Botulinum toxin type A in cervical dystonia.

Cervical dystonia is a syndrome characterized by anomalous postures and unintentional repetitive movements of the head and the neck. Aim of this study is to show the effectiveness of the combined treatment botulinum toxin-FKT through the use of a recent methodic of investigation, myoton, and of the classical clinical evaluations, such as Tsui and VAS scales. 15 patients (9 females, 6 males) have been selected. During the initial visit and in the following controls, performed every month, patients have been submitted to physiatric examination, clinical evaluation of the dystonia through the Tsui scale, clinical evaluation of the pain through the VAS scale, myometric evaluation. We obtained a statistical reduction of muscular tone values in passive elongation (t0=16,34±1,23) until 4 month (t4 =16,11±1,23), when we performed a second infiltration. After 4 months from the second infiltration (t8=15,99±1,11) value did not present more some statistical correlation and was necessary to perform a new infiltration. Values of elasticity, stiffness, Tsui and VAS scale followed the same course of the tone. Our study demonstrates that combined treatment botulinum toxin-FKT is effective. It emerges that if to the treatment with botulinum toxin follows a suitable FKT treatment, the effect of the drug lasts 4 months and not 3 months as described by the literature. Our study has underlined that using myometric measurement we can consider least changes in muscular tone, elasticity and stiffness; we have a broader view of the spastic muscle, and we can better plan appropriate rehabilitative care for each patient.

Three persons with multiple disabilities accessing environmental stimuli and asking for social contact through microswitch and VOCA technology.

BACKGROUND: Direct access to environmental stimuli and opportunity to ask for social contact/attention may be considered highly relevant objectives for persons with multiple disabilities. We assessed the possibility of enabling three of these persons (two children and one adolescent) to combine two microswitches (for accessing environmental stimuli) and a Voice Output Communication Aid (VOCA), which allowed them to ask for caregiver's attention. METHODS: Initially, the participants were required to use each of the two microswitches individually and then together. Thereafter, they were taught to use the VOCA. Eventually, the VOCA was available together with the microswitches, and the participants could use any of the three. RESULTS: The results, which support preliminary data on this topic, showed that all participants (1) were able to operate the two microswitches as well as the VOCA; and (2) used all three of them consistently when they were simultaneously available. CONCLUSIONS: Teaching persons with multiple disabilities to combine a VOCA with conventional microswitches may enrich their general input, emphasize their active social role and eventually enhance their social image.

Behavioural flexibility in individuals with Angelman syndrome, Down syndrome, non-specific intellectual disability and Autism spectrum disorder.

BACKGROUND: Little is known about behavioural flexibility in children and adults with Angelman syndrome and whether people with this syndrome have more or less problems in being behaviourally flexible as compared with other people. METHOD: Behavioural flexibility scores were assessed in 129 individuals with Angelman syndrome using 11 items from the Behavioural Flexibility Rating Scale-Revised (Green et al. 2007). Level of behavioural flexibility scores in individuals with Angelman syndrome (N = 129) was compared with that of people with non-specific intellectual disability (ID) (N = 90), Down syndrome (N = 398) and Autism spectrum disorder (N = 235). RESULTS: Comparative analyses show that individuals with Angelman syndrome were more flexible than those with non-specific ID (P < 0.001) and those with Autism spectrum disorder (P < 0.01). There were no differences in behavioural flexibility scores between individuals with Angelman syndrome and those with Down syndrome (P = 0.94). CONCLUSION: It is concluded that individuals with Angelman syndrome are comparatively flexible in their behaviour.

Promoting step responses of children with multiple disabilities through a walker device and microswitches with contingent stimuli.

Children with severe or profound intellectual and motor disabilities often present problems of balance and locomotion and spend much of their time sitting or lying, with negative consequences for their development and social image. This study provides a replication of recent (pilot) studies using a walker (support) device and microswitches with preferred stimuli to promote locomotion in two children with multiple disabilities. One child used an ABAB design; the other only an AB sequence. Both succeeded in increasing their frequencies of step responses during the B (intervention) phase(s). These findings support the positive evidence already available on the effectiveness of this intervention in motivating and promoting children's locomotion.

Microswitch for vocalization responses: comparing single- versus dual-microphone arrangements for a man with multiple disabilities.

Microswitches are technical devices designed to enable persons with multiple disabilities to control environmental events, generally preferred stimuli such as music and lights, using simple/feasible responses. For example, a sound-detecting microswitch may allow a person to turn on brief periods of preferred stimulation through simple vocal responses. This study compared the accuracy of two versions of a sound-detecting microswitch, i.e., one using a throat microphone and the other both throat and airborne microphones, for a man with multiple disabilities. Analysis showed that the latter microswitch version radically reduced the false activations present with the former microswitch version. Thus, the latter version seemed to have a clear accuracy advantage over the former with important practical implications.

Eye- and mouth-opening movements replacing head and hand responses in a microswitch program for an adolescent with deteriorating motor condition.

This study assessed the possibility of replacing head and hand responses (no longer feasible) with minimal movements within the microswitch-based program of an adolescent with deteriorating motor condition and multiple disabilities. The new movements, i.e., eye- and mouth-opening, were introduced individually and then combined through the simultaneous availability of the related microswitches. Data showed the participant acquired the new movements (responses) successfully and retained them at a 2-mo. postintervention check. Mood improvements, i.e., increases in indices of happiness, also occurred through the program. The conclusion was that a person with deteriorating motor conditions may be able to revitalize a microswitch-based occupational program and retain a constructive engagement if new, feasible responses are identified.

Small hand-closure movements used as a response through microswitch technology by persons with multiple disabilities and minimal motor behavior.

This study assessed small hand-closure movements as a potential response for microswitch activation with two participants with profound multiple disabilities of 5.2 and 20.6 yr. of age. The microswitch consisted of a two-membrane thin pad fixed to the palm of the hand and a control system. The outer membrane (the one facing the fingers) was a touch-sensitive layer; the inner membrane was activated if the participant applied a pressure of over 20 gm. The activation of either membrane triggered an electronic control system, which in turn activated one or more preferred stimuli for 6 sec. except in baseline phases. Each participant received an ABAB sequence, in which A represented baseline and B intervention phases, and a 1-mo. postintervention check. Analysis showed both participants increased their responding during the intervention phases and maintained that responding at the postintervention check. Implications of the findings are discussed.

Enabling two adolescents with multiple disabilities to choose among environmental stimuli through different procedural and technological approaches.

Two single-case studies were carried out using different procedural and technological approaches to enable two adolescents with multiple disabilities to choose among environmental stimuli. Study I focused on replicating a recently developed procedure, which relied on samples of the auditory stimuli available as cues for choice responses. Study II assessed a new procedural and technical setup relying on the use of pictorial representations of the stimuli available as cues for choice responses. The auditory samples and the pictorial representations were presented through computer systems. The participants' choice responses relied on microswitches connected to the computer systems. The data of Study I fully supported previous findings with the same procedural approach. The participant learned to choose preferred stimuli and bypass nonpreferred ones. The data of Study II showed that the participant learned to concentrate his choice. responses on a few stimuli, suggesting that these stimuli were actually preferred and that responding was purposeful. Implications of the results were discussed.

Enabling a young man with minimal motor behavior to manage independently his leisure television engagement.

Persons with severe spastic tetraparesis and minimal motor behavior may be confined to a wheelchair or bed and have virtually no chances of constructive engagement with their immediate environment. A possible way to modify this situation may involve the use of technology. The present study (a) assessed specific technology to enable a young adult to manage his leisure television engagement independently and (b) carried out a social validation assessment of the technology-supported performance involving 90 teacher trainees as raters. The intervention period with the new technology included 67 sessions, during which the participant performed independently 392 of the 408 television-management responses, i.e., turning on the television, finding a channel with a preferred program, setting the volume, and turning off the television. He also indicated preference for using the technology as opposed to not using it. The raters provided relatively high (positive) scores for the technology-supported performance compared to the baseline performance. Implications of the findings are discussed.

Enabling persons with multiple disabilities to choose among environmental stimuli and request stimulus repetitions through microswitch and computer technology.

This study assessed microswitch and computer technology to enable two participants with multiple disabilities, 32 and 19 years of age, to choose among environmental stimuli and request their repetition whenever they so desired. Within each session, 18 stimuli (12 preferred and 6 nonpreferred) were available. For each stimulus, a computer system provided a sample of 3 sec. duration. During the intervention, participants' vocal responding in relation to a stimulus sample activated a microswitch and a computer system turned on that stimulus for 15-30 sec. When participants did not vocally respond, the computer system paused briefly and then presented the next scheduled stimulus sample. When participants vocally responded at the end of a stimulus presentation, the stimulus was repeated. Intervention data showed that the participants learned to choose preferred stimuli and bypass nonpreferred ones as well as to request repetitions of the preferred stimuli. This performance was maintained at a 1-mo. postintervention check and transferred to sets of stimuli not used in the intervention. The implications of these results were discussed.

Enabling a man with multiple disabilities and limited motor behavior to perform a functional task with help of microswitch technology.

This study evaluated the extent to which two microswitches used as interfaces would enable a man with multiple disabilities and limited motor behavior to operate an electric door opener at the entrance of the day center that he attended. Analysis showed the man (a) learned to use the two microswitches, (b) preferred the microswitch placed at his wheelchair's footrest (which also allowed more effective responding) over the microswitch placed at his wheelchair's armrest, and (c) maintained his positive performance at the 1- and 2-mo. postintervention checks. The results were discussed from a technical and practical standpoint and in terms of implications for the quality of life of persons with disabilities.

Writing program with word prediction for a young man with multiple disabilities: a preliminary assessment.

This study assessed a writing program with word prediction, which completed the writing of a word and spoke it out only when there was certainty that such a word was the correct one. The assessment was carried out with a young man with multiple disabilities, who typically used a word-processing program without word prediction. The two programs were compared on the time required by the participant for writing general sentences, through a social validation assessment, and eventually a check on the participant's preference. Analysis showed the program with word prediction was significantly more effective in terms of the writing time, which was halved, and was deemed preferable by 40 raters (psychology students) involved in the social validation assessment and by the participant himself. Implications of the findings are discussed.

Multiple microswitches for persons with multiple disabilities: a basic procedure to examine the persons' choice behavior.

This study examined a basic choice-analysis procedure to clarify choice between two responses and related stimuli by five persons with multiple disabilities ages 17.1 to 50.2 yr. The procedure was based on reversing the links between responses and stimuli to assess whether the stimuli accounted for differences in response levels. When this was not the case, the procedure added extra stimuli for the less preferred response to judge whether such an increase would compensate for the apparent disadvantage of that response, e.g., its higher physical cost. Analysis showed that the choice of three of the five participants seemed motivated by the stimuli following the responses. The choice of the other two persons seemed related to a preference for one of the responses which was partially or largely modified when extra stimuli were added.

An overview of research on increasing indices of happiness of people with severe/profound intellectual and multiple disabilities.

PURPOSE: This paper was to provide an overview of research studies aimed at increasing indices of happiness of persons with severe/profound intellectual and multiple disabilities. METHODS: Computerized and manual searches were carried out to identify the studies published from 1990 to 2004 (i.e., the period during which the issues of quality fo life and happiness in people with disabilities have become more prominent). Twenty-four research studies were identified. They involved the use of six different procedures, that is, structured stimulation sessions, microswitch-based simulation sessions, leisure activities and favourite work tasks or conditions, positive environment or positive behaviour support programmes and mindful caregiving, favourite stimulation automatically delivered on exercise engagement, and snoezelen. RESULTS AND CONCLUSION: Data tended to be positive with increases in the participants' indices of happiness, but some failures also occurred. The outcomes were discussed in relation to (a) methodological issues, such as designs of the studies, length of the intervention, and number of participants, and (b) personal and practical implications of the procedures. Some suggestions for future research (particularly focused on extending evidence and overcoming present methodological weakness) were also examined.

Microswitch clusters to enhance adaptive responses and head control: a programme extension for three children with multiple disabilities.

PURPOSE: We assessed the possibility of extending adaptive responding and head control in three children with multiple disabilities through the use of microswitch clusters. METHOD: The children had previously learned to perform an adaptive hand response and to control head position during that response. They were now taught one or two new adaptive responses (foot lifting, leg touching, or vocalization) and to combine such responses with appropriate head position. Microswitch clusters served to ensure that an adaptive response was followed by positive stimulation only if it was combined with appropriate head position. RESULTS: The results were positive with the children learning the new adaptive responses and combining them with appropriate head position. This performance was maintained during two- or three-month post-intervention checks. During these checks, the children were also successful in using the old adaptive hand response with appropriate head position. CONCLUSIONS: The use of microswitch clusters was effective to extend the level of adaptive responding and enhance appropriate head position during this responding. This outcome, which indicates a successful technical replication and procedural extension of previous work in the area, has positive practical implications for educational and occupational programmes for children with multiple disabilities.

Assessing a microswitch-based stimulation procedure for eye-blinking responses in a young woman with profound multiple disabilities.

We applied a new microswitch-based stimulation procedure for eye-blinking responses with a young woman with profound multiple disabilities, and compared effects of this procedure on the eye-blinking responses and smiling with the effects of a caregiver-based stimulation condition. Analysis showed that the microswitch-based stimulation procedure, with stimulation occurring contingent on eye-blinking responses, increased the frequencies of these responses significantly above the levels recorded in baseline and caregiver-based stimulation conditions. No changes in smiling frequencies occurred. Implications of the findings in terms of alertness, learning, and quality of life are discussed.

Automatic prompting to reduce persistent tongue protrusion in a woman with severe to profound mental retardation.

This study assessed whether an intervention approach relying on auditory prompting delivered automatically through a portable device was effective to reduce tongue protrusion in a woman with severe to profound mental retardation. The device involved (a) an optic sensor, i.e., a miniphotocell kept under the lower lip with medical tape, (b) a small signal transmission box, and (c) a Walkman for presenting the prompts. Initially, the automatic prompting condition was combined with occasional praise from a research assistant for having the tongue in the mouth. Analysis showed that the occurrence of tongue protrusion dropped from about 65% of the observation time during the initial baseline to less than 5% through the intervention. The study lasted 4.5 mo.

A microswitch cluster to enhance arm-lifting responses without dystonic head tilting by a child with multiple disabilities.

This study assessed whether a microswitch duster with favorite stimulation could help a 9.1-yr.-old child with multiple disabilities increase arm-lifting responses without dystonic, sideways head tilting. The microswitch cluster involved two microswitches, one at the child's arm to monitor the lifting response, and the other at the child's head to monitor whether it was tilted during arm lifting. Analysis showed that the percentage of arm-lifting responses occurring without head tilting increased most visibly when favorite stimulation focused on those (appropriate) responses.

Further evaluation of microswitch clusters to enhance hand response and head control in persons with multiple disabilities.

This study was a further evaluation of microswitch clusters (combinations of two microswitches) to improve adaptive responding together with correct head position in two persons with multiple disabilities. The two participants were 19.7 and 6.6 yr. old and had profound intellectual disabilities, spastic tetraparesis, and visual impairment. They were initially taught an adaptive hand response that activated a pressure microswitch and produced favorite stimulation. Thereafter, their performance of the hand response produced favorite stimulation only when it was combined with a correct head position (detected through a mercury microswitch). Analysis showed that both participants increased the frequency of the hand response and, subsequently, the percentage of times they emitted this response in combination with correct (upright) head position. In essence, they were able to coordinate constructive occupation with exercise of appropriate posture. Performance was maintained at a 2-mo. postintervention check.