Diagnosis and management of Guillian Barre Syndrome in children and adolescents

1- The diagnosis of GBS should be based on clinical characteristics, a relevant constellation of clinical findings, and the exclusion of other relevant potential causes.

Requirements for the diagnosis of sensory- motor or motor GBS are:

 • Bilateral flaccid paralysis of extremities

• Absent or decreased deep tendon reflexes in affected limbs

 • Progressive worsening for no more than 4 weeks  (strong recommendation)

2- Diagnostic lumbar puncture with subsequent CSF protein analysis and a cell count is done when the diagnosis is uncertain or when an alternative diagnosis needs to be excluded. Results supportive of GBS are an increased CSF protein concentration, and a normal or only slightly increased CSF white blood cell count (usually <5 cells/μL, rarely 5–50 cells/μL (known as cyto- albuminological dissociation) . A CSF white blood cell count of >50 cells/μL should raise suspicion for alternative diagnoses (strong recommendation)

We suggest that for diagnostic certainty in the event of an initially-normal CSF finding, a lumbar puncture may be repeated 7-10 days after the first symptoms appear, if it is deemed necessary due to unclear clinical or electrophysiological results. (Good practice statement)

3-  Electrophysiological examination for confirming GBS diagnosis is recommended   as it is necessary for obtaining the highest level of diagnostic certainty ( Strong recommendation). If the diagnostic tool is not available, refer the patient to an appropriate clinical centre.

We suggest that for confirmation of diagnosis in the event of an initially normal electrophysiological finding, the procedure may be repeated 1 or 2 weeks later if it is deemed necessary because of other unclear data. (conditional recommendation)

4- Performing MRI of brain and spinal cord is done when the available findings are ambiguous or if there is evidence of pathology at either the spinal level (e.g., bladder dysfunction at the onset of disease, definable motor or sensory lesion) or central level (encephalopathy, pyramidal signs) (strong recommendation)

5- Microbiological and serological diagnostics may be carried out as the diagnosis of GBS is more likely if there is a history of recent infection. Asking about these antecedent events may increase diagnostic confidence and is generally more useful when present than absent (Conditional recommendation)

6- It is not advised to do  routine testing for serum antibodies against gangliosides in most patients with motor-sensory GBS, because of their low–moderate sensitivity and frequent delay in reporting results of antibody assays beyond the therapeutic window (good practice statement)

If  MFS (or MFS spectrum) is suspected, test for serum antibodies against GQ1b  can be done especially when there is some clinical doubt and test results can be obtained within reasonable time (Conditional Recommendation)

7- Starting IVIg or plasma exchange as soon as possible is recommended in patients unable to walk unaided (GBS- DS grade 3 or more) if still within the first 2 weeks from onset of weakness ( strong recommendation)

• Using the most frequently used and proven effective standard course of IVIg (0.4 g/kg/day for 5 days) rather than a low- dose (0.4 g/kg/day for 3 days) or a high- dose (0.4 g/kg/day for 6 days) regimen or a 2- day regimen (1 g/kg/day) or using four to five plasma exchanges over 1– 2 weeks with a total exchanged volume of 12– 15 L (good practice statement)

We advise to start IVIg (or PE four to five exchanges) in patients who are still able to walk unaided (GBS- DS grade 2) within 4 weeks from onset of weakness, but who have a fast rate of deterioration, a risk of requiring ventilatory support, swallowing difficulties, autonomic disturbances or poor prognostic factors (good practice statement)

9- We don’t recommend the use of oral corticosteroids or IV methylprdnisone (IVMP) for the treatment of GBS. (strong recommendation)

10- We don’t recommend using eculizumab for treatment of GBS.(conditional recommendation) because of  the lack of demonstrated efficacy, the known adverse effects (all patients had some adverse effects) and the high cost currently.

11- We advise  using a prognostic model at hospital admission to quantify the risk of requiring mechanical ventilation. This can be quantified using the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS)( table4) OR

Using the following risk factors for requiring mechanical ventilation during hospital admission:

Rapid progression of limb weakness during hospital admission

GBS- DS grade 4 (unable to walk 5 m even with aid)

Neck flexion, facial, or bulbar weakness

The inability to cough

Autonomic instability such as fluctuations in blood pressure or heart rate. (good practice statement)

• We advise regularly assessing any decline in respiratory function by measuring forced vital capacity (FVC), and single breath count (SBC). Fig (good practice statement)

12- Changing the diagnosis from GBS to A-CIDP is suggested after a few weeks from onset in some patients initially diagnosed with GBS, especially if the patient worsens after initial improvement or stabilization (known as a treatment-related fluctuation, TRF), or presents as mild or slowly progressive GBS and continues to worsen.(conditional recommendation)

A-CIDP is more likely if there are three (or more) TRFs.

A-CIDP cannot be confirmed unless there is further worsening at least 8 weeks after onset.

In case of a TRF, consider re-treatment with IVIg or PE.(good practice statement)