Bachmann G, Streppel M, Krug B, Neuen-Jacob E
Klinik und Poliklinik für Hals-Nasen-Ohrenheilkunde, Universität zu Köln, Germany.
Dysphagia. 2001 Fall;16(4):244-8. doi: 10.1007/s00455-001-0082-8.
Hypertrophy of the cricopharyngeal muscle is a serious clinical condition that can cause severe dysphagic symptoms, including prolonged deglutition and postdeglutitive aspiration. Although the therapeutical concepts are well established, the pathogenic mechanism of cricopharyngeal hypertrophy remains unclear. We present a patient with a ten-year history of progressive dysphagia. The neurological and MRI findings were normal. However, videocineradiography showed severe hypertrophy of the cricopharyngeal muscle. This condition was first treated by injections of botulinum toxin, which did not alleviate the symptoms. Next, myotomy and muscle biopsy were performed. Histological evaluation disclosed lymphoplasmacellular florid myositis, single-fiber atrophy, and muscle fiber necrosis with phagocytosis. There were no signs of inclusion body myositis or oculopharyngeal muscular dystrophy. Our finding of severe cricopharyngeal muscle hypertrophy associated with myositis has been published previously (n = 34). The study presented here shows cricopharyngeal dysphagia associated with various systemic diseases, including motor neuron disease, general granulomatous disease, dermatomyositis, or inclusion body myositis. Isolated changes of the cricopharyngeal muscle were described in 65% of the cases.
环咽肌肥大是一种严重的临床病症，可导致严重的吞咽困难症状，包括吞咽时间延长和吞咽后误吸。尽管治疗理念已很成熟，但环咽肌肥大的发病机制仍不清楚。我们报告一例有十年进行性吞咽困难病史的患者。神经学检查和磁共振成像结果均正常。然而，电视荧光吞咽造影显示环咽肌严重肥大。该病症首先采用肉毒杆菌毒素注射治疗，但症状未缓解。接下来进行了肌切开术和肌肉活检。组织学评估显示有淋巴细胞和浆细胞丰富的肌炎、单纤维萎缩以及伴有吞噬作用的肌纤维坏死。没有包涵体肌炎或眼咽型肌营养不良的迹象。我们关于严重环咽肌肥大伴肌炎的发现此前已有报道（n = 34）。此处呈现的研究表明环咽肌吞咽困难与多种全身性疾病有关，包括运动神经元病、全身性肉芽肿病、皮肌炎或包涵体肌炎。65%的病例中描述了环咽肌的孤立性改变。
