Biomechanics, diagnosis, and treatment outcome in inflammatory myopathy presenting as oropharyngeal dysphagia

Abstract

Aims: In patients with inflammatory myopathy and dysphagia, our aims were to determine: (1) the diagnostic utility of clinical and laboratory indicators; (2) the biomechanical properties of the pharyngo-oesophageal segment; (3) the usefulness of pharyngeal videomanometry in distinguishing neuropathic from myopathic dysphagia; and (4) clinical outcome. Methods: Clinical, laboratory, and videomanometric assessment was performed in 13 patients with myositis and dysphagia, in 17 disease controls with dysphagia (due to proven CNS disease), and in 22 healthy age matched controls. The diagnostic accuracy of creatine kinase (CPK), erythrocyte sedimentation rate, antinuclear antibody, and electromyography (EMG) were compared with the gold standard muscle biopsy. The biomechanical properties of the pharyngo-oesophageal segment were assessed by videomanometry. Results: Mean time from dysphagia onset to the diagnosis of myositis was 55 months (range 1-180). One third had no extrapharyngeal muscle weakness; 25% had normal CPK, and EMG was unhelpful in 28%. Compared with neurogenic controls, myositis patients had more prevalent cricopharyngeal restrictive disorders (69% v 14%; p=0.0003), reduced upper oesophageal sphincter (UOS) opening (p=0.01), and elevated hypopharyngeal intrabolus pressures (p=0.001). Videomanometric features favouring a myopathic over a neuropathic aetiology were: preserved pharyngeal swallow response, complete UOS relaxation, and normal swallow coordination. The 12 month mortality was 31%. Conclusions: The notable lack of supportive clinical signs and significant false negative rates for laboratory tests contribute to the marked delay in diagnosis. The myopathic process is strongly associated with restricted sphincter opening suggesting that cricopharyngeal disruption is a useful adjunct to immunosuppressive therapy. The condition has a poor prognosis.A broad spectrum of disorders are potentially responsible for neurogenic dysphagia without obvious cause.

Effect of Bolus Volume and Viscosity on Pharyngeal Automated Impedance Manometry Variables Derived for Broad Dysphagia Patients

“…Most importantly we observed increased Peak P in relation to volume which has been previously shown to increase with bolus volume (5, 6). As has already been shown, using combined high-resolution manometry and videofluoroscopy, intra-bolus pressures in particular are elevated in the presence of pathology that limits opening of the UES (7,9,10). Such patients were not well represented in our patient cohort and the impact of obstruction of AIM variables and the SRI is currently the subject of ongoing study.…”

Movement of the hyoid bone and the epiglottis during swallowing in patients with dysphagia from different etiologies

“…If more cases from various etiologies and location of lesions were analyzed, additional sub-classifications might have been possible. Williams et al (2003) attempted to distinguish neuropathic dysphagia from a myopathic one using videomanometry. They performed videomanometric assessment in 13 dysphagic patients with myositis and 17 patients with neurogenic dysphagia due to central nervous system disease, and in 22 healthy age matched controls, and compared biomechanical properties of the pharyngo-esophageal segment among them.…”

Dysphagia in Inflammatory Myopathy: Self-report, Incidence, and Prevalence

“…Along with weakness of the quadriceps and finger flexor muscles, dysphagia may be a presenting symptom of IM and can provide direction for diagnosis [6]. In a group of 13 patients with IM, 8 (62%) demonstrated aspiration on videofluoroscopy [7]. Although the highest incidence is reported in IBM, with 65-86% of IBM patients demonstrating dysphagic symptoms [6,[8][9][10][11], it has been reported to affect 30-60% of PM patients [12] and 18-20% of those with DM [13,14].…”