Morning
Report
Alison Fitzgerald, MD
PGY3
2/20/2014
Differential Diagnosis
 Reversible

cerebral vasoconstriction
syndrome (RCVS)

VS
 Primary

angiitis of the central nerv...
So, which is it???
RCVS (a.k.a Call-Fleming syndrome)


Prolonged but reversible vasoconstriction/vasospasm of the cerebral
arteries



Alt...
PACNS


May arise from primary involvement (inflammation) of the blood vessels
(small/medium sized arteries) in the brain...
Comparison
RCVS









Abrupt onset
Often has a precipitant
(postpartum, migraine,
severe HTN, vasoactive
medicati...
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 ...
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CNS vasculitis

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CNS vasculitis

  1. 1. Morning Report Alison Fitzgerald, MD PGY3 2/20/2014
  2. 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. 3. So, which is it???
  4. 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. 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. 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. 7. What it came down to… If repeat angiography is normal within days/weeks, more likely RCVS Still pending….
  8. 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

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