[P1-112] Behavioural management and symptom control in patients with severe anti-NMDA encephalitis
Children with anti-NMDA encephalitis present with subacute behavioural deterioration, psychosis, encephalopathy, dyskinesia and autonomic dysfunction. Recovery in children with severe disease is slow despite aggressive immunotherapy. Severe behavioural agitation, hyperkinesia and dystonia often necessitates deep sedation with ICU care and mechanical ventilation for weeks and months, in an effort to control agitation and reduce violent limb thrashing. Dopamine antagonists (haloperidol, risperidone) often result in a paradoxical worsening of dystonia.
We present 3 children with severe anti-NMDA encephalitis meeting Graus criteria for definite (2 patients) and probable (1 patient) disease. MRI brain was normal and 2 patients had cerebrospinal fluid inflammation. All had subacute behavioural decline, agitation and rage behaviours, encephalopathy, sleep fragmentation, mutism, dysphagia, hyperkinesia with dystonia and chorea. Two patients had seizures and another had neurogenic bladder. Rapid symptom control was achieved over 1-2 weeks with a focus on regulating sleep (choral hydrate and melatonin at bedtime), preventing agitation, impulsivity and hyperkinesia (methylphenidate and benzodiazepines during wakefulness) and improving dyskinesia (trihexyphenidyl). Moderate sedation requiring ICU stay (midazolam infusion 10 mcg/kg/min) was required for only one patient.
All 3 patients received intravenous methylprednisolone, immunoglobulins and rituximab. We observed a similar pattern of recovery in all – being able to wean sleep, followed by behaviour and finally dyskinesia medications. All 3 children also showed an uncanny ability to focus if music was incorporated into rehabilitation therapy.
Although children with anti-NMDA encephalitis have normal cerebrospinal fluid dopamine biochemistry, they appear to show deterioration with dopamine antagonists (risperidone) and improvement with dopamine agonists (methylphenidate).
We present 3 children with severe anti-NMDA encephalitis meeting Graus criteria for definite (2 patients) and probable (1 patient) disease. MRI brain was normal and 2 patients had cerebrospinal fluid inflammation. All had subacute behavioural decline, agitation and rage behaviours, encephalopathy, sleep fragmentation, mutism, dysphagia, hyperkinesia with dystonia and chorea. Two patients had seizures and another had neurogenic bladder. Rapid symptom control was achieved over 1-2 weeks with a focus on regulating sleep (choral hydrate and melatonin at bedtime), preventing agitation, impulsivity and hyperkinesia (methylphenidate and benzodiazepines during wakefulness) and improving dyskinesia (trihexyphenidyl). Moderate sedation requiring ICU stay (midazolam infusion 10 mcg/kg/min) was required for only one patient.
All 3 patients received intravenous methylprednisolone, immunoglobulins and rituximab. We observed a similar pattern of recovery in all – being able to wean sleep, followed by behaviour and finally dyskinesia medications. All 3 children also showed an uncanny ability to focus if music was incorporated into rehabilitation therapy.
Although children with anti-NMDA encephalitis have normal cerebrospinal fluid dopamine biochemistry, they appear to show deterioration with dopamine antagonists (risperidone) and improvement with dopamine agonists (methylphenidate).