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Specific disease-modifying treatment is becoming a clinical reality in some disorders of the motor neuron highlighting the importance of a timely and specific diagnosis.
These two cases add to the increasing evidence that motor neuron disease comprises a heterogeneous group of disorders.
These findings imply varying resistance to the disease in different pools of motor neurons.
The present case and two reported similar cases may constitute a new subgroup of motor neuron disease.
This mechanism may be particularly important in a subset of patients with motor neuron disease.
These results give a quantitative indication of the dependency in a population of patients with motor neurone disease.

Related Questions

What are the symptoms of motor neuron diseases?4 answersMotor neuron diseases (MND) are characterized by symptoms such as muscle weakness, muscle atrophy, fasciculations, hyperreflexia, spasticity, and respiratory muscle weakness. These diseases can affect both the upper motor neurons (UMN) and lower motor neurons (LMN). Other symptoms include fatigue, weight loss, cramps, and diffuse fasciculation. MNDs can manifest as weakness, fasciculations, spasticity, cramps, and pathologic hyperreflexia. Sleep disturbances, sensory disturbances, and pain can also be present in some cases. The disease course is typically progressive, with advancing weakness of the extremities and bulbar and respiratory muscles. It is important for clinicians to distinguish MND from other diseases that can mimic its symptoms, as some of these diseases can be potentially treated. Electrodiagnostic evaluation, including nerve conduction studies and needle electromyography, is key in assisting with the diagnosis of MND.
What are the latest treatments for motor neuron disease?5 answersThe latest treatments for motor neuron disease include gene therapy approaches using viral vectors and RNA modulating approaches. Other potential treatments involve cell-based therapies for motor neuron replacement and support, as well as the delivery of neuroprotective molecules. Additionally, up-regulating the expression of IGF-II or guanine deaminase in cells has shown promise as a therapeutic strategy for motor neuron diseases. Riluzole is currently used as a disease modifying drug, and multidisciplinary team care, noninvasive ventilation, botulinum toxin B, and palliative care are recommended for patient management. Further research is needed in the management of various symptoms such as dysphagia, bronchial secretion, pseudobulbar affect, spasticity, cramps, insomnia, cognitive impairment, and communication in motor neuron disease.
Are motor neurons mentioned?5 answersMotor neurons are mentioned in the abstracts. Kemmerer argues that motor regions in the frontal and parietal lobes play a significant role in the perception and interpretation of actions. Manzo's thesis describes motor neurons that control pumping behavior and innervate the proboscis musculature in Drosophila. Manzo, Silies, Gohl, and Scott examine motor neurons that regulate pumping and ingestion in Drosophila, showing that inactivating or activating these neurons disrupts or elicits arrhythmic pumping, respectively. Faravelli et al. discuss the importance of stem cell-derived motor neurons in disease modeling and potential therapeutic approaches for motor neuron diseases. Brodie and Slavin disclose a method of generating motor neurons by up-regulating or down-regulating miRNA levels in mesenchymal stem cells.
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