segmental angiitis of small vessels, which is granulomatous in 88 p.100 and non granulomatous in 12 p.100 of cases. The pathogenesis is unknown. Spontaneous evolution is generally fatal. Cyclophosphamide associated with prednisone considerably improves the prognosis, especially when initiated early in the course of the disease.
Hallak
H
H
Département de Neurologie, CHU, Angers.
Fressinaud
C
C
Emile
J
J
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D001706:Biopsy; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008578:Meninges / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use*; D020293:Vasculitis, Central Nervous System / Q000188:drug therapy* / Q000473:pathology
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone