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Open Access Case report

Chronic Bickerstaff’s encephalitis with cognitive impairment, a reality?

Mathilde Renaud1, Jérôme Aupy1, Guillaume Camuset5, Nicolas Collongues14, Jean-Baptiste Chanson1, Jérôme de Seze14 and Frédéric Blanc123*

Author Affiliations

1 University Hospital of Strasbourg, Neuropsychology Unit, Neurology Service and CMRR (Centre Mémoire de Ressources et de Recherche), 1 avenue Molière, 67098 Strasbourg Cedex, France

2 University of Strasbourg and CNRS, ICube laboratory UMR 7357 and FMTS (Federation de Médecine Translationnelle de Strasbourg), team IMIS/Neurocrypto, Strasbourg, France

3 University Hospital of Strasbourg, CMRR (Memory Resources and Research Centre), Strasbourg, France

4 University of Strasbourg, INSERM U119 laboratory, Strasbourg, France

5 University Hospital of la Réunion, Infectious Diseases Unit, Saint Pierre, La Réunion, France

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BMC Neurology 2014, 14:99  doi:10.1186/1471-2377-14-99

Published: 6 May 2014

Abstract

Background

Bickerstaff’s encephalitis (BE) is an acute post-infectious demyelinating disease with albuminocytological dissociation. A chronic form has rarely been described previously.

Case presentation

A 44-year-old man was hospitalized for drowsiness, cognitive complaint limb weakness, ataxia and sensory disturbance after diarrhea. Neuropsychological evaluation showed slowing, memory and executive function impairment, while analysis of the CSF showed albuminocytological dissociation. Immunologic tests showed positive anti-ganglioside antibodies (anti-GM1 IgM, anti-GD1a IgG and anti-GD1b IgM). Brain MRI was normal but SPECT showed bilateral temporal and frontal hypoperfusion. Outcome under immunoglobulin treatment (IVIG) was favorable with an initial improvement but was marked by worsening after a few weeks. Consequently, the patient was treated with IVIG every 2 months due to the recurrence of symptoms after 6 weeks.

Conclusion

This case raises the question of the existence of a chronic form of BE with cognitive impairment, in the same way as chronic inflammatory demyelinating polyneuropathy is considered to be a chronic form of Guillain–Barré syndrome.

Keywords:
Bickerstaff’s encephalitis; Anti-ganglioside antibodies; Chronic encephalitis; Campylobacter jejuni; Molecular mimicry; Cognitive dysfunction; Dementia; Mild cognitive impairment