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BMC Neurology
Published in: BMC Neurology 1/2014

Open Access 01-12-2014 | Case report

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

Authors: Mathilde Renaud, Jérôme Aupy, Guillaume Camuset, Nicolas Collongues, Jean-Baptiste Chanson, Jérôme de Seze, Frédéric Blanc

Published in: BMC Neurology | Issue 1/2014

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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.
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Metadata
Title
Chronic Bickerstaff’s encephalitis with cognitive impairment, a reality?
Authors
Mathilde Renaud
Jérôme Aupy
Guillaume Camuset
Nicolas Collongues
Jean-Baptiste Chanson
Jérôme de Seze
Frédéric Blanc
Publication date
01-12-2014
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2014
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/1471-2377-14-99

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