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Pediatric Dermatology Vol. 29 No.

6 738748, 2012

A Significant Proportion of Children with


Morphea En Coup De Sabre and Parry-Romberg
Syndrome Have Neuroimaging Findings
Yvonne E. Chiu, M.D.,* Sheetal Vora, M.D., Eun-Kyung M. Kwon, B.A.,*
and Mohit Maheshwari, M.D.
*Division of Pediatric Dermatology, Department of Dermatology, Medical College of Wisconsin, Milwaukee,
Wisconsin, Division of Rheumatology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee,
Wisconsin, Division of Pediatric Radiology, Department of Radiology, Medical College of Wisconsin, Milwaukee,
Wisconsin

Abstract: En coup de sabre (ECDS) and Parry-Romberg syndrome


(PRS) are variants of linear morphea on the head and neck that can be
associated with neurologic manifestations. Intracranial abnormalities on
computed tomography (CT) and magnetic resonance imaging (MRI) are
present in a significant proportion of individuals with these conditions.
We describe 32 children from our institution with ECDS or PRS;
neuroimaging was performed in 21 cases. We also review 51 additional
cases from the literature. Nineteen percent of the children at our
institution with ECDS or PRS had intracranial abnormalities on MRI, half
of whom were asymptomatic. Hyperintensities on T2-weighted sequences
were the most common finding, present in all children with intracranial
abnormalities on MRI. Seizures (13%) and headaches (9%) were the most
common neurologic symptom. Neurologic symptoms were not correlated
with neuroimaging abnormalities, with two asymptomatic children having
marked MRI findings and only two of nine symptomatic children having an
abnormal MRI. Similarly the severity of the superficial disease did not
predict neurologic involvement; a child with subtle skin involvement had
striking MRI findings and seizures, whereas another with a bony defect
had no brain parenchymal involvement. Neurologic symptoms and
neuroimaging abnormalities are found in a surprisingly substantial
percentage of children with ECDS and PRS. Early recognition of neuro-
logic involvement is necessary because it affects treatment choices.
Because clinical predictors of intracranial abnormalities are poor, strong
consideration should be given to obtaining an MRI before treatment
initiation to assist in management decisions and establish a baseline
examination.

Address correspondence to Yvonne E. Chiu, M.D., Department


of Dermatology, Medical College of Wisconsin, 8701 Watertown
Plank Rd., Milwaukee, WI 53226, or e-mail: ychiu@mcw.edu.

DOI: 10.1111/pde.12001

738 2012 Wiley Periodicals, Inc.


Chiu et al: Neuroimaging of Morphea En Coup De Sabre 739

Morphea, also known as localized scleroderma, is pediatric cases of ECDS and PRS in which neuro-
a rare disease seen in adults and children. Most imaging was performed. Search terms used were
children have the linear subtype, which can extend localized scleroderma, morphea, scleroderma
deeply into the subcutaneous tissue, muscle, and en coup de sabre, Parry Romberg, facial hemi-
bone. Linear morphea on the head and neck, called en atrophy, progressive facial hemiatroph*, pro-
coup de sabre (ECDS), and Parry-Romberg syn- gressive hemifacial atroph*, neuro*, X-ray
drome (PRS), also called progressive hemifacial computed tomography, CT scan, magnetic reso-
atrophy, are felt to be related variants within the nance imaging, magnetic resonance spectroscopy,
morphea spectrum of disease (1). ECDS and PRS MRI, neuroimag*, and tomography. Reports
may be associated with cerebral inammation and were excluded if the publication was not written in
neurologic abnormalities. A variety of neurologic English, the imaging was performed when the subject
symptoms have been reported, most commonly sei- was age 18 or older, or there was insucient detail
zures and headaches (2). Computed tomography (CT) about the imaging results. The publications were
and magnetic resonance imaging (MRI) can reveal reviewed to collect details about cutaneous manifes-
calvarial and intracranial abnormalities, even in tations, associated neurologic symptoms, and CT and
asymptomatic individuals (3). The use of neuroimag- MRI results.
ing in evaluating ECDS and PRS is not standardized
and varies between providers and institutions, yet
early recognition of neurologic involvement in these RESULTS
children is important so that appropriate treatment
Retrospective Chart Review
with systemic medications may be initiated. In this
article we describe the neurologic symptoms and Thirty-two children with ECDS and PRS were iden-
neuroimaging abnormalities seen in a signicant tied from the chart review (Table 1). Three (patients
portion of children with ECDS and PRS based on 13, 24, 29) had been included in a previous report
an institutional retrospective review. We performed a from our institution (4). Twenty-one were female
systematic review of the literature to illustrate the (66%) and 11 were male (34%), and the average onset
range of neurologic involvement that can be seen in of disease was at the age of 6.9 years (range 1
this population. 15 years). ECDS alone was present in 24, and an
overlap of ECDS and PRS was present in 8. Twenty-
one of these children had MRI, CT, or both of the
MATERIALS AND METHODS
head performed, and 18 radiographic examinations
Institutional review board approval was obtained to were available for review. Nine of these children had
conduct a retrospective chart review of children with imaging performed because of neurologic complaints,
morphea seen at the Childrens Hospital and Health whereas 12 were asymptomatic. Three of the radio-
System in Milwaukee, Wisconsin. Children seen graphic examinations were performed at another
between 2000 and 2011 were identied from a search facility and could not be reviewed; the radiology
of the hospital database using International Classi- reports of these examinations were all normal. The
cation of Diseases, Ninth Revision, discharge diagno- average age at the time of rst imaging was 9.3 years
ses of 701.0 (circumscribed scleroderma) and 710.1 (range 317 years).
(systemic sclerosis). Each chart was reviewed to iden- Of the 21 children with imaging, 2 had CT of the
tify children diagnosed by the treating clinician with head alone, 16 had MRI of the brain alone, and 3 had
ECDS or PRS and to exclude those with lichen both. Thirteen (62%) had evidence of scalp or
sclerosus, systemic sclerosis, and other diagnoses. For calvarial atrophy. Four (19%) who were imaged had
the purposes of this study, ECDS was dened as linear brain parenchymal abnormalities ipsilateral to their
plaques aecting the head and neck, whereas PRS was cutaneous lesion on MRI (Fig. 1AD). No intracra-
dened as hemifacial atrophy, although we consider nial abnormalities were seen on CT, although the four
the two diagnoses to be in the same spectrum of disease. children with intracranial MRI ndings did not have a
The charts of children with ECDS and PRS were then CT scan performed. All four had T2 hyperintensities,
reviewed to extract cutaneous manifestations and one had chronic blood products or calcication, and
associated neurologic symptoms. A pediatric neurora- one had mild mass eect with eacement of the
diologist (M.M.) reviewed the CT and MRI scans. cortical sulci. Two of these children (10% of those
Ovid Medline was used to search the medical imaged) had neuroradiologic abnormalities without
literature from 1946 to October 2011 to identify neurologic symptoms.
740 Pediatric Dermatology Vol. 29 No. 6 November/December 2012

TABLE 1. Thirty-Two Children with ECDS and PRS

Age of Cutaneous
Patient Sex onset, years involvement Neurologic involvement Neuroimaging ndings

1 F 9 Left ECDS None CT: ipsilateral focal scalp atrophy.


MRI: N/A
2 M 4 Right ECDS Seizures Headaches Impaired CT: changes of suboccipital
cognition and memory Arm decompression for Chiari I
and leg pain and spasms malformation; ipsilateral focal
(Patient has a history of scalp and calvarial atrophy
Arnold-Chiari I MRI: changes of suboccipital
malformation status decompression for Chiari I
post-craniectomy, although many malformation; no brain
symptoms could not be parenchymal abnormality;
attributed to prior ipsilateral focal scalp atrophy
abnormality or surgery.)
3 F 1 Right ECDS None N/A
4 M 11 Right ECDS None CT: N/A
MRI: no intracranial
abnormality; ipsilateral focal
scalp atrophy
5 F 12 Right ECDS None CT: N/A
MRI: no intracranial
abnormality; ipsilateral focal
scalp atrophy
6 F 10 Right ECDS None N/A
7 F 10 Left ECDS, right PRS None N/A
8 F 3 Left ECDS None N/A
9 F 2 Left ECDS Headaches CT: ipsilateral focal scalp atrophy
MRI: no intracranial
abnormality; ipsilateral focal
scalp atrophy
10 F 9 Left ECDS None N/A
11 M 8 LEFT ECDS and PRS None CT: N/A
MRI: ipsilateral caudate nucleus
heterogenous T2 signal
(calcication or chronic blood
products); ipsilateral focal scalp
and calvarial atrophy.
12 F 6 Left ECDS Behavioral issues CT: N/A
MRI: normal*

13 F 5 Left ECDS Seizures CT: N/A
MRI: ipsilateral frontal T2 HI
with patchy enhancement
14 M 4 Right ECDS None CT: N/A
MRI: normal*
15 F Unknown Left ECDS and PRS None N/A
16 F 10 Right ECDS and PRS None N/A
17 M 6 Left ECDS None N/A
18 F 5 Left ECDS None CT: N/A
MRI: no intracranial
abnormality; ipsilateral focal
scalp atrophy
19 F 3 Left ECDS Headaches CT: N/A
MRI: no intracranial
abnormality;
20 M 5 Right ECDS None N/A
21 F 9 Left ECDS None N/A
22 M 13 Right ECDS and PRS None CT: N/A
MRI: no intracranial
abnormality; hypoplastic right
maxilla
23 M 2 Left ECDS None CT: N/A
MRI: ipsilateral frontoparietal T2
HI; mild eacement of sulci;
ipsilateral focal scalp atrophy
24 F 4 Left ECDS Seizures
Chiu et al: Neuroimaging of Morphea En Coup De Sabre 741

TABLE 1. Continued

Age of Cutaneous
Patient Sex onset, years involvement Neurologic involvement Neuroimaging ndings

CT: N/A
MRI: no intracranial
abnormality; ipsilateral focal
scalp and calvarial atrophy
25 M 14 Right ECDS and PRS None CT: N/A
MRI: normal*
26 F 14 Left ECDS None N/A
27 F 5 Left ECDS None CT: N/A
MRI: no intracranial abnormality
28 F 4 Right ECDS and PRS; Tongue deviation CT: no intracranial abnormality;
left ECDS ipsilateral scalp atrophy
MRI: N/A
29 M 5 Right ECDS Strabismus (attributed to lateral CT: N/A
rectus muscle involvement) MRI: no intracranial
Slurred speech abnormality; focal scalp and soft
tissue atrophy; ipsilateral lateral
rectus muscle T2 HI
30 M 1 Left ECDS and PRS None CT: ipsilateral lytic lesion with soft
tissue swelling
MRI: soft tissue thickening with
underlying calvarial defect; mild
eacement of sulci from mass
eect of the soft tissue lesion
31 F 15 Left ECDS None CT: N/A
MRI: no intracranial
abnormality; ipsilateral focal
scalp atrophy
32 F 5 Right and left ECDS Bells palsy CT: N/A
Facial weakness MRI: bilateral cerebellar T2 HI;
Sensory defects right thalamic T2 HI; right focal
Dysmetria scalp atrophy
C78 radiculopathy
CT, computed tomography; N/A, not available; HI, hyperintensity.
*Magnetic resonance imaging scans not available for review.
Previously reported by Holland et al (4).

Nine of the 32 (28%) had neurologic symptoms In our retrospective review, cutaneous severity,
attributed to the ECDS or PRS. The most common neuroimaging ndings, and neurologic symptoms did
neurologic abnormality was seizures, reported in four not appear to correlate. For example, a 5-year-old
children (13%), followed by headache in three (9%). girl (patient 13) had no evidence of soft tissue or
Two (6%) each reported abnormalities of the cranial cranial atrophy on clinical examination or MRI,
nerves and motor and sensory defects in the extrem- yet she had a large ipsilateral T2 hyperintensity
ities. Additional complaints included cognitive prob- (Fig. 1B) and unremitting complex partial seizures
lems, behavioral issues, dysmetria, and slurred speech (previously reported by Holland et al) (4). Another
in one child each. All nine of the symptomatic children 5-year-old boy (patient 30) had severe soft tissue
had neuroimaging performed, but only two (22%) of thickening and complete absence of bone underlying
these symptomatic children had intracranial abnor- his ECDS, but no brain parenchymal involvement.
malities on MRI. All four children with neuroimaging ndings and
The onset of neurologic symptoms was concurrent eight of the nine with neurologic symptoms had
or within 1 year of onset of the cutaneous disease in ECDS alone without PRS.
six children. Two children developed neurologic The MRI ndings altered the treatment plan for
symptoms 4 and 11 years after cutaneous lesions one child. A 13-year-old asymptomatic boy (patient
were rst noticed, although both had ongoing active 22) with ECDS of the right temple was found to have
cutaneous disease at the time. The ninth child had a hypoplasia of the ipsilateral maxillary sinus without
tongue deviation that was noted incidentally on overlying skin changes; there was no other clear
examination 3 years after initial onset of her contin- etiology for the sinus hypoplasia other than the
ued cutaneous disease. morphea ECDS (Fig. 2A,B). Treatment with systemic
742 Pediatric Dermatology Vol. 29 No. 6 November/December 2012

A B

C D

Figure 1. (A) Patient 11: axial T2-weighted image demonstrating left scalp atrophy with T2 hyperintensities and chronic
blood products or calcification in the head of the left caudate nucleus. (B) Patient 13: coronal fluid attenuated inversion
recovery (FLAIR) image demonstrating T2 hyperintensity in the left frontal white matter without associated scalp or calvarial
atrophy. (C) Patient 23: coronal FLAIR image demonstrates small multifocal T2 hyperintensities in the left frontoparietal
white matter and ipsilateral scalp atrophy. (D) Patient 32: axial FLAIR image demonstrates ill-defined T2 hyperintensities in
the cerebellum.

immunosuppressants was started in place of photo- Thirty-three of the 51 (65%) had neurologic symp-
therapy. toms. The most common symptoms were seizures
(n = 22, 43%), headaches (n = 12, 24%), hemiparesis
(n = 7, 14%), abnormal deep tendon reexes (n = 5,
Literature Review
10%), sensory defects (n = 4, 8%), and vision loss
Fifty-one children with ECDS and PRS with neuroi- (n = 4, 8%). Six had symptoms without radiographic
maging results were identied from the literature abnormalities, although three were evaluated using
search (Table 2). Six were evaluated using CT, 23 only CT, and the other three had MRI alone.
using MRI, and 22 using both. Thirty-seven of the 51
(73%) had intracranial abnormalities; soft tissue and
DISCUSSION
cranial atrophy were not consistently reported. Five
had MRI abnormalities not detected on CT scan. The This study demonstrates that a substantial portion of
most common ndings were T2 hyperintensities children with ECDS and PRS have neurologic
(n = 25, 49%), calcications (n = 13, 25%), and involvement in the form of neuroimaging abnormal-
ipsilateral cerebral atrophy (n = 9, 17%). Nine ities or neurologic symptoms. Given the bias inherent
(17%) had MRI abnormalities without neurologic in reporting positive ndings, it is not surprising that
symptoms. the literature review found a high rate of neuroimag-
Chiu et al: Neuroimaging of Morphea En Coup De Sabre 743

A B

Figure 2. (A) Patient 22: morphea en coup de sabre of the right temple. (B) Coronal T1-weighted MRI shows right maxillary
sinus hypoplasia.

ing abnormalities (73%) and neurologic symptoms less clear; an inammatory process, particularly a
(65%), yet the results of the institutional retrospective vasculitis, is suspected. Brain biopsies have been
review also revealed a substantial rate of neurologic performed in some children and show a perivascular
involvement in the form of abnormal imaging or lymphocytic inltrate with features of vasculitis
symptoms in 34% overall. (4,813). Gliosis (proliferation of astrocytes, usually
At our institution, 19% of the imaged children had leading to scar) and sclerosis of the leptomeninges
brain parenchymal abnormalities found on MRI; have also been reported (8,14). Intraparenchymal and
17% of the asymptomatic children and 22% of the intravascular calcications can be seen (4). Abnormal
symptomatic children had abnormalities detected on and ectatic blood vessels are seen on biopsies and
MRI. The most common imaging abnormalities were angiographic studies, supporting the theory that
hyperintense lesions seen on T2 sequences, consistent vasculopathy or vasculitis are at the root of the
with our review of the literature. A prior review of cerebral changes (4,1517).
adults with ECDS and PRS also found T2 hyperin- There are no published prospective studies on the
tensities to be the most common, although another eects of treatment on neurologic involvement, but
series of nine adults with ECDS and PRS found that systemic antiinammatory and immunosuppressive
all nine had evidence of cerebral edema, whereas only medications are routinely used to treat other vascu-
three had T2 hyperintensities (2,3). The retrospective litides. Additionally, data from case reports suggest
chart review also conrms prior reports that 19% to that disease-modifying treatment improves the neu-
39% of children with ECDS and PRS have neurologic rologic symptoms and abnormal radiologic ndings
symptoms (57). In our study the most common (16,1821). Instances in which neurologic symptoms
neurologic symptom was seizure, again in line with are refractory to systemic therapy may represent late
prior studies (5,6). sequelae such as calcications and brosis, similar to
The association between neurologic symptoms and the cutaneous scarring of supercial disease, and
neuroimaging abnormalities and ECDS and PRS immunosuppressive therapy is unlikely to alter the
cannot be determined with complete certainty. The course in such cases.
severity of the cutaneous manifestations did not The data from our institution show that neurologic
predict neurologic symptoms or imaging ndings, as symptoms did not necessarily predict neuroimaging
demonstrated by patients 13 and 30, although the abnormalities and vice versa. The MRI was abnormal
temporal relationship, with six of the nine children in only two of the nine symptomatic children, and two
developing neurologic symptoms at approximately of the asymptomatic children had marked MRI
the same time as their cutaneous disease, suggests ndings. Further research is necessary to determine
parallel disease courses. the signicance of MRI abnormalities in the absence
The pathogenesis of morphea is unknown, and the of clinical symptoms and whether treatment alters
pathogenesis of the neurologic involvement is even the course of the neurologic disease. Because our
744 Pediatric Dermatology Vol. 29 No. 6 November/December 2012

TABLE 2. Fifty-Two Children with ECDS and PRS Reported in the Literature

Cutaneous Neurologic
References involvement involvement Neuroimaging ndings

Asher 1982 (27) Right PRS Migraine headaches CT: normal


Sensory defects MRI: N/A
Nerve palsies
Abnormal deep
tendon reexes
Vision loss
Asher 1982 (27) Left PRS None CT: contralateral parietal non-enhancing area of increased
density
MRI: N/A
David 1990 (28) Left ECDS Headaches CT: ipsilateral retrobulbar soft tissue mass with proptosis;
Seizures ipsilateral parieto-occipital calcication with enhancement
MRI: N/A
Fry 1992 (29) Right PRS Hemiparesis CT: normal
MRI: N/A
Fry 1992 (29) Right PRS Headaches CT: ipsilateral frontal calcication
MRI: ipsilateral frontal T2 HI
Fry 1992 (29) Right PRS Behavioral disorder CT: ipsilateral frontal calcication
Seizures MRI: ipsilateral frontal T2 HI
Fry 1992 (29) Right PRS None CT: normal
MRI: ipsilateral frontal T2 HI
Fry 1992 (29) Left PRS None CT: ipsilateral frontal calcication
MRI: ipsilateral hemispheric multifocal T2 HI
Fry 1992 (29) Left PRS None CT: N/A
MRI: normal
Liu 1994 (30) Left ECDS None CT: ipsilateral frontal calcication
MRI: ipsilateral frontal neuromigrational abnormality;
ipsilateral frontal calcication and T2 HI
Liu 1994 (30) Left ECDS None CT: N/A
MRI: normal
Liu 1994 (30) Left PRS N/A CT: normal
MRI: normal
Liu 1994 (30) N/A None CT: ipsilateral frontal calcication
MRI: ipsilateral frontal neuromigrational abnormality;
ipsilateral frontal calcication and T2 HI
Liu 1994 (30) N/A None CT: ipsilateral parietal hypoattenuating lesion
MRI: N/A
Liu 1994 (30) N/A None CT: N/A
MRI: normal
Cory 1995 (31) Left PRS Seizures CT: ipsilateral cingulate gyrus calcication; ipsilateral
frontal and parietal subcortical white matter ow densities
MRI: ipsilateral cingulate gyrus T1 and T2 calcication;
ipsilateral corpus callosal genu, subcortical white matter
of cingulate gyrus, deep frontal white matter,
frontoparietal subcortical white matter T2 HI;
encephalomalacia; ipsilateral frontoparietal
pial enhancement
Derex 1995 (32) Left PRS Seizures CT: ipsilateral parietal porencephalic cyst; left frontal and
Mental retardation parietal calcication
MRI: ipsilateral parietal porencephalic cyst
Schievink 1996 (33) Left PRS Vision loss CT: ipsilateral cerebral atrophy; ipsilateral basal ganglia,
Nerve palsies lateral ventricle, and parietal calcications; contralateral
Sensory defects cavernous sinus aneurysm
MRI: N/A
Goldberg-Stern 1997 (19) Left PRS Abnormal deep CT: ipsilateral frontal densities
tendon reex MRI: ipsilateral frontal T2 HI; ipsilateral frontoparietal
Seizures gyral or sulcal enhancement
Menni 1997 (34) Right ECDS and PRS Hemiparesis CT: normal
MRI: ipsilateral hemispheric T2 HI
Taylor 1997 (35) Right ECDS and PRS Seizures CT: ipsilateral temporal calcication
Vision loss MRI: ipsilateral temporal mixed signal abnormality
Higashi 2000 (36) Left ECDS Headaches CT: N/A
Seizures MRI: ipsilateral frontal T2 HI
Dizziness
Chiu et al: Neuroimaging of Morphea En Coup De Sabre 745

TABLE 2. Continued

Cutaneous Neurologic
References involvement involvement Neuroimaging ndings

Yano 2000 (37) Left ECDS and PR Seizures CT: ipsilateral frontal and parietal calcications; ipsilateral
white matter hypodensity
MRI: ipsilateral frontal and parietal T2 HI
Aynaci 2001 (38) Left PRS Adies pupil CT: ipsilateral parietal calcication
MRI: ipsilateral parietal subcortical T2 HI
MRA: normal
Moko 2003 (39) Left PRS Migraine headaches CT: N/A
MRI: ipsilateral cerebral T2 HI
Moko 2003 (39) Left PRS None CT: N/A
MRI: normal
Shah 2003 (13) Right PRS Migraine headaches CT: N/A
Seizures MRI: ipsilateral frontoparietal atrophy;
Hemiparesis ipsilateral frontal T2 HI
Abnormal deep MRA: normal
tendon reexes
Appenzeller 2004 (3) Right ECDS None CT: normal
MRI: abnormal gyral pattern; blurring
of gray-white matter
Appenzeller 2004 (3) Right ECDS None CT: normal
MRI: abnormal gyral pattern; blurring of
gray-white matter; calcication
Finley 2004 (40) Right PRS None CT: N/A
MRI: ipsilateral orbital atrophy
MRA: normal
Hulzebos 2004 (41) Right PRS None CT: N/A
MRI: ipsilateral orbital atrophy
Sandhu 2004 (42) Left PRS Seizures CT: N/A
MRI: ipsilateral frontal subdural hygroma
Sathornsumetee 2005 (43) Right ECDS and PRS Seizures CT: N/A
Dysarthria MRI: ipsilateral cerebral atrophy; hippocampal
Dysphagia asymmetry; ipsilateral subdural uid collection;
Nerve palsies ipsilateral frontal T2 HI
Abnormal deep
tendon reexes
Holl-Weiden 2006 (16) Left ECDS Headaches CT: N/A
MRI: ipsilateral frontoparietal and occipital T2 HI
Okumura 2006 (44) Left PRS None CT: ipsilateral cerebral and contralateral parieto-occipital
hypoattenuating lesions
MRI: ipsilateral cerebral and contralateral
parieto-occipital T2 HI
Paprocka 2006 (15) Left PRS Seizures CT: N/A
Hemiparesis MRI: ipsilateral frontoparietal atrophy; ipsilateral
Mental retardation parietal T2 hyperintensity; contralateral
occipital T2 hyperintensity
MRA: normal
MRS: decreased NAA and creatine; presence of lipid and
lactate (ipsilateral > contralateral)
SPECT: ipsilateral frontoparietal decreased
radiopharmaceutical uptake
Sommer 2006 (1) Right PRS Seizures CT: N/A
Migraine headaches MRI: normal
Sommer 2006 (1) Right PRS None CT: N/A
MRI: normal
Sommer 2006 (1) Left ECDS and PRS Seizures CT: N/A
Migraine headaches MRI: normal
Sommer 2006 (1) Right PRS None CT: N/A
MRI: normal
Sommer 2006 (1) Right PRS Developmental delay CT: N/A
MRI: normal
Carreno 2007 (11) Left PRS Seizures CT: N/A
MRI: ipsilateral cerebral atrophy
Verhelst 2008 (45) Right ECDS and PRS Seizures CT: ipsilateral cortical and subcortical calcications
Developmental MRI: ipsilateral hippocampal atrophy
regression
746 Pediatric Dermatology Vol. 29 No. 6 November/December 2012

TABLE 2. Continued

Cutaneous Neurologic
References involvement involvement Neuroimaging ndings

Chiang 2009 (46) Right ECDS Seizures CT: normal


MRI: ipsilateral cerebral atrophy;
ipsilateral temporo-occipital T2 HI
Menascu 2009 (21) Left ECDS and PRS Migraine headaches CT: N/A
Abnormal deep MRI: ipsilateral frontoparietal and temporal T2 HI
tendon reexes MRA: normal
Fine motor MRS: normal
impairment
Sartori 2009 (47) Left ECDS and PRS Seizures CT: ipsilateral basifrontal and temporal calcication
Behavioral disorder MRI: ipsilateral basifrontal cortical and
subcortical T2 and FLAIR HI
Qureshi 2010 (48) Left PRS Vision loss CT: narrowing of ipsilateral carotid canal
Seizures MRI: ipsilateral cerebral atrophy;
Hemiparesis ipsilateral orbital atrophy
MRA: diuse narrowing of ipsilateral internal
carotid and posterior cerebral arteries
Takenouchi 2010 (49) Right PRS Hemiparesis CT: N/A
Migraine headaches MRI: ipsilateral cerebral and thalamic
Movement disorder atrophy and T2 and FLAIR HI
MRA: normal
Fain 2011 (18) Right ECDS and PRS Seizures CT: N/A
Headaches MRI: ipsilateral sulci eacement; ipsilateral cerebral
Hemiparesis T2 and FLAIR HI; ipsilateral punctate hypointensities
Sensory defects (may represent microhemorrhage or calcication,
possibly a cavernous malformation)
Fain 2011 (18) Right ECDS Seizures CT: ipsilateral parietal high density lesion
Sensory defects MRI: ipsilateral parietal gradient echo hypointensity
suggestive of blood products or calcication,
possibly a cavernous malformation
Longo 2011 (20) Left PRS Seizures CT: N/A
MRI: ipsilateral T2 HI and T1 hypointensity
Not all authors uniformly reported soft tissue and calvarial changes, which are not included in the table.
N/A, not available; HI, hyperintensity; FLAIR, uid attenuated inversion recovery.

understanding of ECDS and PRS is limited, caution is rheumatologists favored (22). Dermatologists pre-
advised in the treatment of these children. scribed methotrexate and systemic corticosteroids for
We recommend that all symptomatic children with fewer than 5% of children with linear morphea, which
ECDS or PRS have an MRI of the brain performed at many argue routinely requires systemic therapy given
initial diagnosis, with the initial ndings and disease the potential for prominent disgurement when on the
course determining further imaging. MRI is preferred face or functional compromise when on a limb (22).
because it is more sensitive than CT in detecting the Although it has been reported that topical therapies
brain parenchymal abnormalities seen in ECDS and and phototherapy are successful in treating the
PRS. supercial manifestations of ECDS, these therapies
Even when children are asymptomatic, brain imag- are not thought to penetrate beyond the dermis and
ing should be strongly considered to guide therapeutic should not be used for deeper disease (23,24). The
decisions. Clinical predictors of intracranial disease presence of intracranial or bony abnormalities on
are poor, and the presence of intracranial involvement MRI should prompt consideration of early systemic
cannot be determined using history and physical therapy rather than topical therapy or phototherapy.
examination. Waiting until symptom development If systemic immunosuppressive treatment will be used
to obtain an MRI and start systemic therapy may to treat the child, neuroimaging may still be helpful as
not be sucient to completely prevent neurologic a baseline examination to determine the presence or
sequelae. A study examining prescribing patterns absence of intracranial abnormalities before starting
found that dermatologists preferentially prescribed therapy.
topical therapy and phototherapy for linear morphea Because ECDS and PRS are rare, participant
over methotrexate and systemic corticosteroids, which accrual for prospective studies is dicult, and small
Chiu et al: Neuroimaging of Morphea En Coup De Sabre 747

participant numbers limit retrospective reviews. facial hemiatrophy: a case series of 12 patients. J Am
Although this study of children with ECDS and Acad Dermatol 2006;54:227233.
2. Kister I, Inglese M, Laxer RM et al. Neurologic
PRS evaluated using neuroimaging is the largest to
manifestations of localized scleroderma: a case report
date, the small numbers and retrospective nature limit and literature review. Neurology 2008;71:15381545.
it. Not all of the children underwent neuroimaging, 3. Appenzeller S, Montenegro MA, Dertkigil SS et al.
introducing the possibility of bias in that symptomatic Neuroimaging ndings in scleroderma en coup de sabre.
children were preferentially referred for radiologic Neurology 2004;62:15851589.
4. Holland KE, Stees B, Nocton JJ et al. Linear sclero-
scans, but a substantial number of asymptomatic
derma en coup de sabre with associated neurologic
children underwent imaging, and rates of abnormal abnormalities. Pediatrics 2006;117:e132e136.
scans were not considerably dierent between asymp- 5. Christen-Zaech S, Hakim MD, Afsar FS et al. Pediatric
tomatic (17%) and symptomatic children (22%). Not morphea (localized scleroderma): review of 136
all MRIs could be reviewed, although the unavailable patients. J Am Acad Dermatol 2008;59:385396.
6. Zulian F, Athreya BH, Laxer R et al. Juvenile localized
MRIs were reported to be normal, and any abnor-
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treatment initiation with topical therapy or photo- Romberg syndrome and linear scleroderma in coup de
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ACKNOWLEDGMENTS encephalitis associated with Parry-Romberg syndrome.
Neurology 2003;61:395397.
We are indebted to Ariel Rosen, research coordinator,
14. Chung MH, Sum J, Morrell MJ et al. Intracerebral
for help with the institutional review board application involvement in scleroderma en coup de sabre: report of
and coordination of the project. We are indebted to a case with neuropathologic ndings. Ann Neurol
Rita Sieracki, librarian, for assistance with the litera- 1995;37:679681.
ture search. This publication was supported by the 15. Paprocka J, Jamroz E, Adamek D et al. Diculties in
dierentiation of Parry-Romberg syndrome, unilateral
National Center for Research Resources and the
facial sclerodermia, and Rasmussen syndrome. Childs
National Center for Advancing Translational Sciences, Nerv Syst 2006;22:409415.
National Institutes of Health (NIH), through grant 16. Holl-Wieden A, Klink T, Klink J et al. Linear
UL1RR031973. Its contents are solely the responsibil- scleroderma en coup de sabre associated with cere-
ity of the authors and do not necessarily represent the bral and ocular vasculitis. Scand J Rheumatol
2006;35:402404.
ocial views of the NIH.
17. Sakai M, Aoki S, Inoue Y et al. Silent white matter
lesion in linear scleroderma en coup de sabre. J Comput
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