6.7.3.2 Acute headache probably attributed to reversible cerebral vasoconstriction syndrome (RCVS)Hartmut Gobel2018-02-06T10:48:54+00:00
Description:
Headache typical for reversible cerebral vasoconstriction syndrome (RCVS), namely thunderclap headache recurring over 1-2 weeks and triggered by sexual activity, exertion, Valsalva manœuvres and/or emotion, but the intracranial arterial beading typical of RCVS has not been demonstrated by cerebral angiography.
Diagnostic criteria:
- Any new headache fulfilling criterion C
- Reversible cerebral vasoconstriction syndrome (RCVS) is suspected, but cerebral angiography is normal
- Probability of causation demonstrated by all of the following:
- at least two headaches within 1 month, with all three of the following characteristics:
- a) thunderclap onset, and peaking in <1 minute
- b) severe intensity
- c) lasting ≥5 minutes
- at least one thunderclap headache has been triggered by one of the following:
- a) sexual activity (just before or at orgasm)
- b) exertion
- c) Valsalva-like manœuvre
- d) emotion
- e) bathing and/or showering
- f) bending
- no new thunderclap or other significant headache occurs >1 month after onset
- at least two headaches within 1 month, with all three of the following characteristics:
- Either of the following:
- headache has resolved within 3 months of its onset
- headache has not yet resolved but 3 months from its onset have not yet passed
- Not better accounted for by another ICHD-3 diagnosis1.
Note:
In particular, aneurysmal subarachnoid haemorrhage has been excluded by appropriate investigations.
Comment:
ICHD-3 does not generally propose criteria for probable secondary headaches. However, the arterial abnormalities of reversible cerebral vasoconstriction syndrome (RCVS) may be difficult to demonstrate. Some RCVS cases need repeated CT- or MR-angiography during 2-3 weeks after headache onset and others need invasive conventional angiography to be detected. In patients who have recurrent, triggered thunderclap headaches typical for RCVS over a period of less than one month but normal initial cerebral angiography, and in whom another cause of the headaches has been excluded by appropriate investigations, a temporary diagnosis of 6.7.3.2 Headache probably attributed to reversible cerebral vasoconstriction syndrome can be made.