Intensive immunmodulatory therapy in a 3 year old child with anti-NMDA-receptor encephalitis

Introduction: An anti-NMDA-receptor encephalitis is a severe, more and more diagnosed form of encephalitis with characteristic clinical features. Anti-NMDA-receptor encephalitis had initially been described in young women with ovarian teratoma, but is also common in children and without neoplasm.

Case report:: A previously healthy 3 year old girl presented with a one week history of abnormal fatigue, mood liability and psychomotor agitation. Over a period of one week there was a rapid clinical deterioration with increasing somnolence, loss of communication and regression of motor skills. She developed orofacial dyskinesia, choreoathetoid arm movements and autonomic dysfunction. Seven days after admission focal seizures evolved with increasing seizure frequency and therapy resistant seizure activity.

Sequential CSF studies showed lymphocytic pleocytosis with increased IgG index and oligoclonal bands. Initial MRI scans of the brain were normal and later showed increased signal intensity on T2 weighted images in both nuclei caudati. Infectious and immunological studies were all negative. Steroid pulses and intravenous immunglobulins were administered and showed no benefit. With confirmation of neuropil antibodies 10 cycles of plasmapharesis were started, followed by 4 cycles of rituximab and monthly pulsed cyclophopspamide infusions in combination with pulsed steroids over the following 6 months. At first seizures stopped and the girl slowly made a complete functional recovery. Screening for occult malignancy was negative.

Conclusion: In children with acute behavior disturbances, seizures, dystonia or dyskinesia in combination with CSF and EEG changes, an anti-NMDA-receptor encephalitis should be considered. In principle, the prognosis is favorable. However, improvement correlates with prompt identification of the disorder and early, sometimes intense immunotherapy and in the case of a malignancy with complete tumor removal.