[National Questionnaire Survey on the Actual Use and Content Evaluation of the human T-cell leukemia virus type I (HTLV-1) -associated Myelopathy (HAM) Practice Guidelines 2019].

It is not enough to just create medical practice guidelines; they are also required to be implemented into practice. Therefore, we surveyed specialists to determine the extent of the dissemination of the "HAM Practice Guidelines 2019," to quantify gaps, identify challenges, and understand needs in daily practice. The survey also revealed that the 25% of the specialists were unaware of the tests required for confirming human T-cell leukemia virus type I (HTLV-1) infection. Additionally, they had insufficient knowledge of the HTLV-1 infection. About 90.7% of the specialists agreed with the policy of determining treatment intensity based on disease activity. However, the implementation rate of cerebrospinal fluid marker measurement, which is useful for this assessment, was as low as 27%. Hence, it is important to use the findings of this study to further promote awareness about this issue.

Health-Related Quality of Life Evaluation Using the Short Form-36 in Patients With Human T-Lymphotropic Virus Type 1-Associated Myelopathy.

Background: Human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy (HAM) is a neuroinflammatory disease, causing various neurological symptoms, including motor, sensory, and bladder and bowel dysfunctions. This study was designed to reveal the impact of HAM and related symptoms on health-related quality of life (HRQoL). Methods: We analyzed the Short Form-36 (SF-36) and clinical data of 538 patients with HAM registered in the HAM-net, a nationwide patient registry for HAM in Japan. HRQoL was evaluated using the SF-6D (a health state utility value calculated from the SF-36) and eight SF-36 subscales. A general liner model was used to estimate the impact of major HAM-related symptoms, including gait dysfunction, sensory disturbance in the legs (pain and numbness), urinary dysfunction, and constipation, on the SF-6D and SF-36 subscale scores. Results: The mean age and disease duration were 62.0 and 16.5 years, respectively. Of the patients, 73.2% needed walking aid; 42.7 and 67.1% had leg pain and numbness, respectively; 92.1% had urinary dysfunction; and 77.9% had constipation. The mean SF-6D score was 0.565, which was significantly lower than the national average (0.674 in the 60-69 years age group; p < 0.001), exceeding the minimal important difference (0.05-0.1). All the major symptoms were significantly associated with a decrease in the SF-6D score. The SF-36 subscale scores were significantly lower than the national standard of 50 (p ≤ 0.001), except for mental health (MH). Gait dysfunction was associated with lower scores in physical functioning (PF), limitations on role functioning because of physical health, bodily pain, general health perception (GH), vitality (VT), and social functioning; however, no association was observed between gait dysfunction and limitations on role functioning because of emotional problems and MH. Meanwhile, sensory disturbance in the legs was associated with a decrease in scores in all subscales. Urinary dysfunction was associated with worse PF, GH, VT, and MH. Constipation was associated only with PF. Conclusion: HRQoL of patients with HAM was worse than that of the general population and was associated with all major symptoms. Thus, patients should be comprehensively managed to achieve better HRQoL.