Megaesophagus associated with Myasthenia Gravis: a rare condition in humans.

We present the case of a 79 year-old patient with megaesophagus and aphagia, who required percutaneous endoscopic gastrostomy (PEG) feeding. Megaesophagus was considered the sole cause of aphagia by the referring secondary hospital. Solid state sensor videofluoromanometry (VFM) showed a substantial decrease in manometric values within the hypopharynx along with good relaxation of the upper-esophageal sphincter (UES). This led to electromyography (EMG), which showed features compatible with the diagnosis of Myasthenia Gravis (MG). The diagnosis of MG was confirmed by an intravenous injection of edrophonium chloride, performed under VFM monitoring. The co-existence of MG and megaesophagus rarely occurs in humans; although, most small animals, such as dogs, cats, and rodents, present with megaesophagus as an initial symptom of MG.

Physiologic variables for videofluoromanometric assessment of dysphagia: an exploratory study.

STUDY AIM: To assess the physiological variables among Upper Esophageal Sphincter Nadir (UESN), Hypopharyngeal Peak Pressure (HPP) and Pharyngo-Esophageal Pressure Gradient (PEPG) for Videofluoromanometry (VFM). PATIENTS & METHOD: Exploratory non-randomised prospective study comparing UESN, HPP and PEPG of three cohorts of individuals presumably presenting very distinctive "manometric signatures" based on McConnel's Piston Model of swallowing: 11 non-dysphagic volunteers called the Control Group (CG), 10 dysphagic patients presenting a Myotonic Dystrophy (MD), at various stages of evolution, and 10 patients presenting a CricoPharyngeal Barr (CPB), with no post-swallow pharyngeal residue at a previous Modified Barium Swallow (MBS). VFM tests are performed using solid-state three unidirectional transducers produced by Gaeltec Inc. The simultaneous display storage of the standard fluoroscopic swallow of 10 ml liquid barium with UESN and HPP measurements, continuously recorded on a 3-channel polygraph, is performed using a Kay-Pentax Swallowing Work Station. PEPG calculations are subsequently made. RESULTS: Significant different HPP and PEPG values were observed between the three cohorts. MD patients presented HPP and PEPG below 100 mmHg while CPB patients presented HPP and PEPG above 200 mmHg. The CG presented HPP and PEPG between 100 and 200 mmHg. UESN values revealed no significant difference between the three cohorts. A reading scale is proposed. The aim of the scale is to make a link between HPP or PEPG values and physiopathological (not diagnostic) conditions. Patients presenting an HPP or PEPG below 100 mmHg indicate a High probability of Pharyngeal Propulsion Impairment while patients presenting an HPP or PEPG above 200 mmHg are more likely to have an Increased Flow Resistance with appropriate Propulsion Response. Pros and cons for calculation of the PEPG, representing a possibly unnecessary step, are discussed. CONCLUSIONS: In our study, the use of HPP or PEPG as physiological variables provides quantitative data that allow VFM to discriminate three very distinctive swallowing conditions. Further studies are needed to assess the HPP and PEPG obtained with other manometic devices within the same specific populations for them to be considered as universal physiological variables. Eventually, further investigations should answer the question as to whether the calculation of the PEPG represents any value in comparison with HPP measurement alone.

Critical illness myopathy unrelated to corticosteroids or neuromuscular blocking agents.

Acute myopathy occurs in critically ill patients, receiving neuromuscular blocking agents or corticosteroids during intensive care hospitalisation. We report three patients with acute quadriplegic myopathy, two of whom were not exposed to corticosteroids or neuromuscular blocking agents. The first of these latter two patients had a history of generalised anoxia with coma related to surgery, complicated by multiple organ failure and sepsis. The second patient, suffering from acute leukaemia, developed sepsis and acute respiratory distress syndrome with the need for mechanical ventilation in the intensive care unit. Electrophysiological studies and muscle biopsy findings were consistent with the diagnosis of critical illness myopathy with loss of myosin filaments. Selective loss of myosin was confirmed by biochemical analysis of muscle. These findings demonstrate that acute myopathy with loss of myosin filaments may occur in patients with severe systemic illness without exposure to corticosteroids or neuromuscular blocking agents.