Ischemic forearm exercise invariably causes muscle cramps and pain in patients with glycolytic defects. We investigated an alternative diagnostic exercise test that may be better tolerated. Nine patients with McArdle disease, one with the partial glycolytic defect phosphoglycerate mutase deficiency, and nine matched, healthy subjects performed the classic ischemic forearm protocol and an identical protocol without ischemia. Blood was sampled in the median cubital vein of the exercised arm. Plasma lactate level increased similarly in healthy subjects during ischemic (Delta5.1 +/- 0.7mmol L(-1)) and non-ischemic (Delta4.4 +/- 0.3) tests and decreased similarly in McArdle patients (Delta-0.10 +/- 0.02 vs Delta-0.40 +/- 0.10mmol L(-1)). Postexercise peak lactate to ammonia ratios clearly separated patients and healthy controls in ischemic (McArdle, 4 +/- 2 [range, 1-12]; partial glycolytic defect phosphoglycerate mutase deficiency, 6; healthy, 33 +/- 4 [range, 17-56]) and non-ischemic (McArdle, 5 +/- 1 [range, 1-10]; partial glycolytic defect phosphoglycerate mutase deficiency, 5; healthy, 42 +/- 3 [range, 35-56]) protocols. Similar differences in lactate to ammonia ratio between patients and healthy subjects were observed in two other work protocols using intermittent handgrip contraction at 50% and static handgrip exercise at 30% of maximal voluntary contraction force. All patients developed pain and cramps during the ischemic test, and four had to abort the test prematurely. No patient experienced cramps in the non-ischemic test, and all completed the test. The findings indicate that the diagnostic ischemic forearm test for glycolytic disorders should be replaced by an aerobic forearm test.

Oxygen desaturation in venous blood from exercising muscle is markedly lower in patients with mitochondrial myopathy than in subjects with other muscle diseases and healthy subjects, suggesting that a forearm exercise test can be a diagnostic screening tool for mitochondrial myopathy.

Perineal descent was studied by defaecography with the patients in the sitting position in 55 healthy volunteers, 21 women with idiopathic faecal incontinence and 8 women with obstructed defaecation. This technique provides data necessary for the evaluation of defaecation disorders, i.e. morphological changes during defaecation as well as the dynamics of the pelvic floor. It was found that the pelvic floor position during rest and during straining is almost the same in women with incontinence and in women with obstructed defaecation. Furthermore patients with normal position of the pelvic floor during rest may exhibit considerable descent during straining while patients with abnormal position of the perineum during rest may show normal descent during straining. This observation may indicate that the first sign of abnormal function may be an increased descent during straining, only later following by descent during rest. The importance of establishing control data is emphasized since differences in defaecographic techniques between different centres may render comparison difficult.

Cineradiographic defaecography combined with measurement of the anorectal angle and descent of the pelvic floor is proposed. The method used in 73 women gave valuable information in 48 patients who complained of anal incompetence, rectal tenesmus, and chronic constipation. In these patients, high and low rectal intussusception, rectocele, and pathologic movement of the pelvic floor were detected. Some of these phenomena could only be diagnosed by the radiologic method here described. Quantitations of the anorectal angle and descent of the pelvic floor placed the group with constipation halfway between normal individuals and those with anal incompetence. The value of this finding is discussed. Recent improvements in anorectal surgery often make videodefaecography decisive for the choice of the optimal operative method. Therefore, videodefaecography together with measurement of the anorectal angle and pelvic floor descent is recommended whenever anorectal surgery for correction of functional disturbances is contemplated.Since the pioneer work of BRODEN & SNELLMAN in 1968 (4), cinedefaecography has only been sporadically mentioned in the literature (1, 5-7). However, during the past few years, anal incompetence has become a less concealed complaint in the female population. The fact that more patients demand help, and the refinement of therapeutic options, make cinedefaecography an important method to reveal abnormalities in the pelvic floor. Dysfunction in the anorectal region of psychologic origin cannot always be excluded (10, II).Since methods for objective diagnostic procedures are indicated, to improve cinedefaecography, we combined real-time videotape recordings with quantitative measurements of the anorectal angle (ARA) and descent of the pelvic floor (D). Material and MethodsVideodefaecography was performed in 73 women with a mean age of 55 years (range 26-82, evenly distributed). Twenty-five women were asymptomatic volunteers with a mean age of 53 years (range 28-78). Of the other 48 women, 25 had anal incompetence, 2 had flatus incompetence, 2 rectal tenesmus, 15 chronic constipation, and 4 patients had different complaints and were examined twice.The patients were prepared in the same way as for barium examination of the colon. Approximately 200 ml of thick barium contrast medium (a mixture of half-volume Mixobar oesophagus and half-volume Mixobar suspension) was instilled through a catheter into the rectum. At the end of instillation, the catheter was carefully withdrawn in order to mark the anal canal with contrast medium. The patient was then placed in a standardized pot chair in front of a fluoroscopic unit, with the lateral view of the rectum positioned in the centre of the field. Fluoroscopy was registered on videotape during rest and evacuation. The fluoroscopic position was held static during the whole procedure. Both the rectum, the anal canal, and the upper edge of the pot chair had to be visible on the TV simultaneously.From the video sequence, static images were acquired during rest and under...