Pediatric Reversible Cerebral Vasoconstriction Syndrome: Two Unique Cases with a Review of all Reported Children

2021 ◽  
Author(s):  
Neelu Desai ◽  
Rahul Badheka ◽  
Nitin Shah ◽  
Vrajesh Udani
Keyword(s):  
Magnetic Resonance ◽  
Cerebral Arteries ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Neurological Deficits ◽  
Cerebral Vasoconstriction ◽  
Magnetic Resonance Imaging Mri ◽  
Reversible Encephalopathy ◽  
Mr Angiogram

AbstractReversible cerebral vasoconstriction syndrome (RCVS) has been well described in adults, but pediatric cases are yet under recognized. We describe two children with RCVS and review similar already published pediatric cases. The first patient was a 10-year-old girl who presented with severe headaches and seizures 3 days after blood transfusion. Brain magnetic resonance imaging (MRI) showed changes compatible with posterior reversible encephalopathy syndrome and subarachnoid hemorrhage. Magnetic resonance angiogram showed diffuse vasoconstriction of multiple cerebral arteries. The second patient was a 9-year-old boy who presented with severe thunderclap headaches. Brain MRI showed isolated intraventricular hemorrhage. Computed tomography/MR angiogram and digital subtraction angiogram were normal. A week later, he developed focal neurological deficits. Repeated MR angiogram showed diffuse vasospasm of multiple intracranial arteries. Both children recovered completely. A clinico-radiological review of previously reported childhood RCVS is provided.

Reversible Segmental Cerebral Vasoconstriction (Call-Fleming Syndrome): Are Calcium Channel Inhibitors A Potential Treatment Option?

Cephalalgia ◽  
2003 ◽  
Vol 23 (3) ◽  
pp. 218-222 ◽  
Author(s):  
DA Nowak ◽  
SO Rodiek ◽  
S Henneken ◽  
J Zinner ◽  
R Schreiner ◽  
...  
Keyword(s):  
Calcium Channel ◽  
Treatment Options ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Sudden Onset ◽  
Neurological Deficits ◽  
Vasoactive Drugs ◽  
Cerebral Vasoconstriction ◽  
Calcium Channel Inhibitors ◽  
Magnetic Resonance Imaging Mri

The Call-Fleming syndrome is characterized by sudden onset of thunderclap-like headache and focal neurological deficits. The pathophysiological correlate is a reversible segmental cerebral vasoconstriction frequently associated with focal cerebral ischaemia. The syndrome has been described in a variety of clinical conditions, and recently an association between the syndrome and exposure to vasoactive drugs was observed. Effective treatment options are not known. A 63-year-old female developed sudden ‘worst ever’ headache. Initial neurological examination, laboratory blood tests, CSF examination and brain magnetic resonance imaging (MRI) were normal. Previous medical history was unremarkable and she did not take vasoactive drugs. Eleven days after the onset of headache she developed visual field impairment and a right-sided hemiparesis. Brain MRI revealed bilateral posterior and left parietal ischaemic strokes. Cerebral catheter angiography showed segmental arterial vasoconstriction. A vasodilatative therapy with calcium channel inhibitors was started and serial transcranial Doppler ultrasonography demonstrated resolution of cerebral arterial vasoconstriction. The present case illustrates that calcium channel inhibitors may be an effective therapy for segmental cerebral arterial vasoconstriction. However, more clinical data are needed to prove this observation.

scholarly journals Diagnosing RCVS Without the CV

2015 ◽  
Vol 6 (3) ◽  
pp. NP1-NP4 ◽  
Author(s):  
Nuri Jacoby ◽  
Ulrike Kaunzner ◽  
Marc Dinkin ◽  
Joseph Safdieh
Keyword(s):  
Magnetic Resonance ◽  
Resonance Imaging ◽  
Multiple Risk Factors ◽  
Mri Brain ◽  
Cerebral Vasoconstriction ◽  
History Of ◽  
Magnetic Resonance Imaging Mri ◽  
Reversible Encephalopathy ◽  
Occipital Lobes ◽  
The Brain

This is a case of a 52-year-old man with a past medical history of 2 episodes of coital thunderclap headaches as well as recent cocaine, marijuana, and pseudoephedrine use, who presented with sudden, sharp, posterior headache associated with photophobia and phonophobia. His initial magnetic resonance imaging (MRI) of the brain, magnetic resonance angiography (MRA) of the head, and magnetic resonance venography (MRV) of the head were all normal as well as a normal lumbar puncture. Given the multiple risk factors for reversible cerebral vasoconstriction syndrome (RCVS), the patient was treated for suspected RCVS, despite the normal imaging. Repeat MRI brain 3 days after hospital admission demonstrated confluent white matter T2 hyperintensities most prominent in the occipital lobes, typical of posterior reversible encephalopathy syndrome (PRES). Repeat MRA of the head 1 day after discharge and 4 days after the abnormal MRI brain showed multisegment narrowing of multiple arteries. This case demonstrates that RCVS may present with PRES on MRI brain and also exemplifies the need to treat suspected RCVS even if imaging is normal, as abnormalities in both the MRI and the MRA may be delayed.

scholarly journals Can we evaluate cranial aneurysms on conventional brain magnetic resonance imaging?

2016 ◽  
Vol 7 (01) ◽  
pp. 83-86 ◽  
Author(s):  
Emine Caliskan ◽  
Yeliz Pekcevik ◽  
Adnan Kaya
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Characteristic Curve ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Kappa Statistics ◽  
Resonance Imaging ◽  
Vascular Structures ◽  
Magnetic Resonance Imaging Mri ◽  
Age Range

ABSTRACT Purpose: To evaluate the contribution of conventional brain magnetic resonance imaging (MRI) for the determination of intracranial aneurysms. Materials and Methods: Brain MRI and computed tomography angiography (CTA) of 45 patients (29 women and 16 men; age range, 32–80 years) with aneurysm were analyzed. A comparison was made between brain MRI and CTA based on size and presence of aneurysm. The comparisons between MRI and CTA were investigated through Bland-Altman graphics, receiver operating characteristic curve, and Kappa statistics. Results: Fifty-seven aneurysms were evaluated. Forty-five percent of 57 aneurysms on CTA were detected on conventional brain MRI. A significant correlation was found between CTA and brain MRI in the diagnosis of aneurysm (P < 0.05). In an analysis of the size measurement, a significant correlation was observed between CTA and brain MRI. Seventy-seven percent of aneurysms <4 mm was not detected and the efficiency of MRI in the detection of aneurysms <4 mm was found to be low. Conclusion: Aneurysms can also be appreciated on conventional brain MRI, and vascular structures should be reviewed carefully while analyzing brain MRI.

scholarly journals A Novel SUCLA2 Mutation Presenting as a Complex Childhood Movement Disorder

2016 ◽  
Vol 32 (2) ◽  
pp. 246-250 ◽  
Author(s):  
Caterina Garone ◽  
Juliana Gurgel-Giannetti ◽  
Simone Sanna-Cherchi ◽  
Sindu Krishna ◽  
Ali Naini ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Movement Disorder ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Mitochondrial Respiratory Chain ◽  
Resonance Spectroscopy ◽  
Homozygous Mutation ◽  
Psychomotor Delay ◽  
Mtdna Depletion ◽  
Magnetic Resonance Imaging Mri

SUCLA2 defects have been associated with mitochondrial DNA (mtDNA) depletion and the triad of hypotonia, dystonia/Leigh-like syndrome, and deafness. A 9-year-old Brazilian boy of consanguineous parents presented with psychomotor delay, deafness, myopathy, ataxia, and chorea. Despite the prominent movement disorder, brain magnetic resonance imaging (MRI) was normal while 1H-magnetic resonance spectroscopy (MRS) showed lactate peaks in the cerebral cortex and lateral ventricles. Decreased biochemical activities of mitochondrial respiratory chain enzymes containing mtDNA-encoded subunits and mtDNA depletion were observed in muscle and fibroblasts. A novel homozygous mutation in SUCLA2, the first one in the ligase coenzyme A (CoA) domain of the protein, was identified. Escalating doses of CoQ10 up to 2000 mg daily were associated with improvement of muscle weakness and stabilization of the disease course. The findings indicate the importance of screening for mitochondrial dysfunction in patients with complex movement disorders without brain MRI lesions and further investigation for potential secondary CoQ10 deficiency in patients with SUCLA2 mutations.

Reversible Cerebral Vasoconstriction Syndrome with Posterior Leucoencephalopathy after Oral Contraceptive Pills

Cephalalgia ◽  
2009 ◽  
Vol 30 (1) ◽  
pp. 42-45 ◽  
Author(s):  
Y Soo ◽  
AB Singhal ◽  
T Leung ◽  
S Yu ◽  
H Mak ◽  
...  
Keyword(s):  
Oral Contraceptive ◽  
Cerebral Arteries ◽  
Haemorrhagic Stroke ◽  
Sudden Onset ◽  
Reproductive Hormones ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Neurological Deficits ◽  
Oral Contraceptive Pills ◽  
Cerebral Vasoconstriction ◽  
Contraceptive Pills

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset recurrent ‘thunderclap’ headaches with reversible multifocal narrowing of the cerebral arteries, often associated with focal neurological deficits from ischaemic or haemorrhagic stroke. It has been associated with exposure to vasoconstrictive drugs, pregnancy, migraine, and a variety of other conditions. Whereas the pathophysiology of RCVS remains unclear, changes in the levels of female hormones are considered important because RCVS predominantly affects women and is frequently associated with pregnancy. We report a patient with angiographically confirmed RCVS whose MRI showed reversible brain oedema, suggesting an overlap between RCVS and the reversible posterior leucoencephalopathy syndrome. The only identified risk factor was oral contraceptive pills started 1 month prior to onset, supporting a role for female reproductive hormones in precipitating this overlap syndrome.

scholarly journals 1415. Use of Adjunctive Steroids in Adults with Encephalitis

2019 ◽  
Vol 6 (Supplement_2) ◽  
pp. S515-S515
Author(s):  
Ambreen Allana ◽  
Mohammed Samannodi ◽  
Michael Hansen ◽  
Rodrigo Hasbun
Keyword(s):  
Clinical Outcomes ◽  
Brain Mri ◽  
Tertiary Care ◽  
Brain Magnetic Resonance Imaging ◽  
Adverse Outcomes ◽  
Neurological Deficits ◽  
Icu Admission ◽  
Magnetic Resonance Imaging Mri ◽  
Tertiary Care Hospitals ◽  
The Impact

Abstract Background To describe the use and the impact on clinical outcomes of adjunctive steroids in adults with encephalitis. Methods We conducted a retrospective observational study of 230 adults (age >17 years) who met the international consortium definition for encephalitis. An adverse clinical outcome was defined as a Glasgow outcome score of 1–4. The study took place at three tertiary care hospitals in Houston TX, between August 2008 and September 2017. Results A total of 230 adults with encephalitis were enrolled, out of which 121 (52.6%) received steroids. Adjunctive steroids were given more frequently to those with focal neurological deficits (P = 0.013), had a positive cerebrospinal fluid (CSF) HSV PCR (P = 0.013), required mechanical ventilation (MV) (P = 0.011), required intensive care unit (ICU) admission (P < 0.001), had white matter abnormalities (P = 0.014) or had cerebral edema on the brain magnetic resonance imaging (MRI) (P = 0.003). An adverse outcome was seen in 139 (60.7%) of patients. Predictors for adverse outcomes included a Glasgow coma score (GCS) < 8, fever, seizures, ICU admission, and presence of edema on brain MRI. The use of adjunctive steroids did not impact clinical outcomes (P = 0.521). Independent prognostic factors on logistic regression analysis were edema on brain MRI (7.780 [1.717–35.263] P = 0.008), GCS < 8 (6.339 [1.992–20.168] P = 0.002), and fever (2.601 [1.342–5.038] P = 0.005). Conclusion Adults with encephalitis continues to be associated with significant adverse clinical outcomes in the majority of patients. Adjunctive steroids are used in the sicker patients and it is not associated with improved clinical outcomes. Disclosures All authors: No reported disclosures.

Abstract P460: Comparison of Hemorrhagic Posterior Reversible Encephalopathy Syndrome and Hemorrhagic Reversible Cerebral Vasoconstriction Syndrome

Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Meghan Purohit ◽  
Monica Scarsella ◽  
Karlo Toljan ◽  
Naresh Mullaguri ◽  
Dolora R Wisco ◽  
...  
Keyword(s):  
Posterior Reversible Encephalopathy Syndrome ◽  
Drugs Of Abuse ◽  
Skilled Nursing Facility ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Neurological Deficits ◽  
Posterior Reversible Encephalopathy ◽  
Presenting Symptoms ◽  
Cerebral Vasoconstriction ◽  
Tertiary Center ◽  
Reversible Encephalopathy

Introduction: Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) are diagnoses that have a clinical and radiographic overlap. One particular overlap includes the presence of hemorrhage, which we studied in each population to determine its effect on outcomes. Objective: To compare characteristics and outcomes of hemorrhagic PRES and hemorrhagic RCVS populations. Methods: We conducted a review of the electronic health record at a single tertiary center from January 2008 to December 2018. Inclusion criteria were 18 years or older with clinical and radiographic evidence of PRES or RCVS. Patient demographics, presenting symptoms, imaging findings, and outcomes were compared between PRES and RCVS groups. Poor outcome was defined as discharge to skilled nursing facility or death. Analysis was performed using Pearson’s Chi-Square test. Results: Among 281 PRES and 98 RCVS cases, intracranial hemorrhage was seen on imaging in 51 PRES cases (18%) and 34 RCVS cases (35%). Headache was present in all patients with hemorrhagic RCVS but only seen in 40% of hemorrhagic PRES. Use of antidepressants or drugs of abuse was more frequent with hemorrhagic RCVS (53%) as compared to hemorrhagic PRES (7%, p <0.05). Among hemorrhagic PRES, presence of intraparenchymal hemorrhage but not subarachnoid hemorrhage was associated with vasoconstriction on arterial imaging (p<0.05). Length of stay was longer for both hemorrhagic PRES and hemorrhagic RCVS (p<0.001) compared those without hemorrhage. Presence of focal neurological deficits, motor and sensory, was associated with poor outcomes in hemorrhagic RCVS patients (p<0.05). Discharge to a rehabilitation facility was associated with hemorrhagic PRES (p<0.05), no association in those without hemorrhage. There is no evidence that discharge location is significantly different between RCVS hemorrhage and non-hemorrhage groups. Conclusion: Hemorrhage in PRES and RCVS is associated with more clinical deficits at presentation and longer hospital stays. Underlying vasoconstriction is frequent and associated with hemorrhagic PRES.

093 Clinical and imaging discordance in reversible cerebral vasoconstriction syndrome

2018 ◽  
Vol 89 (6) ◽  
pp. A37.2-A37 ◽  
Author(s):  
Michael J Devlin ◽  
Archana Dwivedee ◽  
Richard P White
Keyword(s):  
Subarachnoid Haemorrhage ◽  
Cerebral Arteries ◽  
Clinical Status ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Clinical State ◽  
Cerebral Vasoconstriction ◽  
Csf Analysis ◽  
Elevated Protein ◽  
Digital Subtraction Angiogram ◽  
Reversible Encephalopathy

IntroductionReversible cerebral vasoconstriction syndrome (RCVS) is to be suspected in cases of thunderclap headache particularly when associated with convex subarachnoid haemorrhage without cerebral aneurysm. Angiographic changes and clinical state do not always correlate. We present a case report from the Townsville Hospital.CaseA 49 year old female presented to another hospital with recurrent thunderclap headaches first commencing with exercise. The medical history included idiopathic intracranial hypertension in remission, chronic migraine treated with botulinum toxin, and depression treated with amitriptyline and desvenlafaxine. MR imaging revealed cerebral vertex subarachnoid haemorrhage with normal MR angiogram of the cerebral vessels. The patient was transferred to our institution in a confused and restless state for cerebral digital subtraction angiogram, which was normal. A lumbar puncture revealed elevated white cells of 17×106/L with normal CSF protein and opening pressure of 39 cm H2O. Nimodipine that had been commenced before the patient became encephalopathic was ceased. Her confusion and headaches resolved but relapsed after three days. A repeat MR brain revealed multiple T2 hyperintensities in bilateral anterior and posterior circulation territories demonstrating diffusion restriction, and MR angiography revealed new segmental strictures and beading of bilateral middle and anterior cerebral arteries. The patient was diagnosed with overlap RCVS/posterior reversible encephalopathy syndrome; desvenlafaxine was ceased, acetazolamide commenced for raised intracranial pressure and a several week course of nimodipine recommenced. The patient was discharged home after resolution of headache and confusion without neurological deficit.ConclusionThis case highlights the dynamic changes of vasoconstriction and that angiographic findings can be discordant from the clinical status. Serial imaging is required to detect abnormalities of RCVS when the clinical suspicion is high. CSF analysis can be normal, however may reveal elevated protein or pleocytosis. The cause of our patient’s RCVS was thought to be the chronic use of desvenlafaxine.

Diffuse cerebrovascular dilation: Case report of amezinium metilsulfate-induced reversible cerebral vasoconstriction syndrome

Cephalalgia ◽  
2015 ◽  
Vol 36 (3) ◽  
pp. 289-293 ◽  
Author(s):  
Makoto Kobayashi
Keyword(s):  
Case Report ◽  
Magnetic Resonance Angiography ◽  
Cerebral Arteries ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Neurological Deficits ◽  
Case Description ◽  
Cerebral Vasoconstriction ◽  
Sympathomimetic Drug ◽  
Vasoactive Medications

Background Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches with reversible cerebral vasoconstriction, and often precipitated by the postpartum state and vasoactive medications. We describe a case of a patient with RCVS induced by amezinium metilsulfate, a sympathomimetic drug, in whom magnetic resonance angiography (MRA) initially revealed diffusely dilated cerebral arteries. Case description A 34-year-old woman was prescribed amezinium metilsulfate for hypotension. Twelve days later, she suffered from abrupt severe headaches and was referred to our department. She had no neurological deficits; however, MRA revealed diffusely dilated anterior, middle, and posterior cerebral arteries with vasoconstriction. She was tentatively diagnosed with RCVS and successfully treated with verapamil for headache. Nevertheless, follow-up MRAs disclosed widespread segmental vasoconstriction that resolved in two months. Discussion Diffuse cerebrovascular dilation has not been addressed but may be associated with RCVS pathophysiology. In addition, physicians should bear in mind that amezinium metilsulfate can potentially induce RCVS.

scholarly journals Slowly expanding/evolving lesions as a magnetic resonance imaging marker of chronic active multiple sclerosis lesions

2018 ◽  
Vol 25 (14) ◽  
pp. 1915-1925 ◽  
Author(s):  
Colm Elliott ◽  
Jerry S Wolinsky ◽  
Stephen L Hauser ◽  
Ludwig Kappos ◽  
Frederik Barkhof ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Magnetic Resonance ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Tissue Loss ◽  
Jacobian Determinant ◽  
Resonance Imaging ◽  
T2 Lesions ◽  
Magnetic Resonance Imaging Mri

Background: Chronic lesion activity driven by smoldering inflammation is a pathological hallmark of progressive forms of multiple sclerosis (MS). Objective: To develop a method for automatic detection of slowly expanding/evolving lesions (SELs) on conventional brain magnetic resonance imaging (MRI) and characterize such SELs in primary progressive MS (PPMS) and relapsing MS (RMS) populations. Methods: We defined SELs as contiguous regions of existing T2 lesions showing local expansion assessed by the Jacobian determinant of the deformation between reference and follow-up scans. SEL candidates were assigned a heuristic score based on concentricity and constancy of change in T2- and T1-weighted MRIs. SELs were examined in 1334 RMS patients and 555 PPMS patients. Results: Compared with RMS patients, PPMS patients had higher numbers of SELs ( p = 0.002) and higher T2 volumes of SELs ( p < 0.001). SELs were devoid of gadolinium enhancement. Compared with areas of T2 lesions not classified as SEL, SELs had significantly lower T1 intensity at baseline and larger decrease in T1 intensity over time. Conclusion: We suggest that SELs reflect chronic tissue loss in the absence of ongoing acute inflammation. SELs may represent a conventional brain MRI correlate of chronic active MS lesions and a candidate biomarker for smoldering inflammation in MS.

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