PMID- 18062458
OWN - NLM
STAT- MEDLINE
DCOM- 20080123
LR  - 20131121
IS  - 0317-1671 (Print)
IS  - 0317-1671 (Linking)
VI  - 34
IP  - 4
DP  - 2007 Nov
TI  - Reversible cerebral vasoconstriction syndrome or primary angiitis of the central 
      nervous system?
PG  - 467-77
AB  - BACKGROUND: Reversible Cerebral Vasoconstriction Syndrome (RCVS) may present as 
      thunderclap headache (TCH), accompanied by reversible cerebral vasospasm and 
      focal neurological deficits, often without a clear precipitant. RCVS may be 
      mistaken for Primary Angiitis of the Central Nervous System (PACNS) due to the 
      presence of similar angiographic features of segmental narrowing of cerebral 
      arteries. We discuss the clinical features of a young female migraine patient who 
      developed TCH and was found to have RCVS following initial treatment with 
      corticosteroids for PACNS, in the context of a systematic review of the available 
      medical literature. METHODS: A Medline search was performed to identify all case 
      reports since 1966 describing RCVS and PACNS that provide sufficient clinical 
      detail to permit diagnostic classification according to published criteria. RCVS 
      included case studies in which there was angiographic or transcranial Doppler 
      ultrasound evidence of near-to-complete resolution of cerebral vasoconstriction 
      in the absence of a well-recognized secondary cause. PACNS included reports of 
      histologically confirmed PACNS either through biopsy or necropsy. RESULTS: 
      Reversible Cerebral Vasoconstriction Syndrome occurs primarily in females and is 
      characterized by sudden, severe headache at onset, normal CSF analysis, 
      vasoconstriction involving the Circle of Willis and its immediate branches, and 
      angiographic or TCD ultrasound evidence of near-to-complete vasospastic 
      resolution within 1-4 weeks. It occurs typically in the context of 
      vasoconstrictive drug use, the peripartum period, bathing, and physical exertion. 
      CONCLUSION: Initial and follow-up (within 4 weeks) non-invasive angiographic 
      studies are indicated in patients who present with TCH or who have clinical 
      presentations that could be consistent with RCVS or PACNS in the absence of a 
      well-recognized secondary cause, such as subarachnoid haemorrhage. Early 
      reversibility of cerebral vasospasm is the key neuroradiological feature that 
      supports the clinical diagnosis of RCVS.
FAU - Gerretsen, Philip
AU  - Gerretsen P
AD  - Division of Neurology, Mount Sinai Hospital, University Health Network, 
      University of Toronto, Toronto, Ontario, Canada.
FAU - Kern, Ralph Z
AU  - Kern RZ
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - England
TA  - Can J Neurol Sci
JT  - The Canadian journal of neurological sciences. Le journal canadien des sciences 
      neurologiques
JID - 0415227
RN  - 0 (Anti-Inflammatory Agents)
RN  - VB0R961HZT (Prednisone)
SB  - IM
MH  - Adult
MH  - Anti-Inflammatory Agents/therapeutic use
MH  - Cerebrovascular Circulation/*physiology
MH  - Diagnosis, Differential
MH  - Female
MH  - Headache Disorders, Primary/etiology
MH  - Humans
MH  - Magnetic Resonance Angiography
MH  - Prednisone/therapeutic use
MH  - Syndrome
MH  - Vasculitis/drug therapy/*physiopathology
MH  - Vasoconstriction/*physiology
MH  - Vasospasm, Intracranial/*complications/*physiopathology
EDAT- 2007/12/08 09:00
MHDA- 2008/01/24 09:00
CRDT- 2007/12/08 09:00
PHST- 2007/12/08 09:00 [pubmed]
PHST- 2008/01/24 09:00 [medline]
PHST- 2007/12/08 09:00 [entrez]
PST - ppublish
SO  - Can J Neurol Sci. 2007 Nov;34(4):467-77.