Acute Guillain Barre Syndrome in childhood: clinical, electrophysiological and immunological study

ABSTRACT

Acute Guillain Barre Syndrome [acute GBS] is one of the leading causes of acute flaccid paralysis [AFP] in children. The syndrome has been recognized as a heterogeneous disorder with different subtypes, by clinical, electrophysiological and pathologic criteria. The disease may present with a wide variety of clinical presentations as well as prognostic outcome. The study was conducted on all children with acute GBS attending Alexandria University Children's Hospital during the period from September 2000 to September 2001 for the purpose of characterization of acute GBS in Egyptian Children and its relation to presumed infection with Cytomegalovirus [CMV] or Epstien-Barr virus [EBV]. All clinical, laboratory and electrophysiological data of the children with acute GBS who are admitted to the hospital during the period of the study were evaluated. Serum IgM titer against CMV and EPV were assessed and correlated with other patients'data. Thirty five children were admitted to the hospital during that period. Sixty age and sex matched healthy children were included as controls for studying IgM titre of EBV and CMV. Affected children were 22 boys and 13 girls. Their ages ranged between one and 10 years. Eight cases did not have preceding illness before the disease. The initial symptom was motor in 11 cases, sensory in 2 cases and mixed in 22 cases. Ascending paralysis was dominant in all cases except three who had descending paralysis. Only five cases did not exhibit severe pediatric GBS [grade 4 or 5] according to the functional grading system. Twenty nine cases exhibited the major electrophysiological type of acute inflammatory demyelinating polyneuropathy [AIDP]. Albuminocytologic dissociation was encountered in 25 cases. Two cases died in the acute phase, one was left with residual neurological deficit while the rest recovered completely. There was a statistically significant difference between cases and controls as regards the IgM titre against CMV virus [P < 0.05] while IgM titres against EBV were not statistically different from the control group. In addition children with presumed Prior CMV infection tended to be younger in their age significantly more than those with presumed EBV infection [r = 0.382, P = 0.003]. The only significant association was the relation between the type of initial symptoms versus the IgM titre against CMV and EBV where sensory symptoms were prominent more significantly among cases with presumed CMV infection than those with presumed EBV infection [P<0.005]. Acute GBS demonstrates diverse clinical, electrophsyiolgical and laboratory dilemma. Clinical and electrophysiological criteria remain the most important prognostic factors. Despite its initial grave presentation, early institution of therapy using intra-venous immunoglobulin [IVIG] or plasma exchange ensures dramatic recovery in most of cases. The underlying etio-pathologic mechanisms are still not properly understood. Their clarification might have its impact on better delineation of clinical presentations, outcome and prevention