Abstract Details

HTLV-1 Associated Myelopathy/Tropical Spastic Paresis (HAM/TSP) is a progressive, inflammatory neurological disorder caused by the HTLV-1 retrovirus. Current treatment options for HAM/TSP are limited. We report a case of a patient with rapidly progressive HAM/TSP who has had significant, sustained improvement with mycophenolate mofetil (MMF) over 16 months. To our knowledge, this is the first reported case of HAM/TSP with a prolonged clinical response to MMF.

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A 65 year-old female presented with 2 months of progressive bilateral leg weakness and paresthesias. Within 6 months of symptom onset, the patient reported urinary incontinence and required a wheelchair for mobility. Her initial neurological exam demonstrated predominantly proximal, bilateral lower limb weakness, bilateral lower limb hyperreflexia, spasticity, and loss of vibration sense. Her Osame motor disability score (OMDS) within a month of presentation was 9 (range 0-13). Brain and spine MRIs with and without contrast were unremarkable. Further work-up revealed HTLV-1/2 antibodies in cerebrospinal fluid, and she was diagnosed with HAM/TSP. She demonstrated an initial robust response to corticosteroids with a reduction in her OMDS to 5, but had a marked decline in mobility with attempts to taper steroids. MMF was initiated, enabling prednisone to be reduced to a modest maintenance dose. On this regimen, she had significant clinical improvement, with a sustained reduction in her OMDS to 3 over 16 months of follow up. She experienced resolution of her urinary incontinence and improved strength, regaining the ability to ambulate without assistance.

The pathogenesis of HAM/TSP involves T-cell mediated inflammation of the spinal cord. Our case suggests that MMF may be an effective disease modifying therapy in the treatment of HAM/TSP. This robust benefit of MMF may result from its induction of T cell apoptosis and disruption of clonal proliferation of activated T cells.