Gastric volvulus complicating myotonic dystrophy.

Myotonic dystrophy is an autosomal inherited disorder of both striated and smooth muscle, and is considered to be a rare cause of gastrointestinal dilatation and abnormal peristalsis. We report on a patient with myotonic dystrophy complicated by gastric volvulus. A 57-year-old female with myotonic dystrophy suddenly developed abdominal pain, nausea and vomiting. X-ray examinations revealed gastric dilatation and pyloroantral obstruction, consistent with acute gastric volvulus. The patient underwent successful emergency gastrectomy. Gastric volvulus is often an unrecognized surgical emergency, but its clinical and radiographic features are so characteristic that accurate diagnosis is possible if the condition is kept in mind. Thus, the clinician should consider the possibility of gastric volvulus when evaluating gastrointestinal complaints in patients with myotonic dystrophy.     

Abnormal regulation of parathyroid hormone secretion by serum calcium level in myotonic dystrophy

Although the presence of bone abnormalities has been well known in myotonic dystrophy (MyD), details about calcium metabolism in this condition remain unclear. We already reported that the intestinal calcium absorption is increased in MyD patients due to the elevation of plasma 1,25(OH)2D level. We also reported that serum immunoreactive parathyroid hormone (iPTH) levels and Nephrogenous cyclic AMP (NcAMP) levels are increased in MyD patients. This study was designed to elucidate the mechanism of hyperparathyroid state in MyD patients. Intravenous calcium tolerance test was performed in 7 patients with MyD and 7 control subjects, 3 patients with other neuromuscular disorder and 4 healthy subjects. Calcium was infused stepwise at the concentration of 0, 104.7, 194.9 and 235.5mg every hour. The basal plasma calcium levels were significantly higher in MyD (9.79 +/- 0.27 mg/dl, Mean +/- SD) as compared with control subjects (9.50 +/- 0.19; p less than 0.05). Basal serum iPTH levels were also significantly higher in MyD (514.0 +/- 188.9 pg/ml) than those in control subjects (333.7 +/- 113.5; p less than 0.05). After calcium infusion, serum calcium levels were increased in both groups, but the levels of serum calcium remained significantly higher in MyD than those in control subjects. On the other hand, urinary calcium excretion levels were not different between the two groups. At the same time, %TRP and calcium clearance were significantly lower in MyD than control subjects.(ABSTRACT TRUNCATED AT 250 WORDS)     

Impaired vasopressin secretion in patients with myotonic dystrophy

Hypernatremia has occasionally been observed in patients with myotonic muscular dystrophy (MyD). To elucidate the possibility of osmoregulatory dysfunction, we investigated hypothalamo-posterior pituitary function as well as serum electrolytes in eight patients with MyD. Blood samples were obtained early in the morning after overnight dehydration. Renal function was estimated by blood urea nitrogen, serum creatinine and creatinine clearance. Posterior pituitary function was evaluated by direct measurement of plasma vasopressin (AVP) during a 5% hypertonic saline infusion. Plasma AVP concentrations were determined by sensitive radioimmunoassay. In five patients, circulating blood volume (CBV), plasma renin activity (PRA) and serum aldosterone (S-Aldo.) were also measured. The mean serum sodium level (143.9 +/- 1.7mEq/1: Mean +/- SD) was significantly higher than in the controls (139.4 +/- 2.2mEq/1). A 5% hypertonic saline infusion showed a subnormal increase in AVP and diminished thirst, despite sufficient elevation of plasma osmolality, in all patients as compared with healthy adults. Renal function was intact. Biochemical evidence of dehydration, estimated by PRA, S-Aldo and CBV, was unremarkable in four of the five patients. These findings suggest that patients with MyD have neurogenic disorders of osmoregulation in addition to previously reported endocrine abnormalities. Impaired AVP secretion in response to osmotic stimuli and reduced thirst might be responsible for such failure.     

Abnormal cytokine and adrenocortical hormone regulation in myotonic dystrophy

Metabolic-endocrine dysfunctions, including hyperinsulinemia, hypertriglyceridemia, increased fat mass, and dysregulation of the hypothalamic-pituitary-adrenal axis, are common in myotonic dystrophy (MD). We hypothesized that increased production of interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-alpha) may be important underlying mechanisms. We studied the diurnal rhythmicity of cytokines and cortisol, ACTH, and dehydroepiandrosterone in 18 men with adult onset MD and 18 controls. Morning levels of androstenedione, 17-hydroxyprogesterone, testosterone, and insulin were also determined. Genetic analyses were performed, including calculation of allele sizes. Median circulating 24-h levels of IL-6 (P < 0.001), TNF-alpha (P = 0.05), ACTH (P < 0.05), and cortisol (P < 0.05) were all significantly increased in MD, whereas dehydroepiandrosterone levels were decreased (P < 0.001). The diurnal rhythms of these cytokines/ hormones were disturbed in patients. Morning testosterone levels were decreased and insulin levels increased (P < 0.01 for both). Patients with high body fat mass had significantly increased insulin levels and decreased morning levels of cortisol, ACTH, and testosterone. IL-6 and TNF-alpha levels are increased and adrenocortical hormone regulation is disturbed in MD. Adiposity may contribute to these disturbances, which may be of importance for decreased adrenal androgen hormone production and metabolic, muscular, and neuropsychiatric dysfunction in MD.     

Insulin secretion in patients with myotonic dystrophy and their relatives

Summary Oral glucose, i.v. tolbutamide and i.v. arginine tolerance tests were performed in 11 patients with myotonic dystrophy and 9 of their clinically unaffected relatives. Five of the myotonic patients had glucose intolerance; 7 had exaggerated immunoreactive insulin (IRI) response to glucose. One of the 9 relatives demonstrated glucose intolerance and none had exaggerated insulin response to glucose. Three relatives, all obese, hyperresponded to arginine and one of them also responded excessively to tolbutamide. The results indicate that an exaggerated IRI response to glucose is common in myotonic dystrophy. Enhanced responses to other stimuli are less frequent. Although glucose intolerance occurred in half of the patients, the fact that the highest IRI levels were seen in non-diabetic patients suggests that this excessive response may protect against glucose intolerance. Our studies in relatives do not support the potential usefulness of testing for hyperinsulinemia in the early detection of myotonic dystrophy. Presented at the 9th International Diabetes Federation Congress in New Delhi, India, November 2, 1976.     

Gastric emptying and myoelectrical activity in children with nonulcer dyspepsia

We examined the effect of oral cisapride on gastric emptying time and myoelectrical activity using real-time ultrasonography and cutaneous electrogastrography in 10 children with nonulcer dyspepsia. A clear dominant frequency close to 3 cpm was present both at baseline and after eight weeks of cisapride. After cisapride, nine children had an increase in the normal slow wave percentage and the mean percentage of normal slow wave was significantly different (71.90±5.19% vs 79.16±5.54%;P<0.01). Moreover, an increased stability of the dominant frequency, determined by computing the coefficient of variation before and after cisapride, was found (28.12±1.72% vs 23.61±3.47%;P<0.01). At baseline the gastric emptying time, expressed asT1/2, was 139.76±40.04 min and at eight weeks 119.76±30.04 min (P=0.06). As regards the relationship between EGG and gastric emptying, the proportion of children with improved normal slow wave percentage was similar to that with improvedT1/2 emptying (z=0.57,P=0.57). Thus, gastric electrical activity seems to be an important factor in the pathophysiology of nonulcer dyspepsia in children.     

Gastric myoelectrical activity in patients with primary biliary cirrhosis

Objective  To examine gastric myoelectrical activity in patients with primary biliary cirrhosis (PBC). Materials and methods  The study comprised 11 female PBC patients (average age 53.4 years, range 43–70) and two aged-matched control groups: 11 (53.4 years, range 37–78) healthy women, and 10 female patients with chronic hepatitis C, CHC (53.9 years, range 35–66), who were examined prior to administration of an antiviral therapy. Every subject underwent an electrogastrographic recording comprising a 30-min interdigestive and a 120-min postprandial period. Results  Abnormal electrogastrograms, containing prolonged epochs of tachygastria in the postprandial phase were found in 2 out of 11 (18.2%) patients having both stage IV of the Scheuer’s PBC classification, as well as in 1 patient out of 10 (10%) with CHC at stage F2 according to the METAVIR fibrosis score. Conclusion  Electrogastrographic abnormalities do not seem to be pathognomonic for the PBC as a disease, but rather would be considered an unspecific sequel of a morbid liver affection.     

Gastric myoelectrical activity in diabetics with and without diabetic autonomic neuropathy

BACKGROUND/AIMS: The effects of diabetes mellitus on gastric myoelectrical activity has not been fully investigated. The aim of the present study was to investigate the pattern of gastric myoelectrical activity in noninsulin dependent diabetics, detected by electrogastrography in an attempt to clarify the relation between diabetic autonomic neuropathy and gastric myoelectrical abnormalities, if any. METHODOLOGY: The study was carried out on 34 noninsulin dependent diabetes (7 males, 27 females). Their age ranged from 35-60 years with mean age of 51.5 +/- 3.5 years. The EGG was recorded for 30 min in both the fasting and postprandial states, using an ambulatory EGG recording device (Digitrapper EGG, Synectics Medical). The adaptive spectral analysis method was used to assess the normality of the EGG. The EGG was defined as abnormal if: the percentage of normal slow waves (2.5-3.7 cycles/min) was below 70% during either the baseline or postprandial recording or there was a decrease in EGG peak power after the meal, or both. RESULTS: EGG abnormalities were detected in 13 patients (38.2%); 1 had tachygastria, 1 had bradygastria, 7 had dysrhythmias, and 4 had decreased EGG peak power after the meal. All diabetic patients with abnormal EGG suffer autonomic neuropathy. CONCLUSIONS: These results suggest that gastric myoelectrical abnormalities occur in a high proportion of noninsulin dependent diabetics and these abnormalities predominate in those patients with autonomic neuropathy.     

Gastric myoelectrical activity and gastric emptying in patients with functional dyspepsia

OBJECTIVES: The aims of this study were to investigate gastric myoelectrical activity and gastric emptying (GE) and their relationship in patients with functional dyspepsia. METHODS: The study was conducted in 15 healthy volunteers (six women, nine men, mean age: 42 yr) and 15 patients (13 women, two men, mean age: 43 yr) with functional dyspepsia. Gastric myoelectrical activity was recorded using cutaneous electrogastrography (EGG) for 30 min in the fasting state and for 120 min simultaneously with GE monitoring after an isotope-labeled solid meal. The anterior/posterior images of the stomach were taken using a technetium scanner immediately after eating, and then at 1, 2, and 4 h to determine the percentage of gastric retention. The dominant frequency of the EGG, the change of the postprandial EGG peak power (deltaP), and the percentage of normal 2-4 cycles/min (cpm) slow waves during each recording session were calculated and compared between the patients and healthy subjects. RESULTS: The patients had a significantly lower mean percentage of 2-4 cpm slow waves, both in the fed state and in the fasting state, than did healthy subjects. Compared to the EGG in the fasting state, a significant increase of the EGG dominant frequency in the fed state was observed in healthy subjects but not in the patients. The mean postprandial EGG power increase in the patients was substantially less than in the healthy subjects during the first postprandial hour but similar during the second postprandial hour. The mean percentage of gastric retention in patients is substantially higher than in the healthy subjects, both at 2 h after eating and at 4 h after eating. Of 15 patients, nine (60%) had delayed GE (gastric retention at 2 h >50%) and 10 (66%) had abnormal EGGs (percentage of 2-4 cpm <70% and/or deltaP < 0). Eight of these 10 patients (80%) with abnormal EGGs had delayed GE. CONCLUSIONS: A high proportion of adult patients (60%) with functional dyspepsia have abnormally slow GE and abnormalities in gastric myoelectrical activity.     

Gastric myoelectrical activity in patients with gastric outlet obstruction and idiopathic gastroparesis

Objective: The cause of gastroparesis may be uncertain in some patients. Mechanical obstruction of the stomach or duodenum should be excluded in patients with idiopathic gastroparesis. The objective of this study was to compare gastric myoelectrical activity in patients with idiopathic gastroparesis with that of patients with gastroparesis due to mechanical obstruction of the stomach or duodenum.
Methods: Electrogastrography techniques were used to record gastric myoelectrical activity in 20 patients with idiopathic gastroparesis and in nine patients with gastroparesis secondary to gastric outlet obstruction. Four of these nine patients initially were thought to have idiopathic gastroparesis. Electrogastrograms (EGGs) were recorded from 29 healthy subjects who served as controls. EGGs were recorded for 20–30 min 2 h after a standard 200-Kcal meal and were analyzed visually and by computer.
Results: Patients with gastroparesis due to outlet obstruction had high-amplitude and excessively regular 3–cycles-per-minute (cpm) EGG patterns, whereas patients with idiopathic gastroparesis had primarily 1- to 2-cpm patterns and little 3-cpm EGG activity. The percentage of total EGG power in the 3-cpm range was approximately 50% in patients with gastric outlet obstruction compared with 20% in patients with idiopathic gastroparesis (   p < 0.001  ). The percentage of EGG power in the normal 3-cpm range was greater in the obstructed patients (50%) than in the healthy controls (35%;   p < 0.052  ).
Conclusions: Gastric myoelectrical patterns recorded in the EGG distinguish mechanical and idiopathic causes of gastroparesis and may be useful in evaluating patients with nausea, vomiting, and gastroparesis of unknown cause.     

Gastric myoelectrical activity and gastric emptying in diabetic patients with dyspeptic symptoms

INTRODUCTION Recently, electrogastrography(EGG) has received more andmore attention.     

Discordance between growth hormone (GH) responses after GH-releasing hormone and insulin hypoglycemia in myotonic dystrophy

Plasma GH responses to human GHRH, arginine, L-dopa, and insulin-induced hypoglycemia were determined in seven myotonic dystrophy (MD) patients. An iv bolus injection of GHRH-(1-44)-NH2 (1 microgram/kg BW) only slightly increased plasma GH concentrations in MD patients. The mean peak plasma GH level after GHRH injection [4.2 +/- 0.8 (+/- SE) micrograms/L] was significantly lower than that in 10 age-matched normal subjects (26.7 +/- 4.3 micrograms/L) or that in 6 patients with progressive muscular dystrophy (22.8 +/- 6.6 micrograms/L) whose nutritional status was similar to that of the MD patients. Even with a larger dose of GHRH (3 micrograms/kg BW), the plasma GH rises were minimal in the MD patients (mean peak, 5.9 +/- 1.8 micrograms/L). The plasma GH responses to a 30-min iv infusion of arginine (0.5 g/kg BW) and oral ingestion of L-dopa (0.5 g) were attenuated to a similar extent, whereas insulin-induced hypoglycemia caused a significant increase in plasma GH in all seven MD patients [mean peak, 17.4 +/- 4.1 (+/- SE) microgram/L]. The plasma TSH responses to TRH and plasma insulin-like growth factor I levels were similar in the MD patients and normal subjects. These findings suggest that 1) the impaired GH release after GHRH, arginine, and L-dopa administration in MD patients is not due to somatotroph deficiency, since the GH response to hypoglycemia is well preserved; and 2) insulin-induced hypoglycemia may stimulate GH release at least in part via inhibition of somatostatin release.     

Proximal and distal gut hormone secretion in irritable bowel syndrome

Objective. Sensory and motor dysfunctions of the gut are both important characteristics of irritable bowel syndrome (IBS). Several gut peptides contribute to the regulation of gastrointestinal function but little is known about gut hormone secretion in IBS. Material and methods. We evaluated perceptual thresholds and fasting and postprandial plasma levels of proximal (cholecystokinin (CCK), motilin) and distal (peptide YY) gut peptides up to 1 h after ingestion of a high caloric meal in 99 IBS patients and 40 age- and gender-matched healthy controls. Results. Fasting plasma CCK levels were significantly elevated in patients (1.2±0.8 pM) compared with those in controls (0.8±0.7 pM, p=0.006), as was the incremental postprandial CCK response (72±73 versus 40±42 pM·60 min, respectively; p=0.003). No differences in fasting and postprandial motilin or PYY levels were found. The postprandial PYY response was significantly increased in hypersensitive compared to normosensitive patients (215±135 versus 162±169 pM, p=0.048). Patients with a diarrhoea predominant bowel habit had higher fasting motilin levels compared to constipated patients or alternating type IBS patients (82.1±36.5 versus 60.8±25.1 versus 57.5±23.9 pM, one-way ANOVA p=0.003). Conclusions. IBS patients have increased fasting and postprandial plasma levels of CCK. Changes in plasma levels of motilin and PYY may contribute to the clinical expression of IBS, such as the presence of visceral hypersensitivity or a predominant bowel habit.