[Rapidly progressive dementia disclosing primary angiitis of the central nervous system].
Angiitis of the central nervous system involves a wide spectrum of clinical symptoms. Complementary exams (CSF, neuroradiological exams) are not very specific and sensitive. Diagnostic procedures often require cortical and leptomeningeal biopsy. Treatment, based on corticosteroids and cyclophosphamid, have greatly improved prognosis. We report a rapidly progress dementia syndrome that revealed angiitis of the central nervous system. Corticosteroid treatment did not prevent a fatal outcome. Autopsy findings confirmed the diagnosis of central nervous leucocytoclastic angiitis.
Castelnovo
G
G
Service de Neurologie, CHU de Montpellier-Nimes, 2, avenue du Pr. Debre, 30029 Nimes Cedex, France.
Bouly
S
S
Vladut
M
M
Marty-Double
C
C
Labauge
P
P
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001344:Autopsy; D003520:Cyclophosphamide / Q000627:therapeutic use; D015140:Dementia, Vascular / Q000209:etiology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004569:Electroencephalography; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D013256:Steroids; D014057:Tomography, X-Ray Computed; D020293:Vasculitis, Central Nervous System / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D013256:Steroids; D003520:Cyclophosphamide