1. Morning
Report
Alison Fitzgerald, MD
PGY3
2/20/2014

2. Differential Diagnosis
 Reversible
cerebral vasoconstriction
syndrome (RCVS)
VS
 Primary
angiitis of the central nervous
system (PACNS) (a.k.a. Primary CNS
vasculitis or Isolated CNS vasculitis)

3. So, which is it???

4. RCVS (a.k.a Call-Fleming syndrome)

Prolonged but reversible vasoconstriction/vasospasm of the cerebral
arteries

Although the pathology is not known definitively, it is thought SSRIs,
uncontrolled hypertension, endocrine abnormality, and neurosurgical
trauma

Symptoms result from vasospasms that narrow arteries, especially those
around the circle of Willis, which can lead to a dramatic headaches
that are often of the thunderclap headache (sudden-onset)character.

Ischemia is thought to cause various lesions and upper motor neuron
damage in these patients which presents 3–4 days after migraine onset
as focal neurological symptoms such as dysarthria, unilateral weakness,
unsteady gait, and/or hyperreflexia

Vasospasm is common post subarachnoid hemorrhage and cerebral
aneurysm but in RCVS only 25% of patients have symptoms post
subarachnoid hemorrhage.

5. PACNS

May arise from primary involvement (inflammation) of the blood vessels
(small/medium sized arteries) in the brain or spinal cord, or from secondary
involvement of the CNS by a systemic disorder (systemic vasculitis, connective tissue
disease, infectious process)

Affects diffuse areas of the CNS

Diagnostic tests yield non-specific abnormalities

Skipped lesions are common (often found in cerebral cortex and leptomeninges
than rather than subcortical regions)

Rare; 2:1 male predominance, age 42 avg, long prodromal period; symptoms of
systemic vasculitis usually lacking

Symptoms include decreased cognition, headache, seizure, stroke, cerebral
hemorrhage

Angiography reveals findings of ectasia and stenosis (“beading”) usually in the small
arteries; affects several sites of the cerebral circulation, circumferential or eccentric
vessel irregularities, multiple occlusions with sharp cutoffs

6. Comparison
RCVS






Abrupt onset
Often has a precipitant
(postpartum, migraine,
severe HTN, vasoactive
medication)
More common in women
Angiographic changes are
readily reversible within
days to weeks
Will resolve without steroids;
CCB to treat
Normal CSF studies (unless
associated SAH)
PACNS






Sub-acute onset
No precipitant
More common in men
Angiographic changes
are not reversible
Long taper of steroids
to treat
CSF studies abnormal
in 80-90% of patients;
non-specific findings

7. What it came down to…
If repeat angiography is normal within
days/weeks, more likely RCVS
Still pending….

8. References


Hajj-Ali R, Calabrese LH; www.uptodate.com; Primary angiitis
of the central nervous system in adults. May 21, 2013
Reversible cerebral vasoconstriction syndrome,
www.Wikipedia.com