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Fatal reversible cerebral vasoconstriction syndrome.
Amy Kunchok1, Helen C Castley2, Lucie Aldous2
1Department of Neurology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia.
Journal of the Neurological Sciences
|February 7, 2018
Summary
Fulminant reversible cerebral vasoconstriction syndrome can be fatal, contrary to its usual self-limiting course. Corticosteroid treatment, often given for misdiagnosed primary central nervous system vasculitis, may be linked to worse outcomes in these rare cases.
Area of Science:
- Neurology
- Neurocritical Care
- Vascular Neurology
Background:
- Reversible cerebral vasoconstriction syndrome (RCVS) is typically a benign, self-limiting condition.
- RCVS is characterized by temporary narrowing of cerebral arteries.
- Misdiagnosis as primary central nervous system vasculitis can lead to specific treatments.
Purpose of the Study:
- To report fatal cases of fulminant reversible cerebral vasoconstriction syndrome.
- To highlight the potential negative impact of corticosteroid treatment in RCVS.
- To differentiate RCVS from primary central nervous system vasculitis in severe presentations.
Main Methods:
- Retrospective analysis of four fatal cases of RCVS.
- Review of initial diagnoses and treatment regimens.
- Comparison of clinical presentation and outcomes.
Main Results:
- Four patients presented with fulminant RCVS, initially diagnosed as primary central nervous system vasculitis.
- All patients received corticosteroid therapy.
- Outcomes were fatal in these cases, suggesting a potential association between corticosteroids and worse prognosis in severe RCVS.
Conclusions:
- Fulminant RCVS, though rare, can have a fatal outcome.
- Corticosteroid treatment may be detrimental in severe RCVS.
- Accurate diagnosis of RCVS is crucial to avoid potentially harmful treatments.
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