Fernandes P M, Macleod M R, Bateman A, Abrahams S, Pal S
Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK.
Centre for Clinical Brain Sciences, University of Edinburgh & NHS Forth Valley, Edinburgh, UK.
BMC Neurol. 2017 Mar 29;17(1):64. doi: 10.1186/s12883-017-0847-9.
Conjugal amyotrophic lateral sclerosis is rare, with significant effects on psychological and care needs. We report a case of conjugal amyotrophic lateral sclerosis disease from central Scotland. This case is particularly unusual as both patients were diagnosed within an 18-month period and experienced the disease simultaneously, with similar symptomatology and progression.
Patient A was a 71-year-old man who presented with unilateral arm weakness and wasting. Patient B was a 68-year-old woman who presented with unilateral shoulder and elbow weakness. Diagnosis of amyotrophic lateral sclerosis was made within a few months of presentation in both cases, based on typical clinical symptomatology together with supportive neurophysiological testing. Interventions included enteral feeding and non-invasive ventilation. The time period between symptom onset and death was 5 years for Patient A and 3.5 years for Patient B.
This case illustrates two main points: the care issues surrounding cases of conjugal neurological disease, and the psychological issues in these patients. There are significant care issues arising when co-habiting couples both develop severe functionally limiting neurological diseases at the same time. The more slowly progressive nature of Patient A's disease may be at least partially explained by the support he was able to receive from Patient B before she developed symptoms. Secondly, there are important psychological effects of living with someone with the same - but more advanced - progressive and incurable neurological disease. Thus, Patient B was reluctant to have certain interventions that she had observed being given to her husband. Lastly, no plausible shared environmental risk factors were identified, implying that the co-occurrence of ALS in this couple was a random association.
夫妻双方均患肌萎缩侧索硬化症的情况较为罕见，对心理和护理需求有重大影响。我们报告一例来自苏格兰中部的夫妻双方均患肌萎缩侧索硬化症的病例。该病例尤为特殊，因为两名患者均在18个月内被确诊，且同时发病，症状和病情进展相似。
患者A为一名71岁男性，表现为单侧手臂无力和肌肉萎缩。患者B为一名68岁女性，表现为单侧肩部和肘部无力。根据典型的临床症状以及支持性的神经生理学检查，两名患者均在发病后数月内被诊断为肌萎缩侧索硬化症。干预措施包括肠内营养和无创通气。患者A从症状出现到死亡的时间为5年，患者B为3.5年。
该病例说明了两个主要问题：夫妻双方均患神经系统疾病时的护理问题以及这些患者的心理问题。当同居夫妻同时患上严重影响功能的神经系统疾病时，会出现重大的护理问题。患者A病情进展较慢，这至少部分可以解释为在患者B出现症状之前，他能够得到患者B的支持。其次，与患有相同但更严重的进行性和不治之症的神经系统疾病的人一起生活会产生重要的心理影响。因此，患者B不愿意接受她看到给予丈夫的某些干预措施。最后，未发现合理的共同环境风险因素，这意味着这对夫妻同时患肌萎缩侧索硬化症是一种随机关联。
