Reversible brain atrophy in anti-NMDA receptor encephalitis: a long-term observational study.
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.
Department of Neurology, School of Medicine, Kitasato University, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan. takahiro@med.kitasato-u.ac.jp
Takahiro
T
Iizuka
Shintaro
S
Yoshii
Shinichi
S
Kan
Junichi
J
Hamada
Josep
J
Dalmau
Fumihiko
F
Sakai
Hideki
H
Mochizuki
D000293:Adolescent; D000328:Adult; D000906:Antibodies / Q000097:blood / Q000134:cerebrospinal fluid; D001284:Atrophy / Q000209:etiology; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology*; D001931:Brain Mapping; D004660:Encephalitis / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging / Q000379:methods; D019370:Observation; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D015899:Tomography, Emission-Computed, Single-Photon / Q000379:methods
D000906:Antibodies; D016194:Receptors, N-Methyl-D-Aspartate