Case Report

Restrictive cerebral cortical venopathy:
A new clinicopathological entity

Interventional Neuroradiology
0(00) 1–8
! The Author(s) 2019
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DOI: 10.1177/1591019918821861
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Zoya A Voronovich1 , Kathy Wolfe2, Kimberly Foster1, Danielle Sorte1,3
and Andrew P Carlson1

Abstract
We present a case of a novel restrictive cerebral venopathy in a child, consisting of a bilateral network of small to medium
cortical veins without evidence of arteriovenous shunting, absence of the deep venous system, venous ischemia, elevated
intracranial pressure, and intracranial calcifications. The condition is unlike other diseases characterized by networks of
small veins, including cerebral proliferative angiopathy, Sturge-Weber syndrome, or developmental venous anomaly. While
this case may be the result of an anatomic variation leading to the congenital absence of or early occlusion of the deep
venous system, the insidious nature over many years argues against this. The absence of large cortical veins suggests a
congenital abnormality of the venous structure. The child’s presentation with a seizure-like event followed by protracted
hemiparesis is consistent with venous ischemia. We propose that this is likely to represent a new clinicopathological entity.

Keywords
9p21.1 deletion, cerebral proliferative angiopathy, cortical veins, deep venous system, developmental venous anomaly,
intracranial calcifications, intracranial hypertension, LINGO2, stroke-like episode, venous ischemia, Sturge-Weber syndrome
Received 21 September 2018; accepted 20 November 2018

Introduction
Few pediatric diseases of the cerebral venous system
have been identified. These conditions, such as developmental venous anomalies (DVAs) and venous malformations, are described as ‘‘rarely pathologic’’ and
‘‘as a rule, should not be treated.’’1 We present a previously unreported case of a congenital cerebral venopathy characterized by a network of small to medium
veins, causing restricted venous drainage and ischemia.
We argue that this abnormal venous anatomy ultimately leads to profound brain injury, as evidenced by
intracranial calcifications (ICCs) and cerebral atrophy.
Resulting intracranial hypertension was managed with
a ventriculoperitoneal shunt.

Case report
History and presentation
A 16-year-old male with cerebral palsy, well-controlled
focal epilepsy and cognitive impairment presented to
our hospital after sustaining a seizure-like event, with
subsequent persistent left-sided hemiplegia and obtundation of two days’ duration. The child was obtunded
on initial examination, and was withdrawing from central stimulus, with stronger movement of the right

extremities than the left. The child had a history of
seizures, which were predominantly characterized as
focal to bilateral tonic-clonic. He was previously treated with levetiracetam, but the treatment was discontinued after he was seizure free for several years. He
was born at full term via cesarean section and required
a two-month stay in the neonatal intensive care unit
because of respiratory problems, without cardiopulmonary resuscitation. He required surgery for persistent ductus arteriosus, bilateral orchiopexy, and
strabismus. He also had two instances of intussusception that did not require surgical intervention.
Imaging from 12 years ago demonstrated intraparenchymal calcifications and unspecified cerebral venous
anomalies. Testing for TORCH infections was
1

Department of Neurosurgery, University of New Mexico Health Sciences
Center, Albuquerque, USA
2
Department of Neurology, University of New Mexico Health Sciences
Center, Albuquerque, USA
3
Department of Radiology, University of New Mexico Health Sciences Center,
Albuquerque, USA
Corresponding author:
Andrew P Carlson, Department of Neurosurgery, University of New Mexico
Health Sciences Center, MSC 10-5615, 2211 Lomas Blvd NE, Albuquerque,
NM 87131, USA.
Email: AndrewCarlson@salud.unm.edu

2
reportedly negative. There was a question of the patient
having an arteriovenous malformation, and he had at
least one diagnostic cerebral catheter angiogram that
identified venous anomalies, thought to represent
changes related to deep venous occlusion.
Family history is significant for a brother with febrile seizures and hearing deficits. No other history of
seizures, aneurysms, or connective tissue disease was
reported.

Imaging and diagnostic findings
Head computed tomography (CT) demonstrated extensive bilateral intraparenchymal calcifications involving
the cerebral, periventricular, and cerebellar regions,
and associated with volume loss. The bilateral frontoparietal, parietal, and occipital calcifications appeared
to be in a gyral distribution, although the frontal calcifications also had a white-matter component. The cerebellar calcifications were also bilateral and involved the
dentate nuclei (see Figure 1). The calcifications
appeared unchanged when compared to imaging from
13 years prior.
Magnetic resonance imaging (MRI) of the brain
demonstrated extensive restricted diffusion of the
right hemisphere subcortical white matter and extensive
abnormal flow voids in the subarachnoid space (see
Figure 2). T1 signal of the right hemisphere was isointense as compared to the left hemisphere, while T2
signal was slightly increased in the right hemisphere.
The flow voids were similar in appearance to those
demonstrated on MRI of the brain obtained 13 years
prior. However, the findings of diffusion restriction
were not seen on previous studies, and are consistent
with the patient’s new deficits. T2 optic nerve sheath
diameters were 6.2 mm in the right eye and 7.3 mm in

Interventional Neuroradiology 0(00)
the left eye, measuring above the 5.83 mm cutoff for
elevated intracranial pressure (ICP).2
A digital subtraction angiogram (DSA) was performed to better characterize the vascular abnormalities. No abnormalities were seen in the arterial or
capillary phases. However, the venous phase was profoundly abnormal bilaterally, characterized by an
extensive network of small to medium veins that
drained very slowly via extremely tortuous networks
into the superior sagittal sinus. There was a paucity
of large cortical veins. The transverse sinuses, the sigmoid sinuses, and the internal jugular veins were
patent. There was no filling of the deep venous system
or drainage into the vein of Galen and the straight sinus
(see Figure 3). The absence of the deep venous system
and the abundance of small cortical veins were noted
on the child’s DSAs from nine and 13 years prior to
presentation, and appeared similar to the angiogram
performed at presentation. No MR venogram was performed to our knowledge.
The angiographic findings raised the question of
cerebral venous congestion leading to elevated ICP.
Although an ophthalmologic examination did not demonstrate papilledema, a lumbar puncture performed
under sedation demonstrated an opening pressure of
30 cm H2O. A right parietal ventriculoperitoneal
shunt was placed for management of intracranial
hypertension.
The child’s neurologic examination improved during
the hospitalization, with increased wakefulness and
improving left-sided strength. At two-month followup, the patient returned to his neurologic baseline.
MRI obtained four months after presentation demonstrated resolution of diffusion restriction, decreased
optic nerve sheath diameter bilaterally, and decreased
ventricular size.

Figure 1. Head computed tomography (CT) without contrast demonstrated scattered extensive bilateral intraparenchymal calcifications
with diffuse volume loss. (a) The frontoparietal, parietal, and occipital calcifications appeared to be in a gyral distribution. (b) The
periventricular frontal calcifications appeared somewhat more pronounced when compared to the head CT from 12 years prior. (c) The
bilateral cerebellar calcifications involve the dentate nuclei.

Voronovich et al.

3

Figure 2. Magnetic resonance imaging (MRI) of the brain (1.5 Tesla) with and without contrast demonstrated extensive abnormal flow
voids in the subarachnoid spaces, with predominance at the left Sylvian fissure and along the left anterior tentorium. The flow voids are
diffuse as demonstrated by their presence (a) in the basal cisterns and (b) over the convexities. (c) The B1000 sequence demonstrated
extensive diffusion restriction of the subcortical white matter throughout the majority of the right frontal, parietal, temporal, and occipital
lobes, without corresponding T2 fluid-attenuated inversion recovery abnormality (not shown). (d) MRI brain B1000 sequence from nine
years prior to presentation did not demonstrate the pattern of diffusion restriction observed in the MRI at presentation.

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Figure 3. (a and b) The venous phase of the right internal carotid artery injection demonstrated an extensive network of very small veins,
draining slowly into extremely tortuous medium-sized veins, and then into the superior sagittal sinus and the transverse sinuses. There
was no filling of the deep venous system into the vein of Galen and the straight sinus. The torcula was high-riding. The sigmoid sinuses
and the internal jugular veins were patent bilaterally. There was a delay in opacification due to the extremely delayed outflow from the
cortical vessels. (b) and (c) The venous phase of the left internal carotid artery injection and of the right vertebral artery injection (d and e)
demonstrated similar findings.

Voronovich et al.
Genetic testing demonstrated 178 kb deletion of
9p21.1, including the LINGO2 gene.

5
It seems unlikely, therefore, that this is simply a reactive
process to occlusion or to the absence of the deep
venous system.

Discussion
We describe a case of a previously unreported restrictive cerebral venopathy in a 16-year-old male with seizure disorder and developmental delay, consisting of a
diffuse network of small to medium cortical veins without evidence of arteriovenous shunting, and the
absence of the deep venous system. These findings
were associated with ICCs, elevated ICP, and a
stroke-like episode. Our literature review did not yield
any other cases of diffuse networks of small cortical
veins without associated arteriovenous shunting.

Venous anatomy
The venous drainage pattern of the adult supratentorial
structures is described as having a superficial system
and a deep system. The superficial veins lie on the surface of the cerebral cortex, and drain the outer 1 to 2 cm
of cortex and white matter via intracortical and subcortical veins in a centrifugal direction. The superficial
medullary veins drain the deeper or gyral white
matter. The pial veins collect blood from intracortical,
subcortical, and superficial medullary veins, and coalesce into the larger cortical veins, which then continue
toward their respective dural sinuses.3 The superficial
veins ultimately drain to the superior sagittal sinus, the
sphenoparietal sinus and the cavernous sinus, the inferior sagittal sinus and the vein of Galen, and the tributaries of the sinuses related to the tentorium cerebelli.4
The deep venous system drains centripetally from the
more central white matter and paraventricular nuclei,
such as the internal cerebral veins, the basal veins of
Rosenthal, and their tributaries, including those draining the basal surface of the brain. The deep white
matter first drains to the deep medullary veins, which
converge into distinct zones as they transverse the white
matter to connect with the subependymal veins. The
superficial and the deep venous systems do interconnect: The anastomotic medullary veins connect the
superficial and the deep medullary veins, and the transcerebral veins interconnect the pial veins and the subependymal veins.3 Compared to the usual configuration
of the venous system, there is an absence of the basal
veins of Rosenthal and the internal cerebral veins in
this case. Additionally, the cortical veins of the superficial system do not appear to coalesce into larger cortical veins, and there is no clearly defined vein of Labbé,
vein of Trollard, or superficial Sylvian (middle cerebral)
vein. The extensive network of small veins may represent the dilation of the anastomotic medullary veins
and the transcerebral veins to support the drainage of
the deep white matter in the absence of prominent
internal cerebral veins and basal veins of Rosenthal.
However, most of these vessels seem to be in the subarachnoid space rather than the deep white matter.

Cerebral venous development
Developmental events in the late embryologic and early
fetal stages may be at the root of the observed abnormalities. Around week 6 of development, extrinsic diffusion can no longer sustain the neural tissue. The
intrinsic vessels of the brain develop centripetally, originating from the initial surface capillary meshwork
and extending to the metabolically active germinal
zone, where they form arteriovenous loops. Thus, the
true transcerebral veins are the first to develop.
Raybaud5 infers that the subependymal veins are the
next to develop, followed by the cortical-subcortical
veins. He additionally notes that there is no literature
describing how the vasculature of the subependymal
collectors is formed from the vasculature of the germinal zone, nor how these collectors connect to the
dorsal dural veins. Of note, the shift of dorsal venous
drainage from a purely choroid one into the vein of
Markowski to a common choroid and parenchymal
drainage (including the tributaries of the subependymal
veins) through the vein of Galen occurs around week
11. Given that the deep white matter and periventricular structures appear grossly normal in our patient, the
intrinsic venous structures can be inferred to be sufficient for drainage. However, the absence of the vein of
Galen and no notable presence of a persistent vein of
Markowski (i.e. a vein of Galen malformation) are suggestive of a failure of the normal development of dorsal
venous drainage at this stage.

Other entities with abnormal networks of
small veins
The findings of our case are fundamentally different
from those of other entities that involve abnormal networks of small veins, including cerebral proliferative
angiopathy, DVAs, and Sturge-Weber syndrome
(SWS). Cerebral proliferative angiopathy (CPA), as
described by Lasjaunias et al., differs from our case in
that CPA is characterized by ‘‘the small caliber of a
multitude of feeding arteries and draining veins,’’6
while our case involves abnormalities of the venous
phase only and does not have arteriovenous shunting.
More recent reports have also identified arteriovenous
fistula, flow-related aneurysm,7 and intracranial hemorrhage7,8 in CPA, neither of which were observed in our
patient. Leptomeningeal angiomatosis of SWS may
have a similar radiographic appearance to our case.9
Our patient also shares some clinical features with
SWS, including ICCs likely associated with venous congestion,10 a stroke-like episode,11 and the dilation of
small veins.12 Poor drainage of the deep venous
system has also been identified in SWS,13 but dilation
of the deep venous system is more frequently cited.14

6
Ultimately, our patient does not have leptomeningeal
angiomatosis, and does not carry the somatic mutation
in the GNAQ gene, located on 9q21.2, that appears to
be the major genetic factor in SWS.15 Interestingly,
Lasjaunias et al. presented the findings of transcortical
bilateral collateral circulation involving striate veins
bilaterally and the transcallosal vein on the right side
in a 19-month-old-boy presenting with a seizure, persistent hemiparesis, but without port-wine stain.
Although this case demonstrated an extensive bilateral
network of small veins, a few large cortical veins were
also visualized, and the deep venous system was not
only present but appeared to provide a significant
route of cerebral drainage. Cortical vein occlusion without venous sinus impairment was suggested as the
underlying mechanism. The case was thought to be
consistent with the spectrum of cerebrofacial venous
metameric syndrome (CVMS), a term that appears to
have fallen out of favor relative to SWS type III that
accounts for 10% of the cases without cutaneous findings.10 DVAs are characterized by a network of
enlarged subependymal or subpial venous channels of
the caput medusa formation, extending toward one or
more enlarged transependymal veins acting as collectors. The absence or disruption of either the deep or
the superficial venous drainage system has been suggested as the cause of the compensatory development
of the transparenchymal medullary venous drainage
pathway of the DVA.16 The absence of the deep
venous system, and the enlarged venous structures on
the cortical surface are seen in our patient. However,
the angiogram did not demonstrate the coalesced transparenchymal collectors characteristic of DVAs, and our
patient’s angiographic findings are diffuse and involve
both hemispheres.

Intracranial hypertension
We propose that our patient’s abnormal cortical
venous architecture cannot support a normal rate of
venous outflow, thus leading to an imbalance relative
to the arterial inflow, and resulting in increased ICP, as
described by Wilson.17 Interestingly, the appearance of
the small tortuous veins and slow venous drainage we
observed was quite similar to the severe pseudopheibitic
pattern first noted in dural arteriovenous fistulas in
association with venous congestion and intracranial
hypertension.18 Bilateral optic nerve sheath dilation
further supported the concern for intracranial hypertension, which was confirmed with a lumbar puncture.
Lack of papilledema on the ophthalmologic examination can be seen in the setting of acute rise of ICP,19
and does not negate concerns for intracranial hypertension in our patient. Cerebrospinal fluid diversion was
pursued to prevent the sequelae of long-term elevated
ICP, and to lower ICP in hopes of decreasing the size of
the venous infarct, as suggested by recent animal studies.20 Furthermore, there is a lack of techniques to
address small-vein congestion directly, and the child’s

Interventional Neuroradiology 0(00)
venous sinuses were widely patent, with no evidence of
stenosis to consider stenting or open techniques, such as
sino-jugular bypass.21 Although his clinical condition
began to improve even prior to shunting, the resolution
of ischemia without progression to infarct may have
been helped by the decrease in ICP, as evident by the
reduction in the optic nerve sheath diameters on followup MRI.

Venous ischemia and the stroke-like episode
The MRI findings of diffusion restriction, without associated fluid-attenuated inversion recovery change, are
suggestive of cytotoxic edema as would be seen in
early stages of ischemia.22 Our patient’s recovery of
function, the resolution of diffusion restriction, and
the lack of encephalomalacia on follow-up are consistent with a venous etiology of ischemia. The ischemic
penumbra and the number of cells that are only functionally impaired are thought to be larger in venous
stroke as compared to arterial stroke, which explains
the relatively indolent course and good clinical recovery
that can be seen in venous stroke.23 The event may have
been triggered by the seizures sustained by the patient,
as these would lead to hyperperfusion in the ictal-onset
zone,24 and thus exacerbate the venous congestion. The
absence of recent seizures as a trigger may explain why
the patient has not had a prior episode of hemiparesis.
On the other hand, another physiologic event, such as
dehydration and/or fever related to the patient’s recent
illness, may have led to worsened venous congestion
and/or tolerance thereof, resulting both in venous ischemia and seizures. Of note, similar episodes of protracted—but ultimately recovered—hemiparesis are
also reported in SWS as ‘‘stroke-like episodes,’’ and
were similarly attributed to venous congestion and
ischemia.25,26 Interestingly, findings of venous wall
thickening and ‘‘sluggish flow’’ have also been correlated to the brain lesions related to the stroke-like episodes of the very different disorder of mitochondrial
encephalopathy with lactic acidosis and stroke-like
episodes.27

Calcifications
Intracranial calcifications (ICCs) observed in our
patient may be the result of cell damage from prior
insults, or from deposition of calcium in the vessels.
ICCs found in patients younger than 20 years old and
located outside the choroid plexus and the pineal gland
are pathologic. Although specific associations, such as
those seen in Aicardi-Goutiéres syndrome and in postinfectious etiologies, are recognized,28 ICCs are usually
an ‘‘epiphenomenon of low diagnostic specificity.’’29
Vascular pathology is typically thought to result in dystrophic ICCs, where damaged cell membranes allow
excess calcium influx.30,31 However, histologic analysis
of ICCs in SWS revealed them to be of the vascular
type secondary to ‘‘calcium incrustation’’ of the

Voronovich et al.

7

vessels,30 rather than due to the traditionally proposed
cell damage due to ‘‘chronic hypoxic state caused by
inadequate venous drainage and venous hypertension’’32 that we also would have expected to see in
our patient. There were no notable changes in our
patient’s ICCs over the years preceding his presentation, or on the follow-up MRI SWI sequence, within
the limitations of the shunt valve artifact. The lack of
recent changes is unsurprising given the reversal of the
ischemia as described above. Similar to observations
made in SWS, our patient’s ICCs may contribute to
his epilepsy and cognitive delay.33

Conclusion
We present a case of a novel cerebral venopathy in a
child, characterized by a diffuse network of small cortical veins without evidence of arteriovenous shunting,
absence of the deep venous system, ICCs, elevated ICP,
and a stroke-like episode. The condition is unlike other
diseases characterized by networks of small veins,
including CPA, SWS, or DVA. Our case is most similar
to a case of CVMS briefly described by Lasjaunias et al.,
yet is still more extreme because of the absence of large
cortical veins. The development of subependymal veins
and their connection to the vein of Galen may be the key
to understanding this malformation, but these are
unfortunately poorly understood. The case is ultimately
illustrative of acute and chronic consequences of venous
congestion, such as ischemia and calcifications. We look
forward to learning of similar cases in hopes of gaining a
better understanding of this condition.
Acknowledgment
We thank Dr Adnan Siddiqui for valuable discussion of
this case.

Declaration of conflicting interests
The authors declared no potential conflicts of interest with
respect to the research, authorship, and/or publication of this
article.

Funding
The authors received no financial support for the research,
authorship, and/or publication of this article.

ORCID iD
Zoya A Voronovich

http://orcid.org/0000-0001-5322-5316

References
1. Smith ER and Scott RM. Cavernous and venous malformations. In: Albright AL, Pollack IF and Adelson PD
(eds) Principles and practice of pediatric neurosurgery.
New York: Thieme, 2014, pp.858–865.
2. Geeraerts T, Newcombe VF, Coles JP, et al. Use of
T2-weighted magnetic resonance imaging of the optic
nerve sheath to detect raised intracranial pressure. Crit
Care 2008; 12: R114.

3. Pearl M, Gregg L and Gandhi D. Cerebral venous development in relation to developmental venous anomalies
and vein of Galen aneurysmal malformations. Semin
Ultrasound CT MRI 2011; 32: 252–263.
4. Morris P. The venous system. In: Practical neuroangiography. Philadelphia: Lippincott Williams & Wilkins,
2007, pp.271–288.
5. Raybaud C. Normal and abnormal embryology and
development of the intracranial vascular system.
Neurosurg Clin N Am 2010; 21: 399–426.
6. Lasjaunias PL, Landrieu P, Rodesch G, et al. Cerebral
proliferative angiopathy: Clinical and angiographic
description of an entity different from cerebral AVMs.
Stroke 2008; 39: 878–885.
7. Maekawa H, Terada A, Ishiguro T, et al. Recurrent periventricular hemorrhage in cerebral proliferative angiopathy: Case report. Interv Neuroradiol 2018; 24: 713–717.
8. Giragani S, Pavunesan SK and Balasubramaniam A.
Targeted endovascular treatment of haemorrhagic posterior fossa proliferative angiopathy. Interv Neuroradiol
2018; 24: 440–443.
9. Pinto AL, Chen L, Friedman R, et al. Sturge-Weber syndrome: Brain magnetic resonance imaging and neuropathology findings. Pediatr Neurol 2016; 58: 25–30.
10. Lasjaunias PL, ter Brugge KG, Berenstein A. SturgeWeber syndrome. In: Surgical neuro-angiography.
Volume 3. Clinical and interventional aspects in children.
Berlin: Springer, 2006, pp.455–507.
11. Lo W, Marchuk DA, Ball KL, et al. Updates and future
horizons on the understanding, diagnosis, and treatment
of Sturge-Weber syndrome brain involvement. Dev Med
Child Neurol 2012; 54: 214–223.
12. Mentzel HJ, Dieckmann A, Fitzek C, et al. Early diagnosis of cerebral involvement in Sturge-Weber syndrome
using high-resolution BOLD MR venography. Pediatr
Radiol 2005; 35: 85–90.
13. Hamano K, Ito M, Inai K, et al. A case of Sturge-Weber
syndrome with peculiar venous abnormalities. Childs
Nerv Syst 1993; 9: 491–493.
14. Comi AM. Presentation, diagnosis, pathophysiology, and
treatment of the neurological features of Sturge-Weber
syndrome. Neurologist 2011; 17: 179–184.
15. Shirley MD, Tang H, Gallione CJ, et al. Sturge-Weber
syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med 2013; 368: 1971–1979.
16. Linscott LL, Leach JL, Jones BV, et al. Developmental
venous anomalies of the brain in children—imaging spectrum and update. Pediatr Radiol 2016; 46: 394–406.
17. Wilson MH. Monro-Kellie 2.0: The dynamic vascular
and venous pathophysiological components of intracranial pressure. J Cereb Blood Flow Metab 2016; 36:
1338–1350.
18. Willinsky RA, Goyal M, TerBrugge K, et al. Tortuous,
engorged pial veins in intracranial dural arteriovenous
fistulas: Correlations with presentation, location, and
MR findings in 122 patients. Am J Neuroradiol 1999;
20: 1031–1036.
19. Hayreh SS. Pathogenesis of optic disc edema in raised
intracranial pressure. Prog Retin Eye Res 2016; 50:
108–144.
20. Wang W, Lin C, Hong J, et al. Effects of increased intracranial pressure gradient on cerebral venous infarction in
rabbits. World Neurosurg 2018; 120: e161–e168.

8
21. Sindou MP, Auque J and Jouanneau E. Neurosurgery
and the intracranial venous system. Acta Neurochir
Suppl 2005; 94: 167–175.
22. Ho ML, Rojas R and Eisenberg RL. Cerebral edema.
AJR Am J Roentgenol 2012; 199: W258–W273.
23. Valdueza JM, Schreiber SJ, Röhl JE, et al. Pathogenesis
of stroke. In: Neurosonology and neuroimaging of stroke:
A comprehensive reference. 2nd ed. New York: Thieme,
2017, pp.90–105.
24. Van Paesschen W. Ictal SPECT. Epilepsia 2004;
45(Suppl 4): 35–40.
25. De la Torre AJ, Luat AF, Juhász C, et al. A multidisciplinary consensus for clinical care and research needs for
Sturge-Weber syndrome. Pediatr Neurol 2018; 84: 11–20.
26. Kumar KR, Hon K, Schultz D, et al. Transient changes
on brain magnetic resonance imaging in a patient with
Sturge-Weber syndrome presenting with hemiparesis.
Neurologist 2009; 15: 351–354.
27. Whitehead MT, Wien M, Lee B, et al. Cortical venous disease severity in MELAS syndrome correlates with brain
lesion development. Neuroradiology 2017; 59: 813–818.

Interventional Neuroradiology 0(00)
28. Livingston JH, Stivaros S, Warren D, et al. Intracranial
calcification in childhood: A review of aetiologies and
recognizable phenotypes. Dev Med Child Neurol 2014;
56: 612–626.
29. Livingston JH, Stivaros S, van Der Knaap MS, et al.
Recognizable phenotypes associated with intracranial calcification. Dev Med Child Neurol 2013; 55: 46–57.
30. McCartney E and Squier W. Patterns and pathways of
calcification in the developing brain. Dev Med Child
Neurol 2014; 56: 1009–1015.
31. Bano D and Nicotera P. Ca2þ signals and neuronal
death in brain ischemia. Stroke 2007; 38(2 Suppl):
674–676.
32. Wu J, Tarabishy B, Hu J, et al. Cortical calcification in
Sturge-Weber syndrome on MRI-SWI: Relation to brain
perfusion status and seizure severity. J Magn Reson
Imaging 2011; 34: 791–798.
33. Pilli VK, Behen ME, Hu J, et al. Clinical and metabolic correlates of cerebral calcifications in SturgeWeber syndrome. Dev Med Child Neurol 2017; 59:
952–958.