scholarly journals Reversible cerebral vasoconstriction syndrome promptly diagnosed with magnetic resonance imaging including magnetic resonance angiography during immunosuppressive therapy in a 16-year-old girl with refractory cytopenia of childhood

2016 ◽  
Vol 8 (4) ◽  
Author(s):  
Hideaki Ueki ◽  
Yasushi Sanayama ◽  
Akiyo Miyajima ◽  
Taichiro Tsuchimochi ◽  
Shunji Igarashi ◽  
...  

Reversible cerebral vasoconstriction syndrome (RCVS) is a syndrome characterized by severe headache with segmental vasoconstriction of the cerebral arteries that resolves within 12 weeks. A 16-year-old girl with refractory cytopenia of childhood, who was receiving the immunosuppressant cyclosporine, developed severe headache and was diagnosed with RCVS using magnetic resonance imaging, including magnetic resonance angiography (MRA). MRA is a non-invasive and very effective technique for diagnosing RCVS. MRA should be performed at the onset of severe headache during immunosuppressant administration for children with hematological disorders and may prevent sequelae such as posterior reversible encephalopathy syndrome or ischemic attack.

Cephalalgia ◽  
2017 ◽  
Vol 38 (6) ◽  
pp. 1207-1210 ◽  
Author(s):  
Sho Murase ◽  
Yasufumi Gon ◽  
Akihiro Watanabe ◽  
Kenichi Todo ◽  
Nobuo Kohara ◽  
...  

Background The temporal and anatomical features of vasoconstriction in patients with reversible cerebral vasoconstriction syndrome within hours after symptom onset, in the hyperacute phase, are unclear. Case result Herein we report the cases of two patients with acute severe headache who were diagnosed with reversible cerebral vasoconstriction syndrome. Magnetic resonance imaging within hours after symptom onset revealed multiple areas of isolated cortical vasogenic edema and hyperintense vessel signs of the distal cerebral arteries. Follow-up imaging performed four days later in both cases showed diffuse segmental arterial vasoconstriction in the proximal regions of the cerebral arteries. Both patients received antivasoconstrictive therapy shortly after admission, and neither had neurological sequelae at discharge. The magnetic resonance imaging findings improved gradually within three months after symptom onset. Conclusion Isolated cortical vasogenic edema and hyperintense vessel signs, when observed within hours from sudden severe headache onset, may be useful early markers of reversible cerebral vasoconstriction syndrome.


Cephalalgia ◽  
2010 ◽  
Vol 30 (10) ◽  
pp. 1271-1275 ◽  
Author(s):  
Benjamin Verillaud ◽  
Anne Ducros ◽  
Hélène Massiou ◽  
Patrice Tran Ba Huy ◽  
Marie-Germaine Bousser ◽  
...  

We report two patients with reversible cerebral vasoconstriction syndrome (RCVS) and carotid glomus tumour. The first patient presented with multiple thunderclap headaches. Cervical and cerebral magnetic resonance imaging showed diffuse cerebral vasoconstriction on magnetic resonance angiogram (MRA) and a carotid glomus tumour. The second patient presented with a cervical mass and was diagnosed with a non-secreting paraganglioma of the carotid body. Surgery with pre-operative angiography was followed by thunderclap headaches and MRA showed segmental cerebral vasoconstriction. Both patients were treated with nimodipine and headaches stopped. Both had normal cerebral arteries on the control MRA at 3 months. These two cases suggest that a paraganglioma may increase the susceptibility to develop RCVS. As a consequence, patients with RCVS should be investigated for a carotid glomus tumour, and patients with paraganglioma reporting severe headaches should have a cerebral MRA in order to rule out cerebral vasoconstriction.


2020 ◽  
pp. 22-26
Author(s):  
M. Yu. Afanasyeva ◽  
V. V. Goldobin ◽  
E. G. Klocheva

The data of patients with reversible cerebral vasoconstriction syndrome (RCVS) who were examined and treated in 2013–2020 are presented. 136 patients were examined (37.3 ± 11.4 years) – 107 (78.7 %) women, 29 (21.3 %) men. Detailed neurological examination, magnetic resonance imaging of the brain, magnetic resonance angiography and magnetic resonance venography were performed. Results. Primary RCVS was detected in 29 (21.3%), secondary RCVS – in 69 patients (50.7%), and a combination of primary and secondary RCVS factors – in 38 (28.0%) patients. In 95 (69.8%) patients (80 women, 15 men; p < 0.05) signs of cerebral artery angiodysplasia were identified. Hypoplasia of venous sinuses (transverse and/or sigmoid) was found in 34 patients (29 women and 5 men). Conclusions. RCVS is developed in women more often than men, but there was no significant difference in risk factors between men and women. Predisposing factors of both primary and secondary RCVS can be revealed simultaneously. Women were more likely to have variants of brachiocephalic artery development. Clarification of angiodysplasia variant influence to the development and severity of RCVS requires further study.


2019 ◽  
Vol 19 (2) ◽  
pp. 154-163
Author(s):  
Siriporn Hirunpat ◽  
Nuttha Sanghan ◽  
Adchara Wan-ai ◽  
Natee Ina ◽  
Pornrujee Hirunpat

Summary: In spite of the recent acceptance to be a common cause of acute severe headaches, reversible cerebral vasoconstriction syndrome (RCVS) is still commonly missed. The syndrome is characterized by acute severe headaches due to the vasoconstriction of the cerebral arteries, which eventually resolve spontaneously. We present a reported case of RCVS with a typical magnetic resonance angiography (MRA) appearance and a review of the literature.


2011 ◽  
Vol 26 (12) ◽  
pp. 1580-1584 ◽  
Author(s):  
Partha S. Ghosh ◽  
A. David Rothner ◽  
Kenneth G. Zahka ◽  
Neil R. Friedman

Reversible cerebral vasoconstriction syndrome is characterized by a reversible segmental and multifocal vasoconstriction of cerebral arteries, and severe headaches with or without focal neurologic deficits or seizures. A 15-year-old boy presented with thunderclap headache. He had severe hypertension, although his neurologic examination was normal. Initial workup for thunderclap headache to exclude subarachnoid or intracranial hemorrhage, meningitis, pituitary apoplexy, or venous sinus thrombosis was negative. Brain magnetic resonance angiography and cerebral angiography demonstrated bilateral anterior and posterior circulation diffuse, multifocal, vascular irregularities (beading and stenosis) suggestive of underlying vasculopathy or vasculitis. He was started on verapamil. There was complete reversal of the vascular abnormalities in 6 weeks evident by magnetic resonance angiography, with resolution of headache and normalization of blood pressure. Reversible cerebral vasoconstriction syndrome has been rarely reported in children. This case report highlights the diagnostic dilemma and management of the rare childhood presentation of this condition.


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