Department of Neurohabilitation, Oslo University Hospital Ullevål, Oslo, Norway.
NORMENT, KG Jebsen Centre of Psychosis Research, Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
Acta Neurol Scand. 2019 Jun;139(6):526-532. doi: 10.1111/ane.13086. Epub 2019 Mar 25.
Myotonic dystrophy type 1 (DM1) is a slowly progressive multisystem disorder. Guidelines recommend multidisciplinary follow-up. We aimed to investigate the presence of unmet health and social care needs among patients with DM1 and whether unmet needs correlated with motor function, cognitive impairments, or quality of life.
Patients were 22 adults with DM1. "Needs and Provisions Complexity Scale" (NPCS) was applied to evaluate the individual's needs and provision of health and social services. The Muscular Impairment Rating Scale (MIRS) was used to measure motor function and disease stage. All patients underwent neuropsychological testing. The EQ-5D-3L questionnaire was used to evaluate the patients' health-related quality of life (HRQoL).
Median time from diagnosis was 11 years (range: 1-40). Twenty patients had developed needs related to social care, personal care, and rehabilitation that had not been met, whereas need for medical follow-up was largely met. The more pronounced the muscular impairment, the more unmet needs were experienced by DM1 patients (r = 0.50, P = 0.019). Degree of unmet needs did not correlate with full-scale IQ (r = -0.27, P = 0.23) or HRQoL (r = -0.14, P = 0.55).
Using NPCS, we discovered that patients with DM1 had unmet needs with respect to social care, personal care, and rehabilitation although their need for medical follow-up was met. Thus, the use of NPCS helped bring our practice in better accordance with guidelines. A higher MIRS grade should alert the clinician to the likelihood of unmet needs.
肌强直性营养不良 1 型（DM1）是一种进行性缓慢的多系统疾病。指南建议进行多学科随访。我们旨在调查 DM1 患者是否存在未满足的健康和社会护理需求，以及未满足的需求是否与运动功能、认知障碍或生活质量相关。
患者为 22 名 DM1 成年患者。应用“需求和供给复杂性量表”（NPCS）评估个体的健康和社会服务需求及供给情况。采用肌肉功能损伤评分量表（MIRS）评估运动功能和疾病阶段。所有患者均接受神经心理学测试。采用 EQ-5D-3L 问卷评估患者的健康相关生活质量（HRQoL）。
诊断后中位时间为 11 年（范围：1-40 年）。20 名患者出现了社会关怀、个人护理和康复方面的需求，但这些需求未得到满足，而医疗随访需求基本得到满足。肌肉损伤越严重，DM1 患者的未满足需求越多（r=0.50，P=0.019）。未满足需求的程度与全量表智商（r=-0.27，P=0.23）或 HRQoL （r=-0.14，P=0.55）均无相关性。
使用 NPCS，我们发现 DM1 患者存在社会关怀、个人护理和康复方面的未满足需求，尽管他们的医疗随访需求得到满足。因此，NPCS 的使用有助于使我们的实践更符合指南。更高的 MIRS 等级应提醒临床医生注意未满足需求的可能性。
