• No results found

Cerebral Perfusion Abnormalities in Children With Sturge-Weber Syndrome Shown by Dynamic Contrast Bolus Magnetic Resonance Perfusion Imaging

N/A
N/A
Protected

Academic year: 2020

Share "Cerebral Perfusion Abnormalities in Children With Sturge-Weber Syndrome Shown by Dynamic Contrast Bolus Magnetic Resonance Perfusion Imaging"

Copied!
9
0
0

Loading.... (view fulltext now)

Full text

(1)

ARTICLE

Cerebral Perfusion Abnormalities in Children With

Sturge-Weber Syndrome Shown by Dynamic Contrast

Bolus Magnetic Resonance Perfusion Imaging

Amlyn L. Evans, FRCRa, Elysa Widjaja, FRCRb, Daniel J. A. Connolly, FRCRa,c, Paul D. Griffiths, PhDb,c

aDepartment of Radiology, Royal Hallamshire Hospital, Sheffield, United Kingdom;bUnit of Academic Radiology, University of Sheffield, Sheffield, United Kingdom; cDepartment of Radiology, Sheffield Children’s Hospital, Sheffield, United Kingdom

The authors have indicated they have no financial relationships relevant to this article to disclose.

ABSTRACT

OBJECTIVE.Sturge-Weber syndrome is characterized by leptomeningeal angiomatosis

and a facial naevus that is usually unilateral. Magnetic resonance imaging is the cornerstone of confirming the disease and judging the extent of the abnormalities. It has been shown, however, that brain perfusion abnormalities on nuclear med-icine imaging often are more extensive than the abnormal leptomeningeal en-hancement on magnetic resonance. In this article, we assess the utility of magnetic resonance perfusion in demonstrating perfusion abnormalities in pediatric cases of Sturge-Weber syndrome.

METHODS.Magnetic resonance perfusion studies were performed on 7 consecutive

children who presented to our department with clinically suspected Sturge-Weber syndrome. The extent of time to peak abnormality on dynamic gadolinium bolus magnetic resonance perfusion imaging was compared with the extent of lepto-meningeal enhancement and the presence of venous abnormalities.

RESULTS.Good magnetic resonance perfusion data were obtained in all 7 cases.

Perfusion abnormalities were closely anatomically related to meningeal enhance-ment on postcontrast T1-weighted imaging. However, perfusion abnormalities were found consistently in the vicinity of developmental venous anomalies that were present in 4 of 7 cases. In 1 child, there was a perfusion deficit in the cerebellar lobe contralateral to the leptomeningeal angiomatosis, consistent with crossed cerebellar diaschisis.

CONCLUSIONS.Magnetic resonance perfusion is a sensitive indicator of perfusion

abnormalities in Sturge-Weber syndrome and can be performed easily at the same time as the diagnostic scan. Magnetic resonance perfusion imaging therefore is useful in the assessment of this disease. This approach has the extra advantage of correlating the perfusion abnormalities with the high-resolution imaging that is provided from magnetic resonance imaging.

www.pediatrics.org/cgi/doi/10.1542/ peds.2005-1815

doi:10.1542/peds.2005-1815 Key Words

cerebral blood flow, phakomatoses, magnetic resonance

Abbreviations

SWS—Sturge-Weber syndrome FLAIR—fluid-attenuated inversion recovery

SPECT—single photon emission computed tomography MR—magnetic resonance FmMTT—first mean transit time

Accepted for publication Nov 10, 2005

Address correspondence to Amlyn L. Evans, FRCR, Neuroradiology Department, Inpatient X-Ray, Royal Hallamshire Hospital, Sheffield S10 2JF, United Kingdom. E-mail: amlyn. evans@sth.nhs.uk

(2)

S

TURGE-WEBER SYNDROME (SWS),otherwise known as encephalotrigeminal angiomatosis or encephalofa-cial angiomatosis, is 1 of the spectrum of diseases that are classified under the phakomatoses.1It was probably first

described by Schirmer in 18602; however, Sturge in 1879

is credited with the eponymous syndrome, having given a clinical account of the classic syndrome and postulated that an intracranial angiomatous malformation was re-sponsible for the neurologic symptoms.3Weber is

cred-ited with the first radiologic report of intracranial calci-fication on a plain skull radiograph.1 Although the

syndrome is congenital, there is generally no heritability, although a few familial cases are described.1

The syndrome is characterized by a facial cutaneous naevus (the so-called naevus flammeus or port-wine stain) that is associated with neurologic symptoms such as focal seizures and/or hemiplegia that affects the op-posite side of the body to the naevus. Hemianopsia and intellectual impairment also are common.4,5The

cutane-ous naevus is usually found in the territory of the tri-geminal nerve, in particular the first division, and there also may be associated orbital abnormalities that consist of buphthalmos (congenitally enlarged globe), choroidal hemangioma, and glaucoma. The pathologic changes usually are unilateral, and although bilateral cutaneous involvement may be seen in 32.5% of cases, bilateral intracranial involvement is seen in only 7.5% of cases.1

Patients with bilateral intracranial disease usually are more severely affected with intractable seizures; there-fore, the diagnosis of unexpected bilateral SWS is clini-cally important. It usually is a neurologiclini-cally progressive disorder, ultimately associated with profound neurologic decline, the mechanism of which is incompletely under-stood.6

Findings on brain computed tomography and MRI reflect the underlying pathology. Superficial calcifica-tion,7focal parenchymal atrophy, and the

leptomenin-geal enhancement are widely recognized, common find-ings.8Leptomeningeal enhancement commonly is used

to judge the extent of the disease process in SWS, and this has previously been assessed on MRI using T1-weighted imaging after gadolinium chelates have been injected.9The T1 enhancement is thought to represent

leakage of contrast medium into the interstitium be-tween the leptomeningeal vessels and into the first cor-tical layer, which frequently undergoes astrogliotic scar-ring secondary to chronic hypoxia and results in breakdown of the blood– brain barrier. It has been re-ported that there is a diminishing amount of superficial enhancement over time, whereas the deep veins become more prominent. This is thought to be attributable to chronic progressive thrombosis, which is supported by post mortem studies that show thickened, fibrotic veins as a result of chronic venous hypertension.10,11

Alterna-tively, leptomeningeal enhancement might represent slow flow throughout the abnormal, tortuous pial

vas-culature. Transient enhancement has been reported12

and is thought to be attributable to the reversibility of the venous hypertension. Seizures may be a causative factor in the venous hypertension or may be a conse-quence of the tissue hypoxia. As pial damage accumu-lates, the leptomeningeal enhancement would be ex-pected to be permanent.

MRI, in particular postcontrast T1 or fluid-attenuated inversion recovery (FLAIR) imaging, is currently the cornerstone of diagnosis in SWS because of its ability to show abnormal leptomeningeal enhancement.9

Perfu-sion abnormalities also are seen on nuclear medicine imaging and are reported to be related to the leptomen-ingeal angiomatosis. In this article, we compare the ex-tent of perfusion abnormalities in children with SWS against the extent of leptomeningeal disease that is de-tected on postcontrast MRI and with other abnormalities that are shown on MRI. Perfusion status is currently assessed using nuclear medicine studies, such as single photon emission computed tomography (SPECT) or positron emission tomography, which use relatively high doses of ionizing radiation.13–15 It is possible that

MRI may be able to replace these methods.

METHODS

Seven children who were referred for clinical MRI in-vestigations because of high clinical suspicions of SWS were included; all had facial nevi. These were consecu-tive referrals during a 4-year period (from September 2000 to October 2004). The routine MRI assessment of children with SWS includes axial fast spin echo dual echo, sagittal and axial spin echo T1-weighted imaging, diffusion-weighted imaging, and time-of-flight magnetic resonance (MR) venography before the injection of gad-olinium chelate. Dynamic contrast-enhanced perfusion imaging (described below) then is performed, and axial spin echo T1-weighted and fast spin echo T1-weighted FLAIR images are taken on a 1.5-T MR scanner (Infin-ion; Philips Medical Systems, Cleveland, OH). Dedicated orbital imaging was performed when ocular abnormali-ties were suspected on routine imaging. The dynamic bolus MRI perfusion scan used a gradient echo T2*-weighted echo planar imaging sequence with the follow-ing parameters: time to repeat 1400 ms, echo time 60 ms, flip angle 90 degrees, thickness 7 mm, field of view 24 cm, matrix 216 ⫻ 216 (Resolution-Aided Matrix factor 2), echo train length 108, and fat saturation, with images taken every 1.4 seconds after an intravenous bolus of gadolinium with a dose of 0.2 mL/kg body weight given as a 1.0 molar chelate (Gadovist; Schering, Berlin, Germany). The perfusion data were analyzed using the proprietary software making assessments of first mean transit time (FmMTT) and relative cerebral

(3)

blood volume. Perfusion abnormalities were considered to be present when the FmMTT was increased by⬎1.5 seconds in a region when compared with the opposite side or with adjacent brain tissue in cases of bilateral abnormalities.

RESULTS

The 7 children (4 girls, 3 boys) had an age range of 15 years (2 months to 15 years; mean: 5.5; SD: 6.3). All 7 patients had neuroimaging or ocular changes that were consistent with SWS, and the results are summarized in Table 1. One patient did not have intracranial abnormal-ities on MRI (patient 3) but had a diffuse choroidal angioma with choroidal detachment and naevus flam-meus sufficient to confirm the diagnosis of SWS. This patient had no abnormalities on FmMTT or relative ce-rebral blood volume.

Of the 6 children with intracranial disease, 4 had unilateral disease and 2 had bilateral involvement, de-fined by the presence of bilateral leptomeningeal en-hancement (eg, Fig 1). In both of these cases, the bilat-eral nature was not suspected clinically; a small contralateral naevus flammeus was recognized retro-spectively in 1 case, and the cutaneous lesion was truly unilateral in the other. Four patients had venous anom-alies (2 bilateral [eg, Fig 2], 1 of which had unilateral leptomeningeal enhancement only). The venous anom-alies consisted of developmental venous anomanom-alies and a radial transmedullary vein.

The regions of the brain that were affected by lepto-meningeal enhancement showed the most extensive FmMTT changes, and the area of abnormality broadly matched the extent of leptomeningeal enhancement in 5 of 6 cases. There was more extensive change than ex-pected in 1 case (patient 4). However, the areas around

venous anomalies also showed abnormal FmMTT in all cases in which they were present. This was less extensive than seen in the areas of leptomeningeal enhancement. FmMTT changes were seen bilaterally in 1 case, in which there was only unilateral leptomeningeal enhancement (patient 2) and the contralateral FmMTT change was along the course of a developmental venous anomaly. One patient showed increased FmMTT in the cerebellar hemisphere contralateral to the affected hemisphere. This was interpreted as showing crossed cerebellar dias-chisis (patient 6; Fig 3).

DISCUSSION

Most children with SWS have normal neurologic func-tion for several months or even years after birth. Visual, motor, or developmental delay may be the first presen-tation of neurologic dysfunction, usually on 1 side of the body and frequently associated with seizures. The sei-zure activity was previously thought to be the major cause of progressive decline in children with SWS; how-ever, in recent literature, it has been suggested that it is more likely to be attributable to progressive venous isch-emia.

It is likely that the abnormalities that give rise to SWS are produced early in fetal development and account for the high frequency of association between unilateral involvement of facial ectoderm, globe, and occipital cor-tex of the brain. Facial and pial vascular anomalies are thought to result from persistence of primordial sinusoi-dal vascular channels that normally are present only from the fourth to the eighth gestational weeks. During that period, the ectoderm that is destined to become the skin of the upper face and the eye overlies the part of the dorsal neural tube that is destined to form the occipital lobes and adjacent structures of the cerebral

hemi-TABLE 1 Details of the Imaging Findings in Patients With SWS Patient

No.

Age Gender Extent of

Leptomeningeal Disease Cerebral Atrophy Venous Anomalies

Perfusion Abnormality Orbit or

Globe Pathology

1 11 mo Female R F P

L O

R P L O

Not present Matched to leptomeningeal disease No

2 10 y Male R F T P O R P O

L none

R O DVA L O

Matched to leptomeningeal disease and along DVA

R choroidal angioma

3 15 y Female No R none

L none

Not present None R choroidal angioma

with established detachment and phthisis bulbi

4 14 mo Female L T P O L T P O

R none

L RMV Greater than leptomeningeal disease and along RMV

No

5 11 y Male L F T P O

R F

L F T P O R none

R T DVA Matched to leptomeningeal disease and along DVA

No

6 4 mo Female L F T P O L cerebral peduncle

L F T P O R none

L basal ganglia DVA

L hemisphere and R cerebellar hemisphere L buphthalmos and choroidal angioma

7 2 mo Male R P O R P O

L none

Not present Matched to leptomeningeal disease No

(4)

spheres. How the cerebral angioma and facial naevus are formed from the persistence of the primordial structures is not clear, but superficial venous aplasia or early thrombotic occlusion may be the cause.16,17 The

lepto-meningeal angiomatosis probably results from the ab-normal persistence of an embryologic venous plexus as a result of venous blood being redirected through the de-veloping leptomeninges. This mechanism also may ex-plain the range of venous abnormalities seen in SWS (4 of 7 in the present study), such as persistent transmed-ullary radial veins, developmental venous anomalies, or dural fistulas. Other abnormalities consist of enlarge-ment and calcification of the choroid plexus on the same side as the affected hemisphere.18

Venous abnormalities are probably attributable to aplasia of superficial cortical veins or venous obliteration early in the postconception period. The effect of absence of a functional superficial venous system is likely to be progressive, and, as the redirected venous pathways fail, ultimately this causes severe tissue hypoxia by venous hypertension. This is proposed as a possible explanation for the saltatory neurologic decline. Prolonged seizures are thought to cause injury by imposing an increased metabolic demand that cannot be met by the inadequate perfusion as a result of focal venous hypertension, and this can lead to venous infarction. Patients with SWS therefore are particularly susceptible to brain injury as a consequence of status epilepticus and require optimal seizure control.19As the ischemic injury progresses, the

cortex is reduced to a nonfunctioning calcified mantle,

FIGURE 1

Images from an 11-month-old with SWS. A, Axial T2-weighted images showing cerebral atrophy in the right posterior frontal and parietal regions (arrow). B and C, Axial FLAIR images after intravenous gadolinium chelate showing areas of leptomeningeal enhance-ment consistent with leptomeningeal angiomatosis in the right posterior frontal and parietal regions (C; arrow) and left medial parietal region (B; arrowhead). D, Axial T1-weighted image after intravenous gadolinium chelate at the same level as in B showing the reduced conspicuity of the leptomeningeal enhancement as compared with post-contrast T1-weighted FLAIR image (arrowhead). E and F, Axial FmMTT images from a dynamic contrast bolus MR perfusion study that was taken through the cerebrum and shows pronounced perfusion defect in the right parietal and frontal lobes and left occip-ital region areas comparable to the areas of leptomeningeal enhancement (B and C). Perfusion deficit: red, high; green, mid; blue, low.

FIGURE 2

(5)

and the overlying leptomeningeal vessels regress, with capillaries no longer being perfused as a result of throm-bosis; for this reason, many clinicians advocate the use of antiplatelet medication. The leptomeningeal angiomato-sis is thought to result from the diversion of blood from the superficial cortex into the developing meninges in the first trimester.

Many authorities have described low-signal regions on T2-weighted images in parts of the brain that are affected by SWS, not associated with calcification. One possible explanation put forward for this observation is advanced or hypermyelination. This theory is not widely held, and the findings of case 6 in our study do not

support hypermyelination. The child who is presented as case 6 was 5 months of age at the time of the MRI, an age when T1-weighted images can be used to assess myeli-nation accurately. There was no evidence of advanced myelination in the affected area; in fact, myelination was shown to be delayed by the lack of high signal in the affected white matter. This is not surprising because many pathologies that produce hypoxia cause delay in myelination, such as birth asphyxia. In that case, we showed direct evidence of perfusion deficit to the area (Fig 3E), and the low signal on the apparent diffusion coefficient map of the diffusion-weighted imaging is likely to be attributable to high levels of deoxyhemoglo-bin.

There are several descriptions of the brain vasculature from both the older catheter-based literature and the more recent MRI literature.20,21 The arterial circulation

usually is not involved in SWS, but venous problems commonly are encountered. Transmedullary radial veins and developmental venous anomalies are readily visible on MR angiographic studies,22but high-quality catheter

angiography may be required to show fistulas. Delays in hemispheric transit times can be shown using high-temporal-resolution angiographic studies indicative of venous hypertension that is sufficient to reduce cerebral blood flow through the affected area. Various newer MRI techniques also have been used to identify the early venous anomalies, including bold oxygen level-depen-dent MR venography (a high-resolution, 3-dimensional, T2-weighted gradient echo sequence) that can visualize veins of⬍0.5 mm in diameter.23

Brain perfusion has been studied extensively using nuclear medicine scans such as 99m

Tc-hexamethylpro-phylene amine oxime SPECT,13,14,24–27 99m

Tc-pertechne-tate, N-isopropyl-p [123I] iodoamphetamine SPECT,28

and 2-deoxy-2 [18F] fluoro-deoxyglucose positron

emis-sion tomography.15,26,29–31 Perfusion changes that are

shown by these modalities have been reported to be present before the other structural abnormalities are demonstrable by neuroimaging, which suggests that “functional” studies may be more sensitive for early disease.25,26 These perfusion changes also have been

shown to track the neurologic decline.15,32 We did not

have the opportunity to compare our MR perfusion findings with nuclear medicine perfusion studies.

In our study of 7 patients with clinically suspected SWS, we have established that MR perfusion deficits are seen to be matched in relation to the areas of enhance-ment that correspond to leptomeningeal disease. In 1 case (patient 4), the area of perfusion abnormality was seen to be more extensive than the area of enhance-ment, suggesting that there may be early venous throm-bosis that is as yet undetectable by postcontrast imaging. Perfusion deficits, as discussed above, have been dem-onstrated previously to be present earlier than other structural abnormalities. This study also may suggest

FIGURE 3

(6)

that MR perfusion is more sensitive than the conven-tional postcontrast imaging in the detection of pathol-ogy.

There is a previously described case of crossed cere-bellar diaschisis in SWS31secondary to cerebral atrophic

changes. We also present a case of crossed cerebellar diaschisis in which there is a perfusion deficit in the posterior right cerebellar hemisphere (patient 6) second-ary to the extensive left cerebral changes (Fig 3). No changes were identified on conventional T2-weighted imaging; therefore, it is proposed that these changes are as a functional consequence of the supratentorial dis-ease.

There was previously a single case report of MR per-fusion in SWS that showed comparable changes to that shown on the T1 postcontrast scan.33That report and the

cases presented here show the advantages of using an integrated multimodality MR-based approach when im-aging children with SWS. This takes advantage of com-bining “functional studies” directly with high-resolution imaging of the brain and vascular compartments.

In our experience, MR perfusion has been shown to be effective and easy to perform at the same time as the patients’ diagnostic MRI. Our results show that, by and large, there is a good anatomic correlation between the extent of leptomeningeal disease and the extent of per-fusion abnormalities. This is not perfect, and sometimes the lobar extent of perfusion defects is greater than the leptomeningeal enhancement. The more important finding of this study, however, was perfusion defects distant from the leptomeningeal disease both ipsilateral and contralateral. In all of our cases, when this was found, some form of venous anomaly was shown on MRI and venography. What is not known at present is how these distant abnormalities relate to neurologic dys-function or seizure activity, if at all.

CONCLUSIONS

We have used perfusion MR for the radiologic assess-ment of SWS and found perfusion abnormalities in ter-ritories other than that suggested by postcontrast T1-weighted scanning. Demonstration of the full extent of perfusion abnormality is important for patients’ progno-sis and in planning surgery to remove the seizure foci. Perfusion MRI therefore also has a role in surgical man-agement and preoperative counseling. The goal of sur-gery in SWS is to prevent seizure activity or to allow intractable seizures to come under medical control and to protect the normal brain from excitotoxic neural dam-age secondary to seizure activity.34It therefore is

essen-tial to recognize the entirety of the abnormality on pre-operative scanning. There has been concern that postcontrast T1 MRI may not reveal the true extent of the abnormality, and certainly there have been cases of children’s having continued seizures after local resection but being cured after limited removal of tissue along the

edge of their previous resection.35This technique of

per-forming perfusion MR to show areas of perfusion abnor-mality that is remote from the areas of presumed lepto-meningeal angiomatosis therefore is important in the preoperative assessment. Use of this technique is partic-ularly useful because it can be performed at the same time as the MRI study to assess the structural deficit. It does not require an additional appointment or possibly a general anesthetic to obtain the perfusion data.

REFERENCES

1. Griffiths PD. Sturge-Weber syndrome revisited: the role of neuroradiology.Neuropediatrics.1996;27:284 –294

2. Schirmer R. Ein fall von teleangiektasie [An account of telan-giectasia].Graefes Arch Ophthalmol.1860;7:119 –121

3. Sturge WA. A case of partial epilepsy apparently due to a lesion of one of the vasomotor centres of the brain.Trans Clin Soc Lond

1879:162–167

4. Roach ES, Bodensteiner JB. Neurologic manifestations of Sturge-Weber syndrome. In: Bodensteiner JB, Roach ES, eds. Sturge-Weber Syndrome. Mt. Freedom, NJ: Sturge-Weber Foundation; 1999:27–37

5. Pascual-Castroviejo I, Diaz-Gonzalez C, Garcia-Melian RM, Gonzalez-Casado I, Munoz-Hiraldo E. Sturge-Weber syndrome: study of 40 patients.Pediatr Neurol.1993;9:283–288

6. Rochkind S, Hoffman H, Hendrick E. Sturge-Weber syndrome: natural history and prognosis.J Epilepsy.1990;3:293–304 7. Kitahara T, Maki Y. A case of Sturge-Weber disease with

epi-lepsy and intracranial calcification at the neonatal period.Eur Neurol.1978;17:8 –12

8. Wasenko JJ, Rosenbloom SA, Duchesneau PM, Lanzieri CF, Weinstein MA. The Sturge-Weber syndrome: comparison of MR and CT characteristics. AJNR Am J Neuroradiol.1990;11: 131–134

9. Griffiths PD, Coley SC, Romanowski CA, Hodgson T, Wilkinson ID. Contrast-enhanced fluid-attenuated inversion recovery im-aging for leptomeningeal disease in children.AJNR Am J Neu-roradiol.2003;24:719 –723

10. Wohlwill FJ, Yakovlev PI. Histopathology of meningo-facial angiomatosis (Sturge-Weber’s disease); report of four cases.

J Neuropathol Exp Neurol.1957;16:341–364

11. Alexander GL, Norman RM.The Sturge-Weber Syndrome. Bristol, United Kingdom: John Wright & Sons; 1960

12. Shin RK, Moonis G, Imbesi SG. Transient focal leptomeningeal enhancement in Sturge-Weber syndrome. J Neuroimaging.

2002;12:270 –272

13. Bar-Sever Z, Connolly LP, Barnes PD, Treves ST. Technetium-99m-HMPAO SPECT in Sturge-Weber syndrome.J Nucl Med.

1996;37:81– 83

14. Griffiths PD, Boodram MB, Blaser S, Armstrong D, Gilday DL, Harwood-Nash D. 99mTechnetium HMPAO imaging in chil-dren with the Sturge-Weber syndrome: a study of nine cases with CT and MRI correlation.Neuroradiology.1997;39:219 –224 15. Lee JS, Asano E, Muzik O, et al. Sturge-Weber syndrome: correlation between clinical course and FDG PET findings.

Neurology.2001;57:189 –195

16. Smirniotopoulos JG, Murphy FM. The phakomatoses.AJNR Am J Neuroradiol.1992;13:725– 846

17. Barkovich AJ. Phakomatoses. In: Barkovich AJ, ed. Pediatric Neuroimaging. New York, NY: Raven; 1990:123–147

(7)

infarction in Sturge-Weber syndrome.Childs Nerv Syst.1998; 14:693– 696

20. Poser CM, Taveras JM. Cerebral angiography in encephalo-trigeminal angiomatosis.Radiology.1957;68:327–336 21. Bentson JR, Wilson GH, Newton TH. Cerebral venous drainage

pattern of the Sturge-Weber syndrome.Radiology.1971;101: 111–118

22. Vogl TJ, Stemmler J, Bergman C, Pfluger T, Egger E, Lissner J. MR and MR angiography of Sturge-Weber syndrome.AJNR Am J Neuroradiol.1993;14:417– 425

23. Mentzel HJ, Dieckmann A, Fitzek C, Brandl U, Reichenbach JR, Kaiser WA. Early diagnosis of cerebral involvement in Sturge-Weber syndrome using high-resolution BOLD MR venography.Pediatr Radiol.2005;35:85–90

24. Chiron C, Raynaud C, Tzourio N, et al. Regional cerebral blood flow by SPECT imaging in Sturge-Weber disease: an aid for diagnosis.J Neurol Neurosurg Psychiatry.1989;52:1402–1409 25. Pinton F, Chiron C, Enjolras O, Motte J, Syrota A, Dulac O.

Early single photon emission computed tomography in Sturge-Weber syndrome. J Neurol Neurosurg Psychiatry. 1997;63: 616 – 621

26. Reid DE, Maria BL, Drane WE, Quisling RG, Hoang KB. Central nervous system perfusion and metabolism abnormalities in Sturge-Weber syndrome.J Child Neurol.1997;12:218 –222 27. Ton-That QT, Picard D, Bissoon-Doyal D, et al.

Technetium-99m HMPAO imaging in Sturge-Weber syndrome. Clin Nucl Med.1990;15:178 –180

28. Horita H, Nozaki H, Hamano S, Aihara T. Single photon emission computed tomography of the brain in Sturge-Weber syndrome using N-isopropyl-p-[123I] iodoamphetamine: a comparative study with X-ray computed tomography [in Japanese].No To Hattatsu.1990;22:341–348

29. Pfund Z, Kagawa K, Juhasz C, et al. Quantitative analysis of gray- and white-matter volumes and glucose metabolism in Sturge-Weber syndrome.J Child Neurol.2003;18:119 –126 30. Chugani HT, Mazziotta JC, Phelps ME. Sturge-Weber syndrome:

a study of cerebral glucose utilization with positron emission tomography.J Pediatr.1989;114:244 –253

31. Yoshikawa H, Fueki N, Sakuragawa N, Ito M, Iio M. Crossed cerebellar diaschisis in the Sturge-Weber syndrome.Brain Dev.

1990;12:535–537

32. Maria BL, Neufeld JA, Rosainz LC, et al. High prevalence of bihemispheric structural and functional defects in Sturge-Weber syndrome.J Child Neurol.1998;13:595– 605

33. Lin DD, Barker PB, Kraut MA, Comi A. Early characteristics of Sturge-Weber syndrome shown by perfusion MR imaging and proton MR spectroscopic imaging. AJNR Am J Neuroradiol.

2003;24:1912–1915

34. Erba G. Sturge Weber syndrome: natural history and indica-tions for surgery.J Epilepsy.1990;3(suppl):287–291

(8)

DOI: 10.1542/peds.2005-1815

2006;117;2119

Pediatrics

Amlyn L. Evans, Elysa Widjaja, Daniel J. A. Connolly and Paul D. Griffiths

Shown by Dynamic Contrast Bolus Magnetic Resonance Perfusion Imaging

Cerebral Perfusion Abnormalities in Children With Sturge-Weber Syndrome

Services

Updated Information &

http://pediatrics.aappublications.org/content/117/6/2119

including high resolution figures, can be found at:

References

http://pediatrics.aappublications.org/content/117/6/2119#BIBL

This article cites 31 articles, 10 of which you can access for free at:

Subspecialty Collections

http://www.aappublications.org/cgi/collection/radiology_sub

Radiology

sub

http://www.aappublications.org/cgi/collection/hematology:oncology_

Hematology/Oncology

following collection(s):

This article, along with others on similar topics, appears in the

Permissions & Licensing

http://www.aappublications.org/site/misc/Permissions.xhtml

in its entirety can be found online at:

Information about reproducing this article in parts (figures, tables) or

Reprints

http://www.aappublications.org/site/misc/reprints.xhtml

(9)

DOI: 10.1542/peds.2005-1815

2006;117;2119

Pediatrics

Amlyn L. Evans, Elysa Widjaja, Daniel J. A. Connolly and Paul D. Griffiths

Shown by Dynamic Contrast Bolus Magnetic Resonance Perfusion Imaging

Cerebral Perfusion Abnormalities in Children With Sturge-Weber Syndrome

http://pediatrics.aappublications.org/content/117/6/2119

located on the World Wide Web at:

The online version of this article, along with updated information and services, is

by the American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

Figure

TABLE 1Details of the Imaging Findings in Patients With SWS
FIGURE 2
FIGURE 3MRIscansofa4-month-oldinfantwithSWS.AandB,AxialFLAIRimagesafterintravenousgadolinium chelate showing avid leptomeningeal enhancement in the temporal, pari-etal, and occipital regions

References

Related documents

be save in adapted breadth in billow storage.. Here accessible benefactor monitors all

In both cases a transition from the isotropic phase to the nematic was observed as the temperature was reduced (the terms ’isotropic* and ’nematic* used here

The sufficiency results are shown to be sharp and, as a special case, yield a global version of the central limit theorem for independent random variables obeying the

(Siemens, 1996); data reduction: SAINT ; program(s) used to solve structure: SHELXS 97 (Sheldrick, 1997); program(s) used to re®ne structure: SHELXL 97 (Sheldrick, 1997);

The anion contains an approximate mirror plane, which passes through the plane of the dicyanocarbene ligand, the Mo1 atom and the pyrazolyl ring atoms N31, N32 and C33±C35.. We

Solvent THF was removed under vacuum at ambient temperature, and the residue rinsed with pentane (50 ml) and toluene (50 ml), and then redissolved into a small amount of DME

Learning in a healthcare environment using the visualisation case study is consistent with the models of Nonaka and Tecuchi (1995) and Nonaka et al (2000) where learning is regarded

‘As a programmer design on Unity is better’ (Logic of subjectivation/ community spirit Logic of subjectivation/ community spirit Logic of subjectivation/ community spirit Logic