Thunderclap headache and benign CNS angiopathy

Chen SP et al. Reccurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder? Neurology 2006; 67:2164-2169.

56 patients with recurrent thunderclap headache were recruited. Segmental vasoconstriction was found in 22 (39 %). HA recurred .7 times per day for fourteen days. Each attack lasted 3 hours, and 84 % of patients reported at least one trigger. Nimodipine aborted attacks in 83 % and stopped headaches within 3 months. 4 patients (7%) developed stroke. Authors note that this entity is identical to benign CNS angiopathy. Exertion and defecation are greater triggers in vasoconstriction group.  Other triggers are emotional situations,laughing, bathing, bending down.

Blogger note: this is identical to Call Fleming syndrome frequently discussed in USA. See http://strokenotes.blogspot.com/search?q=call for more minutiae about it.

Review article
Ducros A. Reversible cerebral vasoconstriction syndrome.  Lancet Neurology 2012;11:906-917

additional pearls

1.  Pain peaks in 1-2 weaks, angiogram findings in about 3 weeks.

2.  Many synonyms exist (see below)

3.  Thunderclap headache usually peaks within 1 minute, unilateral or bilateral and mimics aneurysm rupture; each episode may last 3 hours but can vary with up to 3 day duration, and 4 attacks over the 3 month period with moderate headache in between

4.  Ten percent have focal findings; ten percent may have seizures including inaugural, and they rarely recur.  Marching numbness can mimic migraine, focality, a stroke.

5. Beware of neck pain (dissection).  Other differentials are CVT (check d Dimer and MRV), transfusion or products (red cell transfusion, alpha interferon, IVIG), phenytoin intoxication, CEA, low pressure headache.

6.  May screen for pheochromocytoma or glomus tumor or carcinoid. Do a drug screen in some patients.  Not only is cocaine in differential, cannabis is a trigger as well as LSD.  Other triggers include SSRI's, triptans, ergot derivatives, nicotine patches, ginseng and binge drinking.  Inquire about scorpion poison and star fruit.

7.  Convexity SAH may be subtle, and missed on CT, strokes occur in watershed areas (including cerebellar watershed areas); brain edema can mimic PRES.

8.  Catheter angiography can trigger TIA. 

9. Postpartum cases occurs in first week after delivery.  Inquire about exposure to vasoconstrictors during anesthesia, depression. 

10.  Synonyms and old names:  isolated benign CNS vasculitis, acute benign cerebral angiopathy, reversible cerebral segmental vasoconstriction, Call Fleming syndrome, CNS pseudovasculitis, benign angiopathy of CNS, postpartum angiopathy, migraine angiopathy, migraine angiitis, migraine vasospasm, primary thunderclap headache, cerebral vasculopathy, vasospasm infatal migrainous infarction

11.  Factors that independently make RCVS worse:  those who receive glucocorticoids, serotonergic antidepressants, patients with ischemic stroke (Singhal AB, Topcuoglu MA. Glucocorticoid associated worsening in reversible cerebral vasoconstriction syndrome.  Neurology 2017; 88:228-236.