Cerebral amyloid angiopathy-related inflammation

Epidemiology
In a series of 56 patients, average age was 69 years old, 60% female.

Signs and Symptoms

 * Cognitive/behavioral change: 58%
 * Headache: 42%
 * Seizure: 42%
 * Focal neurological deficit: 19%

Diagnosis

 * MRI
 * Subcortical T2 hyperintensity: 94%
 * Microhemorrhages: 87%, with more than 50 microhemorrhages in 48%
 * Contrast enhancement of lesions: 34%. When present nearly always involves meninges.
 * Acute or subacute infarcts: 22%
 * Superficial siderosis: 19%
 * Acute subarachnoid hemorrhage: 19%
 * Old ICH: 16%
 * Lacunar infarcts: 13%
 * CSF
 * Elevated protein: 92%
 * Elevated WBCs: 26%
 * Normal glucose
 * Can have normal CSF

Treatment
Immunosuppresive treatments including steroids, cyclophosphamide, and mycophenolate have been usedm, and in one retrospective series of 48 patients any immunosuppressive treatment was highly associated with clinical improvement (aOR 26.4, 95% CI 2.36-295, p=0.008) and radiographic improvement (aOR 56.6, 95% CI 3.39-947 when controlling for number of microbleeds, and aOR 8.85, 95% CI 1.00-78.4 when controlled for APOE ε4). APOE ε4 number was also associated with radiographic improvement when controlling for immunosuppressive treatment (aOR 4.49, 95% CI 1.11-18.2). Immunosuppresive treatment was also associated with a decreased likelihood of recurrence (OR 0.19, 95% CI 0.07-0.48, p<0.001).