Abnormal Motor Patterns Research Articles

Severe mental retardation, seizures, and hypotonia due to deletions of MEF2C

We present four patients, in whom we identified overlapping deletions in 5q14.3 involving MEF2C using a clinical oligonucleotide array comparative genomic hybridization (CGH) chromosomal microarray analysis (CMA). In case 1, CMA revealed an approximately 140 kb deletion encompassing the first three exons of MEF2C in a 3-year-old patient with severe psychomotor retardation, periodic tremor, and an abnormal motor pattern with mirror movement of the upper limbs observed during infancy, hypotonia, abnormal EEG, epilepsy, absence of speech, autistic behavior, bruxism, and mild dysmorphic features. MRI of the brain showed mild thinning of the corpus callosum and delay of white matter myelination in the occipital lobes. In case 2, an approximately 1.8 Mb deletion of TMEM161B and MEF2C was found in a child with severe developmental delay, hypotonia, and seizures. Patient 3 had epilepsy, hypotonia, thinning of the corpus callosum, and developmental delay associated with a de novo approximately 2.4 Mb deletion in 5q14.3 including MEF2C and five other genes. In case 4, a de novo approximately 5.7 Mb deletion of MEF2C and five other genes was found in a child with truncal hypotonia, intractable seizures, profound developmental delay, and shortening of the corpus callosum on brain MRI. These deletions further support that haploinsufficiency of MEF2C is responsible for severe mental retardation, seizures, and hypotonia. Our results, in combination with previous reports, imply that exon-targeted oligo array CGH, which is more efficient in identifying exonic copy number variants, should improve the detection of clinically significant deletions and duplications over arrays with probes spaced evenly throughout the genome.

Technical advances in monitoring human motility patterns

Abnormal motor patterns are implicated in many motility disorders. However, for many regions of the gut, our knowledge of normal and abnormal motility behaviors and mechanisms remains incomplete. There have been many recent advances in the development of techniques to increase our knowledge of gastrointestinal motility, some readily available while others remain confined to research centers. This review highlights a range of these recent developments and examines their potential to help diagnose and guide treatment for motility disorders.

Identification of abnormal motor cortex activation patterns in children with cerebral palsy by functional near-infrared spectroscopy.

We demonstrate the utility of functional near-infrared spectroscopy (fNIRS) as a tool for physicians to study cortical plasticity in children with cerebral palsy (CP). Motor cortex activation patterns were studied in five healthy children and five children with CP (8.4+/-2.3 years old in both groups) performing a finger-tapping protocol. Spatial (distance from center and area difference) and temporal (duration and time-to-peak) image metrics are proposed as potential biomarkers for differentiating abnormal cortical activation in children with CP from healthy pediatric controls. In addition, a similarity image-analysis concept is presented that unveils areas that have similar activation patterns as that of the maximum activation area, but are not discernible by visual inspection of standard activation images. Metrics derived from the images presenting areas of similarity are shown to be sensitive identifiers of abnormal activation patterns in children with CP. Importantly, the proposed similarity concept and related metrics may be applicable to other studies for the identification of cortical activation patterns by fNIRS.

Proximal colonic propagating pressure waves sequences and their relationship with movements of content in the proximal human colon

Abnormal colonic motor patterns have been implicated in the pathogenesis of severe constipation. Yet in health, the mechanical link between movement of colonic content and regional pressures have only been partially defined. This is largely due to current methodological limitations. Utilizing a combination of simultaneous colonic manometry, high-resolution scintigraphy and a quantitative technique for detecting discrete episodic flow, our aim was to examine the propulsive properties of colonic propagating sequences (PS) in the healthy colon. In six healthy volunteers a nasocolonic manometry catheter was positioned to record colonic pressures at 7.5 cm intervals from terminal ileum to the splenic flexure. With subjects positioned under a gamma camera, 30 MBq of (99m)Tc sulfur colloid was instilled into the terminal ileum, 22.5 cm proximal to the ileocolonic junction. Isotopic images were recorded (10 s/frame) and synchronized with the manometric trace. In the proximal colon we identified 137 antegrade PSs, of which 93% were deemed to be associated temporally with movements of luminal content. Low amplitude PSs, with component pressure waves between 2 mmHg and 5 mmHg, were as likely to be associated with colonic movements as higher amplitude PSs. As such there was no correlation between the amplitude of the PS and the temporal relationship with colonic movements. Within the proximal colon, 24 retrograde PSs were identified, 23 of which were associated with retrograde movements of colonic content. We conclude that proximal colonic PSs are highly propulsive and are a major determinant of proximal colonic flow.

Association of therapeutic occasion, gross motor function grading and developmental level with gross motor functional recovery in children with cerebral palsy

Background The abnormal posture and motor pattern have not stabilized in children with cerebral palsy at early period, thus timely treatment can establish normal postural reflex and motor pattern, and prevent complications of muscle contracture, ankylosis, skeletal deformity, etc. The clinical factors affecting the rehabilitative efficacy of gross motor function in children with cerebral palsy should be observed. Objective To observe the effects of therapeutic occasion, grading of gross motor function and developmental level on the rehabilitative efficacy in children with cerebral palsy. Design A case-controlled analysis. Setting Qilu Children's Hospital of Shandong University. Participants Totally 138 children with cerebral palsy, who were hospitalized for 12 months in the Rehabilitation Center of Qilu Children's Hospital, Shandong University, were selected from April 2004 to September 2006, and all the children were diagnosed to be accorded with the standard set by the national seminar on cerebral palsy in 2004. There were 97 males and 41 females, including 55 cases of 0–2 years old, 47 cases of 2–4 years and 36 cases of 4–6 years. Informed contents were obtained from relatives of all the children. Methods ▪ Comprehensive rehabilitation treatment: Vojta method was to induce the children to turn over the body and crawl by stimulating reflective turn over and crawling on belly. Bobath method including trainings of head control, turning over body, keeping sitting position, keeping balance, crawling, keeping standing position, and walking, etc.; The children were massaged by using the maneuvers of push, press, rub, pull, wave, etc. according to the sites and types of palsy. Acupuncture was performed mainly at bilateral motor areas, the needle was retained for 1 hour per time, 6 days continuously every week, and followed by a 1-day interval. ▪ Prognosis assessment: The gross motor functional grading of the children with cerebral palsy at admission was assessed using the Chinese version of gross motor function classification system (GMFCS). The gross motor functions of the children with cerebral palsy were assessed before rehabilitation treatment and 12 months after rehabilitation treatment by using gross motor function measure (GMFM). The improved GMFM total score was calculated. Developmental quotient was evaluated using Gesell developmental schedules (GDS) after 12-month rehabilitation treatment. Univariate analysis of variance was performed using the improved GMFM total score with age, GMFCS grade and developmental level (developmental quotient). Main outcome measures Effects of age started to accept rehabilitation treatment, GMFCS grade and developmental level on the recovery of gross motor function in children with cerebral palsy. Results All the 138 children with cerebral palsy were involved in the analysis of results. The improved GMFM total scores were significantly different among the children with cerebral palsy of different ages started to accept treatment, GMFCS grades and developmental levels ( F=13.464, 37.283, 30.814, P < 0.01). The younger the children started to accept treatment, the lower the their GMFCS grades and the higher their developmental levels, then the higher their improved GMFM total scores. Conclusion The recovery of gross motor function is better in younger children started to accepted treatment with cerebral palsy with lower GMFCS grades and higher developmental levels.

Botulinum toxin injection to facilitate rehabilitation of muscle imbalance syndromes in sports medicine

Intramuscular injection of Botulinum toxin to produce reduction of focal muscle overactivity, and localized muscle spasm, has been utilized therapeutically for almost two decades. Muscle overactivity in neurologically normal muscle, where an imbalance exists between a relatively overactive muscle and its less active synergist or antagonist, can inhibit control of the antagonist producing a functional muscle imbalance. This brief review provides an overview of the role of muscle imbalance in sports-related pain and dysfunction, and outlines the potential for intramuscular injection of Botulinum toxin to be used as an adjunct to specific muscle re-education and functional rehabilitation in this patient group. A comprehensive understanding of normal movement and the requirements of the sporting activity are essential to allow accurate diagnosis of abnormal motor patterns and to re-educate more appropriate movement strategies. Therapeutic management of co-impairments may include stretching of tight soft tissues, specific re-education aimed at isolation of the non-dominant weak muscles and improvement in their activation, ‘unlearning’ of faulty motor patterns, and eventual progression onto functional exercises to anticipate gradual return to sporting activity. Intramuscular injection of Botulinum toxin, in carefully selected cases, provides short term reduction of focal muscle overactivity, and may facilitate activation of relatively ‘inhibited’ muscles and assist the restoration of more appropriate motor patterns.

Arm Trajectories in Dyskinetic Cerebral Palsy Have Increased Random Variability

Dyskinetic cerebral palsy results from injury to the basal ganglia early in life. Symptoms can include hyperkinetic or dystonic arm movements that impair function. It is not known whether these movements comprise a small number of specific abnormal motor patterns or whether they are random and variable. We hypothesize that injury to the basal ganglia leads to impaired filtering and removal of undesired neural signals and that lack of appropriate removal of noisy or irrelevant neural signals leads to random and variable arm movements. To test this hypothesis, we quantified the variability in arm trajectories while seven children with dyskinetic cerebral palsy between the ages of 4 and 13 years old made repeated outward reaching movements. We compared the results with those of 21 healthy children between the ages of 5 and 16 years. The best-fit trajectory to the set of reaching movements for each child was taken as the predictable component of movement. We calculated the ratio of the power in the best-fit trajectory to the total variance. This measure is the signal-to-noise ratio, and it quantifies the extent to which trajectories are predictable. We found that children with dyskinetic cerebral palsy had a significantly reduced signal-to-noise ratio compared with healthy children at similar ages. This result shows that there is increased movement variability, and it is consistent with the hypothesis that inadequate removal of noisy signals could be a cause of the movement disorder in dyskinetic cerebral palsy.

Natural History of Chronic Idiopathic Intestinal Pseudo-Obstruction in Adults: A Single Center Study

Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare disease characterized by episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders. Intestinal motor abnormalities have long been identified in CIIP patients. Little is known of the natural history of the disease in adults. This study evaluated the clinical course of CIIP over time. Fifty-nine consecutive CIIP patients without underlying collagen, vascular diseases, or mitochondrial cytopathies were evaluated between 1985 and 2001. Family history, onset of digestive symptoms, previous surgeries, episodes suggestive of subacute intestinal obstruction, digestive symptoms, body mass index, and feeding habits were recorded. Small bowel manometry was performed by a perfusion technique, and abnormal motor patterns were visually identified. Full-thickness biopsies were available in 11 cases and were processed for immunohistochemical analysis of myogenic and neurogenic components of the gut wall. Patients were prospectively followed up for a median of 4.6 years (range, 1-13 years). Diagnosis was often made several years after symptom onset (median, 8 years). Thus, the majority of patients (88%) underwent useless and potentially dangerous surgeries (mean, 2.96 per patient). Manometry invariably showed abnormal motor patterns. Pathologic findings included neuropathies in all investigated cases and abnormalities of interstitial cells of Cajal in 5 of 11 cases. Long-term outcome was generally poor despite surgical and medical therapies; 4 patients died of disease-related complications, 4 underwent small bowel transplantation, almost one third required long-term home parenteral nutrition, and two thirds had some sort of nutritional limitations. CIIP is a severe, often unrecognized disease characterized by disabling and potentially life-threatening complications over time.

Abnormal predefecatory colonic motor patterns define constipation in obstructed defecation

Background & Aims: The pathophysiology of constipation in the syndrome of obstructed defecation is unknown. Using 24-hour pancolonic manometric recordings of the unprepared colon to record basal pressures and spontaneous defecation episodes, we tested the hypothesis that the frequency, timing, or spatial distribution of propagating colonic pressure waves is abnormal in patients with obstructed defecation. Methods: In 11 patients with obstructed defecation and 16 healthy controls, pressures were recorded using a nasocolonic catheter that was positioned such that 16 recording sites spanned the unprepared colon at 7.5-cm intervals. Results: The overall frequency of propagating sequences (PS) in the colon did not differ between patients and controls. When compared with controls, patients had a significant increase in the frequency of retrograde and antegrade PS ( P < 0.05) in the left colon and a significant reduction in the amplitude of propagating pressure waves throughout the entire colon ( P < 0.03). Defecation occurred in 6 of 11 patients and 9 of 16 controls. In the 15 minutes before defecation, controls showed a highly significant increase in frequency ( P = 0.001) and amplitude ( P = 0.01) of PS. In contrast, patients did not demonstrate this or the typical spatiotemporal organization of PS normally observed before expulsion of stool. Conclusions: Patients with obstructed defecation lack the normal predefecatory augmentation in frequency and amplitude of propagating pressure waves and lack the normal stereotypic spatiotemporal patterning of colonic pressure waves that would normally culminate in effective expulsion of stool.

Musculoskeletal aspects of cerebral palsy.

©2003 British Editorial Society of Bone and Joint Surgery doi.10.1302/0301-620X.85B2.14066 $2.00 J Bone Joint Surg [Br] 2003;85-B:157-66. Cerebral palsy is the most common cause of physical disability affecting children in developed countries, with an incidence of 2.0 to 2.5 per 1000 live births.1 It is not a single entity but a heterogeneous collection of clinical syndromes, characterised by abnormal motor patterns and postures. Although in most parts of the world the orthopaedic burden secondary to poliomyelitis and myelomeningocele is declining rapidly, the prevalence of cerebral palsy is static or increasing. It is the most common diagnosis after trauma in most paediatric orthopaedic units and is therefore of enormous strategic importance in terms of allocation of resources, planning and service delivery. The definitions of cerebral palsy have undergone a number of refinements by developmental paediatricians and neurologists. They stress two features. First, cerebral palsy is the result of a lesion in the immature brain, which is nonprogressive; it is a static encephalopathy.2 It is clearly important to differentiate cerebral palsy from progressive neurological conditions from the standpoint of both taxonomy and clinical management. Secondly, cerebral palsy results in a disorder of posture and movement which is permanent but not unchanging.3 To this we would add a third feature, that it results in progressive musculoskeletal pathology in most affected children.4 It is inappropriate to emphasise that the cerebral lesion is static without clearly stating that the musculoskeletal pathology will be progressive. In Little’s original description5 of spastic diplegia, prominence was given to the description of the musculoskeletal deformities. The newborn child with cerebral palsy usually has no deformities or musculoskeletal abnormalities at birth. Scoliosis, dislocation of the hip and fixed contractures develop during the rapid growth of childhood. Cerebral palsy is a useful term which describes a large group of children with motor impairment from many causes and expressed as a wide variety of clinical syndromes. The preferred term is therefore ‘the cerebral palsies’.6 It was formerly considered that most cases of cerebral palsy were the result of obstetric misadventure. Careful epidemiological and brain-imaging studies suggest that it frequently has antenatal antecedents and is often multifactorial. Recent studies also point to an increasing number of specific aetiological factors including intrauterine infections and inherited malformations.1 These investigations will in time lead to both primary prevention and secondary minimisation of cerebral injury. The increase in the incidence of cerebral palsy in preterm infants is because of neonatal intensive care and a rise in multiple births. The rates of cerebral palsy in babies born at term are steady, despite strategies to reduce birth asphyxia. The most important issue in cerebral palsy is the elucidation of the causal pathways from population-based epidemiological studies. This will lead to primary prevention, which in a chronic, incurable condition is clearly the most humane and cost-effective strategy. Cerebral palsy is subdivided according to the movement disorder and its topographical distribution. Spastic and mixed motor disorders account for more than 85% of children on current registers; dyskinetic cerebral palsy is much less common.1,6 The most common topographical syndromes are spastic hemiplegia, spastic diplegia and spastic quadriplegia which is also known as ‘whole-body involvement’.1,3,6

Paralysie du plexus brachial d’origine infectieuse : à propos de quatre cas

Background. – Brachial plexus palsy (BPP) may occur after septic osteoarthritis of the shoulder. BPP is a rare, well recognized complication. The pathophysiology is thought to be either ischemic nerve damage secondary to occlusion of vasa nervorum or extrinsic compression. Early diagnosis and treatment of the infection are needed. Case reports. – We report the cases of three infants aged less than three months and one six-day-old newborn infant who presented with an authentic BPP due to an osteoarthritis of the shoulder. The diagnosis of osteoarthritis relied on clinical, biological and radiological signs. In one case, the causal germ (Enterobacter) was isolated. The diagnosis of the palsy was based on clinical signs. EMG performed on one patient, showed signs of abnormal motor pattern. Following intravenous antibiotics and percutaneous drainage, all infants completely recovered.

Oesophageal motor and sensitivity abnormalities in non-obstructive dysphagia.

To evaluate oesophageal sensitivity to balloon distension in patients with non-obstructive dysphagia (NOD), and to determine its relationship with the motility pattern in response to food ingestion. Twenty-one healthy volunteers and 19 consecutive patients complaining of NOD with normal standard manometry were included. An oesophageal sensitivity test was carried out before the manometry study with liquid and solid swallows. The median threshold to distension was 9 ml in control subjects and 5 ml in patients (P < 0.002). Dysphagia or odynophagia were reproduced in 15/19 (78.9%) patients during manometry with solid swallows only. The percentage of swallows with abnormal motility patterns was higher in patients than control subjects (P < 0.001). Compared with control values, sensitivity abnormality was defined by a distension threshold of < 6 ml. Motor abnormality was defined by > 19% of swallows occurring with one or more abnormal motor profiles. A total of 8/19 (42%) patients presented with the association of an abnormal sensitivity threshold and an abnormal motor pattern; 5/19 (26%) presented with isolated motor abnormalities; 4/19 (21%) patients presented with isolated abnormal sensitivity thresholds; and 2/19 (11%) patients presented without any abnormality. Manometry with solid swallows and oesophageal balloon distension are useful in characterizing NOD.

Disorders of gastrointestinal motility: towards a new classification.

can be learnt from the patient. In the last decade, the ‘Delphic’ technique has been used to try and define combinations of symptoms in the belief, or hope, that specific symptom patterns correspond to specific underlying disorders. The ‘Rome criteria’ for the definition and diagnosis of functional gastrointestinal disorders have received much attention. Unfortunately, consensus of opinions by experts does not, per se, confer scientific validity. Evidence-based medicine requires not consensus, but evidence. We have reappraised the problem of classifying motor disorders by relying on what can be established by the detection of abnormal motor patterns, usually, but not invariably, associated with the altered movement of the contents of the digestive tube. In some, but not yet all, disorders, this approach is reinforced by identification of underlying pathological change in enteric innervation or musculature. While we remain aware that the association between symptoms—the perception that drives patients to seek help—and motor abnormalities is not always clear, we have taken the view that objectively reproducible alterations in organ function provide a robust basis for taxonomy. Such problems are not unique to gastroenterology; as an example, the association between dyspnea and specific pulmonary pathologies is not always clear, but dyspnea is a useful indication of abnormal respiratory function indicative of disease. Clinicians may feel dismayed that we have not elected to define two commonly used terms: ‘functional dysINTRODUCTION

Clinical use of manometry for the diagnosis of intestinal motor abnormalities.

Digestive symptoms suggestive of intestinal motor disorders, such as abdominal pain and distension, fullness, vomiting, constipation and diarrhoea, are very common and non-specific, and may be clinical manifestations of both organic and functional diseases. Both radiology and endoscopy are important in the diagnosis of structural gastrointestinal lesions that can affect motility and offer indirect signs of impaired gastrointestinal functions, but the diagnosis of gut motility disorders currently relies on the manometric assessment of contractile activities. Small bowel manometry helps to identify normal motility features and consequently to identify abnormal motor patterns. Small bowel manometry can help to differentiate mechanical obstruction from pseudo-obstruction and neurogenic from myogenic motor disorders. Manometry is an invasive technique which is not well accepted by patients and requires specific skills from investigators. Also, manometric assessment is limited to referral centres with a specific interest in the field of digestive functions, in general, and motility, in particular. Only patients who remain undiagnosed after extensive traditional work-up and fail repeated courses with medical therapy should be referred for the manometric test. Understanding the underlying pathophysiologic mechanisms of abnormal motility and developing new therapies are the goals of the current research in this fascinating field of medicine.

Small bowel motor abnormalities in malnourished symptomatic adults complaining of severe digestive symptoms with and without functional subocclusive episodes

Aim. To compare small bowel manometric findings in patients with severe digestive symptoms and inability to maintain a normal body weight, with or without recurrent subocclusive episodes in the absence of mechanical obstruction. Methods. Small bowel manometry was performed (fasting> 6h, post-prandial> Ih) by a traditional stationary perfused technique in 62 patients (34.5 ± 12.8 yrs, mean + SD; 57 F) with severe GI symptoms and inability to maintain a normal body weight (BMI 2) radiologically proven subocclusive episodes (distended intestinal loops I airfluid levels) (GROUP A), while the remaining 37 (34.0± 11.9 yrs, mean+SD; 35 F) had not (GROUP B). Organic. systemic and metabolic diseases were ruled out in all patients. The following abnormal motor patterns were visually analysed: abnormalactivity fronts (AbnAFs), bursts, hypermotility, inability to produce a fed pattern (No-FED), clustered contractions (clusters). RESULTS. Patients in GROUP A had undergone a higher number of abdominal surgeries before coming to our laboratory (3.80±2.51 vs 0.94±1.58; p<O.OOI, Mann-Whitney). At least one motor abnormality was recorded in all patients in both groups (see table). CONCLUSIONS. Small bowel motor abnormalities are invariably present in patients with severe digestive symptoms and inability to maintain a normal body weight, regardless of the presence of functional intestinal subocclusions. Patients with recurrent subocclusions have a significatively higher frequency of abnormal motor patterns suggestive of a greater derangement of the control mechanisms of gut motility.

Early identification of cerebral palsy using a profile of abnormal motor patterns

Physical growth of preterm infants relates to many medical factors, such as birthweight, severity of medical illnesses, and nutritional status. We previously reported that maternal behaviors influence developmental outcomes in low birthweight infants (birthweight < 1600 g); we now hypothesize that maternal behaviors also influence physical somatic growth in low birthweight (LBW) infants. We serially followed 218 mother-infant pairs from birth through 12 months of age. One-hundred thirteen LBW infants were categorized based on severity of early medical complications. Low Risk infants (LR, n = 71) had acute respiratory distress and/or grade 1–2 intraventricular hemorrhage (IVH), or grade 3 IVH without hydrocephalus. High Risk infants (HR, n = 42) had chronic lung disease, grade 3 IVH with hydrocephalus or grade 4 IVH, and/or periventricular leukomalacia. We also studied 105 socioeconomic (SES) matched Full Term (FT) controls. Maternal behaviors were assessed during home visits with global ratings of Warm Sensitivity and Punitiveness. Infant weight was measured at birth and at 38 weeks, 6 months and 12 months corrected gestational age. We examined alternative expressions of weight growth across the three groups by developing the Weight Quotient (WQ), which is the ratio of actual measured weight to the median weight for age. For each infant the regression of the WQ ratio against correct gestational age was analyzed. We used a General Linear Model to compare the relation of the maternal variables to the weight quotients for the three groups. We determined the catch up growth as the slope of each regression. Results indicated that higher levels of maternal Punitiveness were related to slower rates of growth for High Risk (R2 = 0.36), but not Low Risk or Full Term. For maternal Warm Sensitivity (R2 = 0.36), there were significant (P < 0.05) inverse relations with weight gain growth for the Full Term infants. These data suggest that some maternal behaviors are related to the growth of term and premature infants, although the mechanisms through which this occurs is unclear.

EARLY IDENTIFICATION OF CEREBRAL PALSY USING A PROFILE OF ABNORMAL MOTOR PATTERNS

Objective To determine whether a profile of abnormal motor patterns can identify children with cerebral palsy (CP) in the first year of life. Methods The Early Motor Pattern Profile (EMPP) consists of 15 items reflecting variations in muscle tone, reflexes, and movement that have been organized into a standardized format to provide the clinician with an objective picture of neurologic status. A three-point scoring system was applied to each item, delineating a clearly normal response from a clearly abnormal one and placing all partial or inconsistent responses in the middle. Twelve hundred forty-seven high-risk infants who were enrolled in a neonatal intensive care unit follow-up program were examined at 6 and/or 12 months' corrected age using the EMPP. These infants were followed to at least 36 months of age to distinguish those with CP from those with normal motor outcome or minimal impairment (no CP). Results Predictive validity of the EMPP at the 6- and 12-month examinations was determined using various pass-fail cutoffs. The optimal cutoff score at 6 months was between 9 and 10, at which the positive predictive value was 89.4, sensitivity was 87.1, and specificity was 97.8. The optimal cutoff score at 12 months was between 3 and 4, at which the positive predictive value was 91.0, sensitivity was 91.5, and specificity was 97.9. Conclusions The EMPP offers the clinician an effective instrument to identify children in the first year of life who are at greatest risk for the development of CP. The EMPP can be incorporated into a routine health maintenance visit, adding only a few minutes to the process, and has high sensitivity and specificity.

Early Identification of Cerebral Palsy Using a Profile of Abnormal Motor Patterns

To determine whether a profile of abnormal motor patterns can identify children with cerebral palsy (CP) in the first year of life. The Early Motor Pattern Profile (EMPP) consists of 15 items reflecting variations in muscle tone, reflexes, and movement that have been organized into a standardized format to provide the clinician with an objective picture of neurologic status. A three-point scoring system was applied to each item, delineating a clearly normal response from a clearly abnormal one and placing all partial or inconsistent responses in the middle. Twelve hundred forty-seven high-risk infants who were enrolled in a neonatal intensive care unit follow-up program were examined at 6 and/or 12 months' corrected age using the EMPP. These infants were followed to at least 36 months of age to distinguish those with CP from those with normal motor outcome or minimal impairment (no CP). Predictive validity of the EMPP at the 6- and 12-month examinations was determined using various pass-fail cutoffs. The optimal cutoff score at 6 months was between 9 and 10, at which the positive predictive value was 89.4, sensitivity was 87.1, and specificity was 97.8. The optimal cutoff score at 12 months was between 3 and 4, at which the positive predictive value was 91.0, sensitivity was 91.5, and specificity was 97.9. The EMPP offers the clinician an effective instrument to identify children in the first year of life who are at greatest risk for the development of CP. The EMPP can be incorporated into a routine health maintenance visit, adding only a few minutes to the process, and has high sensitivity and specificity.

Effect of intestinal resection on human small bowel motility.

Few data are available on adaptive changes of human small bowel motility after intestinal resection. To characterise jejunal motility after extensive and limited distal intestinal resection. Seven patients with a short bowel syndrome after total ileal and partial jejunal resection (residual jejunal segments between 60 and 100 cm) and six patients with limited distal ileal resection (resected segment between 30 and 70 cm) underwent ambulatory 24 hour jejunal manometry 15 (6-24) months after the operation. Normal values were obtained from 50 healthy subjects. Fasting motility and the motor response to a 600 kcal solid meal were analysed visually and by a computer program. Limited ileal resection did not result in changed jejunal motility. After extensive distal resection, patients had a significantly shorter migrating motor complex (MMC) cycle and a significantly shorter duration of the postprandial motor response compared with controls (p < 0.005). Intestinal resection had no influence on jejunal contraction frequency and amplitude and did not lead to any abnormal motor pattern. Extensive distal resection of the small intestine produces distinct abnormalities of fasting and postprandial motility in the intestinal remnant. The shortening of digestive motility and the increased frequency of MMC cycling could contribute to malabsorption and diarrhoea in the short bowel syndrome.

Abnormal intestinal motor patterns explain enteric colonization with gram-negative bacilli in late radiation enteropathy

Background & Aims : Bacterial overgrowth and intestinal pseudo-obstruction may succeed abdominal radiotherapy, and absence of intestinal migrating motor complex (MMC) has been reported in bacterial overgrowth. The aims of this study were to address the relationship between intestinal patterns of motility and gastrointestinal microflora and to elucidate the pathogenesis of late radiation enteropathy. Methods : Fortyone consecutive female patients with symptoms of late radiation enteropathy were examined by prolonged ambulatory manometry, culture of gastric and duodenal samples with quantification of gram-negative bacilli (GNB) by the glucose gas test, the [ 14C] d-xylose breath test, and determination of pH and short-chain fatty acids in gastric juice. Results : The intensity of MMC explained 61% ( P < 0.001) and 71% ( P < 0.001) of the variability of GNB in the stomach and duodenum, respectively, corresponding to the severity of disease. Abnormal MMC index and presence of irregular bursts were the best predictors of GNB (86%; P < 0.001, multiple regression). Fasting gastric pH explained gastric bacterial counts (63%; P < 0.001) but did not predict GNB. Conclusions : Impaired motility emerges as a causal factor for gastrointestinal colonization with GNB, whereas hypochlorhydria facilitates unspecific gastric colonization. Abnormal motility and GNB in the proximal small intestine are essential factors in the pathogenesis of severe late radiation enteropathy.