Background:
Amyloid β Related Angiitis (ABRA) is a rare inflammatory central nervous system (CNS) vasculitis.
Aims:
To describe the clinical, radiological and pathological findings of ABRA.
Methods:
A single case report at our institution.
Results:
A 61 year old male presented with a witnessed Generalised Tonic Clonic Seizure (GTCS) on the background of a GTCS a week prior to presentation and a past medical history of hypertension, hypertrophic cardiomyopathy, hypercholesterolaemia, gout and a radical prostatectomy for prostate cancer.
On examination, there was no neurological deficits. MRI Brain showed multiple abnormal tortuous vessels within the left frontal region, with associated extensive hemosiderin staining suggestive of prior subarachnoid haemorrhage. A formal angiogram revealed a cluster of abnormal vessels in the left frontal lobe. Neurosurgical biopsy macroscopically revealed numerous superficial serpiginous arterioles. Histopathology showed features of CNS vasculitis evident by lymphohistiocytic inflammation and fibrinoid necrosis of small and medium vessels along with amyloid deposits confirmed by Congo Red stain in keeping with ABRA. The patient was started on oral corticosteroid immunosuppressive therapy with clinical response to seizure frequency.
Conclusion:
ABRA requires early multi disciplinary input and brain biopsy to confirm diagnosis.