A rare case of severe postpartum anti-NMDAR encephalitis

Excerpt

A 27-year-old woman with abnormal behavior and balderdash was mandatorily hospitalized in the psychiatry department for 1 week. She showed hypochondriac and claimed that she had no brain, no teeth, and believed she was dead, however, had no prior psychiatric history. She was a multiparous woman who had given birth to her second healthy daughter 1 month ago via normal vaginal delivery. Computed tomography (CT) of brain and electroencephalogram (EEG) showed no obvious abnormalities. Therefore, she was diagnosed with Cotard's syndrome and treated with antipsychotic drugs. However, her psychiatric symptoms deteriorated gradually and during treatment, she developed status epilepticus, accompanied by generalized tonic–clonic seizure. Thus, she was immediately transferred to the neurology department of a general hospital. On neurological examination, she lapsed into unconsciousness with fever (38.5 ℃) and only neck stiffness was observed. Subsequently, enhanced magnetic resonance imaging (MRI) of the brain revealed multiple abnormal signals in bilateral cerebral hemispheres, brainstem, and cerebellum but no enhancement (Fig. 1a–d). Meanwhile, cervical MRI indicated abnormal signals in cervical spinal cord (Fig. 1e). In addition, re-examination of EEG suggested a severe abnormality. Therefore, encephalitis was highly suspected and lumbar puncture was carried out instantly. Cerebrospinal fluid (CSF) revealed 1 leucocyte/μl (reference value: < 10 leucocytes/μl), total protein 0.284 g/l (reference value: 0.150–0.450 g/l). Laboratory tests for herpes virus, cytomegalovirus, bacteria, acid-fast bacilli, cryptococcosis, and human immunodeficiency virus were all negative, but with autoimmune encephalitis antibody-associated being positive. The anti-NMDAR antibodies titers of CSF and serum were 1:32 and 1:320, respectively (reference value: negative). Taken together, the patient is definitively diagnosed anti-NMDAR encephalitis and treated with intravenous immunoglobulin (IV-Ig) at the dosage of 0.4 g/kg per day for five consecutive days and intravenous methylprednisolone 1000 mg daily. Considering that anti-NMDAR encephalitis was frequently associated with ovarian teratoma, transvaginal ultrasonography detection was arranged promptly, which showed a strong echo, so teratoma in the left ovary is highly suspected, which is with clear boundary and has no obvious blood flow signal. She underwent laparoscopic removal of the ovarian tumor. Finally, the pathological examination confirmed a mature cystic teratoma (Fig. 1f). Nevertheless, the patient’s condition did not improve despite receiving 2-week aggressive treatment. Whereafter, plasma exchange was implemented to help eliminate pathogenic factors and rituximab was added to the therapeutic regimen. However, her condition continued to deteriorate and she died of multiple organ failure unfortunately.

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