Supporting daily activities and indoor travel of persons with moderate Alzheimer's disease through standard technology resources.

These two studies were aimed at evaluating standard technology resources for supporting activity and travel among patients with moderate Alzheimer's disease. Specifically, Study I assessed a pictorial instruction program relying on the use of a portable computer and a commercially available and inexpensive video editing software for supporting the performance of daily activities with three patients. Study II assessed the indoor travel performance of four patients (i.e., the three involved in Study I and a fourth patient with no previous research exposure) using a commercially available, basic doorbell system with sound and light cues. The percentages of correct activity steps obtained with the instruction program used in Study I were relatively high and largely similar to the percentages reported in previous studies using more sophisticated technology. During Study II, the percentages of correct travels of two patients matched the data of the most successful patients involved in previous studies with more sophisticated technology. The percentages of the other two patients tended to be lower than those obtained previously, but were still practically relevant. The implications of the results of the two studies and a number of issues for new research are discussed.

Epileptic seizures, movement disorders, and breathing disturbances in Rett syndrome: diagnostic relevance of video-polygraphy.

Epileptic seizures, movement disorders and breathing disturbances may be observed in Rett syndrome, and correct diagnosis is mandatory for the management. We evaluated the usefulness of video-polygraphy in the differential diagnosis between epileptic and non-epileptic paroxysmal events in eight patients with Rett syndrome. Based on video analysis, myoclonic seizures were usually misdiagnosed as movement disorders and stereotypies; the events identified by parents as generalized tonic-clonic seizures included episodes of motor activity and breathing abnormality. Myoclonic seizures aggravated by inappropriate treatment were evident in four patients; hyperventilation and apnea during wakefulness were present in all patients, while central sleep apneas were present in one patient; sinus tachycardia and cardiac arrhythmias emerged in six patients; cortical myoclonus was disclosed in five patients. In Rett syndrome, video-polygraphy is essential in characterizing the clinical features of paroxysmal events, determining autonomic dysfunctions, documenting myoclonic motor phenomena, and evaluating the responses to the treatment of epilepsy.

Persons with Alzheimer's disease make phone calls independently using a computer-aided telephone system.

This study assessed whether four patients with a diagnosis of Alzheimer's disease could make independent phone calls via a computer-aided telephone system. The study was carried out according to a non-concurrent multiple baseline design across participants. All participants started with baseline during which the telephone system was not available, and continued with intervention in which such system was used. It involved a net-book computer provided with specific software, a global system for mobile communication modem (GSM), a microswitch, and photos of the persons available for the phone calls. All participants learned to use the system and made phone calls independently to a variety of partners such as family members, friends and staff personnel. The positive implications of the system were discussed in relation to previous data in this area and the possibility of helping persons with Alzheimer's disease restore an important, instrumental daily ability and engage in communication with distant partners.

Technology-aided pictorial cues to support the performance of daily activities by persons with moderate Alzheimer's disease.

We developed a technology-aided intervention strategy relying on pictorial cues alone or in combination with verbal instructions and assessed these two versions of the strategy with three persons with moderate Alzheimer's disease. In Section I of the study, the strategy version with pictorial cues plus verbal instructions was compared with an existing technology-based strategy with verbal instructions. Each strategy was used with one specific activity. In Section II of the study, the strategy version with pictorial cues alone was compared with the aforementioned strategy with verbal instructions. Again, each strategy was used with one activity. Both strategy versions were effective with all three participants. The percentages of correct activity performance observed with those versions increased to above 90, and were comparable with those obtained with the existing verbal instructions strategy. A social validation assessment of the version with pictorial cues alone and the existing strategy with verbal instructions (employing university psychology students as raters) showed differences in favor of the latter strategy in terms of practicality and in favor of the former in terms of respect of participants' dignity. The implications of the findings were discussed.

Persons with mild or moderate Alzheimer's disease use a basic orientation technology to travel to different rooms within a day center.

This study assessed whether three patients with Alzheimer's disease could learn to use a basic orientation technology to reach different rooms within a day center. At each travel instance, the technology provided verbal messages (cues) from the room to reach. For the first two patients, the messages were presented at intervals of about 15s. For the third patient (who had more extensive orientation problems), shorter intervals combined with the voice of the research assistant were initially used. Results showed that all three patients were successful in using the technology to orient their travel and find the rooms correctly. A social validation assessment, in which university psychology students were asked to rate the patients' travel performance with the technology and with the help of a caregiver, provided generally higher (more positive) scores for the technology-assisted performance. The implications of the findings for daily programs of patients with Alzheimer's disease are discussed.

Technology-aided verbal instructions to help persons with mild or moderate Alzheimer's disease perform daily activities.

These two studies extended previous research on the use of verbal instructions and support technology for helping persons with mild or moderate Alzheimer's disease perform daily activities. Study I included seven participants who were to carry out one of two previously targeted activities (i.e., either coffee preparation or table setting). Study II included four participants who were to carry out two new activities (i.e., preparation of a fruit salad and of a vegetable salad). The effects of activity engagement on mood (i.e., indices of happiness) were assessed by recording the participants' behavior during the activity trials and parallel non-activity periods. The participants of Study I reached percentages of correct activity performance, which normally exceeded 85. Five of them also showed higher indices of happiness during the activity trials as opposed to the non-activity periods. Three of the participants of Study II reached high percentages of correct performance on both activities available. One of these participants also showed higher indices of happiness during the activity trials. The findings were discussed in relation to previous research outcomes and in terms of their practical implications for intervention programs.

Persons with Alzheimer's disease perform daily activities using verbal-instruction technology: a maintenance assessment.

OBJECTIVE: To assess whether verbal-instruction technology could help persons with mild and moderate Alzheimer's disease maintain their recaptured performance of daily activities. METHODS: This study followed nine patients who had participated in previous studies aimed at helping them recapture one or more functional daily activities (i.e. table setting, coffee, tea or snack preparation, use of make-up and shaving). The plan was to follow each patient for at least 6 months after the intervention, unless his/her condition called for an earlier end of the study. RESULTS: The study was interrupted after 5 months for two patients who developed serious behavioural problems and continued for 6-14 months for the other seven patients who had largely accurate performance with some adaptations of instructions/steps. Most patients also showed mood improvement during activity. CONCLUSION: Verbal-instruction technology might be considered a critical tool to help persons with Alzheimer's disease enhance their activity and mood.

Persons with mild and moderate Alzheimer's disease use verbal-instruction technology to manage daily activities: effects on performance and mood.

OBJECTIVE: To extend the evaluation of verbal-instruction technology for helping persons with mild and moderate Alzheimer's disease recapture daily activities and improve their mood. METHODS: Two studies targeted two activities (i.e. snack preparation/sharing and shaving) with six and three new participants, respectively. Intervention effects on activity performance were assessed through non-concurrent multiple baseline designs across participants. The impact of intervention (activity) on mood was assessed by recording indices of happiness or indices of unhappiness during activity and non-activity trials. RESULTS: The use of a technology providing verbal instructions helped all participants perform the target activities. Performance was largely accurate with seven of the participants. Eight of the participants also showed mood improvement (i.e. increases in indices of happiness or decreases in indices of happiness) during activity. CONCLUSION: These results suggest that the approach reported may be a useful strategy for helping persons with Alzheimer's disease enhance their activity and mood.

Central sleep apnoea in Rett syndrome.

Breathing disturbances in Rett syndrome were reported almost entirely during wakefulness, with normal respiration during sleep. We studied a case of a proven MECP2 mutation in a girl, whose videopolygraphic and polysomnographic monitoring suggested the evidence of central apnoeas not only during awake, but also during sleep. Apart from prevalent awake respiratory dysfunction, central apnoeas in Rett syndrome may be also present during sleep.

Myoclonic status misdiagnosed as movement disorders in Rett syndrome: a video-polygraphic study.

Myoclonic jerks and myoclonic status (MS) are sometimes difficult to distinguish clinically from movement disorders such as hand stereotypies, tremor, and dystonia in Rett syndrome. We describe a rare and complete video-polygraphic study of a girl with Rett syndrome (MECP2 mutation) and MS misdiagnosed as movement disorders and disclosed after video-polygraphic recordings. Corresponding to closely recurring activity of diffuse spike and polyspikes-wave-type paroxysms, rhythmic and, especially, arrhythmic myoclonias, usually asymmetrical and asynchronous, involving mainly right muscle deltoid and rarely followed by an inhibitory phenomenon, appeared. The MS improved and, most importantly, disappeared after the use of levetiracetam, with an evident antimyoclonic efficacy and a marked improvement of daily life for the patient and her caregivers. The difficulty in differentiating some typical nonepileptic behavioral features and movement disorders of patients with Rett syndrome from seizures was overcome using prolonged video-polygraphic recordings in our case.

Persons with moderate Alzheimer's disease improve activities and mood via instruction technology.

BACKGROUND: Three studies assessed the (a) effectiveness of verbal instructions presented via technology in helping persons with moderate Alzheimer's disease perform daily activities and (b) impact of activity engagement on mood. METHODS: The 3 studies targeted coffee preparation with 2 women, use of make-up with 2 women, and use of make-up and tea preparation with 3 women. Intervention effects on activity performance were assessed through nonconcurrent multiple baseline designs across participants or multiple baseline designs across activities. The impact of activity on mood was assessed by recording indices of happiness during activity trials and parallel nonactivity periods. RESULTS: Verbal instructions presented via technology were effective in helping all participants perform the target activities. The participants also showed mood improvement (ie, increases in indices of happiness) during the activity. CONCLUSION: These results suggest that the approach reported may be a useful strategy for helping persons with Alzheimer's disease.

Helping persons with mild or moderate Alzheimer's disease recapture basic daily activities through the use of an instruction strategy.

PURPOSE: The present three pilot studies assessed the effectiveness of verbal instructions, presented automatically through simple technology, in helping persons with mild-to-moderate Alzheimer's disease recapture basic daily activities. The activities were morning bathroom routine, dressing, and table-setting. METHOD: The studies that focused on morning bathroom routine and on table-setting included three participants each, while the study that focused on dressing involved four participants. A non-concurrent multiple baseline design across participants was used for each study. The instructions and technology were available only during the intervention phases. RESULTS: Data showed that the intervention strategy involving verbal instructions for the single activity steps presented automatically through technology was effective in helping all participants on each of the activities. The participants' mean percentages of correct steps across activities raised from 13 - 54 during the baseline periods to above 80 or 90 during the intervention periods. CONCLUSIONS: The results suggest that the intervention strategy reported may represent a suitable approach for helping persons with mild or moderate Alzheimer's disease to recapture basic daily activities. New research should target other activities and check maintenance and generalization issues.

Persons with mild or moderate Alzheimer's disease managing daily activities via verbal instruction technology.

Four studies assessed the effectiveness of verbal instructions presented via technology in helping persons with mild or moderate Alzheimer's disease perform daily activities. The first 2 studies were replication efforts concerning morning bathroom routine and table setting and included 4 and 2 participants, respectively. The third study targeted coffee preparation with 3 participants. The fourth study assessed maintenance and generalization of morning bathroom routine and dressing with 1 participant. Nonconcurrent multiple baseline designs served for the first 3 studies and a 5-month postintervention data collection for the fourth study. Verbal instructions for the activity steps presented via technology were effective in helping the participants of the first 3 studies reacquire basic daily activities and the participant of the fourth study retain the reacquired activities across time and settings. These results suggest that the approach reported may be a useful strategy for helping persons with Alzheimer's disease.

Adult epileptic spasms: a clinical and video-polygraphic study.

PURPOSE: To analyse the video-polygraphic features of two patients with adult epileptic spasms (ES). METHODS: Two patients with epileptic encephalopathy related to different cortical malformation; epileptic spasms had been present since the age of 18 and 25 years respectively. The patients underwent a clinical and neurophysiological examination (awake and sleep video-polygraphic monitoring). RESULTS: In patient 1, epileptic spasms were characterized by loss of contact and a drooping forward of the head and upper extremities. ES were in clusters, of brief duration, lasting for 20'''-1''; all events occurred during eating. Awake and sleep interictal EEG disclosed asynchronous paroxysmal abnormalities in the temporo-parieto-occipital regions. The ictal EEG showed a diffuse slow wave complex with intermingled fast activity, prevalent on both anterior regions; on polygraphic recordings, the prevalence of the muscle activation, in crescendo-decrescendo, was in the neck and the right side. In patient 2, ES were characterized by loss of awareness, head deviation to the left, jerks of the head and left limbs. ES occurred in cluster, and were of brief duration (< 1"), usually on awakening. Interictal EEG showed epileptiform abnormalities in the right fronto-centro-temporal regions; abnormalities present in the awake EEG were enhanced during NREM sleep. The ictal EEG showed a diffuse, slow wave complex with intermingled fast activity, prevalent in the right regions, with concomitant muscle activation, in crescendo-decrescendo, on the left deltoid and tibialis anterior. CONCLUSIONS: Our two cases of adult ES were characterized by distinct clinical and polygraphic features, which were of brief duration, mild intensity, rare frequency per minute, and with preservation of cluster organization. In one patient, ES were induced by eating (reflex periodic spasms triggered by eating). A firm diagnosis of adult ES can be reached using video-polygraphic monitoring.

Immunomodulatory properties of increased levels of liver X receptor beta in peripheral blood mononuclear cells from multiple sclerosis patients.

Liver X receptors (LXRalpha and LXRbeta) are nuclear transcription factors that inhibit transcription of genes of inflammation while inducing HMGCoA reductase. In this paper we demonstrate increased mRNA levels of LXRbeta in peripheral blood mononuclear cells (PBMCs) from multiple sclerosis patients with respect to other neurological patients and healthy controls (HC) (p<0.01). Agonist-induced activation of LXRs partially counteracts the anti-CD3+ anti-CD28-induced proliferation of T cells (p<0.01) and secretion of IFNgamma (p<0.001) from PBMCs of MS patients as well as of HC. Secretion of IL-4 is not affected. Our findings suggest that regulation of cholesterol metabolism not strictly related to inhibition of HMGCoA reductase can modulate activity of lymphocytes in MS.