Reversible cerebral vasoconstriction syndrome
TL;DR: Reversible cerebral vasoconstriction syndrome is characterised by severe headaches with or without seizures and focal neurological deficits, and constriction of cerebral arteries which resolves spontaneously in 1–3 months, with no definite effect on the haemorrhagic and ischaemic complications.
Abstract: Reversible cerebral vasoconstriction syndrome is characterised by severe headaches with or without seizures and focal neurological deficits, and constriction of cerebral arteries which resolves spontaneously in 1-3 months. It affects females slightly more than males, and mean age of onset is around 45 years. Approximately 60% of cases are secondary, mainly postpartum and after exposure to vasoactive substances. The major complications are localised cortical subarachnoid haemorrhage (22%) and parenchymal ischaemic or haemorrhagic strokes (7%) which may leave permanent sequelae. Diagnosis requires the demonstration of the "string of beads" appearance of cerebral arteries on angiography, with complete or almost complete resolution on repeat angiography 12 weeks after onset. Nimodipine seems to reduce thunderclap headaches within 48 h but has no definite effect on the haemorrhagic and ischaemic complications.
Citations
More filters
TL;DR: Recurrent thunderclap headaches, seizures, strokes, and non-aneurysmal subarachnoid haemorrhage can all reveal reversible cerebral vasoconstriction syndrome, characterised by severe headaches, and segmental constriction of cerebral arteries that resolves within 3 months.
Abstract: Summary Recurrent thunderclap headaches, seizures, strokes, and non-aneurysmal subarachnoid haemorrhage can all reveal reversible cerebral vasoconstriction syndrome. This increasingly recognised syndrome is characterised by severe headaches, with or without other symptoms, and segmental constriction of cerebral arteries that resolves within 3 months. Reversible cerebral vasoconstriction syndrome is supposedly due to a transient disturbance in the control of cerebrovascular tone. More than half the cases occur post partum or after exposure to adrenergic or serotonergic drugs. Manifestations have a uniphasic course, and vary from pure cephalalgic forms to rare catastrophic forms associated with several haemorrhagic and ischaemic strokes, brain oedema, and death. Diagnosis can be hampered by the dynamic nature of clinicoradiological features. Stroke can occur a few days after initial normal imaging, and cerebral vasoconstriction is at a maximum on angiograms 2–3 weeks after clinical onset. The calcium channel blocker nimodipine seems to reduce thunderclap headaches within 48 h of administration, but has no proven effect on haemorrhagic and ischaemic complications.
613 citations
TL;DR: Recent developments in neuroimaging and detection, aetiology, prevalence, pathophysiology and clinical significance of cortical superficial siderosis are focused on, with a particular emphasis on cerebral amyloid angiopathy.
Abstract: Cortical superficial siderosis describes a distinct pattern of blood-breakdown product deposition limited to cortical sulci over the convexities of the cerebral hemispheres, sparing the brainstem, cerebellum and spinal cord. Although cortical superficial siderosis has many possible causes, it is emerging as a key feature of cerebral amyloid angiopathy, a common and important age-related cerebral small vessel disorder leading to intracerebral haemorrhage and dementia. In cerebral amyloid angiopathy cohorts, cortical superficial siderosis is associated with characteristic clinical symptoms, including transient focal neurological episodes; preliminary data also suggest an association with a high risk of future intracerebral haemorrhage, with potential implications for antithrombotic treatment decisions. Thus, cortical superficial siderosis is of relevance to neurologists working in neurovascular, memory and epilepsy clinics, and neurovascular emergency services, emphasizing the need for appropriate blood-sensitive magnetic resonance sequences to be routinely acquired in these clinical settings. In this review we focus on recent developments in neuroimaging and detection, aetiology, prevalence, pathophysiology and clinical significance of cortical superficial siderosis, with a particular emphasis on cerebral amyloid angiopathy. We also highlight important areas for future investigation and propose standards for evaluating cortical superficial siderosis in research studies.
272 citations
Cites background from "Reversible cerebral vasoconstrictio..."
...Long-term prognosis is often favourable and determined largely by the occurrence of stroke, although 55% of the patients develop life-threatening stroke and case-fatality is 51% (Ducros, 2012)....
[...]
...Reversible cerebral vasoconstriction syndrome is a clinical-radiological syndrome (Ducros, 2012) defined as: (i) acute severe (‘thunderclap’) headache at onset with or Figure 3 Differential diagnosis and mimics of cSS and acute cSAH....
[...]
...Reversible cerebral vasoconstriction syndrome is a clinical-radiological syndrome (Ducros, 2012) defined as: (i) acute severe (‘thunderclap’) headache at onset with or Figure 3 Differential diagnosis and mimics of cSS and...
[...]
TL;DR: Seizures do not normally progress to chronic epilepsy so antiepileptic drugs should be discontinued after about 3 months, and recovery within a few days, while the MRI abnormalities resolve much more slowly.
Abstract: The posterior reversible encephalopathy syndrome is an increasingly recognised disorder. Most patients have several symptoms; seizures are the most frequent, often multiple or status epilepticus. A combination of seizures, visual disturbance and/or headache, in particular, should lead to an early brain MRI to reveal the typical pattern of bilateral hyperintensities on fluid attenuated inversion recovery imaging, predominantly in the parieto-occipital region. There seem to be many possible triggers, including abrupt arterial hypertension, impaired renal function, pregnancy, immunosuppressive therapies and various inflammatory conditions. The clinical outcome is excellent, with recovery within a few days, while the MRI abnormalities resolve much more slowly. Little is known about the best management. Seizures do not normally progress to chronic epilepsy so antiepileptic drugs should be discontinued after about 3 months.
213 citations
TL;DR: The differential diagnosis includes reversible cerebral vasoconstriction syndromes and secondary cerebral vasculitis, which generally consist of headache, altered cognition, focal weakness, or stroke.
211 citations
TL;DR: Correlation of imaging findings with clinical presentation and laboratory test results helps establish the diagnosis of CNS vasculitis.
Abstract: Cerebral vasculitis is characterized by inflammation of the walls of blood vessels and may affect vessels of any size. The pathogenesis of vasculitis remains poorly understood. Vasculitis may affect large vessels (Takayasu arteritis, giant cell arteritis), medium-sized vessels (Kawasaki disease, polyarteritis nodosa), small vessels (immunoglobulin A vasculitis, microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis), or variable-sized vessels (Behcet disease, Cogan syndrome). Primary angiitis of the central nervous system (CNS) is an idiopathic disorder with no evidence of generalized inflammation that may simulate reversible cerebral vasoconstriction syndromes. Vasculitis may be secondary to systemic disease, infection, malignancy, drug use, or radiation therapy. Imaging findings vary from small ischemic changes to frank infarction, hemorrhage, and white matter edema and may show contrast material enhancement. The cerebral arteries may demonstrate a beaded appearance with variable degrees of stenosis, occlusion, and contrast enhancement of the vessel wall. Correlation of imaging findings with clinical presentation and laboratory test results helps establish the diagnosis of CNS vasculitis.
178 citations
Cites background from "Reversible cerebral vasoconstrictio..."
...Reversible Cerebral Vasoconstriction Syndromes The term reversible cerebral vasoconstriction syndrome refers to various disorders that are characterized by brain vasoconstriction (ie, Call-Fleming syndrome, postpartum angiopathy, migrainous vasospasm, and benign angiopathy of the CNS) (56,57)....
[...]
...Major complications of RCVS include brain edema (38% of cases), localized convexity subarachnoid hemorrhage (22%), posterior reversible encephalopathy syndrome (9%–14%), and, less frequently, ischemic or hemorrhagic stroke (57,58)....
[...]
References
More filters
TL;DR: Reversible, predominantly posterior leukoencephalopathy may develop in patients who have renal insufficiency or hypertension or who are immunosuppressed and the findings on neuroimaging are characteristic of subcortical edema without infarction.
Abstract: Background and Methods In some patients who are hospitalized for acute illness, we have noted a reversible syndrome of headache, altered mental functioning, seizures, and loss of vision associated with findings indicating predominantly posterior leukoencephalopathy on imaging studies. To elucidate this syndrome, we searched the log books listing computed tomographic (CT) and magnetic resonance imaging (MRI) studies performed at the New England Medical Center in Boston and Hopital Sainte Anne in Paris; we found 15 such patients who were evaluated from 1988 through 1994. Results Of the 15 patients, 7 were receiving immunosuppressive therapy after transplantation or as treatment for aplastic anemia, 1 was receiving interferon for melanoma, 3 had eclampsia, and 4 had acute hypertensive encephalopathy associated with renal disease (2 with lupus nephritis, 1 with acute glomerulonephritis, and 1 with acetaminophen-induced hepatorenal failure). Altogether, 12 patients had abrupt increases in blood pressure, and 8...
2,944 citations
TL;DR: From this issue Cephalalgia will become a monthly journal and has a strong throughput of excellent work and rather than raise the rejection rate, and somewhat arbitrarily dismiss work that readers might be very interested in seeing, it is timely to increase to 12 issues a year.
Abstract: Cephalalgia has been successful by a number of measures in the last 12 months: our circulation is up particularly through electronic penetration in libraries, our submission rate has risen substantially and our impact factor remains high amongst clinical neuroscience journals. We are entering our fifth and last year of the current publisher’s contract. In the life of the contract we have switched to a very successful on-line submission system, finalized details for a new CDROM containing all issues of Cephalalgia and Headache, and will publish this year the revised International Headache Society classification for headache (1). All of this is excellent progress The journal continues to attract a range of important, interesting and often very thought-provoking submissions. We have benefitted greatly from the broad range of interests of the Associate Editors we are able to recommend and solicit high-quality expert reviews that keep the scientific standards high. In keeping with our general philosophy there is a need for renewal amongst the Associate Editors, and Professor Peer Tfelt-Hansen will be standing down. Peer is widely regarded as one of the most authoritative figures on clinical trial methodology in headache, his wide contributions to the field have appropriately lead to his election as President of the International Headache Society. The journal thanks him for his contributions, and congratulates him on his election. After wide consultation I have invited Professor Nahib Ramadan, University of Chicago, to join the Associate Editors. Professor Ramadan was extensively involved in the journal under the previous Editor-in-Chief, Professor K. Michael Welch, and brings both excellence in academic life and a stint in industry with that important perspective to the Board. I welcome his acceptance of the invitation. From this issue Cephalalgia will become a monthly journal. We have a strong throughput of excellent work and rather than raise the rejection rate, and somewhat arbitrarily dismiss work that readers might be very interested in seeing, we felt it timely to increase to 12 issues a year. We hope this will allow more rapid publication of your accepted work and a greater range of interesting papers for readers in any one year. Let me close by thanking the Editorial Assistant Faye Cheeseman for her sterling efforts at the Blackwell’s Office in keeping the journal ticking over, our referees whose expertise is the key to our success, the Associate Editors who do so much for the journal, and you the reader for your continued support for Cephalalgia.
2,385 citations
TL;DR: Posterior reversible encephalopathy syndrome is a neurotoxic state coupled with a unique CT or MR imaging appearance, recognized in the setting of a number of complex conditions.
Abstract: SUMMARY: Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state coupled with a unique CT or MR imaging appearance. Recognized in the setting of a number of complex conditions (preeclampsia/eclampsia, allogeneic bone marrow transplantation, organ transplantation, autoimmune disease and high dose chemotherapy) the imaging, clinical and laboratory features of this toxic state are becoming better elucidated. This review summarizes the basic and advanced imaging features of PRES, along with pertinent features of the clinical and laboratory presentation and available histopathology. Many common imaging/clinical/laboratory observations are present among these patients, despite the perception of widely different associated clinical conditions.
959 citations
"Reversible cerebral vasoconstrictio..." refers background in this paper
...23 Haemorrhage (focal haematoma or subarachnoid blood) is seen in about 15% of cases.(21) The posterior reversible encephalopathy syndrome was initially described in association with eclampsia or during ciclosporin treatment after transplantation, always in the setting of severe hypertension....
[...]
TL;DR: The data suggest that RCVS is more frequent than previously thought, is more often secondary particularly to vasoactive substances, and should be considered in patients with recurrent thunderclap headaches, cSAH or cryptogenic strokes with severe headaches.
Abstract: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the association of severe headaches with or without additional neurological symptoms and a 'string and beads' appearance on cerebral arteries, which resolves spontaneously in 1-3 months. We present the clinical, neuroimaging and outcome data of 67 consecutive patients prospectively diagnosed over 3 years in our institution with an angiographically confirmed RCVS. There were 43 females and 24 males with a mean age of 42 years (19-70). RCVS was spontaneous in 37% of patients and secondary in the 63% others, to postpartum in 5 and to exposure to various vasoactive substances in 37, mainly cannabis, selective serotonin-recapture inhibitors and nasal decongestants. The main pattern of presentation (94% of patients) was multiple thunderclap headaches recurring over a mean period of 1 week. In 51 patients (76%), headaches resumed the clinical presentation. Various complications were observed, with different time courses. Cortical subarachnoid haemorrhage (cSAH) (22%), intracerebral haemorrhage (6%), seizures (3%) and reversible posterior leukoencephalopathy (9%) were early complications, occurring mainly within the first week. Ischaemic events, including TIAs (16%) and cerebral infarction (4%), occurred significantly later than haemorrhagic events, mainly during the second week. Significant sex differences were observed: women were older, had more frequent single-drug exposure and a higher rate of stroke and cSAH. Sixty-one patients were treated by nimodipine: 36% had recurrent headaches, 7% TIAs and one multiple infarcts. The different time courses of thunderclap headaches, vasoconstriction and strokes suggest that the responsible vasospastic disorder starts distally and progresses towards medium sized and large arteries. No relapse was observed during the 16 +/- 12.4 months of follow-up. Our data suggest that RCVS is more frequent than previously thought, is more often secondary particularly to vasoactive substances, and should be considered in patients with recurrent thunderclap headaches, cSAH or cryptogenic strokes with severe headaches.
859 citations
"Reversible cerebral vasoconstrictio..." refers background or result in this paper
...Acute alcoholic intoxication may be an additional precipitating factor but has only been incriminated in association with exposure to other drugs, such as cannabis and/or ecstasy/ and or cocaine.(3)...
[...]
...31 Non-invasive angiography (MRA or CTA) was only 80% sensitive in our series compared with the gold standard of catheter angiography which is by definition 100% sensitive (because it defines the syndrome), although nowadays rarely necessary (fig 6).(3) If another condition or another lesion is very unlikely, and if the initial MRA/CTA is definitely normal, and if there is no cortical SAH and no stroke on MRI, we do not perform a catheter angiogram....
[...]
...4, 13, 16, 27–29 Focal intracerebral haemorrhage occurred in 6% of our 67 cases.(3) This may be single or multiple, cortical or deep and of variable volume (fig 2)....
[...]
...Headache is often the only symptom, as in 75% of our French series.(3) It is severe in most...
[...]
...temporal pattern of the clinical features and the associated arterial abnormalities (table 3).(3)...
[...]
TL;DR: Systemic features in PRES appear to render strong support for the older theory of vasoconstriction coupled with hypoperfusion as the mechanism, including supporting evidence and current available imaging/clinical data related to the conditions that develop PRES.
Abstract: SUMMARY: Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state accompanied by a unique brain imaging pattern typically associated with a number of complex clinical conditions including: preeclampsia/eclampsia, allogeneic bone marrow transplantation, solid organ transplantation, autoimmune diseases and high dose cancer chemotherapy. The mechanism behind the developing vasogenic edema and CT or MR imaging appearance of PRES is not known. Two theories have historically been proposed: 1) Severe hypertension leads to failed auto-regulation, subsequent hyperperfusion, with endothelial injury/vasogenic edema and; 2) vasoconstriction and hypoperfusion leads to brain ischemia and subsequent vasogenic edema. The strengths/weaknesses of these hypotheses are reviewed in a translational fashion including supporting evidence and current available imaging/clinical data related to the conditions that develop PRES. While the hypertension/hyperperfusion theory has been most popular, the conditions associated with PRES have a similar immune challenge present and develop a similar state of T-cell/endothelial cell activation that may be the basis of leukocyte trafficking and systemic/cerebral vasoconstriction. These systemic features along with current vascular and perfusion imaging features in PRES appear to render strong support for the older theory of vasoconstriction coupled with hypoperfusion as the mechanism.
843 citations
"Reversible cerebral vasoconstrictio..." refers background in this paper
...24 Like the reversible cerebral vasoconstriction syndrome, the exact pathophysiology of the posterior reversible encephalopathy syndrome is unknown, and two hypotheses are debated.(25) The most popular is that severe arterial hypertension leads to a failure of cerebral autoregulation with subsequent hyperperfusion and vasogenic oedema....
[...]