Immersive Virtual Environments and Wearable Haptic Devices in rehabilitation of children with neuromotor impairments: a single-blind randomized controlled crossover pilot study.

BACKGROUND: The past decade has seen the emergence of rehabilitation treatments using virtual reality. One of the advantages in using this technology is the potential to create positive motivation, by means of engaging environments and tasks shaped in the form of serious games. The aim of this study is to determine the efficacy of immersive Virtual Environments and weaRable hAptic devices (VERA) for rehabilitation of upper limb in children with Cerebral Palsy (CP) and Developmental Dyspraxia (DD). METHODS: A two period cross-over design was adopted for determining the differences between the proposed therapy and a conventional treatment. Eight children were randomized into two groups: one group received the VERA treatment in the first period and the manual therapy in the second period, and viceversa for the other group. Children were assessed at the beginning and the end of each period through both the Nine Hole Peg Test (9-HPT, primary outcome) and Kinesiological Measurements obtained during the performing of similar tasks in a real setting scenario (secondary outcomes). RESULTS: All subjects, not depending from which group they come from, significantly improved in both the performance of the 9-HPT and in the parameters of the kinesiological measurements (movement error and smoothness). No statistically significant differences have been found between the two groups. CONCLUSIONS: These findings suggest that immersive VE and wearable haptic devices is a viable alternative to conventional therapy for improving upper extremity function in children with neuromotor impairments. Trial registration ClinicalTrials, NCT03353623. Registered 27 November 2017-Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT03353623.

Gait in normal pressure hydrocephalus: characteristics and effects of the CSF tap test.

INTRODUCTION: Normal pressure hydrocephalus (NPH), described by Hakim and Adams in 1965, is characterized by gait apraxia, urinary incontinence, and dementia. It is associated with normal cerebrospinal fluid (CSF) pressure and ventricular dilation that cannot be attributed to cerebral atrophy. OBJECTIVES: To evaluate gait characteristics in patients with idiopathic NPH and investigate the effect of the CSF tap test (CSF-TT) on gait. METHODS: Twenty-five patients diagnosed with probable idiopathic NPH were submitted to the CSF-TT. The procedure aimed to achieve changes in gait parameters. RESULTS: Fifteen gait parameters were assessed before and after the CSF-TT. Five showed a statistically significant improvement (p < 0.05): walking speed (p < 0.001), cadence (p < 0.001), step length (p < 0.001), en bloc turning (p = 0.001), and step height (p = 0.004). CONCLUSION: This study demonstrated that gait speed was the most responsive parameter to the CSF-TT, followed by cadence, step length, en bloc turning, and step height.

Gait in normal pressure hydrocephalus: characteristics and effects of the CSF tap test / Marcha na hidrocefalia de pressão normal: características e efeitos do tap-test

ABSTRACT Normal pressure hydrocephalus (NPH), described by Hakim and Adams in 1965, is characterized by gait apraxia, urinary incontinence, and dementia. It is associated with normal cerebrospinal fluid (CSF) pressure and ventricular dilation that cannot be attributed to cerebral atrophy. Objectives: To evaluate gait characteristics in patients with idiopathic NPH and investigate the effect of the CSF tap test (CSF-TT) on gait. Methods: Twenty-five patients diagnosed with probable idiopathic NPH were submitted to the CSF-TT. The procedure aimed to achieve changes in gait parameters. Results: Fifteen gait parameters were assessed before and after the CSF-TT. Five showed a statistically significant improvement (p < 0.05): walking speed (p < 0.001), cadence (p < 0.001), step length (p < 0.001), en bloc turning (p = 0.001), and step height (p = 0.004). Conclusion: This study demonstrated that gait speed was the most responsive parameter to the CSF-TT, followed by cadence, step length, en bloc turning, and step height.

RESUMO A hidrocefalia de pressão normal (HPN), descrita por Hakim-Adams em 1965, caracteriza-se por apraxia de marcha, incontinência urinária e demência e está associada com pressão normal do líquido cefalorraquidiano e dilatação ventricular não atribuída a atrofia cerebral. Objetivos: Avaliar as características da marcha em pacientes com HPN idiopática e o efeito do "tap-test" (TT) na marcha. Métodos: Vinte e cinco pacientes com o diagnóstico HPN idiopática provável, foram avaliados com o TT. O procedimento tem como objetivo causar mudanças nas características da marcha. Resultados: Quinze parâmetros da marcha foram avaliados com o TT. Cinco mostraram melhora estatisticamente significativa (p < 0,05): velocidade da marcha (p < 0,001), cadência (p < 0,001), comprimento do passo (p < 0,001), giro em "bloco" (p = 0,001) e altura do passo (p = 0,004). Conclusão: Este estudo demonstrou que a velocidade da marcha foi o parâmetro que mais respondeu ao efeito do TT, seguido da cadência, comprimento do passo, giro em "bloco" e altura do passo.

Apraxia of gait- or apraxia of postural transitions?

"Apraxia of gait" is not a useful concept and freezing of gait should also not be considered an apraxia. The concept of apraxia may, however, be applied to distortions of postural transitions that can accompany fronto-parietal lesions.

Balance impairment does not necessarily coexist with gait apraxia in mild and moderate Alzheimer's disease.

OBJECTIVES: To assess correlations among gait apraxia, balance impairment and cognitive performance in mild (AD1, n = 30) and moderate (AD2, n = 30) AD. METHOD: The following evaluations were undertaken: gait apraxia (Assessment Walking Skills); balance performance (Berg Balance Scale); Clinical Dementia Rating and Mini-mental State Examination (MMSE). RESULTS: While disregarding AD subgroups, Berg Balance Scale and the MMSE correlated significantly with Assessment Walking Skills and 23% of all subjects scored below its cut-off. After stratification, Berg Balance Scale correlated significantly with Assessment Walking Skills in both AD subgroups, and with the MMSE only in AD1. CONCLUSIONS: Balance impairment does not necessarily coexist with gait apraxia. Gait apraxia is more prevalent in moderate AD when compared with mild AD.

Balance impairment does not necessarily coexist with gait apraxia in mild and moderate Alzheimer's disease / Comprometimento do equilíbrio e apraxia da marcha não necessariamente coexistem na doença de Alzheimer leve e moderada

ABSTRACT Currently, there are no studies reporting how much balance impairment coexists with gait apraxia in mild and moderate Alzheimer’s disease (AD). Objectives To assess correlations among gait apraxia, balance impairment and cognitive performance in mild (AD1, n = 30) and moderate (AD2, n = 30) AD. Method The following evaluations were undertaken: gait apraxia (Assessment Walking Skills); balance performance (Berg Balance Scale); Clinical Dementia Rating and Mini-mental State Examination (MMSE). Results While disregarding AD subgroups, Berg Balance Scale and the MMSE correlated significantly with Assessment Walking Skills and 23% of all subjects scored below its cut-off. After stratification, Berg Balance Scale correlated significantly with Assessment Walking Skills in both AD subgroups, and with the MMSE only in AD1. Conclusions Balance impairment does not necessarily coexist with gait apraxia. Gait apraxia is more prevalent in moderate AD when compared with mild AD.

RESUMO Apraxia da marcha e desequilíbrio são condições subinvestigadas na doença de Alzheimer (DA) leve e moderada. Objetivo Verificar a correlação da apraxia da marcha com desequilíbrio e cognição em 30 idosos com DA leve (DA1) e 30 idosos com DA moderada (DA2). Método Foram feitas as seguintes avaliações: apraxia da marcha (Assessment Walking Skills); equilíbrio (Berg Balance Scale); Clinical Dementia Rating e Mini-exame do estado mental – MEEM. Resultados Desconsiderando-se os grupos, Berg Balance Scale e MEEM correlacionaram-se significativamente com a Assessment Walking Skills, enquanto 23% dos participantes pontuaram abaixo da note de corte da mesma. Considerando-se os grupos, Berg Balance Scale correlacionou-se significativamente com a Assessment Walking Skills em ambos os grupos, embora o MEEM o tenha feito apenas em DA1. Conclusões Desequilíbrio e apraxia da marcha não necessariamente coexistem com apraxia da marcha. Prevalência de apraxia da marcha foi maior na DA moderada do que na DA leve.

Feasibility of external rhythmic cueing with the Google Glass for improving gait in people with Parkinson's disease.

New mobile technologies like smartglasses can deliver external cues that may improve gait in people with Parkinson's disease in their natural environment. However, the potential of these devices must first be assessed in controlled experiments. Therefore, we evaluated rhythmic visual and auditory cueing in a laboratory setting with a custom-made application for the Google Glass. Twelve participants (mean age = 66.8; mean disease duration = 13.6 years) were tested at end of dose. We compared several key gait parameters (walking speed, cadence, stride length, and stride length variability) and freezing of gait for three types of external cues (metronome, flashing light, and optic flow) and a control condition (no-cue). For all cueing conditions, the subjects completed several walking tasks of varying complexity. Seven inertial sensors attached to the feet, legs and pelvis captured motion data for gait analysis. Two experienced raters scored the presence and severity of freezing of gait using video recordings. User experience was evaluated through a semi-open interview. During cueing, a more stable gait pattern emerged, particularly on complicated walking courses; however, freezing of gait did not significantly decrease. The metronome was more effective than rhythmic visual cues and most preferred by the participants. Participants were overall positive about the usability of the Google Glass and willing to use it at home. Thus, smartglasses like the Google Glass could be used to provide personalized mobile cueing to support gait; however, in its current form, auditory cues seemed more effective than rhythmic visual cues.

Gait dyspraxia as a clinical marker of cognitive decline in Down syndrome: A review of theory and proposed mechanisms.

Down syndrome (DS) is the most common genetic cause of intellectual disability in children. With aging, DS is associated with an increased risk for Alzheimer's disease (AD). The development of AD neuropathology in individuals with DS can result in further disturbances in cognition and behavior and may significantly exacerbate caregiver burden. Early detection may allow for appropriate preparation by caregivers. Recent literature suggests that declines in gait may serve as an early marker of AD-related cognitive disorders; however, this relationship has not been examined in individuals with DS. The theory regarding gait dyspraxia and cognitive decline in the general population is reviewed, and potential applications to the population with individuals with DS are highlighted. Challenges and benefits in the line of inquiry are discussed. In particular, it appears that gait declines in aging individuals with DS may be associated with known declines in frontoparietal gray matter, development of AD-related pathology, and white matter losses in tracts critical to motor control. These changes are also potentially related to the cognitive and functional changes often observed during the same chronological period as gait declines in adults with DS. Gait declines may be an early marker of cognitive change, related to the development of underlying AD-related pathology, in individuals with DS. Future investigations in this area may provide insight into the clinical changes associated with development of AD pathology in both the population with DS and the general population, enhancing efforts for optimal patient and caregiver support and propelling investigations regarding safety/quality of life interventions and disease-modifying interventions.

Clinical and MRI characterization of MS patients with a pure and severe cognitive onset.

BACKGROUND AND OBJECTIVE: Cognitive and behavioural symptoms are common in multiple sclerosis (MS), but they are rarely the inaugural and predominant manifestation of the disease. Our objective is to characterize the clinical and radiological features of cognitive-multiple sclerosis (cog-MS), defined as MS subjects who entered into the disease with cognitive symptoms, which subsequently remain the predominant manifestation. METHODS: We describe the disease course, and clinical and radiological features of 18 subjects with a cognitive form of MS. RESULTS: Memory loss and behavioural changes were the primary symptoms at disease onset. They remained prominent and led to severe cognitive impairment during disease course. The main associated manifestations were depression, pathological laughing and/or crying, urinary incontinence and gait disturbance suggestive of high-level gait disorder. Motor, sensory or cerebellar abnormalities were uncommon. During disease course, superimposed neurological relapses occurred in 61% of cases. Brain MRI revealed multiple periventricular lesions that were extensive and confluent in half of cases, and a severe atrophy measured as an increase in the third ventricular width compared to age-matched healthy controls. Gadolinium-enhancing lesions were common (72%). The mean diagnosis delay from disease onset was 2 years. A principal component analysis on the neuropsychological results revealed that verbal memory assessment is complementary to global cognitive functioning evaluation in these patients with severe cognitive deficit. Verbal memory deficit was associated with high EDSS. CONCLUSIONS: cog-MS patients might represent a challenging diagnosis, which needs to be individualized for an early management.

Non-invasive serial casting to treat idiopathic toe walking in an 18-month old child.

BACKGROUND: Idiopathic toe walking is characterized by persistent toe walking in the absence of clinically diagnosed neuromuscular disease. Treatment options in children diagnosed with idiopathic toe walking include: observation, physical therapy, serial casting, or Achilles tendon (heel cord) lengthening surgery. OBJECTIVE: In this case report, we present a non-invasive serial casting protocol to treat severe and persistent toe walking in an 18-month old child, diagnosed as an idiopathic toe walker following neurological examination. METHODS: A series of below knee casts was used to provide a consistent stretch to the plantar flexor muscles. Upon removal of each set of casts, passive range of motion at the ankles was measured with a goniometer. RESULTS: Four sets of casts, each lasting approximately one week, increased passive ankle dorsiflexion to 10° of neutral and established a heel-toe walking gait. Improvements in ankle range of motion and gait were maintained upon repeated examinations at 3, 7, and 12 months post-casting. CONCLUSIONS: These results demonstrate that non-invasive procedures, such as serial casting, can be successful in very young children diagnosed as idiopathic toe walkers. Early identification and intervention for this diagnosis may eliminate the need for invasive surgeries and associated risks in this population.

High beta activity in the subthalamic nucleus and freezing of gait in Parkinson's disease.

OBJECTIVE: Oscillatory activity in the beta band is increased in the subthalamic nucleus (STN) of Parkinson's disease (PD) patients. Rigidity and bradykinesia are associated with the low-beta component (13-20Hz) but the neurophysiological correlate of freezing of gait in PD has not been ascertained. METHODS: We evaluated the power and coherence of the low- and high-beta bands in the STN and cortex (EEG) of PD patients with (p-FOG) (n=14) or without freezing of gait (n-FOG) (n=8) in whom electrodes for chronic stimulation in the STN had been implanted for treatment with deep brain stimulation. RESULTS: p-FOG patients showed higher power in the high-beta band (F=11.6, p=0.002) that was significantly reduced after l-dopa administration along with suppression of FOG (F=4.6, p=0.042). High-beta cortico-STN coherence was maximal for midline cortical EEG electrodes, whereas the low-beta band was maximal for lateral electrodes (χ(2)=20.60, p<0.0001). CONCLUSIONS: The association between freezing of gait, high-beta STN oscillations and cortico-STN coherence suggests that this oscillatory activity might interfere in the frontal cortex-basal ganglia networks, thereby participating in the pathophysiology of FOG in PD.

Neurophysiologic monitoring of the spinal accessory nerve, hypoglossal nerve, and the spinomedullary region.

This review of hypoglossal nerve, spinal accessory nerve, and spinomedullary region intraoperative monitoring details pertinent central and extramedullary anatomy, an updated understanding of proper free-run EMG recording methods and recent developments in stimulation technique and instrumentation. Mapping and monitoring the floor of the fourth ventricle, especially the vagal/hypoglossal trigone region, are emphasized. Although cranial nerve transcranial electrical motor evoked potential recordings can afford appreciation of corticobulbar/corticospinal tract function and secure a more dependable measure of proximate extramedullary somatoefferents, the sometimes difficult implementation and the, as yet, unresolved alert criteria of these recordings demand critical appraisal. Nearby and intimately associated cardiochronotropic and barocontrol neural networks are described; their better understanding is recommended as an important adjunct to "routine" neural monitoring. Finally, an Illustrative case is presented to highlight the many strengths and weaknesses of "state of the art" lower cranial nerve/spinomedullary region monitoring.

[Vascular or "lower body Parkinsonism": rise and fall of a diagnosis]. / Vascularis vagy "lower body Parkinsonizmus". Egy diagnózis tündöklése és Bukása.

UNLABELLED: The "arteriosclerotic parkinsonism", which is called vascular parkinsonism (VP), was first described by Critchley'. The broad based slow gait, reduced stride lenght, start hesitation, freezing and paratonia was mentioned as "lower body parkinsonism" (LBP) which can be associated by slow speech, dysexecutive syndrome, and hand tremor of predominantly postural character. In VP the DAT-scan proved normal dopamine content of the striatum in contrast with Parkinson's disease (PD). Additionally, Lewy bodies of brainstem type were not found in VR Probability of VP increases if central type pathologic gait is prominent; the hands are slightly involved, the MRI indicates transparent periventricular white substance and/or brain atrophy. In some cases differentiation of gait apraxia and parkinsonism could be challenging. There is no rigor of the lower limbs at rest in neither of them, the disturbance of movement is evoked by the gait itself. Three subtypes of "gait ignition failure" has been recently described: (1) ignition apraxia, (2) equilibrium apraxia and (3) mixed gait apraxia. The primary progressive freesing gait was considered as a Parkinson-plus syndrome. Freesing occurs more frequently in diseases with pakinsonism than in PD. The grade of ventricle dilatation and the frontal leukoaraiosis was similar in LBP and gait apraxia. In cases of normal pressure hydrocephalus the impaired gait may mimic PD. Pathologic gait in VP can be explained by the lesions of the senso-motor association pathways in dorsal paramedian white substance within the vulnerable borderzone region. These may be colocalized with the representation of the lower extremities in the posterior third of the supplementer motor area. Rektor2 proposed to change the name of LBP to "cerebrovascular gait disorder". Notwithstandig central type gait disorder develops also in many degenerative diseases other than cerebro-vascular origin. The neuronal net controling the regulation of movement is widespread, therefore several cortical and subcortical lesions could elicit large variations of pathologic gait, ie.: ataxia, apraxia, ignition failure, akinesis etc. IN CONCLUSION: most of the central gait disorders regarding the pathology and their appearance can not be called "parkinsonism"; these are much closer related to the localization of lesions rather than to the diagnostic categories.

Characterizing gait, locomotor status, and disease severity in children and adolescents with Friedreich ataxia.

BACKGROUND AND PURPOSE: The purpose of this study was to describe gait parameters in children and adolescents with a diagnosis of Friedreich ataxia (FA) and examine the relationship between disease severity, measured by the Friedreich Ataxia Rating Scale (FARS) and gait parameters. The study examined whether FARS scores can discriminate between those who walk independently and those who require assistance. METHODS: Thirty-eight children (aged 5-11 years) and adolescents (aged 12-17 years) with genetically confirmed FA were divided into two groups based on locomotor status: group 1, subjects who were able to walk independently, and group 2, subjects who required assistance for walking. Temporal and spatial gait parameters were collected using the Stride Analyzer computerized foot switch system and compared with age-matched normative data. The FARS was used to measure disease severity. Correlation coefficients and the Mann-Whitney U test of differences were used to evaluate associations and discern differences between groups. RESULTS: In subjects with FA, gait parameters of velocity and cadence were slower and stride length was shorter compared with age-matched children without disabilities. These parameters were significantly correlated with FARS score (r = 0.696, 0.667, 0.537; respectively, all P values <0.001). Total FARS scores were correlated with locomotor status (ç value r = 0.623; P < 0.01) and could categorize subjects into groups based on independent walking or need for assistance, 73% and 87% of the time, respectively. DISCUSSION AND CONCLUSION: Subjects with FA exhibited specific abnormal gait characteristics relative to age-matched individuals. Disease severity, as measured by the FARS, was associated with gait velocity, stride length, and cadence. FARS scores can be used to categorize subjects by locomotor status and may be a useful screening tool to identify those requiring assistance.

Primary gait ignition disorder: report of three cases.

Gait ignition failure (GIF) classifications all had major limitations. Few years ago, a new and simpler classification was proposed by Liston. The aim of this paper is to discuss three GIF patients with respect to this new classification. All three patients presented with hesitation to start walking and turning and their neurological examination revealed start and turn hesitation without any other abnormality. We classified our patients according to Liston's classification as ignition apraxia, which enabled us to approach the patients in a practical way. This classification helps to understand the underlying pathologies and combines clinical characteristics and pathophysiology. We reported our experience with pergolide in the treatment of patients suffering from primary GIF and underline the fact that more research is needed on the treatment of this condition.

Higher level gait disorders in subcortical chronic vascular encephalopathy: a single photon emission computed tomography study.

BACKGROUND: the so-called higher level gait disorders include several types of gait disorders in which there are no major modifications in strength, tone, sensitivity, coordination and balance. Brain activation sites related to walking have been investigated using SPECT in humans. The aim of the study was to investigate brain activation during walking in subjects with high-level gait disorders due to chronic subcortical vascular encephalopathy. SUBJECTS: twelve patients with a chronic vascular encephalopathy were enrolled in the study. Seven subjects had apraxic gait while in the other five the gait was normal. All patients had undergone a recent cerebral magnetic resonance that revealed diffused chronic ischemic lesions within the white matter. METHODS: all 12 patients underwent a regional cerebral blood flow (rCBF) brain SPECT study with (99m)Tc-Bicisate on two separate days and under two different conditions: at rest (baseline) and while walking (functional). RESULTS: the rCBF increase induced by the treadmill test (functional-baseline), bilaterally in the medial frontal gyrus and in the anterior lobes of the cerebellum, resulted significantly (P < 0.001) lower in patients with gait apraxia versus those without it. CONCLUSIONS: this study of the brain with SPECT records the areas of perfusion deficit that appear in apraxic subjects when they walk, compared with the recordings obtained with the same investigation performed at rest.

Locomoción y trastornos de la marcha / Locomotion and gait disorders

Resumen. Introducción. El ser humano se caracteriza por la especialización de determinadas funciones, como son el lenguaje o la marcha. Esta capacidad la hemos llevado a cabo gracias al desarrollo de múltiples interconexiones entre diferentes áreas del sistema nervioso central y periférico, así como a la adaptación musculoesquelética. Todas ellas son fundamentales para una correcta realización de la deambulación y el mantenimiento del equilibrio. Desarrollo. Los trastornos de la marcha, en la actualidad, están cobrando especial importancia en las consultas de neurología, hecho que está directamente relacionado con el fenómeno de envejecimiento de la población, dado que es una patología especialmente prevalente en la población anciana. Uno de los pilares básicos en el estudio de estos trastornos es la diferenciación de los distintos patrones clínicos de marcha y la clasificación según el sistema neuronal dañado. La observación, el empleo de diferentes maniobras en la exploración y la búsqueda de otros signos clínicos asociados nos permiten una buena aproximación diagnóstica, que posteriormente podremos confirmar con técnicas complementarias más específicas. Conclusiones. Desde el punto de vista terapéutico, una intervención multidisciplinar precoz por parte de neurólogo, atención primaria, rehabilitadores y fisioterapeutas mejora la calidad de vida de los pacientes y disminuye la comorbilidad y mortalidad asociada, lo que permite, además, la reducción de los recursos sociosanitarios (AU)

Summary. Introduction. Human beings are characterised by the specialisation of certain functions, such as language or the ability to walk. We have achieved this capacity thanks to the development of multiple connections among different areas of the central and peripheral nervous system, together with adaptation of the musculoskeletal system. These are all essential to be able to walk correctly and to keep our balance. Development. Gait disorders are currently receiving a great deal of attention in neurology departments, and this fact is directly related to the phenomenon of the ageing of the population, since it is a pathology that is particularly prevalent among the elderly. One of the fundamental mainstays in the study of these disorders is being able to distinguish between the different clinical gait patterns and their classification according to the neural system that has been damaged. Observation, the use of different manoeuvres in the examination and the search for other associated clinical signs all enable us reach a good diagnostic approximation, which will later be confirmed with more specific complementary techniques. Conclusions. From the therapeutic point of view, an early multidisciplinary intervention y the neurologist, primary care, specialists in rehabilitation and physiotherapists improves patients’ quality of life and lowers the rate of associated comorbidity and mortality, which also results in a reduction in spending on community health resources (AU)

A pathophysiological model of freezing of gait in Parkinson's disease.

Freezing of gait (FOG) is a common phenomenon in Parkinson's disease (PD) affecting over half of those in the advanced stages of the disease and often does not respond to standard drug therapies. This article proposes a possible mechanism by which this disorder of movement comes about. Co-ordinated neural activities are dependent on a series of parallel neuronal networks passing through the basal ganglia connecting and integrating functions. In healthy subjects, these competing, yet complementary, networks permit tight regulation in the broad domains of motor, cognitive, and limbic functions. In patients with PD, the loss of striatal dopamine coupled with the limited repertoire of the output nuclei within these pathways allows for an element of 'cross-talk' between competing inputs, which in turn could lead to a paroxysmal excessive inhibition of the thalamus and pedunculopontine nucleus triggering freezing of gait. It is further postulated that this phenomenon may be acting via a transient period of increased synchronization within the basal ganglia oscillations.