Regional cerebral blood flow characteristics of the Sturge-Weber syndrome

Riela, Anthony R.; Stump, David A.; Roach, E. Steve; McLean, William T.; García, Juan Carlos

doi:10.1016/0887-8994(85)90042-6

Cited by 32 publications

(21 citation statements)

References 16 publications

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“…This may occur alone or in combination with the V2 and V3 distributions, by cerebral venous malformations (leptomeningeal angiomatosis), and by glaucoma with ocular capillary venous vascular malformations. 1,2 …”

Section: Advances and Questions In The Definition Neurological Symptmentioning

confidence: 99%

Updates and future horizons on the understanding, diagnosis, and treatment of Sturge–Weber syndrome brain involvement

Marchuk

Ball

et al. 2011

Develop Med Child Neuro

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AIM To review recent developments in the understanding, diagnosis, and treatment of Sturge–Weber syndrome (SWS). METHOD Members of the Brain Vascular Malformation Consortium Sturge–Weber National Workgroup contributed their expertise, to review the literature, and present promising directions for research. RESULTS The increasing number of reports dealing with SWS over the last decade reflects progress in the diagnosis and understanding of the neurological involvement. The proliferation of centers and advocacy groups to care for patients with SWS and to stimulate research has aided the development of new insights into the clinical manifestations and the pathophysiology of neurological progression, and the development of novel hypotheses to direct future research. Many key questions remain, but the tools and networks to answer them are being developed. INTERPRETATION This review summarizes important new knowledge and presents new research directions that are likely to provide further insights, earlier diagnosis, improved treatments, and possibly, prevention of this syndrome.

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Section: Advances and Questions In The Definition Neurological Symptmentioning

confidence: 99%

Updates and future horizons on the understanding, diagnosis, and treatment of Sturge–Weber syndrome brain involvement

Marchuk

Ball

et al. 2011

Develop Med Child Neuro

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“…Other studies have found this same inverse association; for example, Licht et al demonstrated the inverse link between CBF and periventricular leukomalacia in a study of 25 term infants with congenital heart disease 5 while Fukuda et al found this in two studies, one with 36 25 and one with 67 low birth weight infants. 26 Reila et al showed that regional CBF was decreased in the region of the lesions in Sturge-Weber syndrome in children, 27 however, this has been also demonstrated in adults; for example, Doi et al 28 found microbleeds inversely associated with CBF in Alzheimer’s disease.…”

Section: Discussionmentioning

confidence: 99%

Neurological Injury and Cerebral Blood Flow in Single Ventricles Throughout Staged Surgical Reconstruction

Fogel

Elci

et al. 2017

Circulation

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Background Single ventricle patients experience a high rate of brain injury and adverse neurodevelopmental outcome, however, the incidence of brain abnormalities throughout surgical reconstruction and its relationship with cerebral blood flow, oxygen delivery and carbon dioxide reactivity remains unknown. Methods Single ventricle patients were studied with MRI scans immediately prior to bidirectional Glenn (pre-BDG), prior to Fontan and then 3–9 months after Fontan reconstruction. Results One hundred and sixty eight consecutive subjects recruited into the project underwent 235 scans: 63 pre-BDG (mean age 4.8+1.7 months), 118 BDG (2.9+1.4 years) and 54 after Fontan (2.4+1.0 years). Non-acute ischemic white matter changes on T2 weighted imaging, focal tissue loss, and ventriculomegaly were all more commonly detected in BDG and Fontans compared to pre-BDG (P<0.05). BDG patients has significantly higher CBF than Fontan patients. The odds of discovering brain injury adjusting for surgical stage as well as 2 or more co-existing lesions within a patient all decreased (63–75% and 44% respectively) with increasing amount of cerebral blood flow (P<0.05). In general, there was no association of oxygen delivery (with the exception of ventriculomegaly in the BDG group) or carbon dioxide reactivity with neurological injury. Conclusion Significant brain abnormalities are commonly present in single ventricle patients and detection of these lesions increase as children progress through staged surgical reconstruction with multiple co-existing lesions more common earlier than later. In addition, this study demonstrated that BDG patients had greater CBF than Fontan patients and that there exists an inverse association of various indices of CBF with these brain lesions, however, CO2 reactivity, oxygen delivery (with one exception) were not associated with brain lesion development.

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“…Previous studies have shown hypoperfusion (Riela et al 1985, Chiron et al 1989, and hypometabolism (Chugani et al 1989) in affected hemispheres, often over a wider area than that obviously affected by the angioma. Bilateral hypometabolism appears to be associated with learning disability even with a unilateral pial nevus (Chugani et al 1989).…”

Section: Discussionmentioning

confidence: 99%

“…Transient hemiparesis without evidence of ictal activity is well described in SWS and is considered to be related to an abnormal vasomotor response (Arzimanoglou and Aicardi 1992). In addition, reductions in the cerebral blood flow (CBF) as a response to carbon dioxide (Riela et al 1985) and acetazolamide (Okudaira et al 1997) have been demonstrated, which suggest that the cerebral circulation in SWS can mount only a limited vasodilator response, and thus increase in oxygen delivery, to an increase in metabolic demand.…”

mentioning

confidence: 99%

Sturge–Weber syndrome: cerebral haemodynamics during seizure activity

Aylett

Neville

Cross

et al. 1999

Develop Med Child Neuro

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The aim of this study was to examine the haemodynamic response to seizures in three infants with Sturge–Weber syndrome by measuring regional cerebral blood flow using transcranial Doppler sonography and 99mTc HMPAO SPECT. Time‐locked video/digital EEG recording was carried out for ictal studies. MRI was performed in all subjects. SPECT showed hemispheric hypoperfusion interictally in all three patients and also ictally in one of the three; a small region of hyperperfusion was seen on the same ictal scan in the latter, ie. the patient with interictal and ictal hypoperfusion. In the two older children middle cerebral artery velocity (MCAV) was reduced by between 29 and 62% in the middle cerebral artery of the predominantly affected hemisphere compared with the contralateral side. During seizures, increases of 6 to 30% in MCAV were recorded for the clinically seizing hemisphere compared with 24 to 170% for the contralateral side in four of the seizures recorded. In one infant, MCAV fell bilaterally during a seizure that generalized (–18 and –43% in the predominantly affected and contralateral side respectively). Sequential recordings in one infant suggested that, with time, the haemodynamic response to seizures of the unaffected hemisphere may decrease. These findings suggest that the venous malformation in SWS is associated with an impairment of the cerebral haemodynamic response to seizure activity.

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Regional cerebral blood flow characteristics of the Sturge-Weber syndrome

Cited by 32 publications

References 16 publications

Updates and future horizons on the understanding, diagnosis, and treatment of Sturge–Weber syndrome brain involvement

Updates and future horizons on the understanding, diagnosis, and treatment of Sturge–Weber syndrome brain involvement

Neurological Injury and Cerebral Blood Flow in Single Ventricles Throughout Staged Surgical Reconstruction

Sturge–Weber syndrome: cerebral haemodynamics during seizure activity

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