411 - ABRAcadabra! Important Hematologic Considerations of a Patient with Amyloid-Beta Related Angiitis throughout a Course of Inpatient Rehabilitation: A Case Report

Thursday, February 22, 2024

5:00 PM – 6:30 PM EDT

Presenting Author(s)

MG

Michael Galibov, DO

Resident Physician
JFK Johnson Rehabilitation Institute
Woodbridge, New Jersey, United States

Co-Author(s)

MA

Motasem Abul-Huda, BA

Medical Student
Rowan School of Osteopathic Medicine
Lodi, New Jersey, United States
DS

David Shukhman, DO

Resident Physician
Northwell Health Plainview Hospital
New Brunswick, New Jersey, United States
FJ

Faraz Jamal, DO

Resident Physician
JFK Johnson Rehabilitation Institute
Hoboken, New Jersey, United States

Case Diagnosis: A 72-year old female with focal neurologic findings and bleeding secondary to Amyloid-Beta Related Angiitis (ABRA)

Case Description: Patient arrived with primary complaints of recurrent falls and left-sided weakness. MRI brain with and without contrast demonstrated increased complement of protein/hemorrhage in the cortical subarachnoid spaces and overlying leptomeningeal enhancement. Right frontal lobe specimen revealed reactive gliosis with no tumor/neoplasm identified. Vascular and fibrinoid necrosis were present with strong positive staining for beta-amyloid within the subarachnoid and parenchymal blood vessels. Clinically, the patient demonstrated left lower extremity weakness as well as multiple episodes of epistaxis, gum bleeding and bruising - which all obstructed her progression through a comprehensive inpatient rehabilitation regimen. Patient was additionally prescribed Cytoxan, corticosteroids, and gabapentin with improvement in weakness. Anticoagulation was ultimately discontinued due to increased bleeding risk from amyloid angiopathy, which led to increased patient tolerance of skilled therapy sessions.

Discussions: ABRA is a rare cause of cognitive and neurologic symptoms in elderly patients. It is preceded by cerebral amyloid angiopathy, which is characterized by amyloidosis primarily affecting the cortical and leptomeningeal vessels within the cerebrum. MRI and brain biopsy are the preferred diagnostic modalities, with typical findings including transmural infiltration of vessel walls by lymphocytes accompanied by granuloma formation. ABRA occurs when the impaired production and clearance of AB amyloid from the brain is complicated by vasculitis, which may be due to an autoimmune response to the amyloid. Notably, ABRA patients are at increased risk for bleeding events and therefore must be monitored closely with regards to anticoagulation. The mainstay of treatment is immunosuppressive therapy.

Conclusions: Amyloid-Beta Related Angiitis should be considered in patients presenting with cognitive and neurologic dysfunction, particularly in the setting of characteristic neuroimaging and biopsy findings. In addition to administering immunosuppressive therapy, careful attention should be given to monitor for hematologic events resulting from vasculitis.