The Lack of Ad Hoc Neuropsychological Assessment in Adults with Neurofibromatosis: A Systematic Review.

Background: Neurofibromatosis Type 1 (NF1) is a genetic autosomal dominant disorder that affects both the central and peripheral nervous systems. Children and adolescents with NF1 commonly experience neuropsychological, motor, and behavioral deficits. The cognitive profile hallmark of this disorder includes visuospatial and executive function impairments. These cognitive disorders may persist into adulthood. This study aims to analyze previous research studies that have described cognitive dysfunctions in adults with NF1. The purpose of this analysis is to review the neuropsychological and psychological assessment methods used. Methods: A total of 327 articles were identified based on the search terms in their titles and abstracts. The evaluation was conducted by scrutinizing each article's title, abstract, and text. Results: Only 16 articles were found to be eligible for inclusion based on the pre-defined criteria. The selected studies primarily focus on the development of diagnostic protocols for individuals with NF1. Conclusions: The management of NF1 disease requires a multidisciplinary approach to address symptoms, preserve neurological functions, and ensure the best possible quality of life. However, cognitive impairment can negatively affect psychological well-being. This study suggested that cognitive functions in NF1 patients were not tested using specific measures, but rather were evaluated through intelligence scales. Additionally, the findings revealed that there is no standardized neuropsychological assessment for adults with NF1. To address this gap, it would be helpful to create a specific neuropsychological battery to study cognitive function in NF1 patients during clinical studies. This battery could also serve as a tool to design models for cognitive rehabilitation by using reliable and sensitive measures of cognitive outcomes.

Rehabilitation of somatoparaphrenia with misoplegia: insights from a single case-pilot study.

Somatoparaphrenia lacka ownership of a paralyzed limb, i.e., the illusion that one's limbs belong to someone else. Somatoparaphrenia is one of the many forms of body misperceptions. We report a case of somatoparaphrenia with misoplegia, characterized by the absence of anosognosia for hemiplegia and personal neglect, following a surgical operation for left parietal meningioma. The patient received a novel multidisciplinary treatment, including motor rehabilitation training, traditional physiotherapy and robotic rehabilitation using the Hunova Movendo Technology and psychological counseling. At the end of the training, the patient improved in global cognitive functioning, mood, motor abilities, and the perception of herself and her body, reducing the sense of estrangement and repulsion in the lower right limb. Our result showed the importance of a specific neuropsychological assessment in patients with parietal brain lesions and the usefulness of an integrated psychological and motor approach in rehabilitating patients with somatoparaphrenia, primarily when associated with misoplegia.

Effects of Robotic Neurorehabilitation on Body Representation in Individuals with Stroke: A Preliminary Study Focusing on an EEG-Based Approach.

Patients with stroke can experience a drastic change in their body representation (BR), beyond the physical and psychological consequences of stroke itself. Noteworthy, the misperception of BR could affect patients' motor performance even more. Our study aimed at evaluating the usefulness of a robot-aided gait training (RAGT) equipped with augmented visuomotor feedback, expected to target BR (RAGT + VR) in improving lower limb sensorimotor function, gait performance (using Fugl-Meyer Assessment scale for lower extremities, FMA-LE), and BR (using the Body Esteem Scale-BES- and the Body Uneasiness Test-BUT), as compared to RAGT - VR. We also assessed the neurophysiologic basis putatively subtending the BR-based motor function recovery, using EEG recording during RAGT. Forty-five patients with stroke were enrolled in this study and randomized with a 1:2 ratio into either the RAGT + VR (n = 30) or the RAGT - VR (n = 15) group. The former group carried out rehabilitation training with the Lokomat©Pro; whereas, the latter used the Lokomat©Nanos. The rehabilitation protocol consisted of 40 one-hour training sessions. At the end of the training, the RAGT + VR improved in FMA-LE (p < 0.001) and BR (as per BES, (p < 0.001), and BUT, (p < 0.001)) more than the RAGT- did (p < 0.001). These differences in clinical outcomes were paralleled by a greater strengthening of visuomotor connectivity and corticomotor excitability (as detected at the EEG analyses) in the RAGT + VR than in the RAGT - VR (all comparisons p < 0.001), corresponding to an improved motor programming and execution in the former group.We may argue that BR recovery was important concerning functional motor improvement by its integration with the motor control system. This likely occurred through the activation of the Mirror Neuron System secondary to the visuomotor feedback provision, resembling virtual reality. Last, our data further confirm the important role of visuomotor feedback in post-stroke rehabilitation, which can achieve better patient-tailored improvement in functional gait by means of RAGT + VR targeting BR.