Anti-NMDA receptor encephalitis is an acute form of lethal encephalitis which has a high probability for recovery with timely diagnosis and treatment. Therefore, as frontline physicians it is important for us to be aware of this disease.
Anti-NMDA Encephalitis is an autoimmune disease, where the primary target is NR1/NR2 subunit of the NMDA receptor (N-Methyl D-Aspartate). This disease has recently received press over the last decade but it is suspected that this entity still remains under-recognised.
Clinical Presentation
Typically NMDA-Encephalitis starts with a flu like illness(weeks to months before) followed by personality changes, psychotic behaviour, disorientation, confusion, paranoia, dyskinesia, seizures, hallucinations and autonomic dysfunction. The condition is associated with tumours, mostly teratomas of the ovaries. Male to Female ratio is 9:1 and the typical patient is a young female.
Work-up
- Routine Bloods (CBC, Renal, Liver, Kidney Profiles, TSH)
- Head Imaging (SOLs, r/o other causes
- Lumbar Puncture - NR1 and NR2 antibodies in CSF
- Pelvic ultrasound (to look for teratomas)
Differential Diagnosis
- Psychiatric Illnesses
- Substance Abuse
- Other forms of Viral Encephalitis
- Limbic System Encephalitis
Treatment
- Pelvic Tumor Removal
- Immunotherapy - corticosteroids, intravenous immunoglobulin and plasmapheresis
- Rehabilitation
The recovery process usually takes several months and paradoxically, the symptoms can reappear but as the recovery process continues, the psychosis eventually fades away.
Take Home:
It is important to consider anti-NMDA receptor encephalitis as a possible cause of acute psychosis in young patients with no past neuropsychiatric history before we label it as a "Mental Health" problem.
Posted by:
Lakshay Chanana
Speciality Doctor
Northwick Park Hospital
Department of Emergency Medicine
England
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