The Turkish Journal of Pediatrics
[Mail to Editor ]
A Patient with Behçet’s Disease Presenting with Acute Urinary Retention
Sare Gülfem Akyüz1, Aysun Çaltık2, Mehmet Bülbül1, Özlem Aydoğ1, Gülay Demircin3,
Ayşe Aksoy4, Berna Uçan5
Departments of 1Pediatric Nephrology, 4Pediatric Neurology, and 5Radiology, Dr. Sami Ulus Children’s and Maternity
Hospital, and 2Keçiören Training and Research Hospital, and 3TOBB University Faculty of Medicine, Ankara, Turkey.
|Behçet’s disease (BD) is a multisystemic inflammatory disorder of unknown
etiology. Neurologic involvement is known to be the most devastating feature
of BD. The frequency and types of neurologic involvement in the pediatric age
group are not clear, and the available information is limited to case reports.
Here, we report a BD patient who presented with urinary incontinence as
the initial feature of spinal cord involvement.|
Behçet’s disease, urinary retention, spinal cord.
|Behçet’s disease (BD) is a multisystemic
inflammatory disorder of unknown etiology.The
reported incidence of neurologic involvement
varies from 5-10% in non-selected series,, and
it may be parenchymal or non-parenchymal.
Encephalomyelitis, spinal cord abnormalities,
aseptic meningitis, and benign intracranial
hypertension may be recognized in patients with
BD. Here, we report a 16-year-old boy who
presented with urinary retention as a feature
of spinal cord involvement of BD.|
|Case Presentation |
|A 16-year-old boy was admitted with urinary
retention for three days. He had a history of
BD for five years. He had been diagnosed as
BD on the basis of recurrent oral and genital
ulcerations and positive pathergy reaction. He
had been using only colchicine, but had not
presented for follow-up visits for two years.
In his physical examination on presentation,
there were no mucocutaneous, musculoskeletal,
cardiovascular, or ocular findings. On the
neurological examination, his cognitive,
cerebellar, cerebral, and cranial nerve functions
were intact; deep tendon reflexes were normal
in both upper and lower limbs. A Foley catheter
On laboratory investigations, complete blood
count, acute phase reactants, and renal and liver function tests were in normal
limits. Urine analysis showed eumorphic
erythrocytes. Abdominal ultrasonography and
echocardiography were normal.
During his follow-up, he developed paresthesia
of the legs, difficulty walking and gait
disturbance. Motor function in the legs was
diminished bilaterally (3/5). Deep tendon
reflexes were exacerbated, and cremasteric and
abdominal reflexes were absent. Cerebrospinal
fluid (CSF) examination could not be done
because his family did not give permission.
Cranial magnetic resonance imaging (MRI)
was normal. MRI of the lumbar spine was
normal on T1- and T2-weighted images, but
revealed contrast enhancement after gadolinium
injection, and these findings were consistent
with myelitis (Fig. 1).
According to the patient’s history of BD and
lumbar spine MRI findings, our patient was
diagnosed as neuro-Behçet’s disease (NBD)
with spinal cord involvement.
He was treated first with intravenous (i.v.)
pulse methylprednisolone (30 mg/kg/day,
for 3 days). Motor functions in his legs
normalized, but urinary retention did not
resolve. Thus, i.v. pulse cyclophosphamide
therapy (750 mg/m2/monthly, twice) was
added, and clean intermittent catheterization for urinary retention was continued. We also
continued tapering the dose of prednisolone
orally (starting dose of 2 mg/kg/day, tapered
to 0.75 mg/kg/day in 45 days). His muscle
strength had almost fully recovered in the
second month of the treatment, but he still
required clean intermittent catheterization.
| ||Fig.1. Continuous diffuse pial postcontrast enhancement
is observed at the level of T11-L1 A, at T1 weighted
sagittal magnetic resonance imaging (thin arrows).|
|Behçet’s disease (BD) is a multisystemic
inflammatory disorder of unknown etiology.
A higher incidence in families with more
than one member with BD suggests a genetic
anticipation. Although the usual onset of
BD is in the third or fourth decade, pediatric
cases have also been reported,. In addition
to mucocutaneous and ocular involvement, BD
may also involve the joints, blood vessels, and
gastrointestinal and nervous systems,.
Neurologic involvement was reported in 5-10%
of the cases in a large series of BD. NBD is
mainly in two major forms: parenchymal central
nervous system involvement and cerebral
venous sinus thrombosis (CVST). CVST mainly
occurs in the pediatric age group as compared
to adult-onset NBD, in which parenchymal
neurologic involvement is more frequent.
The frequency and types of the neurologic
involvement in the pediatric age group are
not clear, and available information is limited
to case reports,.
In NBD, while the brainstem and diencephalon
are affected frequently, spinal cord involvement
is detected rarely. Spinal cord involvement
in NBD has been known since the 1950’s.
Whereas initial reports on this issue were based
on histopathological studies, cases reported
more recently were diagnosed by spinal MRI
scans. The rate of spinal cord involvement has
varied between 2.5-30% in various studies. In
a study of Uludüz, only one child had spinal
cord involvement among 26 patients with
pediatric BD. Her neurological picture was
consistent with a relapsing myelitis, resolving
without sequelae over many years. In a study
of Shakir, neurologic involvement of BD was
reviewed, and paraparesis, sensory deficits
indicating a spinal level, sphincter and/or sexual
dysfunction, and CSF lymphocytic pleocytosis
were reported to be cardinal features of spinal
cord involvement. Persistent disability was only
noted in patients with spinal cord involvement.
In our patient, although the motor function in his legs normalized, urinary retention has
not yet resolved, and he is still on treatment
with clean intermittent catheterization in the
first month of the treatment.
There are no controlled studies concerning
the treatment of pediatric NBD. Treatment
focuses on the reduction of the inflammation
that causes vascular or parenchymal lesions.
Treatment choices in neurological diseases
are based mainly on anecdotal reports and
experience. High- dose corticosteroids are
given during attacks, followed by maintenance
oral corticosteroids tapered over 2-3 months.
If the patient has poor prognostic factors
(spastic paraparesis, sensory deficits, sphincter
and/or sexual dysfunction, CSF lymphocytic
pleocytosis, or persistent disability) or a second
neurologic episode has occurred, another
immunosuppressant agent such as azathioprine,
i.v. cyclophosphamide or methotrexate should
be added. Interferon (IFN)-α and tumor
necrosis factor (TNF)-α antagonists have
been used with some success in resistant
cases-. Because the urinary incontinence
of our patient did not resolve with pulse i.v.
methylprednisolone, we added high-dose i.v.
In conclusion, neurologic involvement is known
to be the most devastating involvement in BD.
Spinal cord involvement is very rare, and might
be overlooked. Considering the dismal outcome
of spinal cord presentation in young males,
early and vigorous treatment is recommended
in this group of NBD.
1. Yazıcı H, Yurdakul S, Hamuryudan V. Behçet’s
syndrome. Curr Opin Rheumatol 1999; 11: 53-57.
2. Al-Araji A, Kidd DP. Neuro -Behçet’s disease:
epidemiology, clinical characteristics and management.
Lancet Neurol 2009; 8: 192-204.
3. Akman-Demir G, Serdaroğlu B, Taşçi B; Neuro-
Behçet Study Group. Clinical patterns of neurological
involvement in Behçet’s disease: evaluation of 200
patients. Brain 1999; 122: 2171-2181.
4. Yeşilot N, Mutlu M, Güngör O, Baykal B, Serdaroğlu
P, Akman-Demir G. Clinical characteristics and course
of spinal cord involvement in Behçet’s disease. Eur
J Neurol 2007; 14: 729-737.
5. Uludüz D, Kürtüncü M, Yapıcı Z, et al. Clinical
characteristics of pediatric-onset neuro-Behçet disease.
Neurology 2011; 77: 1900-1905.
6. Siva A, Saip S. The spectrum of nervous system
involvement in Behçet’s syndrome and its differential
diagnosis. J Neurol 2009; 256: 513-529.
7. Shakir RA, Sulaiman K, Kahn RA, Rudwan M.
Neurological presentation of neuro-Behcet’s syndrome:
clinical categories. Eur Neurol 1990; 30: 249-253.
8. Hatemi G, Silman A, Bang D, et al.; EULAR Expert
Committee. EULAR recommendations for the
management of Behçet disease. Ann Rheum Dis
2008; 67: 1656-1662.
9. Nichols JC, İnce A, Akduman L, Mann ES. Interferon-α2a
treatment of neuro-Behçet disease. J Neuroophthalmol
2001; 21: 109-111.
10. Fujikawa K, Aratake K, Kawakami A, et al. Successful
treatment of refractory neuro-Behçet disease with
infliximab: a case report to show its efficacy by magnetic
resonance imaging, transcranial magnetic stimulation
and cytokine profile. Ann Rheum Dis 2007; 66: 136-
11. Alty JE, Monaghan TM, Bamford JM. A patient with
neuro-Behçet’s disease is successfully treated with
etanercept: further evidence for the value of TNF-α
blockade. Clin Neurol Neurosurg 2007; 109: 279-281.
12. Ribi C, Sztajzel R, Delavelle J, Chizzolini C. Efficacy
of TNF-α blockade in cyclophosphamide resistant
neuro-Behçet disease. J Neurol Neurosurg Psychiatry
2005; 76: 1733-1735.
[Mail to Editor ]