Severe Motor Disorders Research Articles

New Insight Into Intestinal Motor Function via Noninvasive Endoluminal Image Analysis

Evaluation of small bowel motility by intestinal manometry is invasive and requires expertise for interpretation. Our aim was to use capsule technology for evaluation of small bowel motor function based on a fully computerized image analysis program. Thirty-six consecutive patients with severe intestinal motor disorders (19 fulfilling manometric criteria of intestinal dysmotility and 17 not) and 50 healthy subjects received the endoscopic capsule (Pillcam; Given Imaging, Yokneam, Israel). Endoluminal image analysis was performed with a computer vision program specifically developed for the detection of contractile patterns (phasic luminal closure and radial wrinkles by wall texture analysis), noncontractile patterns (tunnel and wall appearance by Laplacian filtering), intestinal content (by color decomposition analysis), and endoluminal motion (by chromatic stability). Automatic classification of normal and abnormal intestinal motility was performed by means of a machine-learning technique. As compared with healthy subjects, patients exhibited less contractile activity (25% less phasic luminal closures, P < .05) and more noncontractile patterns (151% more tunnel pattern, P < .05), static sequences (56% more static images, P < .01), and turbid intestinal content (94% more static turbid images, P < .01). On cross validation, the classifier identified as abnormal all but 1 patient with manometric criteria of dysmotility and as normal all healthy subjects. Out of the 17 patients without manometric criteria of dysmotility, 11 were identified as abnormal and 6 as normal. Our study shows that endoluminal image analysis, by means of computer vision and machine-learning techniques, constitutes a reliable, noninvasive, and automated diagnostic test of intestinal motor disorders.

Manometric findings in adult eosinophilic oesophagitis: a study of 12 cases

To describe the manometric findings detected in adult patients with dysphagia that were diagnosed of eosinophilic oesophagitis, and to compare with the cases of eosinophilic infiltration of the oesophagus reported in the literature. We present 12 adult patients diagnosed as suffering from this disorder in our department in a 1.5-year period, according to histological criteria and discarding any other cause of eosinophilic infiltration of the oesophagus. Stationary oesophageal manometry using a hydropneumocapillary perfusion system was performed in every case. The recommendations of the Spanish Group of Digestive Motility were followed for the interpretation of the results. In seven patients who presented motor disorder in manometric evaluation, treatment with steroid oesophageal lavage using fluticasone propionate was carried out and these patients were subsequently re-evaluated. All patients were young predominantly men, and the first endoscopic examination showed regular concentric stenosis or a 'ring oesophagus'. Six patients had a severe nonspecific oesophageal motor disorder characterized by up to 80% of nontransmitted or very low-amplitude waves in the lower two-thirds of the organ. Three patients presented a manometric disturbance characterized by hyperkinetic peristaltic waves in distal oesophageal third. One patient had an alteration of the oesophageal motor dynamics characterized by 80% of deglutory complexes formed by a primary simultaneous wave in the two lower oesophageal thirds followed by a secondary peristaltic wave in 50% of cases that had a normal duration and amplitude. The remaining two patients had normal oesophageal motility. The upper oesophageal sphincter showed no alterations, and the manometric evaluation of the lower oesophageal sphincter tone proved normal in 10 patients, with slight hypotension in two cases. In seven of the nine patients who presented an oesophageal motor disorder, treatment with steroid oesophageal lavage using fluticasone propionate was administered and a new oesophageal manometry was performed afterwards, in which the motor disorder was clearly improved as soon as dysphagia, endoscopic lesions and histopathologic alteration disappeared. In the literature, 61 cases of eosinophilic infiltration of the oesophageal mucosa subjected to oesophageal manometric study had been described, and 60.6% of them showed evidence of different types of manometric alterations, mainly with spastic or hypercontractility characteristics. Although six of our cases showed very deficient peristalsis with very low-amplitude or nontransmitted waves, and in another three high-amplitude peristaltic waves were recorded. Motor disorders improved parallel to the disappearance of the eosinophilic infiltration of the mucosa. These data suggest that motor disorders in eosinophilic oesophagitis are a consequence of eosinophil infiltration of the oesophagus and should be considered in the differential diagnosis of dysphagia. These manometric alterations could be considered as primary nonspecific disorders and included in the 'ineffective oesophageal motility' group.

Changes in postural alignment when using kneeblocks for children with severe motor disorders

Purpose. Children with severe motor disorders often use adaptive seating devices to improve their postural alignment with aim of helping to prevent the development of deformity. Little objective evidence exists about their effectiveness, and this study sought to measure changes in postural alignment when using/not using the active element in one adaptive seating system.Method. A seating system that uses a sacral pad and kneeblock was examined. This seating device aims to neutralize the angular deviation of the hips and pelvis in order to give a stable base for functional sitting. Change in postural alignment was measured in 23 children with severe motor impairment (GMFCS 5) when using and when not using the kneeblock devices, immediately, and after a delay of one month.Results. We found that the removal or replacement of kneeblocks effected hip abduction and rotation on one side only, but no other immediate effect on joint angles and overall body posture.Conclusions. The postural management of children with neurological conditions requires further objective outcome measurement on which to base clinical practice, and improvement of posture is one of a number of factors which should be considered when providing adaptive seating to this group of children.

Leakage Testing at the Time of Surgical Oesophageal Myotomy

Background: Surgical myotomy is a well-established and validated method to treat severe gastro-oesophageal motor disorders such as achalasia. The benign character of these diseases further substantiates the importance of operating with greatest possible safety margins. We presently report our experiences with the use of perioperative leakage testing. Materials and Methods: Thirty-seven consecutive patients are reported of whom 30 had a laparoscopic, 3 a thoracoscopic and 4 an open operation. The indications for an operation were in 3 patients oesophageal spasm, in 30 patients newly diagnosed achalasia and 4 patients had an open reoperation due to a previous incomplete myotomy plus epiphrenic diverticulum. Thirty patients had a perioperative endoscopy with gas insufflation and a leakage test, whereas the others did not. Results: A previously unrecognised oesophageal mucosal tear was discovered during the test and repaired in 4 of the 30 tested cases whereafter everyone had an uneventful postoperative recovery. Among the remaining 7 untested patients, 3 developed clinical signs of leakage of whom 1 had an immediate reoperation. The postoperative courses were in all those prolonged and complicated. Conclusion: Perioperative use of endoscopy at the time of completion of the surgical myotomy is a useful tool to document leakage. Thereby the safety profile of the operation can be further enhanced.

Detection of movement-related potentials from the electro-encephalogram for possible use in a brain-computer interface.

Brain-computer interfaces are devices for enabling patients with severe motor disorders to communicate with the world. One method for operating such devices is to use movement-related potentials that are generated in the brain when the patient moves, or imagines a movement of, one of his limbs. An algorithm for detecting movement-related potentials using a small number of EEG channels was developed. This algorithm is a combination of the matched filter, a non-linear transformation previously developed as part of a similar detector, and a classifier. The algorithm was compared with previous designs of similar detectors by both theoretic analysis and off-line evaluation of performance on data recorded from five subjects. It is shown that the performance of the algorithm was superior to that of previous methods, improving the area under the receiver operating characteristic curve to 87.8%, an improvement of 25% compared with the best previously suggested detection method. Finally, the probable sources for false detections were identified, and possible ways to minimise them are proposed.

Computerized approaches to communication retraining after stroke.

Augmentative and alternative communication (AAC) strategies traditionally have been designed for individuals for whom oral speech is not a viable option. Common clinical practice has expanded AAC use to a tool for cognitive retraining or remediation. Although technologic developments offer the potential for vastly improved communication and inclusion for some individuals with severe communication disorders, technology alone does not result in successful communication. Although AAC interventions have become increasingly common across the age range, the research base underlying the design of AAC technology, intervention strategies, and treatment potential across communication disorders remains quite limited. To move beyond the traditional application of AAC strategies as a communication modality for persons with severe motor disorders to applications for persons with cognitive/linguistic disorders, three interrelated areas of research requiring further investigations are discussed. These include the following: use of AAC as a research tool to provide theoretical insights into the nature of normal and impaired language and cognition; development of AAC strategies as treatment tools to facilitate language recovery and use (production and comprehension); and studies that evaluate the efficacy of applying AAC strategies as communication modalities for individuals with acquired cognitive/linguistic communication disorders.

Polyunsaturated Fatty Acids Decrease Induced Pathological Disturbances in Rat Pups

Early recognition of pathological processes in CNS by the biotesting method allows correcting these processes since the prenatal period. A natural product, epaden, produced from fat of marine hydrobionts ( ω-3-polyunsaturated fatty acids) has an antiaggregation and antioxidant activity, as well as a pronounced adaptogenic, protective, and reducing action. Addition of epaden to diet of pregnant rats decreases twofold fetal lethality under effect of peptide factors causing disturbances of motor activity in newborns, increases viability index, leads to normalization of simple motor acts, and prevents from development of severe motor disorders in rat pups. A preliminary administration of epaden and its simultaneous administration with pathogenic factors produce a more pronounced positive effect than the subsequent treatment of induced embryological and motor disturbances.

Gastric electrical stimulation is superior to standard pharmacological treatment in reducing health care costs and hospital stays and in improving quality of life in patients with severe upper GI motor disorders

Gastric electrical stimulation is superior to standard pharmacological treatment in reducing health care costs and hospital stays and in improving quality of life in patients with severe upper GI motor disorders

上位頚椎手術症例の検討

Diseases of the upper cervical spine tend to cause severe instability, motor, sensory and respiratory disorders. We evaluated 27 patients who underwent upper cervical spine operations at our hospital to 1990. The patients consisting of 7 males and 20 females were followed up postoperatively for 3 months to 11 years (mean follow-up: 7 months and 3 years). Thirteen patients had rheumatoid arthritis, 5 congenital anomalies, 2 cerebral palsy, and 4 suffered traumatic injuries. We divided the atlanto-axial subluxation patients into 2 groups, H and V. The H group consisted of patients with horizontal dislocation. While the V group consisted of patients with vertical dislocation. Those in the H group were for the most part moderately young RA, congenital, traumatic patients, and those in the V group were old female RA patients. Most of the male patients were congenital and traumatic, with 6 belonging to the H group. In both groups, ADL and pain improved, but severe myelopathic symptoms did not improved significantly. After several days' halo-vest reducation, posterior spinal fusion, and bone graft were performed on each patients. In patients with vertical dislocation, C1 decompression was necessary; but because of instability after one fascet fusion and impossible to reduce, occipital fusion was performed. Both groups showed improvement inatlanto-axial instability. Improvement of ADI was better in the H group, which may be due to decreased pain.

Patterns of relative cerebral blood flow in minor cognitive motor disorder in human immunodeficiency virus infection.

Individuals infected with HIV are at risk to develop cognitive impairment during the course of their disease. Although many patients develop an HIV-associated dementia, others may develop the less severe minor cognitive motor disorder (MCMD). In this study, relative cerebral blood flow was measured with PET imaging in HIV+ MCMD patients, HIV+ control subjects, and HIV- control subjects; analyses were performed by using statistical parametric mapping. Comparing a short-term memory task versus a rest state yielded activation in superior temporal cortex, postcentral gyrus, and cerebellum in all three subject groups. Comparing long- and short-term memory tasks yielded activation throughout the frontal cortex, including BA46. Activation in this area was reduced in the HIV+ control subjects and further reduced in the MCMD+ patients. Thus, brain activation associated with lower-level, automatic processing appears normal in HIV+ MCMD+ subjects, but activation associated with effortful retrieval and organizational processes is abnormal.

Ablation of Cerebellar Golgi Cells Disrupts Synaptic Integration Involving GABA Inhibition and NMDA Receptor Activation in Motor Coordination

The role of inhibitory Golgi cells in cerebellar function was investigated by selectively ablating Golgi cells expressing human interleukin-2 receptor α subunit in transgenic mice, using the immunotoxin-mediated cell targeting technique. Golgi cell disruption caused severe acute motor disorders. These mice showed gradual recovery but retained a continuing inability to perform compound movements. Optical and electrical recordings combined with immunocytological analysis indicated that elimination of Golgi cells not only reduces GABA-mediated inhibition but also attenuates functional NMDA receptors in granule cells. These results demonstrate that synaptic integration involving both GABA inhibition and NMDA receptor activation is essential for compound motor coordination. Furthermore, this integration can adapt after Golgi cell elimination so as not to evoke overexcitation by the reduction of NMDA receptors.

Outcome of recommendations for augmentative communication in children.

Some children with severe motor disorders have unintelligible speech, and may be recommended augmentative communication systems, such as a symbol chart or a voice output aid. The paper reports the outcome after 15-18 months for 35 children of recommendations for augmentative communication. Using structured questionnaires, parents were asked whether equipment was provided as recommended. Their perception of success in children's use of augmentative aids was recorded and related to potentially influential factors. Twenty-five symbol systems, 10 speech output devices and 11 switches were received; 18 symbol systems were used for communication and 10 were used frequently. Seven speech output devices were used for communication but only two were reported to be used frequently. Factors leading to more successful outcomes include early receipt of the aid, perceived adequate local training in the use of the aid, and children aged 6 years or more at initial assessment. The findings also suggest that referring professionals will need to be better informed about the nature and limitations of augmentative communication aids, and that improved local professional input and careful interagency planning and co-ordination are required to achieve optimal outcome.

Patterns of colonic motility as recorded by a sham fecaloma reveal differences among patients with idiopathic chronic constipation.

By using a technique designated sham fecaloma, we were able to identify two types of segmentary motor phenomenon: displacement motor phenomena and nondisplacement motor phenomena. The aim of the study contained herein was to evaluate for identification of patients with different types of slow-transit constipation. Studies were performed in healthy subjects (n = 5; colonic transit time <30 hours) and in constipated patients (n = 6; colonic transit time >125 hours; normal rectoanal manometry). A Foley-type recording probe with two perfused catheters (proximal and distal) was used. A rigid sigmoidoscope was used to place the probe at the sigmoid colon. Values recorded by the distal catheter were subtracted (point by point) from the values recorded by the proximal catheter. Subtraction curves were analyzed to quantify characteristics of displacement motor phenomena (an anally directed pressure gradient) and nondisplacement motor phenomena (an orally directed pressure gradient). All healthy subjects had contractions during the recording session. Displacement motor phenomena were predominant (displacement motor phenomena/30 minutes = 21.2 +/- 13.2; range, 3-39). Constipated patients yielded two different patterns: three patients had a very small number of contractions, and three patients had a prevalence of nondisplacement motor phenomena, with numbers similar to numbers of displacement motor phenomena in healthy subjects. A comparison of the patterns of constipated patients revealed a statistically significant difference (P = 0.039). Sham fecaloma is a simple and safe test. Constipated patients in this study had two different patterns of colonic motility: scarce activity without haustration and normal activity without displacement motor phenomena. This method might be useful as a diagnostic tool for clarification of the pathophysiology of severe colonic motor disorders.

In vitro phosphorylation of medial vestibular nucleus and prepositus hypoglossi proteins during behavioural recovery from unilateral vestibular deafferentation in the guinea pig

Unilateral removal of vestibular nerve input to the vestibular nuclei (e.g. by unilateral labyrinthectomy, UL) results in severe ocular motor and postural disorders which disappear over time (vestibular compensation). We investigated whether recovery of ocular motor function is temporally correlated with changes in protein phosphorylation in the medial vestibular nucleus (MVN) and prepositus hypoglossi (PH; MVN/PH) in vitro. Bilateral MVN/PH were dissected from 48 guinea pigs following decapitation at 10 h, 53 h or 2 weeks post-UL, or -sham operation and frozen. Tissue extracts were incubated with [ γ- 32P]ATP±Ca 2+ plus phorbol 12,13-dibutyrate and phosphatidylserine. UL resulted in a significant bilateral increase in the 32P-incorporation into a 65–85 kDa band (probably the myristoylated alanine-rich C kinase substrate, MARCKS) in compensated animals (53 h post-UL) under conditions which favoured the activation of protein kinase C. Under identical conditions, the labelling of a 42–49 kDa protein (P46) was increased significantly in the bilateral MVN/PH between either 10 h or 53 h and 2 weeks post-UL; there were no significant changes over time in sham controls. These results show that later stages of vestibular compensation are accompanied by changes in the phosphorylation of several likely protein kinase C substrates in the MVN/PH in vitro.

Hidden Intelligence of a Multiply Handicapped Child with Joubert Syndrome

A child with Joubert syndrome (episodic hyperpnoea, abnormal eye-movements and ataxia) was followed from birth to eight years of age. Although severe mental retardation was suspected until approximately five years of age, unexpected and exceptional mental capacities were evident at later follow-up. Adequate testing and appropriate stimulation of children with such a severe early motor disorder are difficult, so they may give a false impression of mental retardation. The history of this child challenges the notion that mental retardation is part of Joubert syndrome.

7 Angina-like chest pain of oesophageal origin

Angina-like chest pain of non-cardiac origin is a major diagnostic and therapeutic problem. The oesophagus is frequently suspected to be the cause of the chest pain in these patients. However, a positive statement for the oesophageal origin of the pain can only be made when during manometry or pH-monitoring the familiar pain attack appears to be accompanied by reflux, severe motor disorders or a combination of both. Due to the intermittent nature of the pain this is only rarely the case during short-listing conventional examinations. Provocation tests have been used to induce the familiar chest pain. The Bernstein acid perfusion test and the edrophonium test yield the best results. Prolonged (24-hour) ambulatory recording of intra-oesophageal pressure and pH to increase the chances of recording chest pain concomitantly with an episode of reflux and/or motor disorders appears to be the most sensitive and also the most physiological test. It is the only test that provides reliable information on the underlying mechanism of the pain, especially in patients with the syndrome of irritable oesophagus, thus contributing in establishing the appropriate therapy for these patients.

24-Hour recording of esophageal pressure and pH in patients with noncardiac chest pain

Sixty patients with anginalike chest pain of noncardiac origin were studied to determine the diagnostic value of 24-h ambulatory esophageal pH and pressure monitoring. The results of these 24-h studies were compared with those obtained by established methods, including x-rays, endoscopy with biopsy, conventional esophageal manometry, and acid perfusion test. Esophageal origin of the chest pain was considered to be likely if the familiar pain sensation was reproduced by the acid perfusion test, or if the pain occurred during an episode of gastroesophageal reflux, severe motor disorders, or both. When the results of established methods were combined and interpreted according to predetermined criteria, esophageal origin of the pain was shown to be likely in 27% of the patients. The 24-h recordings, alone, showed the esophagus to be the likely cause of the pain in 35% of the patients. Combination of all conventional examinations and of 24-h recordings made esophageal origin of the pain likely in 48% of the patients.

Primary Disordered Motor Activity of the Esophagus (Diffuse Spasm): Diagnosis and Treatment

Primary disordered motor activity (PDMA) of the esophagus is characterized by abnormal motor activity in the lower two-thirds of the esophagus. Twenty-one patients with this syndrome were studied by history, roentgenography, endoscopy, manometry, and at operation. The results of a new and more radical approach to esophageal myotomy are evaluated. Pain, dysphagia, and weight loss are characteristic of PDMA. Motor spasm is seen roentgenographically, the esophageal wall is thickened, and with longstanding disease a retention esophagus develops. Manometry showed a very severe motor disorder in the lower two-thirds of the esophagus. Postoperative manometric studies were of value in distinguishing PDMA from achalasia, as proximal esophageal peristalsis was more recognizable. At operation muscle hyperplasia and spasm were found. A myotomy from the stomach to 10 cm. above the aortic arch was done, and it was found necessary to add a Collis gastroplasty to control reflux. With this approach all patients have had relief of pain and dysphagia.