Guillain-Barré Syndrome in India: population-based validation of the Brighton criteria

Objective: Case definitions of GBS were recently developed in response to the 2009 H1N1 vaccination programme but have undergone limited field testing. We validate the sensitivity of the Brighton Working Group case definitions for Guillain-Barré Syndrome (GBS) using a population-based cohort in India.

Methods: The National Polio Surveillance Unit of India actively collects all cases of acute flaccid paralysis (AFP) in children <15 years old, including cases of GBS. Cases of GBS with available cerebrospinal fluid (CSF) and nerve conduction studies (NCS) results, neurological examination, clinical history, and exclusion of related diagnoses were selected (2002-2003). Relevant data were abstracted and entered into a central database. Sensitivity of the Brighton GBS criteria for level 3 of diagnostic certainty which requires no clinical laboratory testing, level 2 which employs CSF or NCS, and level 1 which employs both, were calculated.

Results: 79 cases of GBS (mean age 6.5 years, range 4.0-14.5; 39% female) met the case definition. GBS cases were ascending (79%), symmetrical (85%), and bilateral (100%); involving lower extremity hypotonia (86%) and weakness (100%), upper extremity hypotonia (62%) and weakness (80%), areflexia/hyporeflexia (88%), respiratory muscles (22%), bulbar muscles (22%), and cranial nerves (13%). Four limbs were involved in 80% of cases. Mean time to maximal weakness was 5.2 days (range 0.5-30 days) with nadir GBS disability scores of 3 (7%), 4 (67%), 5 (15%), 6 (10%), or unclear (1%). CSF (mean time to lumbar puncture 29 days) was normal in 29% with cytoalbuminologic dissociation in 65% (mean protein 105 mg/dL, range 10-1000; mean cell count 11/μL, range 0-220, n=4 with >50 cells/μL). Significant improvement occurred in 73% whereas death (9%) occurred 6-29 days after sensorimotor symptom onset. The majority of cases (86%) fulfilled Brighton level 3, level 2 (84%), and level 1 (62%) of diagnostic certainty.

Conclusion: The diagnosis of GBS can be made using Brighton Working Group criteria in India with moderate to high sensitivity. Brighton Working Group case definitions are a plausible standard for capturing a majority of cases of GBS in field operations in low income settings during AFP surveillance.