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. 2020 Feb 4;15(2):e0228679.
doi: 10.1371/journal.pone.0228679. eCollection 2020.

Patients with chronic autoimmune demyelinating polyneuropathies exhibit cognitive deficits which might be associated with CSF evidence of blood-brain barrier disturbance

Yavor Yalachkov  1 , Valerie Uhlmann  1 , Johannes Bergmann  1 , Dilara Soydaş  1 , Stefan Frisch  1   2 , Marion Behrens  1 , Christian Foerch  1 , Johannes Gehrig  1
Affiliations

Patients with chronic autoimmune demyelinating polyneuropathies exhibit cognitive deficits which might be associated with CSF evidence of blood-brain barrier disturbance

Yavor Yalachkov et al. PLoS One. .

Abstract

Background: Chronic autoimmune demyelinating polyneuropathies (CADP) result in impaired sensorimotor function. However, anecdotal clinical observations suggest the development of cognitive deficits during the course of disease.

Methods: We tested 16 patients with CADP (11 patients with chronic inflammatory demyelinating polyneuropathy, 4 patients with multifocal motor neuropathy and 1 patient with multifocal acquired demyelinating sensory and motor neuropathy) and 40 healthy controls (HC) with a neuropsychological test battery. Blood-brain-barrier dysfunction (BBBd) in patients was assessed retrospectively by analysing the cerebral spinal fluid (CSF) status at the time the diagnosis of CAPD was established.

Results: CADP patients failed on average in 1.7 out of 9 neuropsychological tests (SD ± 1.25, min. 0, max. 5). 50% of the CADP patients failed in at least two neuropsychological tests and 44.3% of the patients failed in at least two different cognitive domains. CADP patients exhibiting BBBd at the time of first diagnosis failed in more neuropsychological tests than patients with intact integrity of the BBB (p < 0.05). When compared directly with the HC group, CADP patients performed worse than HC in tests measuring information processing ability and speed as well as phonemic verbal fluency after adjusting for confounding covariates.

Conclusions: Our results suggest that mild to moderate cognitive deficits might be present in patients with CAPD. One possible tentative explanation, albeit strong evidence is still lacking for this pathophysiological mechanism, refers to the effect of autoimmune antibodies entering the CNS via the dysfunctional blood-brain barrier typically seen in some of the CADP patients.

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Conflict of interest statement

Yavor Yalachkov has been supported by travel grants from Novartis and Sanofi Genzyme, has received an honorarium for active participation in an advisory board by Sanofi Genzyme as well as a speaking honorarium by Roche. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

Figures

Fig 1
Fig 1. The y-axis shows the distribution in the number of the failed (i.e., performance > 1 SD below the respective reference mean) neuropsychological tests (min. 0, max. 5, FT = failed test, i.e. below average performance).
The x-axis illustrates the number of patients according to this distribution. The CADP patients with an intact BBB (BBBi) are shown in orange and the patients with a BBB dysfunction (BBBd) are shown in blue.
See this image and copyright information in PMC

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