Upper limb motor function in young adults with spina bifida and hydrocephalus

Objective

The objective of the study was to measure upper limb motor function in young adults with spina bifida meningomyelocele (SBM) and typically developing age peers.

Method

Participants were 26 young adults with SBM, with a Verbal or Performance IQ score of at least 70 on the Wechsler scales, and 27 age- and gender-matched controls. Four upper limb motor function tasks were performed under four different visual and cognitive challenge conditions. Motor independence was assessed by questionnaire.

Results

Fewer SBM than control participants obtained perfect posture and rebound scores. The SBM group performed less accurately and was more disrupted by cognitive challenge than controls on limb dysmetria tasks. The SBM group was slower than controls on the diadochokinesis task. Adaptive motor independence was related to one upper limb motor task, arm posture, and upper rather than lower spinal lesions were associated with less motor independence.

Conclusions

Young adults with SBM have significant limitations in upper limb function and are more disrupted by some challenges while performing upper limb motor tasks. Within the group of young adults with SBM, upper spinal lesions compromise motor independence more than lower spinal lesions.

     

Motor System Pathology in Psychosis

Purpose of Review

Motor abnormalities are an intrinsic feature of psychosis. Neurological soft signs, Parkinsonism, dyskinesia, and other motor phenomena are frequently observed in subjects at clinical or genetic risk for psychosis as well as first-episode patients, chronic patients. Here, we review the most recent literature on motor assessments and pathophysiology in psychosis.

Recent Findings

Instrumental measures of fine motor performance, balance, spontaneous motor activity, and gesture indicated motor abnormalities in subjects at risk and across stages of schizophrenia. Motor phenomena are associated with distinct symptom dimensions and may indicate poor outcomes. Neuroimaging studies demonstrated altered neural maturation within critical motor networks in subjects at risk. Furthermore, specific categories of motor dysfunction were associated with distinct structural and functional alterations in the motor system in schizophrenia.

Summary

Motor abnormalities provide a unique window into the pathobiology of psychosis and have the potential to guide screening, staging, and outcome prediction.

     

Reproducible and persistent weakness in adult rats after surgical resection of motor cortex: evaluation with limb placement test

Object

The purpose of this study was to develop a new rat model for surgical brain injury with motor weakness and to find an adequate behavior test for the application of the model.

Methods

Thirty rats were divided into three groups: craniectomy (n?=?10), durotomy (n?=?10), and corticectomy (n?=?10) groups. The coordinates of the three points from the bregma (coordinate A?=?+4,+1, B?=??2,+1, and C?=?+4,+6). We evaluated right limb motor performance by the modified limb placement test and the cylinder test.

Conclusion

Persistent motor weakness was observed for 2 months in the corticectomy group by the limb placement test, whereas the cylinder test could not detect the weakness. We established a reproducible and persistent rat brain injury model and found that the modified limb placement test is sensitive enough to evaluate residual subtle weakness in this model.

     

Onset of bladder and motor symptoms in multiple system atrophy: differences according to phenotype

Purpose

To compare the order of presentation of bladder and motor symptoms between multiple system atrophy phenotypes.

Methods

Medical records were retrospectively reviewed in 144 patients.

Results

Bladder symptoms occurred either before or within 12 months after onset of motor symptoms in significantly more patients with the cerebellar phenotype than the parkinsonian phenotype (80 vs. 53%, p = 0.003); similar results were observed for urinary incontinence (79 vs. 45%, p = 0.001).

Conclusions

Urinary dysfunction is more likely to appear either before or shortly after motor symptoms in the cerebellar phenotype than in the parkinsonian phenotype.

     

Motor problems in children with neurofibromatosis type 1

Background

Children with the neurogenetic disorder neurofibromatosis type 1 (NF1) often have problems with learning and behaviour. In both parent reports and neuropsychological assessment, motor problems are reported in approximately one third to one half of the children with NF1. Studies using broad motor performance test batteries with relatively large groups of children with NF1 are limited. The aim of this cross-sectional observational study was to describe the severity of motor problems in children with NF1 and to explore the predictive value of demographics, intelligence, and behavioural problems.

Methods

From 2002 to 2014, 69 children with NF1, aged 4 to 16 years (age?=?9.5?±?2.8 years; 29 girls) had a motor, psychological, and neurological evaluation in an NF1 expertise centre. Data were collected about (1) motor performance (M-ABC: Movement Assessment Battery for Children), (2) intelligence, and (3) emotional and behavioural problems as rated by parents.

Results

Sixty-one percent of these children scored within the clinical range of the M-ABC. In ordinal logistic regression analyses, motor problems were associated with symptoms of attention-deficit/hyperactivity disorder (ADHD), symptoms of autism spectrum disorder (ASD), and externalising behavioural problems. Motor outcome was not predicted by age, intelligence, scoliosis, hypotonia, nor hypermobility.

Conclusions

Motor problems are among the most common comorbid developmental problems in children with NF1, and these problems do not diminish with age. Because of their impact on daily functioning, motor problems need to be specifically addressed in diagnosis, follow-up, and treatment of NF1.

     

Repetitive Transcranial Magnetic Stimulation for Upper Extremity Motor Recovery: Does It Help?

Purpose of Review

Repetitive transcranial magnetic stimulation (rTMS) noninvasively modulates brain excitability in humans and influences mediators of plasticity in animals. When applied in humans in the months to years after stroke, potentiation of motor recovery has been limited. Recently, investigators have shifted rTMS administration into the early weeks following stroke, when injury-induced plasticity could be maximally engaged. This article provides an overview of basic mechanisms of rTMS, consideration of its interaction with various forms of neuroplasticity, and a summary of the highest quality clinical evidence for rTMS given early after stroke.

Recent Findings

Studies of repetitive magnetic stimulation in vitro and in vivo have found modulation of excitatory and inhibitory neurotransmission and induction of cellular mechanisms supporting plasticity. A handful of clinical studies have shown sustained improvements in grip strength and UE motor impairment when rTMS is delivered in the first weeks after stroke.

Summary

Though in its infancy, recent research suggests a plasticity-enhancing influence and modest motor recovery potentiation when rTMS is delivered early after stroke.

     

Regulation of Ion Channels by MicroRNAs and the Implication for Epilepsy

Purpose of Review

The goal of this focused review is to describe recent studies supporting a critical role of microRNAs in the regulation of ion channels and discuss the resulting implications for the modulation of neuronal excitability in epilepsy.

Recent Findings

MicroRNA-induced silencing of ion channels has been shown in several different studies in recent years, and some of these reports suggest a prominent role in epilepsy. The ion channels regulated by microRNAs include ligand- and voltage-gated channels and are not only limited to the central nervous system but have also been found in the peripheral nervous system.

Summary

Ion channel-targeting microRNAs can regulate the intrinsic excitability of neurons, and thus influence entire networks in the brain. Their dysregulation in epilepsy may contribute to the disease phenotype. More research is needed to better understand the molecular mechanisms of how microRNAs regulate ion channels to control neuronal excitability, and how these processes are altered in epilepsy.

     

The New Classification of Seizures by the International League Against Epilepsy 2017

Purpose of Review

This review presents the newly developed International League Against Epilepsy (ILAE) 2017 classification of seizure types.

Recent Findings

The fundamental distinction is between seizures that begin focally in one hemisphere of the brain, generalized onset seizures that apparently originate in both hemispheres, and seizures of unknown onset. Focal seizures optionally can be subclassified according to whether awareness (a surrogate marker for consciousness) is intact or impaired. The next level of classification for focal seizures is motor (with subgroups automatisms, atonic, clonic, epileptic spasms, hyperkinetic, myoclonic, tonic), non-motor (with subgroups autonomic, behavior arrest, cognitive, emotional, sensory), and focal to bilateral tonic-clonic. Generalized seizures are categorized as motor (tonic-clonic, clonic, tonic, myoclonic, myoclonic-tonic-clonic, myoclonic-atonic, atonic, epileptic spasms) and non-motor/absence (typical, atypical, myoclonic, eyelid myoclonia).

Summary

The classification allows new types of focal seizures and a few new generalized seizures, and clarifies terms used to name seizures.

     

Effects of early-life exposure to THIP on phenotype development in a mouse model of Rett syndrome

Background

Rett syndrome (RTT) is a neurodevelopmental disorder caused mostly by disruptions in the MECP2 gene. MECP2-null mice show imbalances in neuronal excitability and synaptic communications. Several previous studies indicate that augmenting synaptic GABA receptors (GABA_ARs) can alleviate RTT-like symptoms in mice. In addition to the synaptic GABA_ARs, there is a group of GABA_ARs found outside the synaptic cleft with the capability to produce sustained inhibition, which may be potential therapeutic targets for the control of neuronal excitability in RTT.

Methods

Wild-type and MECP2-null mice were randomly divided into four groups, receiving the extrasynaptic GABA_AR agonist 4,5,6,7-tetrahydroisoxazolo[5,4-c]pyridin-3-ol hydrochloride (THIP) and vehicle control, respectively. Low-dose THIP was administered to neonatal mice through lactation. RTT-like symptoms including lifespan, breathing, motor function, and social behaviors were studied when mice became mature. Changes in neuronal excitability and norepinephrine biosynthesis enzyme expression were studied in electrophysiology and molecular biology.

Results

With no evident sedation and other adverse side effects, early-life exposure to THIP extended the lifespan, alleviated breathing abnormalities, enhanced motor function, and improved social behaviors of MECP2-null mice. Such beneficial effects were associated with stabilization of locus coeruleus neuronal excitability and improvement of norepinephrine biosynthesis enzyme expression.

Conclusions

THIP treatment in early lives might be a therapeutic approach to RTT-like symptoms in MECP2-null mice and perhaps in people with RTT as well.

     

Exploring the relationship between motor impairment,vascular burden and cognition in Parkinson’s disease

Objective

To determine frequency and type of cognitive disorders in cross-sectional analysis of a Parkinson’s disease (PD) cohort, and explore its relations to motor symptoms, modifiable vascular risk factors and white matter lesions (WML) volume.

Methods

In a group of 133 PD patients, mild cognitive impairment (PD-MCI) and dementia (PDD) were diagnosed according to Movement Disorders Society Task Force criteria (level 2 for PD-MCI). Detailed motor measurements were applied, including rigidity, axial, bradykinesia, tremor and postural instability gait disorders (PIGD) scores. Vascular risk was estimated by the Framingham General Cardiovascular Disease risk scoring algorithm and WML volume was measured for whole brain and frontal lobe.

Results

Sixty-one (46.9%) patients fulfilled criteria for PD-MCI, and 23 (17.7%) for PDD. Non-amnestic multiple domain MCI was most frequent (52% of PD-MCI patients). Motor scores were significantly higher in cognitively impaired patients, but only axial score discriminated between MCI and dementia. High vascular risk was related to impaired cognition, bradykinesia, axial, PIGD and freezing of gait (FOG) score, while whole brain WML volume was associated with PDD, FOG and attention deficits. Furthermore, high vascular risk was identified as a potential predictor of both MCI and dementia in PD. Additionally, age and bradykinesia score were independently associated with PD-MCI and age, axial score and whole brain WML volume with PDD.

Conclusion

Cognitive disorders in PD are associated with more severe, predominantly axial motor deficits and increased, but partly modifiable vascular burden, thus opening a possibility for development of preventive strategies in PD.

     

Brain structural investigation and hippocampal tractography in medication overuse headache: a native space analysis

Background

Spatial normalization of brain images, a prerequisite for voxel based morphometry analysis, may account for the large variability of the volumetric data in medication overuse headache (MOH); possibly because this disease concerns patients differing on both sex and age, and hence with different brain size and shape.

Methods

The present study aimed at providing a subject-based analysis of macrostructure using a native space volumes segmentation (Freesurfer), and microstructure using a region of interest (ROI: i.e. hippocampus) tractography approach in MOH patients.

Results

The results show that MOH patients had decreased volumes of left hemisphere temporal gyri (temporal superior, fusiform) and occipital middle gyrus, together with an increased volume of the left inferior (temporal) lateral ventricle. The left temporal volume was negatively correlated with depression score and medication dependence parameters. Seed-based tractography of the hippocampus revealed a decreased number of reconstructed fibers passing through the left hippocampus.

Conclusion

To our knowledge, these alterations have not been described with methods involving brain normalization, and they indicate that left hemisphere temporal areas, including the hippocampus, may play a role in MOH pathophysiology. Trial registration number NCT00833209. Registered 29 January 2009

     

Quantitative assessment of finger tapping characteristics in mild cognitive impairment,Alzheimer’s disease,and Parkinson’s disease

Background

Fine motor impairments are common in neurodegenerative disorders, yet standardized, quantitative measurements of motor abilities are uncommonly used in neurological practice. Thus, understanding and comparing fine motor abilities across disorders have been limited.

Objectives

The current study compared differences in finger tapping, inter-tap interval, and variability in Alzheimer’s disease (AD), Parkinson’s disease (PD), mild cognitive impairment (MCI), and healthy older adults (HOA).

Methods

Finger tapping was measured using a highly sensitive light-diode finger tapper. Total number of finger taps, inter-tap interval, and intra-individual variability (IIV) of finger tapping was measured and compared in AD (n?=?131), PD (n?=?63), MCI (n?=?46), and HOA (n?=?62), controlling for age and sex.

Results

All patient groups had fine motor impairments relative to HOA. AD and MCI groups produced fewer taps with longer inter-tap interval and higher IIV compared to HOA. The PD group, however, produced more taps with shorter inter-tap interval and higher IIV compared to HOA.

Conclusions

Disease-specific changes in fine motor function occur in the most common neurodegenerative diseases. The findings suggest that alterations in finger tapping patterns are common in AD, MCI, and PD. In addition, the present results underscore the importance of motor dysfunction even in neurodegenerative disorders without primary motor symptoms.

     

Neurophysiological hyperresponsivity to sensory input in autism spectrum disorders

Background

Atypical sensory processing is a common clinical observation in autism spectrum disorder (ASD). Neural hyperexcitability has been suggested as the cause for sensory hypersensitivity, a frequently reported clinical observation in ASD. We examined visual evoked responses to parametric increases in stimulus contrast in order to model neural responsivity of sensory systems in ASD.

Methods

Thirteen high-functioning individuals with ASD and 12 typically developing (TD) individuals completed a steady-state visual evoked potential study. Stimuli were vertical circular gratings oscillating at 3.76 Hz at varying contrasts (5, 10, 20,…, 90 % contrast, 10 levels). The average spectral power at the stimulus oscillation frequency was calculated for each contrast level.

Results

The magnitude of evoked sensory responses increased at a significantly greater rate and resulted in disproportionately elevated activation with higher contrasts in the ASD group. Approximately 45 % of ASD participants had rates of response increases greater than any TD participant. This alteration was highly associated with parental reports of these participants’ sensory difficulties.

Conclusions

Greater increases in visual responses over contrast manipulation suggest heightened excitability in the sensory cortex in ASD participants. Heightened neural excitability was observed in a substantial portion but not all of the ASD participants. This pattern suggests that individuals with higher excitability may constitute a neurobiologically distinct subgroup requiring individualized treatment interventions.

     

Mindfulness Meditation for Chronic Pain: Systematic Review and Meta-analysis

Background

Chronic pain patients increasingly seek treatment through mindfulness meditation.

Purpose

This study aims to synthesize evidence on efficacy and safety of mindfulness meditation interventions for the treatment of chronic pain in adults.

Method

We conducted a systematic review on randomized controlled trials (RCTs) with meta-analyses using the Hartung-Knapp-Sidik-Jonkman method for random-effects models. Quality of evidence was assessed using the GRADE approach. Outcomes included pain, depression, quality of life, and analgesic use.

Results

Thirty-eight RCTs met inclusion criteria; seven reported on safety. We found low-quality evidence that mindfulness meditation is associated with a small decrease in pain compared with all types of controls in 30 RCTs. Statistically significant effects were also found for depression symptoms and quality of life.

Conclusions

While mindfulness meditation improves pain and depression symptoms and quality of life, additional well-designed, rigorous, and large-scale RCTs are needed to decisively provide estimates of the efficacy of mindfulness meditation for chronic pain.

     

Perceptions of Neighborhood Park Quality: Associations with Physical Activity and Body Mass Index

Background

Parks are important resources for physical activity (PA), yet few studies have examined how perceptions of park characteristics relate to PA and health.

Purpose

This study investigated associations between perceptions of neighborhood park quality and overall moderate-to-vigorous PA (MVPA), park-based PA, and body mass index (BMI).

Methods

Data were collected via questionnaire from 893 households in Kansas City, Missouri.

Results

The newly developed neighborhood park quality scale demonstrated good test–retest and internal reliability. Residents’ perceptions of neighborhood park quality were related to PA and health outcomes. Perceiving parks as a benefit was positively related to overall MVPA and park-based PA and negatively related to BMI. Perceptions of well-used parks were positively related to BMI, while perceived cleanliness was negatively related to park-based PA.

Conclusions

Better measuring and understanding how perceptions of local parks are associated with PA and health can improve appreciation of how parks facilitate active living.

     

Pallidal deep brain stimulation in juvenile Huntington's disease: local field potential oscillations and clinical data

Background

Recently, therapeutic attempts to control motor choreatic hyperkinesia of Huntington’s disease (HD) by means of pallidal deep brain stimulation (Gp-DBS) were successful. With respect to the clinical effects of Gp-DBS in juvenile hypokinetic-rigid HD (jHD; Westphal variant), only one single-case has been reported up to date. Oscillatory patterns of the Gp in jHD are not known.

Objectives and methods

This work aimed to analyse pallidal local field potential oscillations (LFP) in two patients with jHD treated with Gp-DBS. Safety data and clinical scores up to 12 months after DBS-electrode implantation were collected in the framework of a prospective trial (ClinicalTrials.gov; NCT00902889).

Results

Intraoperative LFP revealed local alpha and beta oscillations similar to those found in other movement disorders with akinetic rigid and dystonic presentation. Significant motor improvement was not found. There were no treatment-related complications or unresolved long-term adverse events.

Conclusions

In spite of similar intraoperative LFP patterns of jHD with those of movement disorders benefitting from DBS, clinical results were not convincing in our patients, so that Gp-DBS in jHD cannot be generally recommended.

     

Devastating Delayed Leukoencephalopathy Associated with Bath Salt Inhalation

Background

“Bath salts” or synthetic cathinone toxicity remains a potentially deadly clinical condition. We report a delayed leukoencephalopathy with persistent minimally conscious state.

Methods

Case report.

Results

A 36-year-old man presents with delayed encephalopathy, dysautonomia, fulminant hepatic failure, and renal failure from severe rhabdomyolysis after consuming bath salts. MRI showed diffusion restriction in the splenium of the corpus callosum and subcortical white matter.

Conclusions

The combination of acute leukoencephalopathy, rhabdomyolysis and fulminant hepatic failure may point to bath salt inhalation and should be known to neurointensivists.

     

Primary lateral sclerosis and the amyotrophic lateral sclerosis–frontotemporal dementia spectrum

Aim

To investigate whether primary lateral sclerosis (PLS) represents part of the amyotrophic lateral sclerosis–frontotemporal dementia (ALS–FTD) spectrum of diseases.

Methods

Comprehensive assessment was taken on 21 patients with PLS and results were compared to patients diagnosed with pure motor ALS (n?=?27) and ALS–FTD (n?=?12). Clinical features, Addenbrooke’s Cognitive Examination (ACE) scores, Motor Neuron Disease Behaviour (Mind-B) scores, motor disability on the ALS functional rating scale (ALSFRS) and survival times were documented. Motor cortex excitability was evaluated using transcranial magnetic stimulation (TMS).

Results

Global cognition was impaired in PLS (mean total ACE score 82.5?±?13.6), similar to ALS–FTD (mean total ACE score 76.3?±?7.7, p?>?0.05) while behavioural impairments were not prominent. TMS revealed that resting motor threshold (RMT) was significantly higher in PLS (75.5?±?6.2) compared ALS–FTD (50.1?±?7.2, p?<?0.001) and ALS (62.3?±?12.6, p?=?0.046). Average short-interval intracortical inhibition (SICI) was similar in all three patient groups. The mean survival time was longest in PLS (217.4?±?22.4 months) and shortest in ALS–FTD (38.5?±?4.5 months, p?=?0.002). Bulbar onset disease (β?=???0.45, p?=?0.007) and RMT (β?=?0.54, p?=?0.001) were independent predictors of global cognition while motor scores (β?=?0.47, p?=?0.036) and SICI (β?=?0.58, p?=?0.006) were significantly associated with ALSFRS.

Conclusion

The cognitive profile in PLS resembles ALS–FTD, without prominent behavioural disturbances. A higher RMT in PLS than ALS and ALS–FTD is consistent with differential cortical motor neuronal abnormalities and more severe involvement of corticospinal axons while SICI, indicative of inhibitory interneuronal dysfunction was comparable with ALS and ALS–FTD. Overall, while these findings support the notion that PLS lies on the ALS–FTD spectrum, the mechanisms underlying slow disease progression are likely to be distinct in PLS.

     

Girls get by with a little help from their friends: gender differences in protective effects of social support for psychotic phenomena amongst poly-victimised adolescents

Purpose

To investigate whether social support is protective for psychotic experiences similarly among poly-victimised adolescent girls and boys.

Methods

We utilised data from the Environmental Risk (E-Risk) Longitudinal Twin Study, a nationally-representative sample of 2232 UK-born twins. Participants were privately interviewed at age 18 about victimisation, psychotic experiences, and social support during adolescence.

Results

Perceived social support (overall and from friends) was found to be protective against psychotic experiences amongst poly-victimised adolescent girls, but not boys. Though boys were similarly protected by family support.

Conclusions

Social support-focused interventions targeting psychotic phenomena amongst poly-victimised adolescents may be more effective for girls.

     

Rosette-forming glioneuronal tumor outside the fourth ventricle: a case-based update

Introduction

Rosette-forming glioneuronal tumors (RGNTs) that do not involve the fourth ventricle are rare. RGNTs were originally thought to be exclusively localized into the fourth ventricle but were found in various anatomical localizations.

Material and methods

We review the literature and found 32 cases of this particular RGNT. The outcome was excellent with no mortality reported after surgical treatment. Only two patients had received adjuvant therapy for progression. We added one case of a RGNT located in the cerebellar hemisphere.

Case and conclusion

She underwent a subtotal removal with no evidence of progression after. This WHO grade I tumor with a specific biphasic histopathology is of a good oncological outcome after surgical treatment. A long follow-up is needed as recurrence or metastatic progressions exist.