E. Steve Roach, MD, Editorial Board Member, Pediatrics in
Review
Most of us learned the basic clinical manifestations of
Guillain-Barré syndrome in medical school and likely have encountered at least
a few children with the condition during residency or in practice. Four January
2018 Pediatrics in Review articles focus on this immune-mediated peripheral
neuropathy, emphasizing some of its less common clinical patterns that are apt
to be less familiar to most physicians.
In their In Brief on the topic, Chung and Deimling summarize
the classic manifestations of Guillain-Barré syndrome. This is an acute-onset,
immunemediated peripheral neuropathy that likely is triggered by an infection
or another immune stimulus. Guillain-Barré syndrome classically presents as an
acute-onset, rapidly progressive, flaccid weakness starting in the legs,
associated with diminished or absent tendon reflexes. The clinical severity of
Guillain-Barré syndrome varies, but at its worst, it can lead to respiratory
insufficiency and death. The cerebrospinal fluid (CSF) typically exhibits an
elevated protein level but not an excess of white blood cells, although this
pattern is not always present initially, and occasionally individuals have a
mildly elevated white blood cell count.
In Index of Suspicion Case 3, Bassal and Lupo present an 11-year-old boy with a 2-day
history of gait unsteadiness and diplopia. His examination revealed initially
diminished then absent tendon reflexes, dysmetria, and partial dysfunction of
cranial nerves III and VI. His extremity muscles were not weak. Results of
imaging and laboratory investigations were normal except for elevation of antiganglioside
anti-GQ1b immunoglobulin G antibodies.
The differential diagnosis of acute ataxia is extensive. This boy’s diagnosis is Miller Fisher
syndrome. Generally considered to be a milder variant of Guillain-Barré
syndrome, Miller Fisher syndrome features a triad of areflexia, ataxia, and
cranial neuropathy. Most individuals
with Miller Fisher syndrome recover completely, with or without therapy.
Nonetheless, prompt recognition of Miller Fisher syndrome may prevent
unnecessary diagnostic tests, and occasionally patients progress to the riskier
Guillain-Barré syndrome.
In Index of Suspicion Case 1, Shah et al describe a
5-year-old boy with right leg pain and difficulty walking for 5 days. His right
leg was weak and tender to palpation. Tendon reflexes were absent in the right
leg. Left leg strength was normal, but even on the left his reflexes were
diminished. This presentation might suggest a localized joint problem, but hip
magnetic resonance imaging (MRI) findings were normal. Weakness in 1 limb would
once have suggested poliomyelitis, which often presents in this manner. More
recently, asymmetrical flaccid paralysis due to acute myelitis has been
described.
This boy too proved to have a variant of Guillain-Barré
syndrome. His CSF revealed increased protein and immunoglobulin G levels. An
MRI of the thoracic and lumbar spine documented asymmetrical contrast
enhancement of ventral and dorsal nerve roots, and a nerve conduction velocity
test confirmed axonal polyneuropathy in both legs. Back or leg pain is common
in children with Guillain-Barré syndrome. The weakness of Guillain-Barré
syndrome is typically symmetrical, but as this child illustrates, it isn’t always.
In Index of Suspicion Case 2, Bashir and Aarons describe a
2-year-old girl who refused to walk for 3 weeks. Based on the tests and
treatments initially recommended, her symptoms at first were attributed to
musculoskeletal disease. When examined, she had no tendon reflexes in the legs
and could not dorsiflex the right foot. Movement of her legs seemed to elicit
pain. Eventually an MRI demonstrated abnormal spinal nerve roots, and a lumbar
puncture confirmed an elevated CSF protein level in the absence of inflammatory
cells.
Persistent, painful dysfunction without complete paralysis
has a broad differential diagnosis that includes several conditions that are
far more common than Guillain-Barré syndrome. The symptoms of toxic synovitis
usually improve with the use of nonsteroidal anti-inflammatory agents and heat.
Septic arthritis, diskitis, and osteomyelitis were eliminated because of her
lack of fever and normal laboratory test results. Postinfectious myositis could
have explained her signs and symptoms. A spinal cord lesion, such as a tumor or
epidural empyema, must be considered in this setting.
It is important to recognize not only classic Guillain-Barré
syndrome but also its less obvious patterns. Children often improve
spontaneously or in response to intravenous immunoglobulin therapy or
plasmapheresis, and as is often the case, earlier treatment is generally more
effective. There are potentially life-threatening complications of
Guillain-Barré syndrome, including respiratory failure and autonomic
dysfunction with systemic hypertension or cardiac arrhythmias. These 4 recent
articles together provide an excellent overview of both the classic and the
atypical manifestations of the syndrome.
Chung A, Deimling M. Guillain Barré syndrome. Pediatr Rev.
2018;39(1):53–54
Bassal F, Lupo P. Case 3: Ophthalmoplegia and Unsteady Gait
in an 11-year-old
Boy. Pediatr Rev. 2018 Jan;39(1):39.
Shah S, Diaz-Medina G, Chen J. Unilateral leg pain and
difficulty walking. Pediatr Rev. 2018;39(1):36–37
Bashir A, Aarons E. Refusal to walk in a 2-year-old girl.
Pediatr Rev. 2018;39(1):38
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