Guillain-Barré syndrome is a rare neurological disorder where the immune system attacks the peripheral nervous system, causing muscle weakness and lack of reflexes. It ranges in severity from mild weakness to nearly complete paralysis. Most cases are preceded by a respiratory or gastrointestinal infection. Treatment involves plasma exchange or IVIG for severe cases to reduce antibodies, as well as monitoring for respiratory and autonomic issues. With treatment, most children recover fully within 2-3 months.

1. Guillain-
Barré syndrome

2. CONTENTS
Overview
Etiology
Epidemiology
Clinical Manifestations
Variant forms
Diagnosis
Treatment
References

3. INTRODUCTION
 Guillain-
Barré syndrome is a
rare neurological
disorder in which the
immune system
mistakenly attacks
part of the peripheral
nervous system
 GBS can range from a
very mild case with
brief weakness to
nearly devastating
paralysis

4. ETIOLOGY
About two-thirds of GBS patients experience symptoms of an upper respiratory or
gastrointestinal tract infection up to 6 weeks prior to onset of GBS.
Associated pathogens
 Campylobacter jejuni (most common disease associated with GBS)
 Cytomegalovirus (CMV)
 HIV
 Influenza
 Zika virus
 Epstein-Barr virus
 SARS-CoV-2
 Mycoplasma pneumoniae
Vaccination
 There have been reports of small increases in incidence after administration of certain
vaccines.
.

5. EPIDEMIOLOGY
 GBS occurs worldwide with an annual incidence of 0.34 to 1.34 cases
per 100,000 persons aged 18 years or less.
 While all age groups are affected, the incidence is lower in children
than in adults. The incidence increases by approximately 20 percent
with every 10-year increase in age beyond the first decade of life.
 GBS occurs rarely in children younger than two years of age but can
occur even in infants. There is a case report of congenital GBS in the
neonate of an affected mother
 Males are affected approximately 1.5 times more often than females
in all age groups.

6. The classic presentation of GBS begins with paresthesia in the toes and
fingertips followed by lower extremity symmetric or modestly
asymmetric weakness that may ascend over hours to days to involve the
arms and, in severe cases, the muscles of respiration.
The typical clinical features of GBS are progressive, mostly
symmetric or modestly asymmetric muscle weakness and absent or
depressed deep tendon reflexes
The predominant symptoms of GBS at presentation in children are pain
and gait difficulty.
In preschool-aged children, the most common symptoms are refusal to
walk and pain in the legs
Cranial neuropathy most commonly affects the facial nerves, causing
bilateral facial weakness.
CLINICAL MANIFESTATIONS

7. Variant forms of guillain-barré syndrome
 Historically, GBS was considered a single disorder. It is now known to be a
heterogeneous syndrome with several variant forms.
 Acute inflammatory demyelinating polyradiculopathy (AIDP) is the most
common type.
 Each type of GBS has distinguishing clinical, pathophysiologic, and pathologic
features.
 The classic presentation of ascending paralysis is most common, but a number
of atypical variants present with local or regional involvement of particular
muscle groups or nerves
 Several have prominent cranial nerve involvement, including Miller Fisher
syndrome (MFS), Bickerstaff brainstem encephalitis, polyneuritis cranialis, and
pharyngeal-cervical-brachial weakness.

8. VARIANT FORM CHARACTERISTICS
Acute inflammatory demyelinating
polyneuropathy
Prototype of GBS and is the most common form
Acute axonal neuropathies More severe course, involvement of motor or motor and sensory
nerves, and an electrophysiologic pattern suggesting axonal
damage
Acute motor axonal neuropathy Pure motor form of GBS and is associated with a
preceding Campylobacter jejuni infection.
Acute motor-sensory axonal
neuropathy
Resembles the motor axonal variant but has more sensory
symptoms. The course tends to be prolonged and severe
GQ1b syndromes Eye movement abnormalities and ataxia rather than limb
weakness and numbness.
Miller Fisher syndrome characterized by external ophthalmoplegia, ataxia, and muscle
weakness with areflexia. Incomplete forms include acute
ophthalmoplegia without ataxia and acute ataxic neuropathy
without ophthalmoplegia

9. Physical examination findings
 Physical examination typically reveals symmetric weakness with diminished or absent reflexes
and gait abnormalities.
 Sensory symptoms are usually "positive" (eg, pain or paresthesia, reflecting nerve irritability)
rather than "negative" (eg, loss of sensation).
Clinical course
 The clinical course of GBS in children is shorter than in adults and recovery is usually
more complete.
 In patients who did not require mechanical ventilation, the median time to recovery of
independent walking was 43 to 52 days in children compared with 85 days in adults

10. DIAGNOSIS
 The initial diagnosis of GBS is based upon the clinical presentation.
 The weakness can vary from mild difficulty with walking to nearly complete paralysis of
all extremity, facial, respiratory, and bulbar muscles. However, some GBS variants present
with local or regional involvement of particular muscle groups or nerves, and several
have prominent cranial nerve involvement; the variable initial presentations can hinder
early diagnosis.
In patients with GBS, lumbar puncture often reveals an elevated CSF
protein with a normal CSF white blood cell count. This finding, known
as albuminocytologic dissociation, is present in 50 to 66 percent of
patients with GBS in the first week after the onset of symptoms and
≥75 percent of patients in the third week .
The elevated protein may be due to increased permeability of the
blood-nerve barrier at the level of the proximal nerve roots. A
normal CSF protein is found in one-third to one-half of patients
when tested earlier than one week after symptom onset

11. Electrodiagnostic studies
 Electrodiagnostic studies are the most specific and
sensitive tests for diagnosis of GBS and establish the
underlying pathophysiology as either demyelinating
or axonal.
 Electrodiagnostic studies are uncomfortable and can
be technically challenging in small children
 In the demyelinating forms of GBS,
electrodiagnostic studies demonstrating
abnormalities including motor conduction block,
slowing of motor and sensory nerve conduction,
temporal dispersion, and prolonged distal
latencies.
 In the axonal forms of GBS, nerve conduction
studies showing decreased amplitude of motor
responses, with normal conduction velocities

12. Magnetic resonance imaging
 Spinal MRI with administration of gadolinium frequently shows enhancement of the
spinal nerve roots and cauda equina during the first weeks after the onset of GBS in
children. In some cases, nerve root enhancement may be delayed and observed only
on a repeat MRI.
 Nevertheless, spinal nerve root enhancement is a nonspecific finding that can be
seen in a variety of disorders. Thus, the diagnosis of GBS cannot be made by MRI
alone.
Antibodies
 Immune reactions directed against epitopes in Schwann cell surface membrane
or myelin can cause the acute demyelinating form of GBS, while immune reactions
against epitopes contained in the axonal membrane cause the acute axonal
forms of GBS.
 Commercially available testing for serum IgG antibodies to GQ1b is useful for the
diagnosis of Miller Fisher syndrome, having a sensitivity of 85 to 90 percent.

13.  Progressing weakness
 Worsening respiratory status or need for mechanical ventilation
 Significant bulbar weakness
 Inability to walk unaided
Indications for treatment

14.  During the initial phase of GBS, all patients require close monitoring of motor, autonomic
(ie, BP, HR, and sphincter function), and respiratory function.
 Serial PFT should be every four hours at the bedside and closely monitored and observed
for fatigue and other clinical signs of impending respiratory muscle failure. Patients
should be electively intubated if clinical evaluation or pulmonary function tests suggest
impending respiratory failure.
 Mortality is often due to complications such as nosocomial infection, acute respiratory
arrest, deep venous thrombosis with pulmonary embolism, and pneumothorax.
TREATMENT
 Nutritional needs should be addressed early in the disease course. Orogastric tube
feeding, gastrostomy, or parenteral nutrition is often necessary
 The patient's position should be changed frequently for comfort and to avoid skin
breakdown. Intermittent-pressure leg boots are used in the intensive care setting to
prevent deep vein thrombosis
 Physical therapy, occupational therapy, and social services should be involved early.

15. Ventilatory status
 Autonomic dysfunction is a well-recognized feature of GBS and is a significant source of
mortality. Consequently, close monitoring of blood pressure, fluid status, and cardiac rhythm
are essential to the management of these patients.
 Care must also be taken when vasoactive or sedative drugs are used, because GBS-related
dysautonomia may exaggerate the hypotensive responses to these drugs.
Autonomic status
 Approximately 10 to 20 percent of children with GBS require mechanical ventilation for
respiratory failure. The need for tracheal intubation should be anticipated so that it can
be performed as an elective procedure
 Children with a vital capacity approximately one-half the normal value for age or ≤20
mL/kg of body weight generally progress to require ventilatory support. Sedation and
neuromuscular blockade should be avoided where possible in ventilated patients
because they obscure the course of the illness.

16. Plasma exchange
 Plasma exchange is recommended for those patients who have rapidly progressing weakness,
worsening respiratory status, are unable to walk unaided, require mechanical ventilation, or have
significant bulbar weakness.
 As a result of the cost, risk, and discomfort to the patient, plasma exchange generally is not used in
very young children, for ambulatory patients with mild disease, or for patients whose symptoms
have stabilized.
 The mechanism is thought to be removal of antibodies directed against nerves from the
circulation.
Intravenous immune globulin
 Randomized trials in children suggest that IVIG shortens the time to recovery compared with
supportive care alone and it is thought to involve suppression of inflammatory and immune-
mediated processes.
 The total dose of IVIG in children is 2 g/kg, given as 1 g/kg for 2 days or 400 mg/kg for 5 days.
 IVIG is preferred to plasma exchange in children because of the relative safety and ease of
administration
IMMUNOTHERAPIES

17. REFERENCES
 Guillain-Barré syndrome in children: Epidemiology, clinical features, and
diagnosis - Uptodate Free. (n.d.). https://pro.uptodatefree.ir/show/6235
 Guillain-Barré syndrome in children: Treatment and prognosis - Uptodate
Free. (n.d.). https://pro.uptodatefree.ir/show/6204
 Guillain-Barré Syndrome. (n.d.). National Institute of Neurological Disorders
and Stroke. https://www.ninds.nih.gov/health-information/disorders/guillain-
barre-syndrome
 Guillain-Barré syndrome - Knowledge @ AMBOSS.
(n.d.). https://www.amboss.com/us/knowledge/guillain-barre-syndrome/