1/45. Autonomic failure and proximal skeletal myopathy in a patient with primary Sjogren syndrome.Autonomic failure and proximal skeletal myopathy are rare features of the Sjogren syndrome (SS). We describe a 51-year-old woman with primary SS who had development of esophageal dysmotility, urinary retention, severe orthostatism, and skeletal myopathy during a 3-month period after the diagnosis of SS. Her symptoms and signs responded well to corticosteroid therapy. Although dysfunction of the peripheral nervous system has a prevalence rate of 20% in patients with SS, most commonly the nerve dysfunction is a sensory deficit, and autonomic neuropathy is less frequent. Autonomic neuropathy due to SS may be underreported. The cause of our patient's myopathy remains undetermined. We speculate that the myopathy was due to either a form of polymyositis or an immune-mediated neuropathy with muscle involvement.- - - - - - - - - - ranking = 1keywords = motility (Clic here for more details about this article) |
2/45. Esophageal aperistalsis following fundoplication in a patient with trisomy 21.Gastrointestinal abnormalities are frequent in patients with down syndrome (DS), gastroesophageal reflux (GER) being prominent among them. A 10-year-old boy with DS presented with progressive daily vomiting and an upper gastrointestinal study documenting reflux. A laparoscopic Nissen fundoplication was performed uneventfully. Postoperative inability to take solids was noted and a contrast study showed a tight gastroesophageal junction and poor peristalsis. Persistent symptoms were not alleviated by esophageal dilatation, despite a relaxing lower esophageal sphincter. Esophageal manometry documented complete esophageal aperistalsis. A percutaneous endoscopic gastrostomy was placed and the patient required long-term tube feeds. Esophageal aperistalsis is a rare condition in DS, likely superimposed on GER. fundoplication may adversely affect the already abnormal esophageal motility in these children. Esophageal manometry preoperatively will identify motility disorders and assist in selecting the best management for these patients.- - - - - - - - - - ranking = 62.005804973326keywords = motility disorder, motility (Clic here for more details about this article) |
3/45. Dysphagia in oesophageal intramural pseudo-diverticulosis: fibrosis, dysmotility or web?We describe two cases of oesophageal intramural pseudo-diverticulosis associated with a cervical oesophageal web presenting as intermittent dysphagia. In both cases, disruption of the web endoscopically resulted in lasting relief from symptoms. This observation, together with a review of the literature written during the past 39 years, suggests that oesophageal web formation may be under-reported in this condition and may be more important than either dysmotility or submucosal fibrosis and stricturing in the aetiology of the dysphagia seen in these patients. All patients with a radiological diagnosis of oesophageal intramural pseudo-diverticulosis should have an endoscopic examination which may be both diagnostic and potentially therapeutic.- - - - - - - - - - ranking = 5keywords = motility (Clic here for more details about this article) |
4/45. Esophageal dysmotility in brothers with an FG-like syndrome.We present 4 brothers with developmental delay, minor anomalies, and symptoms due to gastrointestinal dysmotility. There was some resemblance with FG syndrome, although none of the brothers had sufficient findings to make this diagnosis. The index case presented with at age 1 month with screaming episodes, mild gastro-esophageal reflux (GER), and severe constipation. Esophageal manometry studies were consistent with the diagnosis of "nutcracker esophagus." Symptomatic and manometric improvement followed treatment with oral calcium channel blockers. Two older and less severely affected brothers had similar manometric findings but did not require treatment. A fourth brother with symptoms in infancy now has normal esophageal manometry findings. These boys in all likelihood have an X-linked syndrome with manifestations of FG syndrome, in which treatment with calcium channel blockers, produces clinical and manometric improvement. The FG syndrome is an X-linked syndrome of multiple congenital anomalies/mental retardation with facultative manifestations of gastrointestinal dysmotility, including gastro-esophageal reflux, severe feeding difficulties, and constipation. Esophageal dysmotility, in particular "nutcracker esophagus," should be suspected in infants with the FG syndrome and screaming attacks.- - - - - - - - - - ranking = 7keywords = motility (Clic here for more details about this article) |
5/45. Two cases of severe non-specific oesophageal dysmotility showing different response to botulinum injection therapy.We report 2 cases where treatment of achalasia type symptoms due to severe non-specific oesophageal dysmotility have shown symptom resolution and manometric improvement to intrasphincteric botulinum injections either by itself or in combination with oesophageal dilatation.- - - - - - - - - - ranking = 5keywords = motility (Clic here for more details about this article) |
6/45. Esophageal hypermotility associated with intramural pseudodiverticulosis. Primary esophageal disease or epiphenomena?Esophageal intramural pseudodiverticulosis is a very rare disease of unclear etiology. The clinical picture is characterized by progressive dysphagia. Because of its frequent association with alcohol abuse and subsequent weight loss, it must be differentiated reliably from esophageal carcinoma. The diagnosis is established by the characteristic detection of multiple intramural contrast accumulations in the barium esophagogram. Additional endoscopic and endosonographic confirmation and histological examination are required to exclude a malignant tumor. Moreover, associated diseases are almost always present and should also be diagnosed by pH-metry, cytology, and esophageal manometry. Good and long-lasting therapeutic success can be achieved by bouginage of the stenosis with concomitant treatment of the associated esophageal diseases. Based on two case reports of patients with this disease, we discuss the unusual association with esophageal hypermotility as well as the symptoms, clinical course, therapy, and pathogenesis of the disease.- - - - - - - - - - ranking = 5keywords = motility (Clic here for more details about this article) |
7/45. Dysphagia in a patient with lateral medullary syndrome: insight into the central control of swallowing.BACKGROUND & AIMS: Central control of swallowing is regulated by a central pattern generator (CPG) positioned dorsally in the solitary tract nucleus and neighboring medullary reticular formation. The CPG serially activates the cranial nerve motor neurons, including the nucleus ambiguus and vagal dorsal motor nucleus, which then innervate the muscles of deglutition. This case provides insight into the central control of swallowing. methods: A 65-year-old man with a right superior lateral medullary syndrome presented with a constellation of symptoms, including dysphagia. The swallow was characterized using videofluoroscopy and esophageal motility and the results were compared with magnetic resonance imaging (MRI) findings. RESULTS: Videofluoroscopy showed intact lingual propulsion and volitional movements of the larynx. Distal pharyngeal peristalsis was absent, and the bolus did not pass the upper esophageal sphincter. manometry showed proximal pharyngeal contraction and normal peristaltic activity in the lower esophagus (smooth muscle), but motor activity of the upper esophageal sphincter and proximal esophagus (striated muscle) was absent. MRI showed a lesion of the dorsal medulla. CONCLUSIONS: These findings are compatible with a specific lesion of the connections from a programming CPG in the solitary tract nucleus to nucleus ambiguus neurons, which supply the distal pharynx, upper esophageal sphincter, and proximal esophagus. There is functional preservation of the CPG control center in the solitary tract nucleus and of the vagal dorsal motor nucleus neurons innervating the smooth muscle esophagus.- - - - - - - - - - ranking = 1keywords = motility (Clic here for more details about this article) |
8/45. Unilateral bronchiectasis and esophageal dysmotility in congenital adult tracheoesophageal fistula.Tracheoesophageal fistulas (TEF) in adults are most commonly neoplastic, and very rarely congenital in nature. We report a 45-year-old Hispanic male with TEF and initial presentation of minimal hemoptysis. The patient had radiographic evidence of unilateral upper lobe (RUL) bronchiectasis, massive esophageal dilatation, and dysmotility. However, there was no evidence of esophageal malignancy, achalasia, or Chagas' disease. bronchoscopy revealed a large TEF in the posterior wall of trachea, which was not visualized on esophagram or esophagoscopy. bronchoalveolar lavage (BAL) cultures grew mycobacterium avium complex (MAC). Our report illustrates that idiopathic, or congenital, TEF can be associated with esophageal dysmotility, adulthood bronchiectasis, and atypical mycobacterial superinfection.- - - - - - - - - - ranking = 6keywords = motility (Clic here for more details about this article) |
9/45. Esophageal dysmotility in an adult with chronic granulomatous disease.Chronic granulomatous disease (CGD) is a group of hereditary disorders of impaired intracellular destruction of phagocytosed bacteria. Gastrointestinal manifestations are present, with hepatic abscess being the most common. In this case report, we present an adult with CGD with esophageal involvement, which has been described in only one other adult. The clinical history, modalities of diagnosis (including endoscopy, barium radiography, and esophageal manometry), and therapeutic strategies pertaining to the esophageal manifestations of CGD are discussed. A review of the pertinent available literature is provided.- - - - - - - - - - ranking = 4keywords = motility (Clic here for more details about this article) |
10/45. chest pain. Differentiating GIT from cardiac causes.BACKGROUND: chest pain is a common presenting symptom in general practice. Although a cardiac cause is not the commonest origin, a high index of suspicion is needed. When the diagnosis is not clear, a cardiac cause should be considered until proven otherwise. A gastrointestinal origin of chest pain is not infrequent and may be due to oesophageal, gastric or biliary disease. Oesophageal causes are most common and include reflux, hypersensitivity or dysmotility. OBJECTIVE: This paper reviews the main gastrointestinal causes that may present with acute chest pain. DISCUSSION: Clinical history taking is the key to decision making and guides the choice of prompt or routine investigation or a therapeutic trial. When reflux is suspected as the cause, a therapeutic trial of high dose antisecretory therapy is appropriate. Investigations may be helpful when typical reflux symptoms are not present or there is a poor response to this approach. Investigations may include endoscopy, ambulatory pH monitoring, barium swallow or oesophageal manometry.- - - - - - - - - - ranking = 1keywords = motility (Clic here for more details about this article) |
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