Recurrence of Lobar Hemorrhage: A Red Flag for Cerebral Amyloid Angiopathy-related Inflammation?

Background. Recurrent lobar intracerebral hemorrhage is more commonly associated with cerebral amyloid angiopathy and less likely associated with hypertension. Cerebral amyloid angiopathy-related inflammation is a subgroup of cerebral amyloid angiopathy that can present with lobar intracerebral hemorrhage, encephalopathy, and seizures; wherein corticosteroids may facilitate favorable outcome. Whether recurrence of lobar intracerebral hemorrhage in cerebral amyloid angiopathy is related to cerebral amyloid angiopathy-related inflammation is unknown. Case presentation. A 68-year-old woman presented with an acute onset of confusion. She was known to have a history of recurrent lobar intracerebral hemorrhage related to cerebral amyloid angiopathy. Brain imaging revealed previous sequelae of cerebral amyloid angiopathy and a new lobar intracerebral hemorrhage. An empirical diagnosis of cerebral amyloid angiopathy-related inflammation was made given the patent's clinical course of recurrence. Utilizing current evidence of criteria used to diagnose cerebral amyloid angiopathy-related inflammation, corticosteroid therapy was initiated with significant improvement in clinical and imaging characteristics. Discussion. Inflammatory pathways incited as a result of cerebrovascular amyloid deposition play a vital role in pathogenesis of cerebral amyloid angiopathy-related inflammation. We highlight the need to consider corticosteroid therapy in patients presenting with recurrent lobar intracerebral hemorrhage in the setting of cerebral amyloid angiopathy since inflammation may play a role in its pathophysiology. Evidence in the literature is sparse to suggest that cerebral amyloid angiopathy-related inflammation might be the root cause for the lobar intracerebral hemorrhage recurrence in cerebral amyloid angiopathy. Further studies are needed to identify mechanisms of recurrent hemorrhage, its correlations with cerebral amyloid angiopathy-related inflammation, and the potential role of corticosteroid therapy.