**1. Introduction**

Guillain Barré Syndrome (GBS) is an acute demyelinating polyradiculoneuropathy, of autoimmune origin, which presents with various heterogeneous clinical variants [1]. In most cases there is an infectious picture prior to the clinical manifestations of GBS (acute paralysis, weak limbs and inability to ambulation) [1, 2].

#### *Demyelination Disorders*

In 1857, Landry described the first cases of GBS. The affected patients presented with predominantly ascending motor paralysis, respiratory failure and death [3, 4]. In 1916, Guillain - Barré Strol [4] demonstrated that these patients presented motor deficits and areflexia, but without sensory affection and with albuminocytological dissociation as part of the integral diagnosis of the SGB [4].

In 1990, Asbury and Comblath established the electrodiagnostic criteria for GBS, characterized by a delay in the conduction velocity of two or more motor nerves [5].

At present, GBS is the most frequent cause of flaccid paralysis in previously healthy children [6]. Worldwide, the annual incidence is 0.6 to 2.4 cases per 100,000 inhabitants, in any age group, affecting both genders with an H relationship./M 1.5: 1 [7].

The main infectious agent reported in outbreaks of GBS is C. jejuni [8]. Other infections associated with GBS are: cytomegalovirus (CMV), Epstein–Barr virus, Influenza A virus, Mycoplasma pneumoniae and Haemophilus influenzae [9].

GBS is defined clinically, pathologically, and electrophysiologically as an acute inflammatory demyelinating polyneuropathy. According to the characteristics of the nerve conduction studies, it was observed that GBS is characterized by; slowing of conduction speeds, conduction block, delayed latencies and/or scattered responses; but over time, the evidence from several studies indicated that there are different clinical, serological, and electrophysiological characteristics in each of the GBS variants.

The following describes in detail the pathophysiology and clinical picture that characterizes each of the GBS variants:


### *Recurrence of Guillain Barré Syndrome in Patient Pediatric with Presentation of Two Different… DOI: http://dx.doi.org/10.5772/intechopen.96358*

The clinical picture is not severe and depends on the extent of axonal injury. Regarding the clinical examination of the patient, the tendon reflexes are preserved and he may even have hyperreflexia. Distal limb involvement shows rapid and complete recovery [18, 20]. Therefore, regardless of GBS variants, axons are the main target for autoimmune injury [21].

Regarding medical treatment, the effect of immunotherapy in GBS has been studied for many years (mainly in studies of randomized controlled trials), establishing that the use of intravenous immunoglobulin (IVIG) and plasma exchange (plasmapheresis) they are effective [22]. The cornerstone of GBS treatment in pediatric patients is based on the use of intravenous immunoglobulin. The treatment can be applied in 2 different therapies; 1st therapeutic (most effective): immunoglobulin dose (2 gr/kg of body weight) administered in two days at 1 gr/kg per day. The 2nd therapy: dose of immunoglobulin at 0.4 gr/kg of body weight administered in 5 days [2, 23].

The specific indications for the use of IVIG in GBS are; rapid progression of muscle weakness, respiratory failure or ventilatory mechanical support, involvement of the bulbar or cranial nerve and inability to ambulation [2]. Plasmapheresis has shown the same efficacy as immunoglobulin but constitutes a more invasive treatment, being reserved only for cases of intolerance or poor response to intravenous immunoglobulin administration [24].

The severity of the clinical picture is important as a prognostic factor in GBS. About 40% of affected children have an inability to ambulate during the acute phase. In severe cases, approximately 25% of patients will require special supports in Intensive Care Units due to the need for support with artificial ventilation secondary to dysautonomias [12, 25–27].

The authors present the case of a 7 year old girl with severe and atypical Guillain Barré syndrome, describing the clinical course and associated complications in a recurrence of GBS in pediatrics.
