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Psychiatric phenomena as initial manifestation of encephalitis by anti‐NMDAR antibodies

Published online by Cambridge University Press:  22 February 2013

Peter Maat ,
Esther de Graaff ,
Nico M. van Beveren ,
Esther Hulsenboom ,
Robert M. Verdijk ,
Kathelijne Koorengevel ,
Martijn van Duijn ,
Herbert Hooijkaas ,
Casper Hoogenraad  and
Peter A. Sillevis Smitt
Peter Maat
Affiliation:
Department of Neurology, Erasmus University Medical Center, Rotterdam, Netherlands
Esther de Graaff
Affiliation:
Department of Neurology, Erasmus University Medical Center, Rotterdam, Netherlands Department of Neurosciences, Erasmus University Medical Center, Rotterdam, Netherlands
Nico M. van Beveren
Affiliation:
Department of Psychiatry, Erasmus University Medical Center, Rotterdam, Netherlands
Esther Hulsenboom
Affiliation:
Department of Neurology, Erasmus University Medical Center, Rotterdam, Netherlands
Robert M. Verdijk
Affiliation:
Department of Pathology, Erasmus University Medical Center, Rotterdam, Netherlands
Kathelijne Koorengevel
Affiliation:
Department of Psychiatry, Erasmus University Medical Center, Rotterdam, Netherlands
Martijn van Duijn
Affiliation:
Department of Neurology, Erasmus University Medical Center, Rotterdam, Netherlands
Herbert Hooijkaas
Affiliation:
Department of Immunology, Erasmus University Medical Center, Rotterdam, Netherlands
Casper Hoogenraad
Affiliation:
Department of Neurosciences, Erasmus University Medical Center, Rotterdam, Netherlands
Peter A. Sillevis Smitt*
Affiliation:
Department of Neurology, Erasmus University Medical Center, Rotterdam, Netherlands
*
Peter A. Sillevis Smitt, Department of Neurology, Erasmus University Medical Center, Erasmus MC room H639, s‐Gravendijkwal 230, 3015 CE Rotterdam, Netherlands. Tel: +31-10-7034415; Fax: +31-10-7033208; E‐mail: p.sillevissmitt@erasmusmc.nl
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Abstract

Objective

Autoimmune encephalitis associated with autoantibodies against the N‐methyl‐d‐aspartate receptor (NMDAR) often presents with behavioural change. Our objective was to describe in detail the psychiatric presentation and pathways to care in order to aid the early diagnosis of NMDAR encephalitis.

Methods

Sera and cerebrospinal fluid (CSF) from patients with suspected NMDAR encephalitis were tested on HEK 293 cells transfected with the NR1 subunit of the NMDAR. Clinical information was obtained from the referring psychiatrists and neurologists and by review of the clinical records.

Results

Samples from 15 patients (13 female, 2 male, mean age 24 years, range 5–56 years) tested anti‐NMDAR positive. Twelve of the 15 patients (80%) presented with prominent psychiatric symptoms and 8 were initially referred to a psychiatric service. The most prominent initial psychiatric symptoms were anxiety in seven (47%), behavioural change (often bizarre) in six (40%) and agitation in five (33%). All patients developed psychiatric symptoms in the first 6 weeks of illness. Thirteen patients received psychotropic medications: antipsychotics in 12 and benzodiazepines in 11. Treating physicians considered the psychotropic medication not effective in 11 patients resulting in many drug switches. At nadir, all patients were in a very poor condition. However, eight patients (53%) recovered (almost) completely. Outcome tended to be better in patients who had received early immunotherapy or tumour removal.

Conclusions

Autoimmune encephalitis and anti‐NMDAR testing in serum and CSF should be considered in patients, especially young females, presenting with atypical psychiatric phenomena. Early diagnosis and treatment will likely improve the prognosis of NMDAR encephalitis.

Keywords

anti‐NMDAR antibodiesanti‐NMDAR encephalitispsychotic disorders
Type
Original Articles
Information
Acta Neuropsychiatrica , Volume 25 , Issue 3 , June 2013 , pp. 128 - 136
Copyright
Copyright © Scandinavian College of Neuropsychopharmacology 2013 

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