Improving and standardising assessment of patients with immune-mediated neuropathies: Peripheral Neuropathy outcome measures Standardisation study (PeriNomS study – part 1)

textabstractIn this thesis, the outline and the results of the fi rst part of the Peripheral Neuropathy outcome measures Standardisation (PeriNomS) study are described. The PeriNomS study aims to improve and standardise the assessment of patients with immune-mediated neuropathies. These disorders are potentially treatable with immuno-modulating agents, therefore, proper outcome measures are needed to detect clinically important improvement or deterioration over time. Immune-mediated neuropathies include Guillain-Barré syndrome (GBS), chronic infl ammatory demyelinating polyneuropathy (CIDP), polyneuropathy associated with monoclonal gammopathy of undetermined signifi cance (MGUSP), and multifocal motor neuropathy (MMN). Electrophysiological examination in these patients generally reveals features of a demyelinating polyneuropathy, although a subgroup of GBS patients may have predominantly axonal features (acute motor axonal neuropathy (AMAN)). Diagnostic criteria for all these neuropathies have been formulated.1-4 From a clinical, electrophysiological, and immunological point of view there is increasing evidence that these illnesses represent part of a continuum, mainly separated by their extent of neuromuscular dysfunction, the evolution of weakness over time, and their response to treatment (table 1). In the following, a brief overview of these neuropathies is given with particular emphasis on their clinical presentation