Morning ReportAlison Fitzgerald, MDPGY32/20/2014

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)

So, which is it???

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.

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

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

What it came down to…

If repeat angiography is normal within days/weeks, more likely RCVS

Still pending….

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