How Important it is to Differentiate AMAN from AIDP in Childhood GBS? A Clinician’s Perspective

Excerpt

Guillain-Barré syndrome (GBS) is a monophasic acute immune-mediated polyradiculoneuropathy. It is now considered a heterogenous disorder encompassing immuno-pathologically distinct entities such as acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), Miller Fisher syndrome (MFS) and other rare variants. The response to treatment and final outcome is majorly determined by the disease subtype and clinical severity. Knowing the prevalence of the various subtypes in our geographical region will not only provide important epidemiological data but will also aid in monitoring, treatment and prognostication of individual cases. Gupta and colleagues have tried to answer this relevant question in their prospective study in 57 children with GBS [1]. In backdrop of discordant literature, no consensus exists regarding the most common subtype of GBS in north India. Gupta et al., observed AIDP and AMAN in equal proportions [1]. Kalita et al. reported AIDP as the most common subtype (three times more common than AMAN) while AMAN was the most common subtype identified by Sankhyan et al. [2, 3]. The pediatricians must understand that all these studies have been conducted in tertiary care referral hospitals which are bound to see severe cases. They are not the true representation of the community level prevalence of GBS subtypes. Moreover, the proportion of children with in-excitable nerves/unclassifiable disease varied in all these studies which could have contributed to the observed discrepancy. Another important observation by Gupta et al. and others has been identification of key clinical handles that can aid in clinical pattern-based diagnosis and differentiate AMAN from AIDP even if electrophysiology is unavailable or non-contributory [1‐3]. …