Interrater reliability of electrodiagnosis in neonatal brachial plexopathy

Introduction: We investigated interrater reliability of overall assessment of nerve root lesions by electrodiagnostic testing (EDX) in neonatal brachial plexus palsy (NBPP). Methods: Two blinded, boardâ certified reviewers retrospectively reviewed deâ identified EDX data from 37 infants with NBPP for 2005â 2012. Only nerve conduction and electromyography needle data were included. The examiners independently assigned 1 of 4 nerve root lesion categories: (1) preâ ganglionic lesion (avulsion), (2) postâ ganglionic lesion (rupture), (3) normal, or (4) â unable to determine.â Simple percentage agreement, the Cohen kappa statistic representing interrater reliability for each nerve root (C5â T1), and overall kappa between examiners were evaluated. Results: Interrater reliabilities were substantial to almost perfect for each nerve root except C5. Considering all nerve roots, overall interrater reliability was substantial (kappa = 0.62); simple percentage agreement was 75% (138/185). Conclusions: Interrater reliability of nerve root assessment by EDX for infants with NBPP was high for C6â T1 root levels, but less reliable for C5 because of technical factors. Muscle Nerve 55: 69â 73, 2017Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/135308/1/mus25193.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/135308/2/mus25193_am.pd

Guillain-Barré syndrome: a century of progress

In 1916, Guillain, Barré and Strohl reported on two cases of acute flaccid paralysis with high cerebrospinal fluid protein levels and normal cell counts — novel findings that identified the disease we now know as Guillain–Barré syndrome (GBS). 100 years on, we have made great progress with the clinical and pathological characterization of GBS. Early clinicopathological and animal studies indicated that GBS was an immune-mediated demyelinating disorder, and that severe GBS could result in secondary axonal injury; the current treatments of plasma exchange and intravenous immunoglobulin, which were developed in the 1980s, are based on this premise. Subsequent work has, however, shown that primary axonal injury can be the underlying disease. The association of Campylobacter jejuni strains has led to confirmation that anti-ganglioside antibodies are pathogenic and that axonal GBS involves an antibody and complement-mediated disruption of nodes of Ranvier, neuromuscular junctions and other neuronal and glial membranes. Now, ongoing clinical trials of the complement inhibitor eculizumab are the first targeted immunotherapy in GBS