A Case of Rapidly Progressive HTLV-1 Associated Myelopathy in a Non-endemic Region
Maya Ramy1, Andrew Morrison1, Alex Chen1, Rohini Samudralwar1, Joseph Berger1, Laura Stein1, Alexandra Pfister1, Esteban Paredes Stanley1, Paul Novello1
1Neurology, Hospital of the University of Pennsylvania
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.
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