Dominant mutations in ubiquitously expressed superoxide dismutase (SOD1) cause familial ALS by provoking premature death of adult motor neurons. To test whether mutant damage to cell types beyond motor neurons is required for the onset of motor neuron disease, we generated chimeric mice in which all motor neurons and oligodendrocytes expressed mutant SOD1 at a level sufficient to cause fatal, e...

Spinal motor neurons are specified to innervate different muscle targets through combinatorial programs of transcription factor expression. Whether transcriptional programs also establish finer aspects of motor neuron subtype identity, notably the prominent functional distinction between alpha and gamma motor neurons, remains unclear. In this study, we identify DNA binding proteins with complem...

Introduction: The term motor neuron disease encompasses a spectrum of disorders in which motor neurons are the lost. Importantly, while some motor neurons are lost early in disease and others remain intact at disease end-stage. This creates a valuable experimental paradigm to investigate the factors that regulate motor neuron vulnerability. Spinal muscular atrophy is a childhood motor neuron di...

Frontotemporal dementia is an important neurodegenerative disorder accounting for a significant proportion of dementia cases with onset before 60 years of age. Apart from the well recognized behavioral changes the disease has many other distinctive features like predominant language involvement alone or associated features of motor neuron disease or parkinsonism etc. which at times may be the p...

Bell's palsy is a benign lower motor neuron facial nerve disorder. It is almost always unilateral. We report a 20-year-old nulliparous woman with five episodes of recurrent Bell's palsy. A review of recent medical literature revealed a paucity of case reports involving an individual with five episodes of recurrent Bell's palsy, with none found in Asian neurology medical literature. Despite the ...

The concurrent validity of the Developmental Coordination Disorder Questionnaire (DCDQ) and the McCarron Assessment of Neuromuscular Development (MAND) was investigated in an Australian sample of 38 girls and 91 boys aged 9-12 years (M = 11.15, SD = 0.81). The MAND identified 44 children with motor impairment (MI) and 85 children without MI. The overall decision agreement between the two measur...

Individual spinal motor neuron identities are specified in large part by the intrinsic repertoire of transcription factors expressed by undifferentiated progenitors and maturing neurons. It is shown here that the transcription factor AML1/Runx1 (Runx1) is expressed in selected spinal motor neuron subtypes after the onset of differentiation and is both necessary and sufficient to suppress intern...

Scientific evidence is emerging to indicate that motor neuron injury in motor neuron disease may reflect a complex interplay between genetic factors, oxidative stress, and imbalance of the glutamatergic excitatory control of motor neurons, which may result in damage to critical target proteins and organelles. The relative importance of these factors is likely to vary in different subgroups of p...

Motor impairment in individuals with autism potentially impacts on their development in all spheres. This paper is particularly concerned with people with severe communication impairments suggesting that recognition of the impact of motor impairments on their lives could lead to more effective interventions being developed. One such intervention is the MORE (Means, Opportunities, Reasons, and E...

Developmental coordination disorder (DCD) is often called a motor learning deficit. The question addressed in this paper is whether improvement of motor skills is just a matter of mere practice. Without any kind of intervention, children with DCD do not improve their motor skills generally, whereas they do improve after task-oriented intervention. Merely offering children the opportunity to pra...