title: [Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuropathy].
abstract: Chronic inflammatory demyelinating polyneuropathy (CIDP) is characterized by an insidious onset showing progression over two months. However, up to 16% of CIDP patients may show acute presentation similar to Guillain-Barré syndrome (GBS). Such cases are termed acute-onset CIDP (A-CIDP). Distinguishing A-CIDP from GBS, especially the acute inflammatory demyelinating polyneuropathy (AIDP) subtype, is critical because therapeutic strategies and outcomes may differ between the two syndromes. Regarding clinical features, A-CIDP is less likely to have autonomic nervous system involvement, facial weakness, a preceding infectious illness, or the need for mechanical ventilation, in comparison with AIDP. Electrophysiological features are usually quite similar between the two, although follow-up studies may elucidate key differences. Around 8%-16% of GBS patients may show clinical deterioration shortly after improvement or stabilization following initial immunological therapy. Such a situation is termed treatment-related fluctuation (TRF; GBS-TRF). The distinction between GBS-TRF and A-CIDP is an important clinical issue because maintenance treatment is often required in CIDP. The diagnosis of A-CIDP should be considered when the condition of a patient with GBS deteriorates after nine weeks from onset, or when deterioration occurs three times or more.
authors.affiliation: Department of Neurology, Teikyo University School of Medicine.
authors.forename: Takamichi
authors.initials: T
authors.lastname: Kanbayashi
authors.forename: Masahiro
authors.initials: M
authors.lastname: Sonoo
mesh_terms: D000328:Adult; D020278:Demyelinating Autoimmune Diseases, CNS / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy*; D003937:Diagnosis, Differential; D020275:Guillain-Barre Syndrome / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D008297:Male; D009431:Neural Conduction / Q000502:physiology*; D011129:Polyradiculoneuropathy / Q000175:diagnosis / Q000503:physiopathology*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy