Digital measures of respiratory and upper limb function in spinal muscular atrophy: design, feasibility, reliability, and preliminary validity of a smartphone sensor-based assessment suite.

Spinal muscular atrophy (SMA) is characterized by progressive muscle weakness and paralysis. Motor function is monitored in the clinical setting using assessments including the 32-item Motor Function Measure (MFM-32), but changes in disease severity between clinical visits may be missed. Digital health technologies may assist evaluation of disease severity by bridging gaps between clinical visits. We developed a smartphone sensor-based assessment suite, comprising nine tasks, to assess motor and muscle function in people with SMA. We used data from the risdiplam phase 2 JEWELFISH trial to assess the test-retest reliability and convergent validity of each task. In the first 6 weeks, 116 eligible participants completed assessments on a median of 6.3 days per week. Eight of the nine tasks demonstrated good or excellent test-retest reliability (intraclass correlation coefficients >0.75 and >0.9, respectively). Seven tasks showed a significant association (P < 0.05) with related clinical measures of motor function (individual items from the MFM-32 or Revised Upper Limb Module scales) and seven showed significant association (P < 0.05) with disease severity measured using the MFM-32 total score. This cross-sectional study supports the feasibility, reliability, and validity of using smartphone-based digital assessments to measure function in people living with SMA.

Practice effects in performance outcome measures in patients living with neurologic disorders - A systematic review.

Background: In this systematic review we sought to characterize practice effects on traditional in-clinic or digital performance outcome measures commonly used in one of four neurologic disease areas (multiple sclerosis; Huntington's disease; Parkinson's disease; and Alzheimer's disease, mild cognitive impairment and other forms of dementia), describe mitigation strategies to minimize their impact on data interpretation and identify gaps to be addressed in future work. Methods: Fifty-eight original articles (49 from Embase and an additional 4 from PubMed and 5 from additional sources; cut-off date January 13, 2021) describing practice effects or their mitigation strategies were included. Results: Practice effects observed in healthy volunteers do not always translate to patients living with neurologic disorders. Mitigation strategies include reliable changes indices that account for practice effects or a run-in period. While the former requires data from a reference sample showing similar practice effects, the latter requires a sufficient number of tests in the run-in period to reach steady-state performance. However, many studies only included 2 or 3 test administrations, which is insufficient to define the number of tests needed in a run-in period. Discussion: Several gaps have been identified. In particular the assessment of practice effects on an individual patient level as well as the temporal dynamics of practice effects are largely unaddressed. Here, digital tests, which allow much higher testing frequency over prolonged periods of time, can be used in future work to gain a deeper understanding of practice effects and to develop new metrics for assessing and accounting for practice effects in clinical research and clinical trials.

Learning to Use Narrative Function Words for the Organization and Communication of Experience.

How do people learn to talk about the causal and temporal relations between events, and the motivation behind why people do what they do? The narrative practice hypothesis of Hutto and Gallagher holds that children are exposed to narratives that provide training for understanding and expressing reasons for why people behave as they do. In this context, we have recently developed a model of narrative processing where a structured model of the developing situation (the situation model) is built up from experienced events, and enriched by sentences in a narrative that describe event meanings. The main interest is to develop a proof of concept for how narrative can be used to structure, organize and describe experience. Narrative sentences describe events, and they also define temporal and causal relations between events. These relations are specified by a class of narrative function words, including "because, before, after, first, finally." The current research develops a proof of concept that by observing how people describe social events, a developmental robotic system can begin to acquire early knowledge of how to explain the reasons for events. We collect data from naïve subjects who use narrative function words to describe simple scenes of human-robot interaction, and then employ algorithms for extracting the statistical structure of how narrative function words link events in the situation model. By using these statistical regularities, the robot can thus learn from human experience about how to properly employ in question-answering dialogues with the human, and in generating canonical narratives for new experiences. The behavior of the system is demonstrated over several behavioral interactions, and associated narrative interaction sessions, while a more formal extended evaluation and user study will be the subject of future research. Clearly this is far removed from the power of the full blown narrative practice capability, but it provides a first step in the development of an experimental infrastructure for the study of socially situated narrative practice in human-robot interaction.

Narrative Constructions for the Organization of Self Experience: Proof of Concept via Embodied Robotics.

It has been proposed that starting from meaning that the child derives directly from shared experience with others, adult narrative enriches this meaning and its structure, providing causal links between unseen intentional states and actions. This would require a means for representing meaning from experience-a situation model-and a mechanism that allows information to be extracted from sentences and mapped onto the situation model that has been derived from experience, thus enriching that representation. We present a hypothesis and theory concerning how the language processing infrastructure for grammatical constructions can naturally be extended to narrative constructions to provide a mechanism for using language to enrich meaning derived from physical experience. Toward this aim, the grammatical construction models are augmented with additional structures for representing relations between events across sentences. Simulation results demonstrate proof of concept for how the narrative construction model supports multiple successive levels of meaning creation which allows the system to learn about the intentionality of mental states, and argument substitution which allows extensions to metaphorical language and analogical problem solving. Cross-linguistic validity of the system is demonstrated in Japanese. The narrative construction model is then integrated into the cognitive system of a humanoid robot that provides the memory systems and world-interaction required for representing meaning in a situation model. In this context proof of concept is demonstrated for how the system enriches meaning in the situation model that has been directly derived from experience. In terms of links to empirical data, the model predicts strong usage based effects: that is, that the narrative constructions used by children will be highly correlated with those that they experience. It also relies on the notion of narrative or discourse function words. Both of these are validated in the experimental literature.

The Role of Autobiographical Memory in the Development of a Robot Self.

This article briefly reviews research in cognitive development concerning the nature of the human self. It then reviews research in developmental robotics that has attempted to retrace parts of the developmental trajectory of the self. This should be of interest to developmental psychologists, and researchers in developmental robotics. As a point of departure, one of the most characteristic aspects of human social interaction is cooperation-the process of entering into a joint enterprise to achieve a common goal. Fundamental to this ability to cooperate is the underlying ability to enter into, and engage in, a self-other relation. This suggests that if we intend for robots to cooperate with humans, then to some extent robots must engage in these self-other relations, and hence they must have some aspect of a self. Decades of research in human cognitive development indicate that the self is not fully present from the outset, but rather that it is developed in a usage-based fashion, that is, through engaging with the world, including the physical world and the social world of animate intentional agents. In an effort to characterize the self, Ulric Neisser noted that self is not unitary, and he thus proposed five types of self-knowledge that correspond to five distinct components of self: ecological, interpersonal, conceptual, temporally extended, and private. He emphasized the ecological nature of each of these levels, how they are developed through the engagement of the developing child with the physical and interpersonal worlds. Crucially, development of the self has been shown to rely on the child's autobiographical memory. From the developmental robotics perspective, this suggests that in principal it would be possible to develop certain aspects of self in a robot cognitive system where the robot is engaged in the physical and social world, equipped with an autobiographical memory system. We review a series of developmental robotics studies that make progress in this enterprise. We conclude with a summary of the properties that are required for the development of these different levels of self, and we identify topics for future research.

Corticostriatal response selection in sentence production: Insights from neural network simulation with reservoir computing.

Language production requires selection of the appropriate sentence structure to accommodate the communication goal of the speaker - the transmission of a particular meaning. Here we consider event meanings, in terms of predicates and thematic roles, and we address the problem that a given event can be described from multiple perspectives, which poses a problem of response selection. We present a model of response selection in sentence production that is inspired by the primate corticostriatal system. The model is implemented in the context of reservoir computing where the reservoir - a recurrent neural network with fixed connections - corresponds to cortex, and the readout corresponds to the striatum. We demonstrate robust learning, and generalization properties of the model, and demonstrate its cross linguistic capabilities in English and Japanese. The results contribute to the argument that the corticostriatal system plays a role in response selection in language production, and to the stance that reservoir computing is a valid potential model of corticostriatal processing.

Exploring the acquisition and production of grammatical constructions through human-robot interaction with echo state networks.

One of the principal functions of human language is to allow people to coordinate joint action. This includes the description of events, requests for action, and their organization in time. A crucial component of language acquisition is learning the grammatical structures that allow the expression of such complex meaning related to physical events. The current research investigates the learning of grammatical constructions and their temporal organization in the context of human-robot physical interaction with the embodied sensorimotor humanoid platform, the iCub. We demonstrate three noteworthy phenomena. First, a recurrent network model is used in conjunction with this robotic platform to learn the mappings between grammatical forms and predicate-argument representations of meanings related to events, and the robot's execution of these events in time. Second, this learning mechanism functions in the inverse sense, i.e., in a language production mode, where rather than executing commanded actions, the robot will describe the results of human generated actions. Finally, we collect data from naïve subjects who interact with the robot via spoken language, and demonstrate significant learning and generalization results. This allows us to conclude that such a neural language learning system not only helps to characterize and understand some aspects of human language acquisition, but also that it can be useful in adaptive human-robot interaction.