PracticeUpdate

Significant Progress Has Been Made in Immune- Mediated Neuropathies The field of treatable, immune-mediated neuropathies is expanding

T he understanding of immunobiology, discov- ery of new antibodies, and evaluation of new diagnostics have opened the way to new treatments for patients with these disorders, con- cluded a presentation at ICNMD 2018. Pieter A. van Doorn, MD, PhD, of the Erasmus Medical Center in Rotterdam, The Netherlands, discussed the expanding field of treatable, immune-mediated neuropathies. Dr. van Doorn explained that immune-mediated neuropathies are an extending field of disor- ders with specific subgroups. Individuals with these disorders need to be diagnosed because their conditions are treatable. Immune-mediated neuropathies range from the heterogeneous acute polyneuropathy Guillain-Barré syndrome to the more chronic, often demyelinating, polyradiculoneuropathies. Chronic inflammatory demyelinating polyneuropa- thy (CIDP) is the most prominent immune-mediated neuropathy. CIDP can be divided into the most frequent symmetric sensory-motor variant and pure motor or sensory subgroups. Focal or multi- focal varieties of CIDP are known as Lewis-Sumner syndrome or multifocal acquired demyelinating sensory-motor neuropathy. A variety termed distal acquired demyelinating sensory neuropathy is also reported. Careful electrophysiological investigation and peripheral nerve high-definition ultrasonography or MRI of the plexus can be helpful in diagnosing these individuals. European Academy of Neurology/ Peripheral Nerve Society guidelines for the diag- nosis and treatment of Guillain-Barré syndrome and CIDP are available or under construction. Dr. van Doorn told Elsevier’s PracticeUpdate , “Results of the large, randomized, controlled Second IVIg Dose trial in patients with GBS with a poor prognosis (SID-GBS) trial are eagerly awaited and will likely be available in early 2019. Poor prognosis was based on results of the modified Erasmus GBS Outcome Score (mEGOS).”

Dr. Pieter A. van Doorn

The primary objective of the SID-GBS trial is to determine whether a second IV immune globulin dose in individuals with Guillain-Barré syndrome with a poor prognosis improves functional outcome after 4 weeks. Secondary objectives are to evaluate: ƒ ƒ Functional outcome or muscle strength after 8, 12, and 26 weeks ƒ ƒ The percentage of individuals needing artificial ventilation and less time (number of days) on a respirator or in intensive care ƒ ƒ Reduction in time to hospital discharge ƒ ƒ Reduction in risk of secondary deterioration due to treatment-related fluctuations ƒ ƒ Development of more complications, possi- bly related to the second IV immune globulin treatment ƒ ƒ Reduced mortality due to Guillain-Barré syndrome ƒ ƒ Whether serum immune globulin increase after the first IV immune globulin dosage will be lower in individuals with poor prognosis ƒ ƒ Whether serum immune globulin G increases further (and to what extent) after administration of a second IV dose

PRACTICEUPDATE CONFERENCE SERIES • ICNMD 2018 14