1/461. Latent coeliac disease. Personal experience. Latent coeliac disease (L.C.D.) is an extremely rare condition to describe. In this study we analyzed three cases of patients affected by L.C.D.: two of them suffered from insulin-dependent diabetes mellitus (IDDM) and the other one from infantile cerebral palsy and eosinophilic gastroenteritis. We confirm the existence of this form of coeliac disease (C.D.), by means of duodenal biopsy, and stress the importance of an early diagnosis in order to prevent the serious consequences caused by untreated C.D. ( info) |
2/461. Hypotonia, congenital nystagmus, ataxia, and abnormal auditory brainstem responses: a report on the first white patient. A white Italian boy, aged 5 years and 8 months, is reported with failure to thrive, hypotonia, truncal ataxia, psychomotor retardation, and congenital horizontal pendular nystagmus with only waves I and II on auditory brainstem responses. Our patient's clinical picture resembles that previously reported in 10 male Oriental patients. He did not manifest spastic diplegia by the age of 2 years, as did the subjects reported in the literature, but knee-jerk hyperreflexia was evident at the most recent clinical reevaluation. Serial brain MRI studies revealed a cystic brain lesion and peritrigonal hyperintensities with no brainstem abnormalities. To date, no other child with a similar syndrome has been described either in europe or in America. The clinical features of this condition are consistent and characteristic. A definitive diagnosis is achieved by demonstrating the absence of all waves following wave I or wave II on auditory brainstem responses as early as 3 months of age. Due to the predominance of males, the occurrence in siblings, the early age at onset, the non-progressive course, and the characteristic auditory brainstem response findings, the syndrome may have a genetic origin and be attributable to a dysgenetic brainstem lesion. ( info) |
3/461. Shortening of a unit rod protruding into the hip joint: case report and description of a surgical technique. A unit rod is a well-accepted method of posterior spinal instrumentation in patients with cerebral palsy (CP). Several types of complications after the use of a unit rod were reported, including penetration through the medial wall of the pelvis. A patient who underwent anterior and posterior spinal fusion with unit-rod instrumentation was found to have penetration of the unit rod into a dysplastic hip joint. The pelvic limb of the rod was shortened with a burr through an anterolateral approach to the iliac bone. This procedure was associated with a lower blood loss than was previously reported for revisions of unit rods. There was no need for hardware removal and therefore no loss of correction. The unit rod may be shortened before surgery to prevent this problem. The described procedure is a simple way of correcting penetration of a unit-rod limb into the hip joint. ( info) |
4/461. Syntax PAL: a system to improve the written syntax of language-impaired users. In our work with children who have difficulty with spelling or with the physical action of writing, we have found a number of children who also have difficulty with written grammar. As an extension of PAL, an existing predictive spelling and typing aid, we have developed a writing aid to help these children with sentence construction. The enhanced system uses the syntax of the initial part of a sentence to enhance the position in the prediction list of syntactically correct words. It was postulated that this would discourage the use of incorrect syntax and encourage the use of correct syntax. In two case studies, the use of Syntax PAL significantly improved the quality and quantity of one child's written output, but had little effect on the other child's work. ( info) |
5/461. Using single-subject design in clinical decision making: the effects of tilt-in-space on head control for a child with cerebral palsy. Single-subject design methodology is a research technique useful in demonstrating the effects of treatment. The technique is described here using the effects of tilt-in-space on the ability to maintain head control. The subject is a 9-year-old child with cerebral palsy. The tilt-in-space angles used are 0 degrees , 15 degrees , and 30 degrees . Data are collected for the three different tilt angles in 10 sessions using an alternating rapid treatment design. Visual analysis of the results indicate that the subject displays greater head control when the wheelchair is placed at 15 degrees or 30 degrees tilt rather than at 0 degrees . This study shows how clinicians can complete single-subject studies to support recommendations for their clients and document the effects of their treatments. ( info) |
6/461. Hip dysplasia in spastic cerebral palsy. Previous reports have noted a relationship between pelvic obliquity and hip dysplasia in spastic cerebral palsy but did not confirm its existence by scientific study. A study is reported that confirms the association of pelvic obliquity with hip dysplasia in spastic cerebral palsy. At presentation of subluxation or dislocation prior to surgery, 80 patients were indexed into five body alignment types. Reclassifications were performed with passage of time in order to study the natural history and effects of surgery. In all cases, hip dysplasia was found to be consistent with the forces related to pelvic obliquity. ( info) |
7/461. independent living and the physical environment: aspects that matter to residents. Field interviews were conducted with seven clients with disabilities for the purpose of developing design guidelines for apartments suitable for independent living. Analysis of these data generated six factors that were highly valued and felt to contribute to the success of these individuals' venture into community living. Control appears to be the central construct and to subsume the other concepts: safety/security, accessibility/mobility, function, flexibility and privacy. These findings are presented and discussed here as a working model of environmental control. These ideas are suggested as hypotheses which would need to be tested and refined further before being used as a model to guide clinical interventions. ( info) |
8/461. A comparison of two computer input devices for uppercase letter matching. OBJECTIVE: To determine whether the touch Window or the mouse with an enlarged on-screen arrow was more effective or efficient for an on-screen letter-matching task completed by a boy 9 years of age with spastic quadriplegic cerebral palsy and visual and cognitive deficits. METHOD: A single-subject research design of 5 treatment phases, A1, B1, A2, B2, and A & B, was used. The total percentage of correct letter matches per treatment session, the total percentage correct per letter, and the amount of time needed to match 5 consecutive letters correctly were evaluated. RESULTS: The range and variability of letters correctly matched per session decreased and trends for correct letter matches accelerated when the participant used the mouse interface. Accuracy with matching 18 of 26 (69%) letters of the alphabet increased when selections were made with the mouse interface. The participant was faster when using the touch Window to match 5 consecutive letters correctly; however, regardless of the interface device used, letter matching remained slow and tedious. CONCLUSION: The mouse with an enlarged on-screen arrow and cursor was the more effective interface device with this child. Making a minor adjustment such as increasing the size of the on-screen arrow can make a common piece of equipment accessible to a person with disabilities. ( info) |
9/461. hope in the midst of challenge: evidence-based pastoral care. Describes the origins and characteristics of evidence-based pastoral care and utilizes a case with a person with cerebral palsy demonstrating this approach. Draws on evidence from research and critically evaluates evidence. Notes the dynamic relationship between research and clinical practice and makes suggestions about utilizing evidence-based pastoral care in chaplaincy. ( info) |
10/461. Extensor tendon dislocation in cerebral palsy. An 18-year-old man with cerebral palsy presented with a flexion deformity of the middle finger particularly at the metacarpophalangeal joint and ulnar dislocation of the extensor tendon. Releasing the tight ulnar sagittal band and imbricating the attenuated radial sagittal band allowed centralization of the extensor tendon. For complete correction of other deformities intrinsic release and extrinsic flexor muscle lengthening were done. Extensor tendon instability in this case was due to the combined forces of the extrinsic and intrinsic muscles on the retinacular system of the extensor mechanism. ( info) |