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

Mathilde Renaud, Jérôme Aupy, Guillaume Camuset, Nicolas Collongues, Jean-Baptiste Chanson, Jérôme de Seze, Frédéric Blanc
BMC Neurol. 2014-05-06; 14(1):
DOI: 10.1186/1471-2377-14-99

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1. BMC Neurol. 2014 May 6;14:99. doi: 10.1186/1471-2377-14-99.

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

Renaud M, Aupy J, Camuset G, Collongues N, Chanson JB, de Seze J, Blanc F(1).

Author information:
(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. .

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

DOI: 10.1186/1471-2377-14-99
PMCID: PMC4040113
PMID: 24885623 [Indexed for MEDLINE]

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