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Pain and the Guillain-Barré syndrome in children

under 6 years old
Dang Khoa Nguyen, MD, Stacey Agenarioti-Bélanger, MD, and Michel Vanasse, MD, FRCP(C)

During a 15-year period, 29 children, under the age of 6 years, with acute
pital (Montreal) from 1981 to 1996. The
clinical series consisted of 29 children
Guillain-Barré syndrome were seen at our institution. A review of their (16 boys and 13 girls) under 6 years old
charts revealed that pain was a symptom in all patients and was present on with a diagnosis of GBS according to
admission in 79% of cases. Pain was often the most important symptom and published criteria.6 Electrophysiologic
led to misdiagnosis in 20 patients (69%). In 11 of these children, symptoms studies were performed in all patients,
were present for more than a week before the correct diagnosis was made. and CSF was analyzed for cell count
The most common pain syndrome was back and lower limb pain, present in and protein concentration in 28 of 29.
83% of patients. Pediatricians should consider Guillain-Barré syndrome in
CSF Cerebrospinal fluid
their differential diagnosis when faced with a child who has lower limb pain
GBS Guillain-Barré syndrome
and areflexia. (J Pediatr 1999;134:773-6) IVIG Intravenous immunoglobulin

Illustrative Case Histories

Guillain-Barré syndrome is a rapidly vated protein, and electrophysiologic PATIENT 1. Three days before admis-
progressive, acute, inflammatory poly- studies demonstrate acute neuropathy.2 sion, a 2-year-old boy presented to the
radiculoneuropathy. It typically pre- Most GBS cases are demyelinating, but emergency department with intermit-
sents as an acute ascending symmetri- axonal GBS is now well recognized. tent backache and abdominal pain after
cal weakness with areflexia. Although Plasmapheresis treatment halts pro- a transitory viral illness. He had refused
the etiology of GBS remains unknown, gression of GBS and hastens recovery. to walk during the painful episode but
it is generally accepted that an immune Intravenous immunoglobulin treatment resumed playing when the pain dissi-
response is directed against antigens on has also been shown to be effective.4 pated. The patient was diagnosed with
the myelin or axon, or perhaps both.1,2 Pain is a frequent feature of GBS5 in constipation, after an abdominal x-ray
An infection within the past month is children, as well as in adults. However, film was obtained, and was discharged
found in 50% to 70% of cases. The inci- its diagnosis may be more difficult in home. The following day, the boy re-
dence of GBS in children less than 17 children because the history and their turned to the emergency department.
years old is estimated at 0.8 per 100,000 cooperation during the neurologic ex- He was now irritable, had difficulty sit-
person-years.3 Biochemical analysis of amination are often limited. In our ex- ting alone, and refused to stand. He
cerebrospinal fluid usually shows ele- perience, this is particularly true in continued to complain of abdominal
younger children and frequently leads pain and occasional hip pain. He was
to misdiagnosis. To verify this theory, discharged with the same diagnosis of
From the Division of Neurology, Department of Pedi- we conducted a retrospective study constipation. The next day, the pain had
atrics, Hôpital Sainte-Justine and Université de Mon- and report a series of GBS in children progressed and spread to the lower
tréal, Montreal, Quebec, Canada. under 6 years old seen at Ste-Justine limbs. The patient was now anorexic
Submitted for publication Feb 20, 1998; revi- Hospital in Montreal. and asthenic. He complained of head-
sions received Sept 4, 1998, and Feb 4, 1999; ache and refused to walk. On physical
accepted Mar 8, 1999.
examination, the boy was noted to have
Reprint requests: Michel Vanasse, MD, Lab-
oratoire EEG, Hôpital Sainte-Justine, 3175
PATIENTS AND some neck stiffness and was hospital-
Côte Sainte-Catherine, Montreal, Quebec, METHODS ized because of suspected meningitis.
H3T 1C5 Canada. Peripheral reflexes were diminished,
Copyright © 1999 by Mosby, Inc. We reviewed the charts of all children but the patient moved all 4 limbs. Rou-
0022-3476/99/$8.00 + 0 9/22/98506 with GBS admitted to Ste-Justine Hos- tine laboratory test results were normal,

JUNE 1999

Table I. Pain in children with GBS Table II. Initial diagnoses (29 children) aching lower limb pain, exacerbated by
No. of patients 29 (100%) No. of straight leg raising. Twenty of the 23
Pain on admission 23 (79%) Diagnosis patients patients who had pain on admission
Pain during course of 29 (100%) presented with lower limb pain, and 4
GBS 9 other patients experienced limb pain
Myositis 5 during the course of illness. Myalgic
Distribution of pain
Meningitis 4 pain was often associated with this syn-
Lower limb pain 24 (83%)
Encephalitis 3 drome because the pain was also elicit-
Neck stiffness 11 (38%)
Cerebellar tumor 1 ed by local muscle palpation. Although
Headache 7 (24%)
Acute cerebellar ataxia 1 no typical pattern was found, headache
Visceral pain 3 (10%)
Discitis 1 was noted in 24% of the patients. Ab-
Dysesthetic extremity 1 (3%)
Femoral head avascular 1 dominal pain was rarely encountered
necrosis and was always associated with leg
Muscular dystrophy 1 pain. Among the children capable of re-
Myasthenia gravis 1 porting sensory symptoms other than
Constipation 1 pain, only one reported dysesthetic ex-
including creatine kinase levels. The Functional (somatization) 1 tremity pain. Eleven patients (38%)
lumbar puncture revealed an increased with leg pain on examination were also
CSF protein concentration of 1.04 g/L found to have neck stiffness.
with 11 white blood cells and 304 red Cranial nerve palsies were rare; only 2
blood cells. Results of a lumbar spine and complete recovery was noted on patients had ophthalmoplegia, and 1 had
scan and scintigraphy were normal. follow-up 3 months later. facial diplegia. Twenty-seven of 29
Electromyographic recordings on the (93%) patients had evidence of lower
10th day of the illness confirmed the limb weakness. On admission, 25 pa-
presence of a demyelinating and axonal RESULTS tients (86%) were found to have areflex-
polyradiculoneuropathy. During the ia in the lower limbs, and the remaining
hospitalization, the patient’s pain de- Between 1981 and 1996, 29 children patients had hyporeflexia (14%). Re-
creased slightly and his gait improved. (16 boys and 13 girls) under 6 years of flexes in the upper limbs varied from
He received physical therapy and re- age were diagnosed with GBS at our normoreflexia to areflexia. Six patients
covered completely in 2 months. hospital. Their mean age was 3 years had labile blood pressure (mostly transi-
and 8 months (range, 11 months to 5 tory hypertension), and 3 had arrhyth-
PATIENT 2. A 4-year-old boy was seen years and 9 months). The onset of mia (2 had bradycardia and 1 had nodal
a week before admission by an ortho- GBS was preceded by an infectious ill- escape rhythm). Three patients had
pedic surgeon because of a 2-week ness in 24 of 29 (83%) of the patients. apnea that resolved spontaneously, and
history of bilateral leg pain that wors- Patients had no prior significant or 2 had respiratory distress. One of these
ened on ambulation. A diagnosis of concomitant diseases, except for one required intubation and assisted ventila-
femoral head avascular necrosis was patient who had a history of apnea tion. She later died of acute respiratory
made. The patient was subsequently spells. However, he did not have any distress syndrome and septic shock at
brought to the emergency department respiratory symptoms. the age of 4 years. Most of our patients
because of persistent leg pain. He was Pain was evident or reported in 23 received only physical therapy because
now refusing to walk and had areflex- patients (79%) on admission (Table I). IVIG and plasma exchanges were not
ia with possible leg weakness that Typically, pain was the initial and most yet recognized treatments for GBS at
could not be better defined. The pres- important symptom in many of these the time of their diagnosis. One patient
ence of an elevated CSF protein con- patients. This led to a misdiagnosis in was treated with plasmapheresis, and 3
centration (0.91 g/L) in an acellular 20 patients (69%) (Table II). More- other patients received IVIG. All 4 ex-
spinal fluid combined with elec- over, as illustrated by the case histories, perienced improvement after treatment.
tromyographic findings of a demyeli- it may take several days or even weeks An additional patient received both
nating polyradiculoneuropathy were before the correct diagnosis of GBS is treatments: IVIG was given initially, re-
compatible with a diagnosis of GBS. made. In fact, 11 children had symp- sulting in some improvement; and
The patient’s condition was stable toms for a week or more before the di- plasmapheresis was given afterward, re-
during the first few days of hospital- agnosis of GBS was made. The promi- sulting in further improvement.
ization. After treatment with IVIG, he nent pain syndrome found in our The most commonly prescribed anal-
began to experience improvement, patient population was bilateral deep gesic was acetaminophen (38%), but 3


patients (10%) required codeine. One obtainable from children under 6 years due to the acute neurogenic changes
patient’s pain was refractory to both old. However, we suspect that it is com- seen in the weak muscles. It is notewor-
medications. This patient demonstrated parable to the pain experienced by thy that one patient had significant re-
a significant reduction in pain after adult patients. Ropper and Shahani11 duction of pain intensity with pred-
IVIG treatment. Another patient had analyzed prospectively the clinical fea- nisone and another with IVIG, both
marked pain reduction with prednisone. tures of pain in adult patients with after an unsuccessful trial with anal-
On follow-up, data were available GBS. They reported that the most gesics and nonsteroidal anti-inflamma-
for 21 of the 28 surviving patients. One common description of pain approxi- tory drugs. This effect may be because
patient had died as the result of an ac- mated the discomfort experienced after prednisone and IVIG have anti-inflam-
cident. The remaining 20 patients had a vigorous exercise (“charley horse”). matory properties at the nerve root
completely recovered, although 4 In another prospective study the most level.6 Reduction of pain with cortico-
showed clumsiness (especially when frequent pain syndrome was described steroids has been described by others.11
they were tired) and some had persis- as deep aching or throbbing pain in the Nevertheless, the drugs of choice in the
tent hyporeflexia. low back region with radiation into the management of back and limb pain re-
buttocks, thighs, and occasionally the main nonsteroidal anti-inflammatory
calves, which was exacerbated by drugs, acetaminophen, and opioid anal-
DISCUSSION straight leg raising.5 gesics; whereas dysesthetic extremity
Detection of limb weakness at the pain should be treated with drugs such
Patients with GBS typically present onset of GBS may be quite difficult in as carbamazepine, gabapentin, and tri-
with ascending motor paralysis with young children, especially in those cyclic antidepressants.5,12,13 Non-phar-
areflexia. However, retrospective and who are unable to express themselves. macologic modalities such as correct
prospective reports on adults with Young children refuse to walk if it is positioning and appropriate splinting of
GBS recognize pain as a common painful, and it is therefore difficult to paretic limbs, pressure-relieving mat-
symptom. In a recent study 89% of assess the presence of limb weakness. tresses, cold or warm pads, massage,
adults with GBS reported pain during Moreover, if they are able to walk, and regular passive physical therapy
the course of their illness. Moreover, their gait may be unsteady because of should not be forgotten.13
pain intensity on admission was found pain and may be misinterpreted as an In summary, this report, as well as
to be correlated with neurologic dis- ataxic gait. The lower limb pain may previous studies, strongly suggests that
ability on admission, but it was not a be so striking that it often leads to di- in GBS, in addition to the classic pre-
predictor of poor prognosis.5 Other in- agnostic difficulties. For instance, sentation with rapid onset of symmet-
vestigators also reported pain as a pri- lower limb pain could lead to a misdi- ric weakness, paresthesias, and are-
mary complaint in pediatric GBS, agnosis of myositis, nuchal rigidity flexia, lower limb pain (and sometimes
sometimes even preceding the onset of caused by meningitis, and unsteady back pain) may be major presenting
paralysis.7-10 Sakakihara et al7 noted gait caused by acute cerebellar ataxia symptoms, especially in children less
that limb pain was the initial symptom or cerebellar tumor. Furthermore, as than 6 years of age. Thus GBS should
in 53.4% of the children over 5 years noted by Royden Jones,10 severe be considered in the differential diag-
old and in 24% of children under 5 lower limb pain with significant irri- nosis of any child presenting with
years old. In the latter group limb pain tability, headache, and vomiting can acute extremity pain associated with
eventually occurred in 52% of the pa- evoke a misdiagnosis of encephalitis. hyporeflexia or areflexia.
tients during the course of the disease. The etiology of back and lower limb
Royden Jones9 reported that pain was pain in GBS is probably multifactorial.
the presenting symptom in 20% of chil- Acute back and leg pain exacerbated
dren, especially in those less than 10 by straight leg raising provides indirect REFERENCES
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the course of illness, this number in- primary rami. However, Ropper and Wiederholt WC. Guillain-Barré syn-
drome: clinicoepidemiologic features
creased to 83%. A more detailed de- Shahani11 suggested that the cause of and effect of influenza vaccine. Arch
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