Pierach C A, Jennewein E
Abbott Northwestern Hospital, Department of Medical Education, Minneapolis, Mn. 55407-3799, USA.
Sudhoffs Arch. 1999;83(1):50-66.
Historical evidence has been collected attempting to diagnose members of royal houses, perhaps most publicized by Macalpine and Hunter (1969) for George III and his assumed porphyria, claiming that his insanity was a classic case of thereof. This rare metabolic disease presents with a variety of signs and symptoms: skin disease, abdominal pain, tachycardia, and neuro-psychiatric findings. The porphyrias are hereditary and since George III and Frederick William I share ancestors it seemed reasonable to investigate if the latter may also have suffered from porphyria. The pathography of both kings is meticulous, showing for both that abdominal pain, erratic behavior, restlessness, and discolored urine were frequently observed and complete recovery interictally was common. Intercurrent illnesses, fasting, alcohol and even tobacco smoking have been shown to be inducers of attacks and these risk factors are well documented in royal history.--The diagnosis of porphyria was not recognized then and other names were used, such as Cachexia hypochondriaca, Asthma spasmodico flatulentum, dolores arthritici.--We propose that Frederick William I suffered from an inducible porphyria.
人们已经收集了历史证据来试图诊断王室成员的病症，其中最广为人知的或许是麦卡尔平（Macalpine）和亨特（Hunter）（1969年）对乔治三世及其疑似血卟啉病的研究，他们声称他的精神错乱就是该病的典型病例。这种罕见的代谢性疾病有多种体征和症状：皮肤病、腹痛、心动过速以及神经精神方面的表现。血卟啉病具有遗传性，由于乔治三世和腓特烈·威廉一世有共同的祖先，因此研究后者是否也患有血卟啉病似乎是合理的。两位国王的病情记录都很详尽，显示两人都经常出现腹痛、行为异常、烦躁不安以及尿液变色的情况，而且发作间期完全康复很常见。并发疾病、禁食、饮酒甚至吸烟都已被证明是发作的诱因，这些风险因素在王室历史中有充分记载。当时血卟啉病的诊断尚未得到认可，人们使用了其他名称，如“疑病性恶病质”“痉挛性胀气哮喘”“关节炎性疼痛”。我们认为腓特烈·威廉一世患有可诱发型血卟啉病。
