Diagnosis, treatment, and follow-up of patients with cerebral amyloid angiopathy-related inflammation

Abstract

Purpose: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare potentially reversible encephalopathy associated with an autoimmune process against proteins deposited in the walls of cortical and leptomeningeal brain vessels. Definite diagnosis requires histopathological features of vascular inflammation and amyloid deposition from brain biopsy. Clinical-neuroradiological criteria have been recently introduced and validated to reduce the need for biopsy. The purpose of this paper is to report a historical retrospective review of clinical-neuroradiological follow-up of two patients with probable CAA-ri and five patients with a reasonably probable suspect of CAA-ri (4 females, 3 males, patient's age at admission: 66-79 years) seen at our institution between 2007 and 2021, focusing on clinical and neuroradiological awareness to this entity and variable response to immunotherapy.

Materials and methods: Clinical features at presentation included subacute to acute confusion (6/7), seizures (4/7), cognitive impairment (5/7), and focal neurological signs (3/7). Neuroradiology included braincomputed tomography followed by magnetic resonance imaging. Infectious diseases and autoimmune workups were then performed.

Results: CSF analysis was performed in two patients. Cerebral angiography was performed in two patients, to rule out vascular malformations. Hemorrhagic posterior reversible encephalopathy syndrome has been suspected in two patients. Four patients underwent immunotherapy with corticosteroids followed by reduction of brain dysfunctions. Three patients did not undergo immunotherapy but underwent clinical and/or neuroradiological remission.

Conclusions: Patients with CAA-ri present a rare steroid-responsive acute to subacute brain dysfunction. Thus, it has to be known and recognized both clinically and neuroradiologically. Spontaneous clinical and/or neuroradiological improvement is possible in patients with mild symptoms.

Keywords: Amyloid-related imaging abnormalities; Cerebral amyloid angiopathy-related inflammation; Computed tomography; Magnetic resonance imaging; Spontaneous remission; Treatment.

Fig. 1

Neuroradiological findings. Patient 1: gradient echo magnetic resonance (MR) axial images at diagnosis (a) and 1.5-month follow-up (b). Patient 2: susceptibility-weighted MR axial images at diagnosis (c) and 1-month follow-up (d). Patient 3: gradient echo (e) and susceptibility-weighted (f) MR axial images at diagnosis. Patient 4: computed tomography (g) and susceptibility-weighted MR (h) axial images at diagnosis. Patient 5: susceptibility-weighted MR axial images at diagnosis (i) and 3-month follow-up (j). Patient 6: computed tomography (k) and susceptibility-weighted MR (l) axial images at diagnosis and computed tomography axial image at 5-month follow-up (m). Patient 7: susceptibility-weighted MR axial images at diagnosis (n) and 1-month follow-up (o)