BACKGROUND: In several studies, antiretroviral drugs (principally zidovudine) have been used with success in the treatment of myelopathy associated with human T-lymphotrophic virus 1 (HTLV-1) (tropical spastic paraparesis-HTLV-1-associated myelopathy). The retrovirus HTLV-1 has been implicated as a causative agent of Sjögren syndrome (SS) in clinical reports and murine experiments. Moreover, a recognized complication of primary SS is a myelopathy, which has been shown in case reports to respond to immunosuppressive treatment. OBJECTIVE: To describe a patient with a rapidly progressive, extensive myelopathy with evidence of HTLV-1 infection and SS (probably secondary to HTLV-1) in whom we achieved spectacular therapeutic success using combined immunosuppressive and antiviral therapy. DESIGN: Case report. SETTING: University hospital. Patient A young Haitian woman diagnosed with HTLV-1 and SS developed extensive myelopathy leading to severe disability. MAIN OUTCOME MEASURES: Clinical and radiological improvement. RESULTS: Spectacular radiological and clinical recovery as well as stabilization were achieved with combined antiviral and immunosuppressant treatment. Follow-up at 2 years showed no signs of relapse. CONCLUSIONS: Both tropical spastic paraparesis-HTLV-1-associated myelopathy and Sjögren myelopathy are potentially very disabling. Rapidly progressive myelopathy secondary to SS necessitates the introduction of immunosuppressant therapies. The presence of HTLV-1 may confer the necessity to add antiviral therapy.
BACKGROUND: In several studies, antiretroviral drugs (principally zidovudine) have been used with success in the treatment of myelopathy associated with human T-lymphotrophic virus 1 (HTLV-1) (tropical spastic paraparesis-HTLV-1-associated myelopathy). The retrovirus HTLV-1 has been implicated as a causative agent of Sjögren syndrome (SS) in clinical reports and murine experiments. Moreover, a recognized complication of primary SS is a myelopathy, which has been shown in case reports to respond to immunosuppressive treatment. OBJECTIVE: To describe a patient with a rapidly progressive, extensive myelopathy with evidence of HTLV-1 infection and SS (probably secondary to HTLV-1) in whom we achieved spectacular therapeutic success using combined immunosuppressive and antiviral therapy. DESIGN: Case report. SETTING: University hospital. Patient A young Haitian woman diagnosed with HTLV-1 and SS developed extensive myelopathy leading to severe disability. MAIN OUTCOME MEASURES: Clinical and radiological improvement. RESULTS: Spectacular radiological and clinical recovery as well as stabilization were achieved with combined antiviral and immunosuppressant treatment. Follow-up at 2 years showed no signs of relapse. CONCLUSIONS: Both tropical spastic paraparesis-HTLV-1-associated myelopathy and Sjögren myelopathy are potentially very disabling. Rapidly progressive myelopathy secondary to SS necessitates the introduction of immunosuppressant therapies. The presence of HTLV-1 may confer the necessity to add antiviral therapy.