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Case Reports
. 2010 Oct;257(10):1686-91.
doi: 10.1007/s00415-010-5604-6. Epub 2010 Jun 2.

Reversible brain atrophy in anti-NMDA receptor encephalitis: a long-term observational study

Affiliations
Case Reports

Reversible brain atrophy in anti-NMDA receptor encephalitis: a long-term observational study

Takahiro Iizuka et al. J Neurol. 2010 Oct.

Abstract

The long-term neuroimaging correlates of clinical recovery have not been described in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. The aim of the study is to evaluate the long-term outcome of brain atrophy in anti-NMDAR encephalitis. Patients were two women (ages 17 and 33 years) with severe anti-NMDAR encephalitis resulting in decreased level of consciousness, autonomic instability, hypoventilation, and dyskinesias requiring continuous infusion of anesthetic agents for 6-7 months. Brain MRI and cerebral blood flow SPECT obtained at the time of maximal neurological disability were compared with similar studies obtained 5-7 years later. Both patients were hospitalized for 9-14 months and developed frontotemporal atrophy and hypoperfusion 7-12 months after symptom presentation. In both patients, cognitive functions gradually improved over the next 4-5 years. Comparative neuroimaging studies obtained 5-7 years after symptom presentation showed dramatic improvement of the atrophy and frontotemporal hypoperfusion. The severe and protracted deficits and the frontotemporal atrophy that occur in some patients with anti-NMDAR encephalitis are potentially reversible. This suggests that a functional rather than a structural neuronal damage underlies the pathogenesis of this disorder.

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Conflict of interest statement

Conflict of interest statement The authors have nothing to disclose regarding conflicts of interest or commercial relationships including grants, honoraria, speaker’s lists, significant ownership, or financial support from pharmaceutical or other companies.

Figures

Fig. 1
Fig. 1. T2-weighted MRI and SPECT of patient #1
a MRI on admission is normal. b MRI obtained 7 months later shows marked frontotemporal atrophy. c Follow-up MRI obtained 7 years 6 months after presentation shows marked improvement of atrophy. d SPECT obtained 12 months after admission shows mediofrontal and temporal hypoperfusion. e Follow-up studies obtained 7 years 9 months after presentation demonstrate improvement of hypoperfusion. Arrows indicate hypoperfusion
Fig. 2
Fig. 2. T2-weighted MRI and SPECT of Patient #2
a MRI is normal on admission. b MRI obtained 8 months later shows moderate frontotemporal atrophy. c Follow-up MRI obtained 5 years 10 months after presentation shows improvement of atrophy. d SPECT obtained 7 months after admission shows prefrontal and mediofrontal hypoperfusion. e Follow-up SPECT obtained 5 years 10 months after presentation shows improvement of initial hypoperfusion. Arrows indicate hypoperfusion

References

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