Epidemiological and clinical aspects of Guillain-Barré syndrome and its variants

Abstract

Background: Guillain-Barré syndrome (GBS), an acute polyradiculoneuropathy that occurs because of an abnormal inflammatory response in the peripheral nervous system, is clinically characterized by acute flaccid paresis and areflexia with or without sensory symptoms. This syndrome can lead to disabling or even life-threatening sequelae. Objective: This study aimed to present the clinical and epidemiological aspects of GBS in patients admitted to a tertiary-level hospital in the Federal District between January 2013 and June 2019. Methods: In this observational, cross-sectional and retrospective study, medical records of patients diagnosed with acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy or acute axonal motor-sensitive neuropathy based on electromyographic findings were included, and clinical data were collected retrospectively. Results: A total of 100 patients (63 males and 37 females; ratio, 1.7:1) aged 2–86 years (mean, 36.4 years) were included. The mean annual incidence rate of GBS was 0.54 cases/100,000 inhabitants, with 52 and 49% of the cases occurring between October and March (rainy season) and between April and September (dry season), respectively. The proportions of patients showing each GBS variant were as follows: demyelinating forms, 57%; axonal forms, 39%; and undetermined, 4%. The mean duration of hospitalization was 8–15 days for most patients (38%). During hospitalization, 14% of the patients required mechanical ventilation and 20% experienced infectious complications. Conclusion: The findings indicate that there was an increase in the incidence of GBS during the rainy season. Moreover, we did not observe the typical bimodal distribution regarding age at onset.

Diagnostic accuracy and mechanical ventilation risk in Guillain-Barré syndrome

“…This difference was 50% greater than that found by IGOS consortium in its multicenter study (1.5:1), but which in any case points to the male gender as a risk factor 12 . In other Brazilian studies this male predominance in the number of cases was considerably lower, around 55-57% of the cases were men 19,20 , except for an observational cross-sectional study that showed similar ratio (1.7:1) 21 . In a Peruvian longitudinal report with 92 patients the male/female ratio was even higher (…”

“…Probably this might be due to the inclusion of pediatric patients, a population in which GBS is less common [18][19][20][21] . The classic sensorimotor form of GBS was the most common (57.6%).…”

“…Although some Brazilian epidemiological studies until then had similar patterns of predisposing factors for GBS 19,20 , there is some evidence of regional variations within Brazilian national territory. In a recent hospital-based epidemiological study in Federal District, the most common immune trigger was gastroenteritis (26%) 21 . Our study observed that the most prevalent immune trigger was, also, gastroenteritis (30.3%) followed by upper airways infection (15.2%) and vaccination (12.1%).…”

Campylobacter jejuni and Postinfectious Autoimmune Diseases: A Proof of Concept in Glycobiology

“…This and subsequent studies, including one of children and young adults from northern China who presented with acute paralytic disease and motor fiber degeneration, led to the characterization of the acute motor axonal neuropathy (AMAN) variant of GBS . Another axonal variant of GBS, acute motor and sensory axonal neuropathy (AMSAN), , presents with axonal degeneration of both sensory and motor fibers, but only minimal demyelination and lymphocytic infiltration, and has increased severity, slower recovery, and worse prognosis. ,,− AMAN and AMSAN are characterized by an autoimmune attack against the neuronal axolemma, immunoglobulin deposition, complement activation, and macrophage infiltration in the periaxonal spaces, but little lymphocytic infiltration. ,,,,,,− The proportion of GBS patients who develop different variants varies considerably around the world. ,, …”

“…45,50,66,68,69,74,80−84 The proportion of GBS patients who develop different variants varies considerably around the world. 17,85,86 MFS, first recognized by James Collier in 1932 87,88 and later reported by Charles Miller Fisher in 1956 as a variant of GBS, 89 is characterized by the triad of ophthalmoplegia, ataxia, and areflexia, or the absence of deep tendon reflexes. 90 It usually affects cranial nerves III, IV, and VI.…”