title: Differentiating Familial Neuropathies from Guillain-Barré Syndrome.
abstract: Differentiating Guillain-Barré syndrome (GBS) from inherited neuropathies and other acquired peripheral neuropathies requires understanding the atypical presentations of GBS and its variant forms, as well as historical and physical features suggestive of inherited neuropathies. GBS is typically characterized by the acute onset of ascending flaccid paralysis, areflexia, and dysesthesia secondary to peripheral nerve fiber demyelination. The disorder usually arises following a benign gastrointestinal or respiratory illness, is monophasic, reaches a nadir with several weeks, and responds to immunomodulatory therapy. Inherited neuropathies with onset before adulthood, whose presentation may mimic Guillain-Barré syndrome, are reviewed.
authors.affiliation: Department of Pediatrics, Section of Hospital Medicine, Nelson Service for Undiagnosed and Rare Diseases, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI, USA. Electronic address: bbordini@mcw.edu.
authors.forename: Brett J
authors.initials: BJ
authors.lastname: Bordini
authors.affiliation: Department of Child and Adolescent Neurology, Medical College of Wisconsin, Milwaukee, WI, USA.
authors.forename: Priya
authors.initials: P
authors.lastname: Monrad
mesh_terms: D002648:Child; D003937:Diagnosis, Differential; D020275:Guillain-Barre Syndrome / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D056747:Immunomodulation; D007167:Immunotherapy; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis*
keywords: Acute inflammatory demyelinating polyradiculoneuropathy; Charcot-Marie-Tooth hereditary neuropathy; Congenital neuropathy; Guillain-Barré syndrome