Abstract
Spinocerebellar ataxia type 6 (SCA6) is an autosomal dominant polyglutamine disease that mainly affects the cerebellum, characterized by a late–onset, slowly progression. Although SCA6 has been thought to exhibit preserved generally swallowing function and thereby relatively good prognosis, we already reported that some patients with SCA6 had severe dysphagia. No established treatment for dysphagia has been reported in this disease. In the present report, imidapril hydrochloride, one of the angiotensin–converting enzyme (ACE) inhibitor, was administered to a 69–year–old man who had SCA6 with mild hypertension and cerebral ischemic changes. He did not recognize dysphagia, but video fluoroscopic examination of swallowing (VF) revealed that he had severe pharyngeal dysphagia with silent aspiration. Because ACE inhibitor treatments have been reported to reduce the risk of aspiration pneumonia in hypertensive patients with stroke and improve swallowing function in elderly people with aspiration pneumonia, we selected imidapril hydrochloride as an antihypertensive drug. After administration of imidapril hydrochloride, VF showed improved swallowing functions with no silent aspiration. He did not develop aspiration pneumonia during one–year of imidapril hydrochloride treatment. Possible pharmacological mechanisms included promotion swallowing reflex by increased substance P in the pharyngeal mucosa and improvement of cerebral microcirculatory perfusion. ACE inhibitors may be a candidate for treatment of dysphagia in neurodegenerative diseases with hypertension.
