Cerebral amyloid angiopathy-related inflammation with posterior reversible encephalopathy syndrome-like presentation: a case report.

BACKGROUND: Cerebral amyloid angiopathy-related inflammation (CAA-RI), which presents with acute or subacute cognitive or functional decline, focal or multifocal neurologic deficits, new onset of seizures, or a combination of seizures and neurologic deficits, shares clinical and radiologic similarities with posterior reversible encephalopathy syndrome (PRES). Differential diagnosis is critical because the treatment principle for these 2 conditions differs greatly. Here, we present a case of PRES-like CAA-RI and the strategy used to discriminate between the 2 conditions.

CASE PRESENTATION: A patient with probable CAA-RI was first thought to suffer from PRES. Initial high-dose methylprednisolone therapy caused rapid improvement of the neurologic symptoms but abrupt discontinuation of corticosteroids resulted in clinical relapse and deterioration. Subsequent reinitiation of high-dose methylprednisolone followed by tapering off of oral prednisone led to clinical and radiologic recovery at the 3-month follow-up.

CONCLUSIONS: We suggest that in cases where it is difficult to distinguish between CAA-RI and PRES solely based on magnetic resonance imaging, a good response to corticosteroids and an apolipoprotein E (ApoE) ε4/ε4 genotype are critical for establishing a diagnosis of CAA-RI. If there is clinical deterioration, sudden withdrawal of high-dose corticosteroid during the active phase of CAA-RI should be avoided.