Guillain-Barre Syndrome in a Three-Month-Old Infant Case Report * Chien-Hung Liu Wen-Kan Feng

Case Report
Guillain-Barre Syndrome in a Three-Month-Old Infant
Wen-Kan Feng1 Kun-Long Hung2,3,* Chien-Hung Liu2
We report a three-month-old infant who suffered from progressive motor weakness within
four days after fistulectomy for anal fistula. From the clinical presentations and the results of
laboratory studies included spinal MR images and electrophysiological study, Guillain-Barre
syndrome(GBS) was diagnosed. Immunotherapy with intravenous immunoglobulin was
prescribed early after diagnosis ascertained. Following rehabilitation was performed that good
outcome had been achieved. To our knowledge, this case might be the youngest patient of noncongenital Guillain-Barre syndrome that had been reported before. GBS in young infant is rare and
clinical presentation is not distinct. It is very important that a infant with suspected GBS is
managed as an emergency. Spinal MR imaging as a supplementary diagnostic modality is
suggested. ( FJJM 2010; 8 (1) : 57-61 )
Key words: Guillain-Barré syndrome, infant, flaccid paralysis, areflexia,
intravenous immunoglobulin
INTRODUCTION
Guillain-Barre syndrome (GBS) is the most
common cause of flaccid paralysis in children and
is characterized by various degrees of motor
weakness, sensory abnormalities and autonomic
dysfunction. Several clinical forms of GBS have
been suggested based on clinical and electrodiagnostic
feactures. In some forms, paralysis progresses very
rapidly that lead to ventilatory failure, life-threatening
arrhythmias and hypertension. Aspiration pneumonia
due to in-coordinate swallowing movement is a
hazard in young patients. The overall mortality in
childhood GBS is about 5 %.
Although GBS is far more common in adult,
it occurs in children that the annual incidence is
Department of Pediatrics, Sijhih Cathay General Hospital, Taipei1
Catholic University, School of Medicine, Taipei County, Taiwan3
Submitted March, 01, 2010; final version accepted March, 31, 2010.
*Correspondence author: [email protected]
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第 8 卷 第 1 期 2010
about 0.1 cases per 100000(3), but infant as young
as 3 months of age had never been reported. We
reported a 3-month-old boy of GBS occuring soon
after fistulectomy for anal abscess. According to
the literature we reviewed, this might be the
youngest case of non-congenital GBS that had been
reported recently.
CASE REPORT
This 3-month-old boy had suffered from anal
fistula with relapsing abscess formation since 15
days old. He admitted to our hospital for surgical
intervention. Fistulectomy was performed smoothly
under general anesthesia with intravenous Ketamin
injection. However, weakness of both lower limbs
Department of Pediatrics, Cathay General Hospital, Taipei2 Fu-Jen
57
Wen-Kan Feng
Kun-Long Hung
Chien-Hung Liu
occurred on the 4th post-operative day.
Under physical examination, the anterior
fontanel was open, flat and normal-sized. Both
lower limbs and right upper limb were flaccid.
Muscle power of both lower limbs was zero and
right upper limb was grade two plus. Deep tendon
reflexes of both lower limbs were absent and
decreased on right upper limb. The left upper limb
was normal. No cranial nerve was involved.
CSF study revealed cell count of 8 x 3/5,
protein level 61 mg/dl, and glucose level 51 mg/dl.
Microbiological evaluations included bacterial
cultures from CSF, urine, blood and feces grew no
aerobic or anaerobic pathogens. No virus was
isolated from CSF, urine, throat swab and rectal
swab. Serological studies included EBV IgM and
CMV IgM were negative, antigen of influenza virus
and parainfluenza virus were also negative. Chlamydia
IgM antibody was positive. A significant abnormality
of clinical neurophysiological studies was noted as
shown in Table 1. The amplitude of the compound
muscle action potential (CMAP) of bilateral peroneal
nerves and left tibial nerve was decreased. There
were also blockade of motor nerve conduction
velocities (MNCV). The F-wave study remained
normal. MRI study (Figure 1) of cervical-thoraciclumbar spine showed enhancement of ventral root
at lumber spines. These findings are compatible
with demyelinating polyneuropathy.
Two successive high doses of intravenous
immunoglobulin at 1g/kg/d infusion for two days
were given at once, then physicotherapy started.
Flaccidity of both lower limbs got little improvement
and right upper limb got much improvement at
discharge. He receives regular rehabilitation at OPD
and is able to stand with support at the age 1 year
and 3 month and walk with support six months
later.
DISCUSSION
Gullian-Barre syndrome(GBS) is an acute,
immune-mediateddemyelinatingpolyradiculoneuropathy.
It is characterized by a classical triad of progressive
motor weakness, areflexia and elevated cerebrospinal
fluid (CSF) protein without pleocytosis. Severe
GBS is associated with progressive motor disability
leading to respiratory failure.
GBS is known to be an immune-mediated
disorder resulting from the autoimmune antibodies
that cross-react with epitopes[1,2]on peripheral
nerves that damages the nerve roots and axons.
The autoantibodies may form in response to a
variety of antigenic stimuli, such as bacterial and
viral infections.
GBS can affect all ages and the annual incidence
Table 1. The amplitude of CMAP of bilateral Peroneal N. and left Tibial N. decreased.
There were blockade of MNCV over Peroneal N.
Nerve Conduction Study
Nerve
Latency(msec)
Voltage(mV)
Distance(cm)
NCV
F-Wave
Proximal
Proximal
Proximal
~ Distal
(m/sec)
(msec)
Distal
Distal
Distal
Lt. Peroneal
5.4
3.2
0.3
3.8
8.5
2.5
38.6
Lt. Tibial
5.7
2.7
1.5
2.7
10
3
33.3
26.5
3.5
28.8
25.7
Rt. Peroneal
Rt. Tibial
58
(Non- Pick Up)
6.1
2.1
0.6
0.5
11.5
Fu-Jen Journal of Medicine Vol.8 No.1 2010
GBS in young Infant
Fig 1. Figure. Post-contrast(right) enhanced T1-FLAIR MRI of the lumber spine showed
prominent enhancement of the ventral root(arrow)
is steadily increased with age at 2 to 4 per 100,000
populations. GBS is less common in children that
the annual incidence is about 0.1 case per 100,000
[3]
. Except the neonatal GBS[4]that caused by blocking
antibodies transmitted from mother with GBS
transplacentally, this GBS patient as young as three
months old in age is extremely rare. The diagnosis
of a flaccid young infant of GBS should be
differentiated to infant botulism, poliomyelitis,
myasthenia gravis and infant muscular atrophy [5].
Failure to recognize the correct diagnosis and
inappropriate treatment increased the morbidity.
Although the diagnosis of GBS is clinical,
electrophysiological studies are helpful. The most
frequent findings on electrodiagnostic testing in
GBS are proximal conduction block with increased
distal latency and generalized slowing. Additionally,
abnormal F wave studies were significantly
characteristic in early GBS[6]. MRI is suggested as
a supplementary diagnostic modality when the
clinical and electrophysiological findings are
equivocal. It revealed contrast enhancement in
varying degrees in the spinal nerve roots surrounding
the conus medullaris and extending the length of
the cauda equine, indicating radicular inflammation,
that anterior nerve roots enhanced more intensely
輔仁醫學期刊
第 8 卷 第 1 期 2010
than the posterior roots[7,8]. Recently, contrastenhanced spinal MR imaging becomes more
important in diagnosing GBS in children, especially
young infants.
More than 70% of the patients had reported
a preceding infection (usually virus-like respiratory
or gastroenteric illness[9]confirms in GBS.
Cytomegalovirus, EBV, mycoplasma pneumoniae
and Campylobacter jejuni infection are particularly
common in this syndrome[10]. Haemophilus influenzae
infection was reported a high incidence of GBS by
a Japanese group[10]. Other events as surgery,
vaccination, malignancy and pregnancy are frequently
associated with GBS. Our case experienced relapsing
anal fistula with abscess and surgical intervention
that might be the trigger factor.
It is very important that a child with suspected
GBS is managed as an emergency. So early diagnosis
and treatment in GBS especially in young patients
. Early treatment with intravenous immunoglobulin
and methylprednisolone was emphasized for shorten
the progressive course, presumably by limiting
nerve damage, and regain independent walking[1,
7,11]
.
59
Wen-Kan Feng
Kun-Long Hung
Chien-Hung Liu
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Gorson KC, Ropper AH, Muriello MA, et al:
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Govoni V, Granieri E, Epidemiology of the GuillainBarre syndrome. Curr Opin Neurol 2001;14:605-613.
Koga M, Yuki N, Hirata K, et al: Antecedent
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Fu-Jen Journal of Medicine Vol.8 No.1 2010
GBS in young Infant
發生於三個月嬰兒之 Guillain-Barre syndrome
馮文侃 1
洪焜隆 2,3,* 劉建宏 2
我們報告一名三個月大發生 Guillain-Barre syndrome (GBS)的嬰孩，這可能是文獻
發表最年輕的非先天性 GBS 病例，本報告分析臨床表現、檢查結果，包括脊椎 MRI 及
電生理學檢查，對正確早期診斷 GBS 很有幫助，早期發現和及時使用靜脈免疫球蛋白
治療，對病患的預後有很好的效果。(輔仁醫學期刊 2010；8 (1)：57-61)
關鍵字：Guillain-Barr 症候群，弛緩性麻痺，反射消失，嬰幼兒，免疫球蛋白
汐止國泰綜合醫院小兒科 1 國泰綜合醫院小兒科 2 輔仁大學醫學系 3
投搞日期：2010 年 03 月 01 日；接受日期：2010 年 03 月 31 日
*通訊聯絡作者: 電子郵件: [email protected]
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