1. Clayton Wiley MD/PhD

2. 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.

4. Question 2
What is the differential diagnosis of a spontaneous intracranial hemorrhage?

5. Answer Metastatic tumor Primary CNS tumor Hemorrhagic Infarct
Vascular Malformation
Amyloid Angiopathy
Coagulopathy
Infection

6. Question 3
The blood was evacuated and the tissue sent for pathologic examination. Describe the findings.
Click here to review the slide

7. Question 4
What immunohistochemical stains would you order to help better characterize the etiology of this hemorrhage?

8. 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

9. Question 5
Based on the clinical history, H&E impression and immunohistochemical findings (see below), what is your diagnosis?

10. Answer
A. Cerebral Amyloid Angiopathy
B. Alzheimer’s Disease Pathology

11. Question 6
What causes cerebral amyloid angiopathy and how is it related to other forms of amyloidosis?

12. Question 7
What is amyloid?

13. Question 8
What is the most common cause of spontaneous intracranial hemorrhage and how is the clinical presentation of cerebral amyloid angiopathy different?

14. 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).

15. Question 11
This patient also had amyloid plaques deposited in the surrounding cortex. How closely associated is CAA with Alzheimer’s disease?