Pediatrics and Neonatology (2018) 59, 573e580

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Original Article

Schizencephaly in children: A single medical

center retrospective study
Po-Cheng Hung a,*, Huei-Shyong Wang a, Ming-Liang Chou a,
Kuang-Lin Lin a, Meng-Ying Hsieh a, I-Jun Chou a,
Alex M-C. Wong b

a
Division of Pediatric Neurology, Chang Gung Children’s Hospital, Chang Gung Memorial Hospital,
Chang Gung University, College of Medicine, Taoyuan, Taiwan
b
Division of Neuroradiology, Chang Gung Children’s Hospital, Chang Gung Memorial Hospital, Chang
Gung University, College of Medicine, Taoyuan, Taiwan

Received Mar 31, 2017; received in revised form Oct 29, 2017; accepted Jan 2, 2018
Available online 6 January 2018

Key words Abstract Background: The aim of this study was to evaluate the clinical, and neuroimaging
associated; features, outcomes, and other associated systemic disorders in children with schizencephaly
cerebral; at a single medical center in Taiwan.
congenital; Methods: We retrospectively reviewed the medical records and magnetic resonance images
disorders (MRI) of children with schizencephaly between January 2000 and December 2014. The MRI find-
ings of schizencephaly were recorded along with the presence of associated cerebral disorders.
Clinical, electroencephalographic and additional systemic disorders were also recorded.
Results: A total of 21 patients (13 males and 8 females) were included in the study. According
to the location of schizencephaly, the patients were classified into two groups: unilateral
(n Z 16) and bilateral (n Z 5). The majority of the patients with neurological deficits were
detected before 1 year of age, especially in bilateral clefts. The most common initial presen-
tation was hemiparesis in unilateral schizencephaly, and seizures in bilateral schizencephalies.
Ventriculomegaly was the most common associated cerebral disorder, and the most common
additional systemic disorders included congenital heart disease, hydronephrosis, and
strabismus. Seventeen patients suffered from epileptic seizures with generalized
tonic-clonic seizures being the most common. Eight patients developed refractory epilepsy.
The majority of the patients had motor deficits, intellectual disabilities, and language deficits,
especially in bilateral clefts.
Conclusions: This study demonstrates that the clinical features of schizencephaly vary widely,
with their severity closely related to the cleft. Determining the type, size, and extent of
schizencephaly is useful to plan management and predict the prognosis.

* Corresponding author. Division of Pediatric Neurology, Chang Gung Children’s Hospital, Chang Gung University, College of Medicine, 5,
Fu-Hsing St., Kwei-Shan, Taoyuan, 333 Taiwan. Fax: þ886 3 3288957.
E-mail address: h2918@cgmh.org.tw (P.-C. Hung).

https://doi.org/10.1016/j.pedneo.2018.01.009
1875-9572/Copyright ª 2018, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. This is an open access article under the CC BY-
NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
574 P.-C. Hung et al

Copyright ª 2018, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. This is an
open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/
by-nc-nd/4.0/).

1. Introduction of Infant Development-third edition, Wechsler Preschool

Schizencephaly is an uncommon congenital central nervous
system malformation characterized by the presence of a
cleft spanning the cerebral hemisphere from the pial
surface to the lateral ventricle lined with heterotopic gray
matter. The defect is a cell migration defect which de-
velops between two and five months of gestation.1 It is
usually found during the evaluation of children or young
adults with epilepsy, neurodevelopmental abnormalities, or
an incidental finding. The pathogenesis of schizencephaly is
not fully understood, but it may be due to an antenatal
environmental incident or of genetic origin.2,3 Schizence-
phaly may occur in isolation, in association with other
developmental anomalies of the brain, or as part of a
multiple congenital anomaly syndrome.4
Although this malformation was first described by
Wilmarth in 1887,5 the term was introduced in 1946 by
Yakovlev and Waldsworth,6,7 who described two types of
schizencephaly: type I (closed-lip), a fused cleft that pre-
vented the passage of cerebrospinal fluid, and type II
(open-lip), a cleft of irregular width permitting cerebro-
spinal fluid to pass between the ventricular cavity and
subarachnoid space. Schizencephaly can occur unilaterally
or bilaterally in any hemispheric region. Bilateral clefts are
usually symmetric in location, but do not necessarily have
the same size. A previous report in Taiwan reported that
unilateral and open-lip schizencephaly were most common,
with cortical dysplasia being the most common associated
cerebral malformation, and that bilateral clefts and
intractable seizures resulted in the worst outcomes.8
However, few studies of childhood schizencephaly have
been undertaken in Taiwan. The purpose of this study was
to evaluate the clinical and neuroimaging features of
schizencephaly over a 15-year period at a single medical
center in Taiwan, and to compare the findings with previous
studies.
and Primary Scale of Intelligence-third edition, or the
Wechsler Intelligence Scale for Children-fourth edition. In
addition, the presence of seizures was recorded, including
seizures types, location of epileptic discharges on electro-
encephalography (EEG), and response to antiepileptic
medications. EEG was performed in each patient using the
international 10e20 system. A diagnosis of schizencephaly
was confirmed with brain magnetic resonance images (MRI).
Brain MRI studies included T1-weighted, T2-weighted, fluid-
attenuated inversion recovery, diffusion-weighted and
post-contrast T1 imaging. The hard copies of brain MRI of all
patients were reviewed by an experienced pediatric
neuroradiologist (AMC W). MRI morphologic findings of
schizencephaly were recorded along with the presence and
type of associated cerebral disorders. Schizencephaly was
classified as unilateral and bilateral closed-lip or open-lip
clefts. Open-lip clefts were defined as small, medium or
large according to Barkovich and Kjos’ classification.4 Full
systemic and neurologic examinations were performed
during visits to our pediatric neurology clinic.
3. Results
During the study period, we enrolled 21 patients with
schizencephaly diagnosed from MRI. The ages at diagnosis
ranged from 1 month to 18 years with a mean age of 3 years

2. Materials and methods

The medical records of patients with schizencephaly diag-

nosed at Chang Gung Memorial Hospital, a medical center
in Taiwan, between January 2000 and December 2014 were
retrospectively reviewed. This study was approved by our
Institutional Review Board. The following information was
collected: initial presentation, intellectual evaluation
based on intelligence quotient (intellectual disability was
defined as mild: 55e70, moderate: 40e55, and severe:
25e40 according to the Diagnostic and Statistical Manual of
Mental Disorders (DSM-IV-TR)), motor deficits (presence or Figure 1 Unilateral schizencephaly in case 8; axial T1-
absence, areas of body involved), language development, weighted magnetic resonance image of a 1 year-old-boy
and associated cerebral and additional systemic disorders. showing closed-lip schizencephaly (white arrow) in the right
Intelligence quotient was assessed using the Bayley Scales frontal lobe.
 Schizencephaly in children
Table 1 Unilateral schizencephaly.
Patient/ Location, type (size) Initial presentation Seizures/type EEG findings Outcome
sex/age
1/M/1 m Right, Frontal-Parietal- Seizures Yes/epileptic spasms Diffuse cortical dysfunction MD (left HP), ID (moderate),
Temporal, Open-lip (LC) RS (2), language deficit
2/M/4 m Right, Frontal, Closed-lip Seizures Yes/epileptic spasms Bilateral multifocal epileptiform ID (mild), RS (3), language deficit
3/F/4 m Left, Occipital, Closed-lip Seizures Yes/generalized tonic-clonic Diffuse cortical dysfunction, Normal
bilateral multifocal epileptiform
4/F/6 m Right, Frontal, Open-lip (SC) Developmental delay Yes/generalized tonic Right cortical dysfunction, MD (left HP), ID (mild), RS (4),
right multifocal epileptiform language deficit
5/M/6 m Right, Parietal, Closed-lip Left limb weakness No N/A MD (left HP), ID (mild)
6/M/8 m Left, Frontal, Open-lip (LC) Right limb weakness Yes/complex partial Bilateral multifocal epileptiform MD (right HP), ID (mild),
language deficit
7/M/11 m Right, Parietal, Closed-lip Developmental delay Yes/complex partial Bilateral multifocal epileptiform ID (mild)
8/M/1 y Right, Frontal, Closed-lip Left limb weakness No Normal MD (left HP), ID (mild),
language deficit
9/F/1 y Right, Frontal, Open-lip (MC) Left limb weakness Yes/complex partial Right focal epileptiform MD (left HP), ID (mild),
RS (3), language deficit
10/M/1 y Left, Frontal, Open-lip (SC) Right limb weakness Yes/simple partial Left cortical dysfunction MD (right HP), language deficit
11/M/1y1m Left, Frontal, Open-lip (MC) Right limb weakness Yes/generalized tonic-clonic Left cortical dysfunction, MD (right HP), ID (mild),
right focal epileptiform language deficit
12/M/1y3m Right, Frontal, Closed-lip Left limb weakness Yes/generalized tonic-clonic Right focal epileptiform MD (left HP), language deficit
13/F/2y1m Right, Frontal, Closed-lip Left limb weakness No N/A MD (left HP), language deficit
14/M/18 y Left, Frontal, Closed-lip Seizures Yes/generalized tonic-clonic Normal IQ (N/A)
15/M/18 y Left, Frontal, Open-lip (SC) Seizures Yes/generalized tonic-clonic Diffuse cortical dysfunction, IQ (N/A)
right focal epileptiform
16/F/18 y Right, Frontal, Open-lip (SC) Headache No N/A IQ (N/A)
HP: hemiparesis, ID: intellectual disability, IQ: intelligence quotient, LC: large cleft, MC: medium cleft, MD: motor deficit, N/A: not available, RS (number of anti-epileptic drugs):
refractory seizures, SC: small cleft.

575
576 P.-C. Hung et al

2 months. The follow-up period ranged from 1 year 11
months to 14 years 4 months with a mean of 6 years 6
months. Thirteen patients were males and eight were fe-
males. Seventeen patients (81%) had epileptic seizures,
eight of whom developed refractory epilepsy. The patients
were classified into two groups, those with unilateral and
those with bilateral schizencephalies.
3.1. Unilateral schizencephaly

(Fig. 1) This group included 16 patients (11 males and five

Figure 2 Bilateral schizencephaly in case 4; coronal T2-
weighted magnetic resonance image of a 5-month-old boy
showing a larger open-lip fronto-temporal schizencephaly in
the right (black arrow) and a smaller open-lip cleft in the left
fronto-temporal lobes (black arrow head). There was no
septum pellucidum.
females) aged 1 month to 18 years, with a mean age of 4
years 1 month. The follow-up period ranged from 1 year 11
months to 14 years 4 months with a mean of 7 years 4
months. Details of the schizencephaly category, initial
presentation, clinical seizures types, EEG findings and
outcomes of these patients are shown in Table 1. Associ-
ated cerebral and additional systemic disorders of these
patients are shown in Figs. 2 and 3. Neurological deficits
were detected in 10 patients (62%) before one year of age.
The most common initial presentation at evaluation was
hemiparesis (eight cases), followed by seizures (five cases).
Most of the patients (10/16) had hemiparesis, which was
contralateral to the cleft in all cases. Language deficit was
found in 10 patients including seven in the right hemisphere
and three in the left hemisphere. Intellectual disability was
found in nine cases. Twelve patients (nine males and three
females), including seven with open-lip and five with
closed-lip defects experienced epileptic seizures, with
generalized tonic-clonic seizures in five, complex partial
seizures in three, epileptic spasms in two, and one case
each of generalized tonic seizures and simple partial sei-
zures. Four patients, including three with open-lip and one
with closed-lip schizencephaly, were refractory to anti-
epileptic drugs. EEG abnormalities were found in 11 pa-
tients and epileptiform abnormalities were noted in nine
patients, all of whom had focal epileptiform activity.
Abnormal background activity was found in six patients.
The cleft was open-lip in eight cases (four small, two me-
dium, and two large) and closed-lip in eight cases. The cleft

14

12

10
Number of Cases

6 Bilateral
Unilateral
4

Associated Cerebral Disorders

Figure 3 Associated cerebral disorders in schizencephaly. AC: arachnoid cyst, ASP: absence of septum pellucidum, AVM: arte-
riovenous malformation, CD: cortical dysplasia, DCC: dysplasia of corpus callosum, HE: heterotopia, HY: hydranencephaly, OND:
optic nerve dysplasia, DWC: Dandy-Walker cyst, P: pachygyria, PMG: polymicrogyria, PVL: periventricular leukomalacia, VM:
ventriculomegaly.
Schizencephaly in children
involved the right or left hemispheres in 10 and six cases
respectively. The frontal lobe was the most common loca-
tion in 13 cases. When the frontal lobe was involved, a
motor deficit was usually present (10/13). All 16 patients
had associated cerebral disorders, with ventriculomegaly
found in eight and an absence of septum pellucidum found
in six. Additional systemic disorders were noted in three
patients with two having strabismus and one having both
congenital heart disease and hydronephrosis.
3.2. Bilateral schizencephalies
(Fig. 4)This group included five patients (three females and
two males) aged one month to one year, with a mean age of
4.8 months. The follow-up period ranged from 2 year 5
months to 4 years 10 months, with a mean of 3 years 8
months. Details of the schizencephaly category, initial
presentation, clinical seizures types, EEG findings, and
outcomes of these patients are shown in Table 2. Associ-
ated cerebral and additional systemic disorders of these
patients are shown in Figs. 2 and 3. Neurological deficits
were detected in all patients before one year of age. The
most common initial presentation at evaluation was sei-
zures in four, followed by developmental delay in two. Two
cases had two initial presentations at evaluation. All of the
patients had language deficits. Four patients had quad-
riparesis, and three had intellectual disabilities. All five
patients experienced epileptic seizures including three
with epileptic spasms and two with generalized tonic-clonic
seizures. Four patients, including three with bilateral open-
lip and one with bilateral closed-lip schizencephaly, were
refractory to anti-epileptic drugs. EEG abnormalities were
found in all five patients. Epileptiform abnormalities were
found in three patients, all of whom had bilateral focal
epileptiform activity. Abnormal background activity was
noted in four patients. The cleft was bilateral open-lip in
four cases and bilateral closed-lip in one case. The frontal
lobe was the most common location. All five patients had
associated cerebral disorders with ventriculomegaly, with
2.5
2
Number of Cases
1.5
1
0.5
0
S CHD
Additional Systemic Disorders
Figure 4 Additional systemic disorders in schizencephaly. CHD: congenital heart disease, DM: diabetes mellitus, GERD: gastro-
esophageal reflex disease, H: hydronephrosis, S: strabismus.
577
an absence of septum pellucidum, dysplasia of corpus cal-
losum, and cortical dysplasia each found in two. Additional
systemic disorders were noted in three patients, with one
having both congenital heart disease and hydronephrosis,
one having gastroesophageal reflex disease, and one having
diabetes mellitus.
4. Discussion
Schizencephaly is an extremely rare central nervous system
developmental disorder, with a reported prevalence at birth
of 0.54e1.54 per 100,000 live births in the United States,
European countries, and Japan.9e11 MRI classification of
schizencephaly into unilateral and bilateral is considered to
be useful because patients in these two groups have
different clinicoradiologic presentations.1e4 Although
several studies have reported that unilateral schizencephaly
is slightly more common than bilateral clefts,8,12 other
studies have reported that bilateral schizencephalies are
slightly more common or as common as unilateral cleft.1,2 In
our case series, unilateral schizencephaly was more com-
mon than bilateral schizencephalies (16:5). In addition,
previous studies have reported that bilateral open-lip (60%)
is more common in bilateral schizencephalies, and that
open-lip (60%) is more common in unilateral cleft.1,8 In our
case series, bilateral open-lip (80%) occurred more
commonly in bilateral clefts, whereas open-lip and closed-
lip occurred equally as commonly in unilateral cleft.
The clinical features of schizencephaly are extremely
variable. Denis et al. reported that the most frequent initial
presentation at evaluation was an asymmetrical muscle
tone in unilateral cleft, and developmental delay when
both hemispheres were involved.2 However, we found that
hemiparesis was the most common presentation in unilat-
eral cleft with seizures in bilateral clefts. In addition,
neurological deficits were detected in most of our patients
during the first year, especially in bilateral clefts. These
findings are in agreement with other studies.2,4 The bilat-
erality of the cleft has been reported to be a major
Bilateral
Unilateral
H GERD DM
578 P.-C. Hung et al

prognostic factor.4,8,13 Previous studies have reported that

ID: intellectual disability, IQ: intelligence quotient, LC: large cleft, MD: motor deficit, N/A: not available, QP: quadriparesis, RS (number of anti-epileptic drugs): refractory seizures, SC:
MD (QP), ID (severe), RS (2),
patients with unilateral schizencephaly present with hem-

MD (QP), IQ (N/A), RS (3),

iparesis and mild mental deficits, and that patients with

MD (QP), ID (moderate),

MD (QP), ID (moderate),
RS (3), language deficit
bilateral clefts present with quadriparesis and severe
mental deficits,2,4,14 which is consistent with our results. In

RS (2), IQ (N/A),
language deficit addition, cleft type, size, and location have also been re-

language deficit

language deficit

language deficit
ported to be important prognostic factors.4,12,13,19 Barko-
vich and Kjos reported that 84% of their cases with frontal
Outcome

lobe involvement had a motor deficit.4 In our case series, a

motor deficit was noted in 15 of 18 patients (including 10/
13 with a unilateral cleft and 5/5 with bilateral clefts) when
the clefts were localized in the frontal lobe. In addition,
Left cortical dysfunction,

previous studies have reported language deficits in 17e40%

multifocal epileptiform
dysfunction, bilateral
of patients with right-sided clefts and in 50e60% of patients
Bilateral multifocal

bilateral multifocal

with left-sided clefts.2 The disorder has also been reported

to be more severe if the cleft is open and involves the
Diffuse cortical

Diffuse cortical

Diffuse cortical

dominant hemisphere.13 In our cases series, a language

epileptiform

epileptiform
dysfunction,
EEG findings

dysfunction

deficit was seen in 70% of the patients with a unilateral

cleft involving the right hemisphere, including three with
open-lip and four with closed-lip. Moreover, 50% of the
cases with a cleft located on the left side had a language
deficit, of whom all had open-lip. Since our patients were
Yes/epileptic spasms

Yes/epileptic spasms

Yes/epileptic spasms

all derived from the same institution, further collaborative

studies are needed to investigate the difference. We also
Yes/generalized

Yes/generalized
Seizures/type

found that all of the patients with language deficits had

tonic-clonic

tonic-clonic

frontal lobe involvement.

Epileptic seizures have been reported in patients with
schizencephaly.1e4,12,14 Denis and colleagues reported
epileptic seizures in 37% of the children in their study (64%
with unilateral cleft and 36% with bilateral clefts).2 Lopes
Initial presentation

et al. also reported epileptic seizures in patients with both

unilateral and bilateral clefts, presenting in 63% patients
Developmental

Developmental
delay, seizures
Macrocephaly,

with unilateral cleft and 55% of those with bilateral

clefts.14 Twelve of our patients (75%) with unilateral cleft
Seizures

Seizures
seizures

and five (100%) with bilateral clefts had epileptic seizures.

delay

Although epileptic seizures have been reported to occur

more frequently in patients with unilateral cleft,2,14
epileptic seizures occurred more frequently in the pa-
Frontal, Open-lip (SC)

Frontal, Open-lip (SC)

tients with bilateral clefts in our case series. This differ-

Frontal, Closed-lip

ence may be due to the low numbers of patients with

Frontal- Parietal,

Frontal- Parietal-
Left hemisphere

bilateral clefts in the current study, and thus larger cohort

Open-lip (LC)

Open-lip (LC)

studies are required to validate our findings. In previous

Temporal,

reports, epileptic seizures have been reported to begin

earlier and to have worse outcome in patients with bilateral
clefts,2,14 as in our case series. Previous studies have re-
ported no correlation between the type of schizencephaly
and the type of epileptic seizures,2,14 which is consistent
Frontal- Parietal, Open-lip (LC)

with our results. In addition, various types of epileptic

Frontal- Parietal- Temporal,
Bilateral schizencephalies.

seizures have been reported, with partial seizures being the

most common.2,14 Among our population, we also found
Frontal, Open-lip (SC)

Frontal, Open-lip (SC)

different types of epileptic seizures, with generalized

Frontal, Closed-lip
Right hemisphere

tonic-clonic seizures being the most common (in five pa-

tients with unilateral cleft and two with bilateral clefts).
Open-lip (LC)

Eight patients, including four with unilateral cleft (three

open-lip and one closed-lip) and four with bilateral clefts
(three bilateral open-lips and one bilateral closed-lips)
were refractory to anti-epileptic drugs. We also found
that the average age at onset of epileptic seizures was
small cleft.

lower in the patients with bilateral schizencephalies, which

2/M/2 m

4/M/5 m
1/F/1 m

3/F/4 m
Patient/
sex/age
Table 2

5/F/1 y

is consistent with previous studies.2,14,15 The most common

EEG abnormality was focal epileptiform discharge in our
series, which is also similar to other studies.2,16
Schizencephaly in children
Many associated cerebral and additional systemic dis-
orders have been reported in patients with schizence-
phaly.2,4,12,17 In the current study, associated cerebral
disorders were detected in all of the patients including
those with unilateral and bilateral clefts, with ven-
triculomegaly being the most common in 13 patients (eight
with unilateral and five with bilateral clefts), followed by
the absence of septum pellucidum in eight patients (six
with unilateral and two with bilateral clefts). The absence
of septum pellucidum has been reported to range from 43
to 75% of patients with schizencephaly.2,18 In our case se-
ries, eight (38%) had an absence of septum pellucidum. In
the literature, the absence of septum pellucidum has been
associated with optic nerve dysplasia, corresponding to
septo-optical dysplasia, in 1e9% of patients.4,19 Two of our
cases (9.5%) had septo-optic dysplasia, including one with
unilateral cleft and one with bilateral clefts. Dysplasia of
corpus callosum has been reported in 30e64% of patients in
the literature2,19,20; however, only four (19%) of our pa-
tients had dysplasia of corpus callosum, including two with
unilateral and two with bilateral clefts. Arachnoid cysts
have also been reported to be commonly associated with
schizencephaly.1,16,19,21 In our case series, two patients
with unilateral schizencephaly had arachnoid cysts. Sener
suggested that the mechanism causing schizencephaly may
also lead to a traction effect and splitting of the lep-
tomeninges, resulting in the formation of an arachnoid cyst
adjacent to the schizencephalic cleft.21 In our case series,
additional systemic disorders were found in six patients
(three with unilateral and three with bilateral clefts)
including congenital heart disease, hydronephrosis, and
strabismus each found in two patients. Regarding the
location of the cleft, the frontal lobe has been suggested to
be the most common location2,4,12 which is consistent with
our case series.
There are several limitations to the present study. The
study population was relatively small and the data were
obtained retrospectively. In addition, our patients were
from a single medical center in Taiwan, and generalizing
the results to the wider Taiwanese population should be
made with caution. Moreover, genetic testing was not
performed in our cohort; therefore, it was difficult to
interpret changes in underlying phenotypic distribution and
heterogeneous etiologies by comparing our results with
those of other case series in which mutation were detec-
ted. Further prospective, collaborative, and larger cohort
studies using genetic testing are warranted.
5. Conclusions
Our results showed that the majority of patients with
neurological deficits were detected before one year of
age, especially those with bilateral clefts. The most
common initial presentation of the patients with unilat-
eral schizencephaly was hemiparesis, compared to sei-
zures in the patients with bilateral schizencephalies. The
majority of patients had motor deficits, intellectual dis-
abilities, and language deficits, especially in those with
bilateral clefts. Among the patients who developed epi-
lepsy, those with bilateral clefts had a lower age at onset
of epileptic seizures, which were also more frequent and
579
intractable. The most common associated cerebral disor-
der was ventriculomegaly, and congenital heart disease,
hydronephrosis, and strabismus were the most common
additional systemic disorders.
Conflict of interest
The authors declare no potential conflicts of interest with
respect to research, authorship, and/or publication of this
article.
Acknowledgments
The authors thank Ms. Vickie Yu Hsin Hung for English
editing of manuscript and Ms. Yu Hsuan Hung for designing
the tables and figures.
References
1. Halabuda A, Klasa L, Kwiatkowski S, Wyrobek L, Milczarek O,
Gergont A. Schizencephaly e diagnostic and clinical dilemmas.
Childs Nerv Syst 2015;31:551e6.
2. Denis D, Chateil JF, Brun M, Brissaud O, Lacombe D, Fontan D,
et al. Schizencephaly: clinical and imaging features in 30 in-
fantile cases. Brain Dev 2000;22:475e83.
3. Verrotti A, Spalice A, Ursitti F, Papetti L, Mariani R,
Castronovo A, et al. New trends in neuronal migration disor-
ders. Eur J Paediatr Neurol 2010;14:1e12.
4. Barkovich AJ, Kjos BO. Schizencephaly: correlation of clinical
findings with MR characteristics. AJNR Am J Neuroradiol 1992;
13:85e94.
5. Wilmarth WA. Presentation to the Philadelphia neurological
society. J Nerv Ment Dis 1887;14:395e407.
6. Yakovlev PI, Wadsworth RC. Schizencephalies; a study of the
congenital clefts in the cerebral mantle; clefts with fused lips.
J Neuropathol Exp Neurol 1946;5:116e30.
7. Yakovlev PI, Wadsworth RC. Schizencephalies; a study of the
congenital clefts in the cerebral mantle; clefts with hydro-
cephalus and lips separated. J Neuropathol Exp Neurol 1946;5:
169e206.
8. Liang JS, Lee WT, Peng SS, Yu TW, Shen YZ. Schizencephaly:
correlation between clinical and neuroimaging features. Acta
Paediatr Taiwan 2002;43:208e13.
9. Curry CJ, Lammer EJ, Nelson V, Shaw GM. Schizencephaly:
heterogeneous etiologies in a population of 4 million California
births. Am J Med Genet A 2005;137:181e9.
10. Szabó N, Gyurgyinka G, Kóbor J, Bereg E, Túri S, Sztriha L.
Epidemiology and clinical spectrum of schizencephaly in south-
eastern Hungary. J Child Neurol 2010;25:1335e9.
11. Hino-Fukuyo N, Togashi N, Takahashi R, Saito J, Inui T, Endo W,
et al. Neuroepidemiology of porencephaly, schizencephaly, and
hydranencephaly in Miyagi Prefecture, Japan. Pediatr Neurol
2016;54:39e42.
12. Granata T, Freri E, Caccia C, Setola V, Taroni F, Battaglia G.
Schizencephaly: clinical spectrum, epilepsy, and pathogenesis.
J Child Neurol 2005;20:313e8.
13. Aniskiewicz AS, Frumkin NL, Brady DE, Moore JB, Pera A.
Magnetic resonance imaging and neurobehavioral correlates in
schizencephaly. Arch Neurol 1990;47:911e6.
14. Lopes CF, Cendes F, Piovesana AM, Torres F, Lopes-Cendes I,
Montenegro MA, et al. Epileptic Features of patients with
unilateral and bilateral schizencephaly. J Child Neurol 2006;
21:757e60.
580
15. Guerrini R, Carrozzo R. Epileptogenic brain malformations:
clinical presentation, malformative patterns and indications
for genetic testing. Seizure 2001;10:532e47.
16. Granata T, Battaglia G, D’Incerti L, Franceschetti S,
Spreafico R, Battino D, et al. Schizencephaly: neurora-
diologic and epileptologic findings. Epilepsia 1996;37:
1185e93.
17. Hayashi N, Tsutsumi Y, Barkovich AJ. Morphological features
and associated anomalies of schizencephaly in the clinical
population: detailed analysis of MR images. Neuroradiology
2002;44:418e27.
P.-C. Hung et al
18. Barkovich AJ, Norman D. Absence of the septum pellucidum: a
useful sign in the diagnosis of congenital brain malformations.
AJR Am J Roentgenol 1989;152:353e60.
19. Packard AM, Miller VS, Delgado MR. Schizencephaly: correlations
of clinical and radiologic features. Neurology 1997;48:1427e34.
20. Barkovich AJ, Norman D. Anomalies of the corpus callosum:
correlations with further anomalies of the brain. AJNR Am J
Neuroradiol 1988;9:493e501.
21. Sener RN. Coexistence of schizencephaly and middle cranial
fossa arachnoid cyst: a report of two patients. Eur Radiol 1997;
7:409e11.