Prevalence and outcomes of Guillain-Barré syndrome among pediatrics in Saudi Arabia: a 10-year retrospective study

Asiri, Safiyyah; Altwaijri, Waleed A; Ba-Armah, Duaa; Al Rumayyan, Ahmed; Alrifai, Muhammad T; Salam, Mahmoud; Almutairi, Adel F

Guillain-Barré syndrome (GBS) is defined as a group of clinical syndromes with acute onset of peripheral neuropathy -axonal or demyelinating -secondary to an immune-mediated process. It usually presents itself with a progressive paralysis that can involve the autonomic, bulbar, and respiratory systems. 1 GBS has four subtypes, which are cute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and Miller Fisher syndrome. The most common subtype of these four is acute inflammatory demyelinating submit your manuscript | www.dovepress.com Dovepress Dovepress 628 asiri et al polyradiculoneuropathy (AIDP) that constitutes 85%-90% of GBS cases, followed by the axonal subtypes (30%-47%) and Miller Fisher syndrome (5%). [2] [3] [4] The annual incidence of GBS as reported in one 10-year study was 0.42 cases per 100,000 persons. 5 Higher incidence of GBS has been reported in younger adults and the elderly aged 50 years and older. 6,7 A 6-year regional study revealed that the peak age of patients diagnosed with GBS was between 41 and 60 years with a median age of 47 years. 8 GBS is in fact the most common form of acute flaccid paralysis among children (25.9%-51%), 9 and its annual incidence in a pediatric population ranged from 0.34 to 1.34/100,000. 1, 10 Reports from the Arab countries showed an incidence of 1.33-1.7 per 100,000 children. 11, 12 Almost 75% of GBS patients are males, 4 and higher rates of GBS are reported during the winter and early summer seasons. 3, 8, 13 GBS has been reported to be either preceded or triggered by previous infections or an immune-mediated process, especially with axonal and peripheral nerve demyelinations. 8 For instance, the most common types of preceding infections were upper respiratory tract infections of viral etiologies, followed by gastrointestinal infections. 4, 8 Campylobacter jejuni was the most common pathogen associated with axonal degeneration and slower recovery in GBS patients. Other pathogens identified included Epstein-Barr virus, cytomegalovirus, Mycoplasma pneumoniae, Hemophilus influenzae, 8 and Zika virus. 14, 15 It was also reported that GBS was observed after vaccination for rabies and swine influenza. 8 The clinical presentation of GBS is usually a progressive flaccid weakness that can proceed to complete paralysis, often witnessed in 74% of the cases. In addition, bulbar involvement, absence of reflexes, respiratory muscle involvement, back and lower limb pain, bilateral facial or ocular weakness, and paresthesia are manifested too. 8, 16 Moreover, autonomic disturbances which cause arrhythmias and blood pressure fluctuations can also be observed in 4%-17% of cases which eventually increases the rates of morbidity and mortality among GBS patients. 4,13 A recent study indicated that the risk of mortality associated with GBS is mostly prevalent among the elderly and severely affected patients, during which the risk increases in the recovery phase. 17 GBS progresses rapidly and reaches the peak within 2 weeks before the patients undergo a plateau phase that persists from days to several months, after which they finally recover. 13 In general, the prognosis of patients depends on the varying signs and symptoms of GBS which made clinicians and researchers classify GBS by the distinct subtypes.