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From the Department of Neurology, School of Medicine, Kitasato University, Kanagawa, Japan (T. Iizuka, F.S., T. Ide, T.M., S.Y., M.I., K.S.); Department of Neurology, Divisions of Neuro-oncology and Pediatric Neurology, University of Pennsylvania, Philadelphia, PA (D.R.L., J.D.); Department of Neurology, School of Medicine, Keio University, Tokyo, Japan (N.S.); and Department of Neurology, Shizuoka Municipal Shimizu Hospital, Shizuoka, Japan (T.H.).
* To whom correspondence should be addressed. E-mail: takahiro{at}med.kitasato-u.ac.jp.
ABSTRACT
Objective: To report the definitive diagnosis of anti-NMDA receptor (NMDAR) encephalitis in four Japanese women previously diagnosed with "juvenile acute nonherpetic encephalitis" of unclear etiology, and to describe their long-term follow-up in the absence of tumor resection.
Methods: We extensively reviewed the case histories with current clinical and laboratory evaluations that include testing for antibodies to NR1/NR2 heteromers of the NMDAR in serum/CSF available from the time of symptom onset (4 to 7 years ago) and the present.
Results: All patients sequentially developed prodromal symptoms, psychosis, hypoventilation, severe orofacial dyskinesias, and bizarre immunotherapy-resistant involuntary movements that lasted 1 to 12 months. Two patients required mechanical ventilation for 6 and 9 months. Initial tests were normal or unrevealing, including the presence of nonspecific CSF pleocytosis, and normal or mild changes in brain MRI. Eventually, all patients had dramatic recovery of cognitive functions, although one had bilateral leg amputation due to systemic complications. Antibodies to NR1/NR2 heteromers were found in archived serum or CSF but not in long-term follow-up samples. An ovarian teratoma was subsequently demonstrated in three patients (all confirmed pathologically).
Conclusion: 1) These findings indicate that "juvenile acute nonherpetic encephalitis" or a subset of this disorder is mediated by an antibody-associated immune response against NR1/NR2 heteromers of the NMDA receptor (NMDAR). 2) Our patients’ clinical features emphasize that anti-NMDAR encephalitis is severe but potentially reversible and may precede by years the detection of an ovarian teratoma. 3) Although recovery may occur without tumor removal, the severity and extended duration of symptoms support tumor removal.
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