Personality Changes and Motor Aphasia as Early Signs

Case Report: We took over a 9-year-old girl after first seizure with reduced vigilance from an external clinic. The previous history and psychomotor development was without pathological findings. At admittance in our ward, the patient was awake and oriented, but showed psychomotor slowing and a slurred speech. The parents reported a change in her personality. After a few days, we recognized a progressive impairment with fluctuating state of consciousness, motor aphasia, orofacial dyskinesia and affected psychomotor status with episodes of fear and aggression. The cerebral MRI first revealed unspecific signal hyperintensities in the white matter and later mesial temporal edemas. We detected a pleocytosis (43 leucocytes/µl) and positive Anti-NMDA receptor antibodies in the cerebrospinal fluid and serum. On the third day, we started a treatment with methylprednisolone (2 pulses, 20 mg/kg/d for 5 days) and expanded the treatment with IVIG (1 g/kg/d for 2 days) and plasmapheresis (4 cycles) due to a lack of response. The neurologic status worsened continuously and changed between episodes of somnolence and agitation. After 4 weeks, we started a b-cell-depleting therapy with Rituximab (375 mg/m², 1/week for 4 weeks). The symptomatic treatment of the behavioral disorders was the most challenging aspect. As a vascular complication, the patient developed a thrombosis of the Sinus sagittalis superior and received a treatment with low molecular weight heparin. A few days before the patient was transferred to a rehabilitation facility, the neurologic status improved reluctantly.

Diagnosis: We suspected an Autoimmune Encephalitis because of the clinical symptoms such as reduced state of consciousness, dyskinetic movement disorders, seizures, behavioral und speech disorders and reduced cognitive skills. This was confirmed by the detection of Anti-NMDA receptor antibodies in the cerebrospinal fluid and serum. The MRI revealed changes compatible with a limbic encephalitis.

Discussion: Anti-NMDA-receptor encephalitis is the most frequent encephalitis in children and adolescents. NMDAR-antibodies bind to the NR1-subunit of the synaptic NMDA-receptor and disturbs the neuronal transmission. An early diagnosis and treatment is of particular importance for the prognosis. The course is often prolonged and recovery occurs after 18–24 months. In our case, immunosuppressive treatment was started early and soon extended to Rituximab due to fast deterioration of the neurologic status. To date there is no evidence concerning dose, interval of application and duration of treatment for children and adolescents. There is also a lack of data concerning the symptomatic treatment of behavioral disorders, which cause a high burden of suffering. There is a need for further research and guidelines for the management of those patients.

No conflict of interest has been declared by the author(s).