Clinical characteristics and outcomes of patients with Guillain-Barré and acquired CNS demyelinating overlap syndrome: a cohort study based on a literature review

Abstract

Background: Some patients with Guillain-Barré syndrome (GBS) also have acquired demyelination of the central nervous system (CNS) (i.e., acquired demyelinating syndrome, ADS). Often, the overlap of GBS and ADS is overlooked. Therefore, we evaluated case reports of GBS/ADS overlap syndrome.

Methods: We mainly performed website-based research based on articles in cases presented with GBS/ADS overlap syndrome. A total of 66 cases were included. Clinical and prognosis data were analyzed.

Results: A total of 85% of patients with simultaneous or consecutive occurrence of GBS and ADS were identified within 4 weeks of the initial diagnosis. Transverse myelitis (TM) (32%) was the most common ADS found in GBS/ADS. Patients with Miller Fisher syndrome (MFS)/ADS overlap syndrome had greater female predominance, mean age, frequency of onset at the same time period, or within a short period, and percentage of sole involvement of the subtentorial region. The outcome was favorable based on the functional status in 74% of patients. The sensory level (OR = 0.182, 95% CI = 0.055-0.598; P = 0.005) was the best predictor of a poor outcome, while visual deficit (OR = 4.667, 95% CI = 1.187-18.352; P = 0.027) predicted a favorable outcome.

Conclusion: The ADS in GBS are diverse, CNS demyelinating may occur at any time, but early in the GBS course (and vice versa). MFS/ADS overlap syndromes is more common. The prognosis is generally good, but patients with sensory level deficit are likely to have a poor prognosis. The features of MFS/other CIS may better reflect involvement of the brainstem in MFS itself, rather than ADS in autoimmune peripheral neuropathies.

Keywords: Central nervous system,; Clinically isolated syndromes,; Demyelinating diseases,; Guillain–Barré syndrome,; Miller Fisher syndrome,; Overlap syndrome,; Transverse myelitis.