uluduz2011

Clinical characteristics of pediatric-onset
neuro-Behc¸et disease
D. Uluduz, MD*
M. Ku¨rtu¨ncu¨, MD*
Z. Yapıcı, MD
E. Seyahi, MD
Ö. Kasapc¸opur, MD
H. Özdog˘an, MD
S. Saip, MD
G. Akman-Demir, MD
A. Siva, MD
ABSTRACT
Objectives: Neurologic involvement in the pediatric population with Behc¸et disease (BD) is limited
to case reports. The aim of this study is to examine the frequency and type of neurologic involve-
ment in pediatric patients with BD.
Methods: Medical records of 728 patients with a diagnosis of neuro-BD (NBD) of 2 large BD cohorts
followed in Istanbul University were included in the study. Patients with an onset of both systemic and
neurologic symptoms at or before age 16 (pediatric neuro-BD) were identified. Demographic and clinical
characteristics of pediatric patients with NBD were compared with adult patients with NBD.
Results: There were 26 cases with pediatric BD (3.6%) and 702 (96.4%) adult-onset patients.
Gender ratio was equal in the general pediatric BD cohort, whereas male/female ratio was 5.5/1
in pediatric NBD cases. Mean age at BD onset and neurologic involvement onset were 13.0 ⫾3.0
and 13.5 ⫾2.4, respectively, and in the adult population mean age at onset of BD was 26.7 ⫾8.0
and neurologic involvement occurred a mean of 5.3 ⫾4.5 years later. Clinical and MRI evaluation
revealed that 3 children had CNS parenchymal involvement and 23 had dural venous sinus throm-
bosis (88.5%). We observed parenchymal involvement in 74.8% of the adults, contrary to the low
17.2% of cases with venous sinus thrombosis.
Conclusions: Pediatric NBD comprises 3.6% of our whole NBD cohort, with a male predominance,
mainly in the form of dural venous sinus thrombosis, whereas in the adult NBD population the
dominant form of neurologic involvement is parenchymal, suggesting that the pathogenesis of
NBD may be different according to the age at disease onset. Neurology
®
2011;77:1900–1905
GLOSSARY
BD ⫽Behc¸et disease; CVST ⫽cerebral venous sinus thrombosis; ISGBD ⫽International Study Group for Behc¸et Disease;
NBD ⫽neuro–Behc¸et disease.
Behc¸et disease (BD), first recognized as a unique clinical entity in 1937 by Hulusi Behc¸et, is an
idiopathic chronic multisystemic vascular inflammatory disease of unknown origin. The most
widely used diagnostic criteria of BD are the International Study Group criteria, which require
recurrent oral ulcerations, plus 2 of the following: recurrent genital ulcerations, skin or eye
lesions, or a positive pathergy test.
1
Although the usual onset of BD is in the third or fourth
decades,
2,3
pediatric cases have been reported,
4-7
with a higher incidence in families with more
than one member with BD, suggesting a genetic anticipation.
8
Neurologic involvement, reported to occur in 5%–10% of the cases in large series of
BD, mainly presents in 2 major forms: parenchymal CNS involvement and cerebral ve-
nous sinus thrombosis.
2
However, the frequency and types of the neurologic involvement
in pediatric population is not clear and the only available information is limited to case
reports.
9-13
CME
Address correspondence and
reprint requests to Dr. Aksel Siva,
Istanbul University, Cerrahpasa
School of Medicine, Department
of Neurology, Istanbul, 34093
Turkey
[email protected]
*These authors contributed equally.
From the Department of Neurology (D.U., S.S., A.S.) and Division of Rheumatology, Departments of Medicine (E.S., H.O
¨.) and Pediatrics (O ¨.K.),
Cerrahpas¸a School of Medicine, Istanbul University; Acıbadem University School of Medicine (M.K.); and Istanbul School of Medicine (Z.Y., G.A.-D.),
Istanbul University, Istanbul, Turkey.
Disclosure: Author disclosures are provided at the end of the article.
1900 Copyright © 2011 by AAN Enterprises, Inc.

The present study aimed to describe the
demographic and neurologic features of pedi-
atric NBD patients and to compare them
with the adult NBD patients seen in 2 univer-
sity hospitals with a large BD practice.
METHODS The records of the patients, seen and followed
since 1984 at the NBD outpatient clinics of the neurology de-
partments of 2 medical schools at Istanbul University (Cerrah-
pasa and Istanbul Medical Schools), were retrospectively
evaluated. These patients were referred from The Behc¸et’s Dis-
ease Research Center in Cerrahpasa School of Medicine and
Behc¸et’s Clinic of Istanbul School of Medicine. We used The
International Study Group for BD Criteria for the definitive diag-
nosis of BD.1The patients with BD who presented at or before 16
years of age with any neurologic involvement consistent with BD2
were included in the study.
Patients with BD younger than 16 years of age with recent onset
of severe headache or other neurologic symptoms, but without any
evidence of objective neurologic findings on examinations and a
normal neurologic workup including cranial MRI and magnetic
resonance venography, and when indicated CSF or neurophysio-
logic studies, were excluded.
Demographics (gender, age at onset of BD, age at onset of
neurologic involvement, family history) and clinical features
such as symptoms/signs at disease onset, the frequencies and dis-
tribution of other systemic findings, neuroimaging, and CSF
findings, as well as clinical outcomes, were studied.
Pediatric patients with NBD were compared with adult pa-
tients with BD and NBD using the database of the patients with
BD in both centers.
The data were analyzed by SPSS statistics. We performed
statistical analysis employing Student ttest for mean values and
␹
2
test for table analysis.
Standard protocol approval, registrations, patient consents.
We obtained consent forms to participate in this study from all
patients. The study was approved by the institutional ethics
committee.
RESULTS Demographic and clinical characteristics
of the pediatric patients with NBD are summarized
in table 1. Neurologic involvement appeared before,
Table 1 Demographic and clinical characteristics of the pediatric NBD patients
Patient
no. Sex
Age at
onset of
BD, y
Age at
onset of
NBD, y
Time between
BD and
NBD, y
Family
history
Type of
neurologic
involvement
Site of
neurologic
involvement Outcome
1M 7 11 4 ST SSS ⫹TS Improvement
2M 11 11 0 1 ST SSS Improvement
3F 7 10 3 ST SSS Improvement
4M 11 12 1 ST SSS Improvement
5M 10 13 3 ST SSS ⫹TS Improvement
6M11 10 ⫺1 1 ST TS Improvement
7M 11 11 0 1 ST SSS ⫹TS Residual deficit
8M 14 14 0 ST SSS ⫹TS Improvement
9M 13 14 1 ST TS Residual deficit
10 M 13 16 3 ST SSS Improvement
11 M 14 14 0 ST SSS Improvement
12 M 16 16 0 ST SSS ⫹TS Improvement
13 M 13 16 5 ST SSS Improvement
14 F 14 14 0 1 ST SSS ⫹TS Improvement
15 M 16 16 0 ST TS Improvement
16 M 15 16 1 ST SSS ⫹TS Improvement
17 M 11 11 0 ST SSS Improvement
18 M 13 14 1 Parenchymal BS Improvement
19 M 12 12 0 1 ST SSS Improvement
20 M 12 14 2 ST SSS Improvement
21 M 16 16 0 ST SSS Improvement
22 F21 8 ⫺13 Parenchymal Spinal Improvement
23 M 16 16 0 ST TS Improvement
24 M 11 14 3 ST SSS ⫹TS Improvement
25 F 15 16 1 1 Parenchymal BS Improvement
26 M 14 16 2 ST SSS Improvement
Abbreviations: BD ⫽Behçet disease; BS ⫽brainstem; NBD ⫽neuro–Behçet disease; SSS ⫽superior sagital sinus; ST ⫽
sinus thrombosis; TS ⫽transverse sinus.
Neurology 77 November 22, 2011 1901

with, or after the onset of other systemic signs of BD.
A total of 26 pediatric patients with NBD among
728 patients with definite NBD (3.6%) were identi-
fied. The prevalence of our pediatric patients with
BD was 2.4% in the total BD cohort, and 10% of
this population had neurologic involvement. The
mean age at onset of BD was 13.0 ⫾3.0 years (range
7–16 years) and NBD was 13.5 ⫾2.4 years (range
8–16 years). The mean age at the time of the study
was 19.0 ⫾5.7 years (range 11–32 years). The mean
follow-up time was 6.0 ⫾5.0 years (range 1–19
years; median 7 years). In our adult patients with
NBD, the mean age at onset of BD and NBD were
26.7 ⫾8.0 and 32.0 ⫾8.7 years, respectively. Time
to the first neurologic episode after the onset of BD
was significantly shorter in the pediatric cohort as
compared to the adult-onset cases (p⫽0.001, Stu-
dent ttest). In 11 cases (42.3%), neurologic involve-
ment occurred simultaneously with the onset of
other systemic findings of BD. Two patients (pa-
tients 6 and 22) presented with a neurologic attack
prior to the systemic manifestations of BD. One had
parenchymal CNS involvement as well as oral aph-
thae at the age of 8 and had fulfilled International
Study Group for Behc¸et Disease (ISGBD) criteria
many years later followed by 2 more neurologic at-
tacks later. The other patient had cerebral venous
sinus thrombosis (CVST) at the age of 10 and ful-
filled ISGBD criteria 1 year later. A positive family
history was noted in 23.1% of patients, whereas in
the adult cohort family history was positive in
10.4%; however, this difference did not reach statis-
tical significance.
The frequencies of other systemic symptoms of
BD in pediatric and adult cohorts are shown in table
2. Major vascular events other than CVST had not
been observed in any of the 26 cases.
The most common initial neurologic symptom in
our pediatric NBD population was headache as seen
in 24 cases and epileptic seizures in 3 patients. Cra-
nial neuropathies, such as the oculomotor or abdu-
cens nerve palsies, were seen in 10 patients, and
hemiparesis in 3 patients. The most common type of
neurologic involvement was CVST, which was de-
tected in 23 patients (88.5%). We confirmed the di-
agnosis of CVST by gadolinium-enhanced MRI in
12 patients and by MRV in 11 patients. Isolated su-
perior sagittal sinus thrombosis was observed in
42.3%, and concomitantly with transverse sinus
thrombosis in another 30%; isolated unilateral trans-
verse sinus thrombosis was seen in the remaining
16.1%. None of these patients had any cortical ve-
nous infarcts. In patients who presented with CVST
without a previous diagnosis of BD, a complete
workup for hypercoagulability states and an extensive
differential diagnosis of probable other etiologic
causes were performed. Human leukocyte antigen–
B51 was positive in 73.4% in the whole cohort. The
remaining 3 patients had parenchymal involvement;
2 of these patients had brainstem involvement and 1
patient had spinal cord involvement. In the adult
population, however, parenchymal involvement was
observed in 74.8% of patients, and CVST in 17.2%
of patients. CVST was significantly more frequent in
the pediatric group (p⬍0.001,
␹
2
test).
Although 2 of the pediatric patients had residual
neurologic deficits, all but one were eventually func-
tionally independent, and the outcome of all patients
was favorable at a median follow-up of 7 years.
Patient 7 lost his sight due to optic atrophy immedi-
ately after developing CVST despite a ventriculo-
peritoneal shunt which was performed in another
institution. He is still under our follow-up with bilat-
eral complete loss of vision. Twenty-seven percent of
the adult population had progressive NBD whereas
none of the pediatric cohort progressed during the
follow-up period.
DISCUSSION In our series, we identified pediatric-
onset neurologic involvement in 3.6% of the whole
NBD cohort. In patients with serious organ involve-
ment due to BD, including CNS, a male predomi-
Table 2 Systemic and neurologic findings in the pediatric and adult patients
with BD
Pediatric NBD
(n ⴝ26)
Adult NBD
(n ⴝ702) p
Gender ratio, M:F 5.5:1 2.6:1 0.261
Mean interval between BD and NBD, y 1.25 ⫾1.5 5.3 ⫾4.5 0.001
Family history, n (%) 6 (23.1) 73 (10.4) 0.085
Systemic findings, n (%)
Oral ulcers 26 (100) 702 (100) 1.000
Erythema nodosum 16 (61.5) 340 (48.4) 0.260
Papulopustular lesions 7 (27.0) 201 (28.6) 0.840
Genital ulcers 14 (53.8) 594 (84.6) ⬍0.001
Uveitis 10 (38.5) 450 (64.1) 0.014
Arteralgia/arthritis 9 (34.6) 394 (56.1) 0.049
Neurologic findings, n (%)
Headache 24 (92.3) 411 (58.5) ⬍0.001
Epileptic seizure 3 (11.5) 53 (7.5) 0.443
Ataxia 2 (7.7) 185 (26.4) 0.037
Pyramidal findings 3 (11.5) 418 (59.5) ⬍0.001
Dysarthria 1 (3.8) 161 (23.0) 0.016
Neurologic involvement, n (%)
CVST 23 (88.5) 121 (17.2) ⬍0.001
Parenchymal involvement 3 (11.6) 525 (74.8) ⬍0.001
Abbreviations: BD ⫽Behçet disease; CVST ⫽cerebral venous sinus thrombosis; NBD ⫽
neuro–Behçet disease.
1902 Neurology 77 November 22, 2011

nance is usually noted, with a male to female ratio of
up to 4:1.
2,14-17
It is notable that male predominance
was even more pronounced in the pediatric group
than the adult onset group; however, this difference
was not statistically significant. Neurologic onset was
around puberty. Regarding the systemic manifesta-
tions of BD, oral aphthous ulcers, skin lesions, and
genital ulcers were the most common symptoms in
our pediatric NBD cohort. However, genital ulcers
were significantly less common than in the adult co-
hort. Uveitis and arthritis were also significantly less
common in the pediatric cohort. There may be racial
disparities in terms of clinical manifestations. In a
study reported from Tunisia, the rate of perianal ul-
cers was higher in pediatric BD cases,
18
and the rate
of ocular involvement was higher in a study carried
out in Turkey.
19
The most common initial neurologic symptom in
our study was severe headache, as in the adult cohort;
however, it was significantly more prevalent. But
weakness, pyramidal signs, and ataxia were also com-
mon symptoms and signs in adults, which were un-
common in the pediatric cohort.
The major finding in our study was that neuro-
logic involvement mainly appeared in the form of
CVST in the pediatric group, as compared to adult
onset NBD, where parenchymal neurologic involve-
ment was more frequent. However, in pediatric pa-
tients with NBD with CVST, the involvement of
cerebral venous sinuses is similar to the distribution
of CVST seen in our adult BD cases, suggesting that
once the pattern of neurologic involvement occurs in
NBD, its behavior is age-independent. The reason
for CVST being more common in younger patients
is unknown. However, also in adult series a tendency
for CVST to start at an earlier age has been noted
before.
2,15,20
It seems that while CVST is the pre-
dominant type of neurologic involvement of chil-
dren, especially in Turkey, parenchymal type of
involvement could be the more dominant type in
other juvenile series reported from France, Israel,
and Saudi Arabia.
16,21,22
In our series, we found parenchymal involvement
only in 3 patients; 2 of them had brainstem involve-
ment similar to the adult NBD cases with an onset at
a relatively older age when compared to CVST cases.
Additionally there was one patient with spinal cord
involvement, in whom systemic manifestations oc-
curred many years later. Her neurologic picture was
consistent with a relapsing myelitis, resolving with-
out sequelae over many years, and could not be ex-
plained with another diagnosis in the long-term
follow-up. This type of spinal cord involvement has
been reported in NBD cases and also seen in our
adult cases.
23-25
It has been noted that concurrent pa-
renchymal CNS involvement in patients with BD
with CVST is uncommon.
2,15
Consistent with these
observations none of our pediatric cases had both
patterns of neurologic involvement and also none de-
veloped further neurologic episodes. The lack of oc-
currence of the 2 major forms of neurologic disease
in the same individual with BD (parenchymal CNS
involvement and CVST) may point to the possibility
that these 2 patterns of involvement might have dif-
ferent pathogenic mechanisms.
14,15,26-28
In a recent
review, however, based on 2 small series where a
mixed pattern of CVST and parenchymal disease was
reported in up to 20% of patients, this hypothesis
was questioned.
3
However, our study, in which the
prevalence of CVST in pediatric patients with NBD
was the reverse of the prevalence in adult patients
with NBD, and none had or developed a mixed dis-
ease pattern during the mean follow-up period of
6.0 ⫾5.0 years, supports that this clinicopathologic
classification has clinical, laboratory, neuroradiologic,
pathologic, and prognostic basis. This further supports
the notion that the underlying mechanisms of these 2
major forms of neurologic disease are indeed associated
with different pathogenic mechanisms.
2
It has also been previously noted that CVST in
BD behaves somewhat differently than CVST due to
other causes, because of less tendency of focal venous
hemorrhagic infarction that is commonly seen in
patients with CVST due to other cases.
28
Our ob-
servations in the pediatric onset neuro-BD pa-
tients supports this observation. None of the
patients with CVST had any cortical infarcts,
which is reflected in the low rate of focal neuro-
logic signs, or residual disability.
CVST in BD is strongly associated with systemic
major vessel disease, to show a tendency to occur more
in male patients with BD and have a better neurologic
prognosis when compared with the parenchymal-CNS
type of neurologic involvement.
20,29
Our cases did not
show any other vascular involvement; however, all other
reported findings were also found to be consistent in
our pediatric NBD cohort.
There are no controlled studies concerning the
treatment of pediatric NBD. The treatment focuses
on the reduction of inflammation that causes vascu-
lar or parenchymal lesions. The reduction of inflam-
mation with high-dose corticosteroids per se is
usually enough for the remission of CSVT. Accord-
ing to our experience, most of the patients with
CVST do not require anticoagulation routinely. In
the long term, immunosuppressants such as low-dose
oral corticosteroids, azathioprine, and cyclophosph-
amide may be needed.
Neurologic involvement is known to be the most
devastating systemic involvement in BD. Previous
Neurology 77 November 22, 2011 1903

studies also suggest that the prognosis of young male
patients with BD is poor.
30
Despite these findings,
our patients had generally a favorable outcome. Only
2 patients had some residual neurologic deficits;
however, all but one were functionally independent.
One of the reasons for this better outcome should be
the high frequency of dural sinus thrombosis in this age
group, which is already known to have a much better
prognosis than parenchymal CNS involvement.
14,15
Pediatric onset neurologic involvement is rela-
tively rare in BD (around 4.0% of all NBD cases). It
affects males more often, dural sinus thrombosis is
more common, and therefore the course is more benign
that adult onset BD. Larger, multicenter, and prospec-
tive studies are needed to elucidate these further.
AUTHOR CONTRIBUTIONS
Dr. Uluduz: collecting and designing the data, writing the manuscript.
Dr. Ku¨rtu¨ncu¨: collecting and designing the data, statistical analysis, writ-
ing the manuscript. Dr. Yapıcı: collecting data. Dr. Seyahi: collecting data
and revising the manuscript. Dr. Kasapc¸opur: collecting data. Dr.
O
¨zdog˘an: collecting data. Dr. Saip: collecting and designing the data,
analyzing or revising the manuscript. Dr. Akman-Demir: collecting and
designing the data, analyzing or revising the manuscript. Dr. Siva: collect-
ing and designing the data, revising the manuscript for content.
ACKNOWLEDGMENT
The authors thank Mefkure Eraksoy, Piraye Serdaroglu, Ahmet Gul,
Ilknur Tugal-Tutkun, Hasan Yazici, Vedat Hamuryudan, Sabahattin
Yurdakul, and Gu¨len Hatemi for contributions in case recruitment.
DISCLOSURE
Dr. Uluduz, Dr. Ku¨rtu¨ncu¨, Dr. Yapıcı, Dr. Seyahi, and Dr. Kasapc¸opur
report no disclosures. Dr. O
¨zdoðan has received speaker honoraria from
Novartis. Dr. Saip reports no disclosures. Dr. Akman-Demir has received
speaker honoraria from Novartis, Bayer Schering Pharma, Merck Serono,
Gen Ilac Ltd. Turkey, and Teva Pharmaceutical Industries Ltd.; and
serves as an Associate Editor for Archives of Neuropsychiatry (Turkey). Dr.
Siva has received honoraria for giving educational lectures, travel and reg-
istration coverage for attending several national or international con-
gresses, or symposia and unrestricted research grants to his department
from Bayer Schering Pharma, Merck Serono, Biogen Idec, Novartis, Teva
Pharmaceutical Industries Ltd./sanofi-aventis, and Allergan, Inc.
Received November 9, 2010. Accepted in final form August 3, 2011.
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