An Unfortunate Immunological Overlap: A Case Report of Neuromyelitis Optica Compounded with NMDA Receptor Antibody Encephalitis
Elizabeth Holland1, Kelly Angelle1, Jessica Gautreaux2
1LSU, 2LSUHSC
Objective:

Review a case of co-existing Neuromyelitis Optica Spectrum Disorder (NMOSD) and anti-NMDA receptor anitbody (NMDAR) encephalitis and the importance of recognizing clinical symptoms associated with all identified autoantibodies.

Background:

A 14 year old female presented with 3 weeks of progressive lower extremity sensory loss followed by disabling motor weakness, incontinence, and blurry vision. MRI spine showed spinal cord edema from the level of T1-T11 confirming the suspicion of transverse myelitis.  Lumbar puncture showed leukocytosis (WBC 402) and CSF positive for oligoclonal bands. Serology was positive for AQP4R antibodies confirming the presence of NMOSD.  Patient was treated with intravenous methylprednisolone and rituximab, regained some strength in lower extremities, and was transferred to inpatient rehabiliation. 

Upon arrival to inpatient rehab, the family reported the patient had been experiencing two weeks of progressive changes in sleeping patterns and personality attributed to adjustment and acute hospitalization. Over the first week of rehab admission, the patient had progressive decline characterized by development of echolalia, hallucinations, delusions, catatonia, blunt affect, and tachycardia. It was found on record review that the patient was positive for NMDA receptor antibodies on initial workup. This combined with her clinical decompensation was consistent with an overlapping anti-NMDAR encephalitis. She was re-pulsed with methylprednisolone and 2g/kg IVIG for treatment of NMDAR encephalitis and ultrasound and PET scans obtained to investigate for underlying ovarian teratoma were negative. She is currently undergoing rituximab treatments outpatient with mild improvement in motor and mental status.

Design/Methods:
N/A
Results:
N/A
Conclusions:
In this patient, signs of progressive encephalopathy in setting of a treated transverse myelitis attack due to NMOSD went unrecognized as clinical progression of anti-NMDAR encephalitis despite the confirmed presence of anti-NMDAR antibodies. This highlights the importance of monitoring for symptoms associated with all identified autoantibodies to initiate early treatment especially when symptoms present in sequence, not simultaneously. 
10.1212/WNL.0000000000206271