Inflammatory cerebral amyloid angiopathy diagnosed by clinicoradiological criteria without brain biopsy

Abstract

Case Presentation: A 72-year-old previously independent woman with hypertension, diabetes mellitus, and dyslipidemia was admitted after two epileptic seizures without recovery of consciousness between events. She was treated with intravenous phenytoin and had no further seizures. She reported fever during the week prior to admission. On arrival, she was disoriented. Brain computed tomography revealed extensive vasogenic edema in the left temporoparietal white matter with mild mass effect. Cerebrospinal fluid analysis showed normal cell count and protein levels. Infectious investigation was negative, including multiplex polymerase chain reaction panel, polymerase chain reaction for tuberculosis, venereal disease research laboratory test, and fungal studies. Electroencephalography demonstrated diffuse slowing without epileptiform discharges. Brain magnetic resonance imaging revealed asymmetric hyperintensities on fluid-attenuated inversion recovery sequences in the bilateral parieto-temporo-occipital regions, predominantly on the left, associated with multiple punctate susceptibility foci with cortical predominance compatible with microhemorrhages. No diffusion restriction was observed. She presented with subacute cognitive impairment with a Mini-Mental State Examination score of 12/30. Considering the clinicoradiological suspicion of inflammatory cerebral amyloid angiopathy, intravenous methylprednisolone was administered for five days, followed by oral corticosteroid therapy at 1 mg/kg/day for six weeks. The patient showed marked clinical improvement, with cognitive recovery to baseline within four weeks. Discussion: Inflammatory cerebral amyloid angiopathy is a rare inflammatory variant of cerebral amyloid angiopathy characterized by perivascular inflammation associated with beta-amyloid deposition in cerebral vessels. Patients commonly present with seizures, subacute cognitive decline, and focal neurological deficits. Neuroimaging typically demonstrates asymmetric vasogenic edema associated with cortical microhemorrhages. When these findings occur in the appropriate clinical context and infectious causes are excluded, a clinicoradiological diagnosis can be established. According to the Boston Criteria version 2.0, the presence of asymmetric white matter hyperintensities associated with multiple lobar microhemorrhages supports the diagnosis of possible inflammatory cerebral amyloid angiopathy. Final Comments: This case illustrates a typical presentation of inflammatory cerebral amyloid angiopathy with seizures and subacute cognitive decline. Early recognition is important because the condition is potentially reversible and usually responds well to corticosteroid therapy, allowing the diagnosis to be established without the need for brain biopsy.