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Abstract Details

Epstein-Barr virus driven acute motor axonal neuropathy with central demyelination following stem-cell transplant
Neuromuscular and Clinical Neurophysiology (EMG)
P12 - Poster Session 12 (12:00 PM-1:00 PM)
1-013

We report a patient with acute lymphoblastic leukemia (ALL) who after stem cell transplant (SCT) developed acute motor axonal neuropathy (AMAN) with central demyelination, and evidence of Epstein-Barr virus (EBV) infection in central nervous system (CNS).
 AMAN is an axonal form of Guillain-Barré syndrome, most common in Asian and Hispanic populations. It is commonly associated with Campylobacter jejuni and anti-GM1 antibody. Prognosis is generally poor with slow recovery and residual neurological deficits. 

Case history and literature review
A 25-year-old Hispanic man with precursor B-cell ALL presented with acute ascending weakness, 50 days after autologous SCT. Within 48 hours of symptom onset, he became bedbound due to severe paraparesis, progressing rapidly to bilateral upper extremities and eventually requiring intubation. Neurological exam showed 0/5 strength and areflexia, with no abnormal sensory findings. MRI-brain revealed multiple T2-hyperintense lesions throughout the white matter. MRI-spine revealed diffuse enhancement of cervical and thoracic nerve roots and cauda equina. Cerebrospinal fluid (CSF) studies showed elevated protein, pleocytosis and presence of EBV DNA PCR. His stool culture eventually revealed Campylobacter jejuni. Electromyography showed absent voluntary motor potentials with intact sensory potentials, supportive of acute, diffuse, pure motor polyradiculoneuropathy. Serum anti-GM1 IgM and IgG were positive, confirming diagnosis of AMAN. Despite 2 rounds of intravenous-immunoglobulin treatment, minimal improvement in his motor function was noted on 3 months follow-up.

To our knowledge, this is the first presentation of concurrent CNS EBV infection with central demyelination identified in the pathogenesis of AMAN associated with anti-GM1 antibody and recent C. jejuni infection. Considering our patient’s immunocompromised state, it is very likely that AMAN was driven by a central EBV infection as evidenced by CSF EBV DNA and pleocytosis. Future monitoring for concurrent infections in patients with AMAN can help in better understanding of molecular mimicry as the pathophysiological etiology of this syndrome.
Authors/Disclosures
Anila Kanna, MD (Neurocare of the South)
PRESENTER 		No disclosure on file
Morad Nasseri, MD (BHC) No disclosure on file
Mihir Kakara, MD Dr. Kakara has nothing to disclose.
Mona Elsayed, MD Dr. Elsayed has nothing to disclose.