J Neurosurg Pediatrics 11:713–716, 2013
©AANS, 2013

Moyamoya disease with mesial temporal sclerosis
Case report
Subash Lohani, M.D.,1 Joseph R. Madsen, M.D.,1 Ann M. Bergin, M.B., Sc.M., 2
and Edward R. Smith, M.D.1
Departments of 1Neurosurgery and 2Neurology, Boston Children’s Hospital, Boston, Massachusetts
The combination of moyamoya syndrome and symptomatic mesial temporal sclerosis (MTS) has not previously
been reported. The authors present the case of a 5-year-old boy with symptomatic MTS who developed progressive
moyamoya syndrome. This combination of progressive moyamoya and a structural seizure focus presented a unique
clinical problem, with the natural history of MTS predicting a high likelihood of needing resection in the future,
which could be challenging following any type of moyamoya-related revascularization surgery. In anticipation of this
problem, the patient underwent resection of the right inferior and mesial temporal lobe followed by right pial synangiosis as a 1-day combined operation. Postoperatively he recovered well without any neurological deficits and had
an uneventful hospital stay. This case of moyamoya is unique in its association with MTS, and for the simultaneous
operations for pial synangiosis and temporal lobectomy, highlighting the importance of surgical planning in patients
with dual pathological processes.
(http://thejns.org/doi/abs/10.3171/2013.3.PEDS12437)

Key Words • moyamoya
•
seizure disorder •
mesial temporal sclerosis
pial synangiosis
•
temporal lobectomy •
vascular disorders

M

oyamoya is a cerebrovascular condition that pre-

disposes affected patients to stroke in association with progressive stenosis of the intracranial
internal carotid arteries and their proximal branches. Although moyamoya is found in association with numerous
medical conditions (including presenting with seizures),
to date we are unaware of reports linking moyamoya
with MTS.7 The presence of MTS is particularly important because it is a distinct cause of epilepsy known to be
amenable to surgical treatment.2 The natural history of
MTS in children predicts a high likelihood of needing operative intervention at some point to control the seizures.
In addition, there are data to support a role for surgical
revascularization of progressive moyamoya in children
(even if they are asymptomatic).6 Coexistence of these 2
distinct pathologies is both interesting and has implications for planning long-term treatment strategies.

Case Report
History and Examination. This patient was born at
full term, without complications. Seizures started at 3
months of age with infantile spasms involving bilateral
arm and leg extension, and evolved over the subsequent
Abbreviations used in this paper: EEG = electroencephalography;
MTS = mesial temporal sclerosis.

J Neurosurg: Pediatrics / Volume 11 / June 2013

•

year to clusters of arm and shoulder stiffening with eye
squeezing. He was evaluated with EEG, which revealed
hypsarrhythmia without focality; and MRI, which revealed a small right temporal arachnoid cyst (2 × 1.5 cm),
a possible right medial temporal cortical dysplasia, and
right MTS (Fig. 1). Of interest, no arteriopathy was noted
at 2 years of age. Initial attempts to treat seizures with
adrenocorticotropic hormone, pyridoxine, Klonopin, and
Topamax were unsuccessful, but ultimately seizure control was obtained with Keppra and zonisamide. Because
of improved medical control of his seizures, a planned
right temporal lobectomy was deferred. At the age of 5
years, he began to manifest new symptoms, described as
brief episodes (seconds to minutes in length) of speech
arrest, left-sided weakness, and lethargy. Repeat imaging revealed a right-sided arteriopathy consistent with
moyamoya. An arteriogram was done that demonstrated
Suzuki Stage III moyamoya on the right side (Fig. 1).
His family history was unremarkable for moyamoya or
other neurovascular disease. Low-dose (41.5 mg) aspirin
therapy was started at that time and he was referred for
potential surgical treatment.
Repeat video-EEG telemetry performed at the age of
5 years captured 18 seizures on EEG studies after antiThis article contains some figures that are displayed in color
on­line but in black-and-white in the print edition.

713

S. Lohani et al.

Fig. 1. Preoperative images. A: Axial T2-weighted MR image revealing a right middle cranial fossa arachnoid cyst. B:
Coronal FLAIR MR image revealing mild asymmetrical increased signal of the medial aspect of the right medial temporal lobe,
representing focal cortical dysplasia. C: Coronal T2-weighted MR image showing atrophic changes of the right hippocampus
with associated ex vacuo dilation of the right lateral ventricle, compatible with MTS. D: Axial FLAIR image demonstrating slow
flow in the right temporoparietal region represented by bright signal in the sulci (ivy sign). E: Right internal carotid artery injection (anteroposterior view) arteriogram showing severe right-sided stenoocclusive changes particularly involving the right M1 with
corresponding perforating vessel proliferation consistent with moyamoya disease, Suzuki Stage III.

convulsant medication withdrawal. Seizures arose in the
right midposterior temporal region, and some were associated with subtle behavioral arrest with low-amplitude,
high-frequency body jerks. These were identified by the
parent as his typical seizures. In many electroencephalographic events, no clinical correlate was appreciated.

Surgical Decision Making. At the time of referral to
neurosurgery for evaluation of his moyamoya, the child
had been followed up for his epilepsy. A multidisciplinary
discussion was undertaken, involving the epilepsy neurologists, neurosurgeons, and neurovascular team. It was
decided that revascularization was needed for the progressive moyamoya, and debate centered on the risks and
benefits of temporal lobectomy during the same operation. Under consideration was the likelihood of needing
surgical treatment of his MTS later in life (despite good
current control of his seizures), given the difficulty in performing a lobectomy in the setting of established surgical
collateral vessels versus the added risk of doing a combined surgery in a single sitting. It was decided to proceed
with a combined operation in the same sitting, including
both mesial temporal lobectomy and pial synangiosis.
714

Operation and Postoperative Course. The patient was
taken to the operating room for a single-stage operation:
right frontotemporal craniotomy, corticography with selective resection of the mesial temporal lobe, and pial
synangiosis. (As part of the approach, the small arachnoid
cyst was fenestrated.) The sequence of the operation was
1) dissection of the superficial temporal artery; 2) opening a wide frontotemporal craniotomy, with emphasis on
exposing the anterior temporal region; 3) mesial temporal
lobectomy (with intraoperative corticography and fenestration of the arachnoid cyst); and 4) pial synangiosis with
wide opening of the arachnoid and placement of the pial
sutures (Fig. 2).
Intraoperative EEG studies demonstrated symmetrical, continuous activity, mostly sharply contoured highvoltage (60–70 mV) beta with admixed theta and delta
activity. There were no clear epileptiform discharges and
no electroencephalographic seizures were seen. No significant electroencephalographic slowing was observed during the case. Intraoperative corticography recording was
performed in the right temporal region. Recordings made
prior to resection showed spikes arising from the mesial
J Neurosurg: Pediatrics / Volume 11 / June 2013

Moyamoya disease with mesial temporal sclerosis

Fig. 2. Intraoperative photographs and schematic drawings. A: Patient positioned supine with his right side up and with
EEG leads attached for intraoperative monitoring, allowing exposure of the operative field. B: Right superficial temporal artery
dissected in preparation of pial synangiosis. C: Right frontotemporal craniotomy is done and the dura mater opened, exposing
the right frontal and temporal lobes. D: Postresection photograph of right temporal lobe structures and arachnoid cyst. E:
Schematic representations of the key steps in pial synangiosis.

temporal structures. Postresection corticography showed
resolution of these spikes.
Postoperatively the patient developed no new neurological deficits. His hospital course was uneventful, and
he was discharged home on postoperative Day 5. He had
no further seizures. A 6-month postoperative MRI study
revealed excellent collateral development and no new
strokes, along with reversal of the previous “ivy sign”—a
linear, high signal that follows the sulci, resulting from
diminished cortical blood flow as seen in FLAIR sequences on MRI.7

Pathological Findings. Cerebral cortex and subcortical white matter demonstrated mild gliosis and focal
neuronal loss. The hippocampus specimen had neuronal
depletion of pyramidal cells and moderate gliosis consistent with MTS. There were some SM131-labeled neurons,
raising the possibility of mild heterotopic change. Fragments of arachnoid and dense fibroconnective tissue were
noted in the arachnoid cyst specimen.

Discussion

This case highlights 2 interesting issues. First is the
association of moyamoya syndrome with MTS. Given
that moyamoya and MTS (and also in this case an arachnoid cyst) may lead to seizures, this case demonstrates
the need to pay careful attention to the clinical presentation.2,3,7,9 This child presented initially with infantile
spasms. Later, seizure semiology changed and clinical
evaluation led to the discovery of the new condition of
moyamoya. This underscores the importance of a careful
and continued evaluation, especially if there is an altered
presentation, because there is always the possibility of a
missed diagnosis influencing the clinical manifestation,
even in patients with an apparently established diagnosis.
J Neurosurg: Pediatrics / Volume 11 / June 2013

The second issue is the challenge faced in surgical
treatment of these 2 conditions. There is abundant evidence supporting the role for surgical revascularization of
both symptomatic and asymptomatic moyamoya in children with operations such as pial synangiosis.5–8 Regarding the MTS, the American Academy of Neurology practice parameter recommends surgery as the treatment of
choice for medically intractable temporal lobe epilepsy.4
The patient’s seizures in this case were controlled with
medication at the time of diagnosis of moyamoya, but the
natural history of MTS suggests that a minority of children will continue to attain seizure control on medication
alone, whereas nearly two-thirds of children can experience long-term seizure control after selective temporal
lobectomy (as studied in a Class I randomized, controlled
trial).1,3,4,10 If the child needed a temporal lobectomy at a
point in time after surgical revascularization for his moyamoya, there would be increased risk of encountering
scar tissue and, more importantly, potentially interrupting
critical surgical collateral vessels. Given these data, and
with documentation that typical seizures arose in the right
temporal lobe, the decision was made to offer a selective
temporal lobectomy at the same time as the pial synangiosis, as described in this report. The case highlights the
need for careful intradisciplinary discussion and preoperative planning.
The combined approach was successful, but several
risks were reviewed with the treating physicians and family. The extensive intraparenchymal dissection required
for the selective lobectomy could have resulted in disruption of existing collateral vessels associated with the moyamoya, or it could have increased the risk of hemorrhage
perioperatively, given the use of low-dose aspirin in the
first few days after surgery. It was unclear if the greater
extent of dissection and length of operating time would
715

S. Lohani et al.
increase the risk of perioperative stroke or seizure in the
setting of the 2 conditions. Last, the additional communication of CSF spaces resulting from the fenestration
of the arachnoid cyst, larger craniotomy, and dissection
inherent to the lobectomy could potentially increase the
risk of CSF leakage postoperatively.
Fortunately, the case was uncomplicated and the
child was discharged after a 5-day hospital stay (including a preoperative admission for planned intravenous hydration). He has done well, with no further seizures and
a 6-month postoperative MRI sequence revealing good
collateral development and no new strokes, along with reversal of the previous ivy sign (indicating improved blood
flow after pial synangiosis).
This case is unique because this is the first one to our
knowledge in which moyamoya was found in association
with MTS. It also elucidates the importance of surgical
planning in similar situations with dual pathological processes. Although this case centers on epilepsy, this type
of team-based strategy and anticipation of future patient
surgical needs may be similarly relevant in the setting of
other conditions, such as tumors, vascular lesions (arteriovenous malformation, aneurysm, and so on), or shunts.
We recognize that this is not a technically novel case, but
rather hope that its illustration may offer an example of a
potential surgical strategy that has been used successfully
in a patient with moyamoya and an associated colocalized
surgical lesion.

Conclusions

This case demonstrates the coexistence of MTS and
newly diagnosed moyamoya—with progression—in 1 patient. The finding of dual intracranial pathological processes in patients with moyamoya mandates careful consideration of potential future surgical needs, to properly
anticipate treatment strategies. Resection of an intraparenchymal lesion can be performed successfully along with
a pial synangiosis during a single induction of anesthesia
when the surgical teams coordinate their approaches; such
an example is demonstrated in this patient.
Disclosure
The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this
paper.
Author contributions to the study and manuscript preparation
in­clude the following. Conception and design: Smith. Acquisition
of da­ta: Lohani. Analysis and interpretation of data: Lohani. Drafting

716

the article: Lohani. Critically revising the article: all authors. Re­­
viewed submitted version of manuscript: all authors. Approved the
final version of the manuscript on behalf of all authors: Lohani.
Ad­min­­i­strative/technical/material support: Madsen, Smith. Study
su­per­vision: Smith.
References
1. Al-Holou WN, Yew AY, Boomsaad ZE, Garton HJL, Muraszko KM, Maher CO: Prevalence and natural history of arachnoid cysts in children. Clinical article. J Neurosurg Pediatr
5:578–585, 2010
2. Cersósimo R, Flesler S, Bartuluchi M, Soprano AM, Pomata
H, Caraballo R: Mesial temporal lobe epilepsy with hippocampal sclerosis: study of 42 children. Seizure 20:131–137,
2011
3. Chern JJ, Patel AJ, Jea A, Curry DJ, Comair YG: Surgical
outcome for focal cortical dysplasia: an analysis of recent surgical series. A review. J Neurosurg Pediatr 6:452–458, 2010
4. Engel J Jr, Wiebe S, French J, Sperling M, Williamson P,
Spencer D, et al: Practice parameter: temporal lobe and localized neocortical resections for epilepsy: report of the Quality
Standards Subcommittee of the American Academy of Neurology, in association with the American Epilepsy Society and
the American Association of Neurological Surgeons. Neurology 60:538–547, 2003
5. Fung LW, Thompson D, Ganesan V: Revascularisation surgery for paediatric moyamoya: a review of the literature.
Childs Nerv Syst 21:358–364, 2005
6. Lin N, Baird L, Koss M, Kopecky KE, Gone E, Ullrich NJ, et
al: Discovery of asymptomatic moyamoya arteriopathy in pe­
diatric syndromic populations: radiographic and clinical progression. Neurosurg Focus 31(6):E6, 2011
7. Scott RM, Smith ER: Moyamoya disease and moyamoya syndrome. N Engl J Med 360:1226–1237, 2009
8. Scott RM, Smith JL, Robertson RL, Madsen JR, Soriano SG,
Rockoff MA: Long-term outcome in children with moyamoya
syndrome after cranial revascularization by pial synangiosis.
J Neurosurg 100 (2 Suppl Pediatrics):142–149, 2004
9. Westermaier T, Schweitzer T, Ernestus RI: Arachnoid cysts.
Adv Exp Med Biol 724:37–50, 2012
10. Wieser HG: ILAE Commission Report. Mesial temporal lobe
epilepsy with hippocampal sclerosis. Epilepsia 45:695–714,
2004
Manuscript submitted August 27, 2012.
Accepted March 5, 2013.
Please include this information when citing this paper: published
online April 5, 2013; DOI: 10.3171/2013.3.PEDS12437.
Address correspondence to: Subash Lohani, M.D., Department of
Neurosurgery, Boston Children’s Hospital, 300 Longwood Avenue,
Hunnewell-2, Boston, Massachusetts 02115. email: subash.lohani
@childrens.harvard.edu.

J Neurosurg: Pediatrics / Volume 11 / June 2013