Abstract Details

Title A Case of Rapidly Progressive HTLV-1 Associated Myelopathy in a Non-endemic Region

Topic Infectious Disease

Presentation(s) P7 - Poster Session 7 (8:00 AM-9:00 AM)

Poster/Presentation
Number 3-015

Objective NA

Background HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a slowly progressive paraparesis infrequently diagnosed outside endemic regions (e.g., Caribbean basin and southern Japan). HAM/TSP is due to chronic neuronal injury from HTLV-1-infected T-cells, and diagnosis is supported by clinical history, neurologic examination, and presence of HTLV-1 in the serum and spinal fluid. MRI of the brain and spine can show atrophy of the cord without lesions, though in rare cases longitudinally extensive myelitis is observed.

Design/Methods Case Report

Results A 46-year-old California-born woman with Puerto Rican heritage presented for evaluation of subacute myalgias and leg weakness. Examination noted hip girdle weakness with diffusely brisk reflexes. Brain and spine MRI showed longitudinally extensive myelitis involving the entire cord. Serum NMO and MOG IgG were negative. Serum HTLV-1 western blot was strongly positive. A short course of pulse IV steroids led to rapid clinical improvement. However, months later she had recurrence of symptoms in the setting of urinary tract infection which improved with antibiosis, and she was maintained on oral steroids with a plan for taper. Repeat MRI showed near-resolution of enhancement and signal abnormalities throughout the cord. Months later she abruptly discontinued steroids due to loss of insurance coverage and developed recurrent symptoms with urinary and bowel retention. Repeat MRI showed recurrence and extension in enhancement of the longitudinally extensive myelitis. HTLV-1 western blot in the serum and CSF was again strongly positive. Testing for alternate active infectious, inflammatory, and neoplastic entities was repeatedly negative, and she was diagnosed with recurrent HAM/TSP. She received pulse IV steroids and prolonged oral steroids with modest clinical improvement.

Conclusions Recognition of HTLV-1 and its associated neurologic diseases in non-endemic regions is essential for appropriate diagnosis and treatment to reduce morbidity, though the identification of effective disease modifying approaches in HAM/TSP remains an active focus of research efforts.

Authors/Disclosures
Maya M. Ramy, MD (.) PRESENTER	Miss Ramy has nothing to disclose.
Andrew Morrison, MD (Hospital of the University of Pennsylvania)	Dr. Morrison has nothing to disclose.
Alex Chen, MD	Dr. Chen has nothing to disclose.
Rohini D. Samudralwar, MD (The University of Pennsylvania)	Dr. Samudralwar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Samudralwar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Samudralwar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Samudralwar has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Multiple Sclerosis Association of America.
Joseph R. Berger, MD, FAAN	Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Bristol Myers Squibb/Celgene. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cycle Pharma. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Dice Therapeutics. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech/Roche. Dr. Berger has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Gilead. Dr. Berger has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Merck/. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Morphic. Dr. Berger has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sandoz. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Takeda. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for MAPI. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ExcisionBio. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Population Bio. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for TG Therapeutics. Dr. Berger has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Assorted .
Laura A. Stein, MD (University of Pennsylvania)	Dr. Stein has nothing to disclose.
Alexandra Pfister, MD (Penn Medicine)	Dr. Pfister has a non-compensated relationship as a Board Member with Tango Therapy Project that is relevant to AAN interests or activities.
Esteban Paredes Stanley, MD	Dr. Paredes Stanley has nothing to disclose.
Paul Novello, MD	Dr. Novello has nothing to disclose.

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