Abstract Details

Title Late Onset Anti-NMDA Receptor Auto-Immune Encephalitis In A Patient With A Double Lung Transplant

Topic Autoimmune Neurology

Presentation(s) Autoimmune Neurology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 087

Objective To describe a case of late onset NMDA-R Encephalitis in a male patient with a double-lung transplant.

Background NMDA-R antibody encephalitis is a well-documented immunoglobulin-mediated phenomenon against synaptic ligand-gated ion channel NMDA antigens. Due to strict immunosuppression regimen after solid organ transplant, the risk of NMDA-R encephalitis is rare in the transplant population. To date, there have only been a handful of cases reported after liver and renal transplant, however never after lung transplant.

Design/Methods N/A

Results We describe a case of a 71-year-old Caucasian male with a history of alpha-1-trypsin deficiency requiring bilateral lung transplant from a 27-year-old Caucasian female donor, who deceased from anoxic brain injury. Neurology was consulted for cognitive decline, tremors, and recurrent falls over a one-and-a-half-month period. A rapidly progressive primary parkinsonian condition was initially suspected. During the hospitalization, the patient’s condition deteriorated with decreased level of consciousness, worsening tremors, and development of SIADH. An extensive work-up was pursued, which revealed elevated CSF protein of 143mg/dL, elevated CSF IgG index, and positive anti-NMDA-R antibody in the CSF but absent in the serum. MRI Brain showed nonspecific chronic microvascular ischemic changes. Malignancy screen was unrevealing. Based on the patient’s clinical history of sub-acute cognitive impairment, parkinsonism, and positive CSF NMDA-R antibody, a diagnosis of probable NMDA-R encephalitis was made. The patient received treatment with a five-day course of intravenous immunoglobulin and Methyl-Prednisolone, after which the patient had dramatic improvement of his mental status and resolution of tremor.

Conclusions

To the best of our knowledge, this is the first reported case of NMDA-R encephalitis in a patient with a double lung transplant. It is important to consider NMDA-R encephalitis in rapidly progressive cognitive decline and dyskinesia, despite immunosuppression. Furthermore, this case raises the question of disease pathophysiology of antibody generation; was it autologous or transmitted from the donor ?

Authors/Disclosures
Elochukwu Ibekwe, MD PRESENTER	Dr. Ibekwe has nothing to disclose.
Hera A. Kamdar, MD	Dr. Kamdar has nothing to disclose.
Amir Adeli, MD	Dr. Adeli has nothing to disclose.

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