重度の嚥下障害を認めたACTH単独欠損症の1症例

A Case Report of Isolated ACTH Deficiency with Severe Dysphasia

はじめに

　ACTH（Adrenocorticotropic hormone，以下ACTH）単独欠損症は6種の下垂体前葉ホルモンのうちACTHのみの分泌障害により副腎不全を来す疾患である．厚生労働省研究班報告^1）によると，ACTH単独欠損症の主要症状としては，全身倦怠感，食欲不振，低血圧症状，低血糖等が挙げられている．また，神経・筋症状としては，四肢筋力低下，関節拘縮，筋硬直，筋萎縮等が報告^2～7）されている．

　今回我々は，嚥下障害が重度でかつ遷延したACTH単独欠損症を経験し，嚥下機能について分析を行った．これまでに嚥下障害について記載のある報告は1例^8）しか認められず，貴重な症例を経験したので文献的な考察を加えて報告する．

Abstract : The following report illustrates a rare case of dysphagia caused by secondary adrenal insufficiency in a patient with isolated ACTH deficiency. A 76-year-old man with progressive appetite loss, weight loss and general fatigue was hospitalized due to dehydration, aspiration pneumonia and hypoglycemia. He was unable to stand and walk by himself. His serum cortisol and ACTH levels were both low. An endocrinological examination resulted in a diagnosis of isolated ACTH deficiency. Following the administration of 15mg of glucocorticoids daily, his symptoms diminished without dysphagia. Furthermore, we investigated his swallowing function using a videofluoroscopic examination of swallowing (VF). The VF showed an impaired laryngeal movement as well as an upper esophageal opening and severe aspiration without a delay in his swallowing reflex. Oral intake was judged to be impossible, but his swallowing function gradually improved and he was able to resume eating all of his meals 2 months after treatment. Dysphagia is not a common symptom in ACTH deficient patients but a few similar cases have been reported. The swallowing function of the patients in these cases was not evaluated in detail, so we evaluated this case according to the results of the VF and the patient's process of recovery. Careful monitoring of swallowing function and appropriate treatment for both dysphagia and adrenocortical failure are required for the recovery of such patients. In conclusion, practitioners should be wary of isolated ACTH deficiency in elderly patients with progressive weight loss and dysphagia.