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
• What is the differential diagnosis of a spontaneous
intracranial hemorrhage?
Answer
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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?