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Clayton Wiley MD/PhD. Question 1. This is a 79-year-old woman who presented to an outside hospital following sudden onset of severe headache and left sided weakness. A CT scan of the head was performed. Describe the CT scan findings. Question 2.
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Question 1 • This is a 79-year-old woman who presented to an outside hospital following sudden onset of severe headache and left sided weakness. A CT scan of the head was performed. • Describe the CT scan findings.
Question 2 • What is the differential diagnosis of a spontaneous intracranial hemorrhage?
Answer • Metastatic tumor • Primary CNS tumor • Hemorrhagic Infarct • Vascular Malformation • Amyloid Angiopathy • Coagulopathy • Infection
Question 3 • The blood was evacuated and the tissue sent for pathologic examination. Describe the findings. • Click here to review the slide
Question 4 • What immunohistochemical stains would you order to help better characterize the etiology of this hemorrhage?
Answer • A-beta amyloid • Smooth muscle actin • Pankeratin (to rule out carcinoma not readily apparent on the slide) • PAS (to rule out CADASIL – see question XX) • Click to see A-beta amyloid, Actin, PAS
Question 5 • Based on the clinical history, H&E impression and immunohistochemical findings (see below), what is your diagnosis?
Answer • A. Cerebral Amyloid Angiopathy • B. Alzheimer’s Disease Pathology
Question 6 • What causes cerebral amyloid angiopathy and how is it related to other forms of amyloidosis?
Question 7 • What is amyloid?
Question 8 • What is the most common cause of spontaneous intracranial hemorrhage and how is the clinical presentation of cerebral amyloid angiopathy different?
Answer • Hypertensive vasculopathy (HV) is the most common (70-90%). • CAA most prominently involves the leptomeningeal and cortical vessels. Commonly found in demented individuals with Alzheimer’s like pathology. • HV typically causes hemorrhages in the basal ganglia (40-50%), lobar regions (20-50%), thalamus (10-15%), pons (5-12%), cerebellum (5-10%), and other brainstem sites (1-5%). More commonly fatal (rapid).
Question 11 • This patient also had amyloid plaques deposited in the surrounding cortex. How closely associated is CAA with Alzheimer’s disease?