Motor neuron disease refers to a group of diseases that result from the degeneration of motor nerves and can lead to various upper and lower motor neuron symptoms.
Motor neuron disease (MND) is characterized by selective degeneration of motor nerves and weakness of muscles. It is a progressive disease. The prognosis of the disease is poor and life expectancy is short. The disease only affects the motor neurons that protect the sensory system.
Causes of Motor Neurone Disease
There is no definitive evidence about the causal or risk factors of the disease.
Amyotrophic Lateral Sclerosis (ALS) is a form of MND characterized by the rapid progression and presence of both UMN and LMN symptoms in the trunk, extremities, and bulbar regions. The life expectancy of patients with ALS is as short as 3 to 5 years from the onset of the disease.
The beginning of the disease may be weakness in the extremities or symptoms of the bulbar region. As the disease progresses, the weakness spreads to all regions.
Symptoms of Motor Neurone Disease
· Weakness in the distal muscles of the extremities,
· Fasciculations and cramps all over the body,
· emotional disorders,
· dysarthria
· dysphagia,
· fatigue
· change in tone
· Reflexes may be exaggerated.
ALS significantly hinders patients' quality of life due to increased dependence on activities of daily living as the disease progresses. The course of the disease varies depending on the pattern of weakness and the rate of progression.
Terminal symptoms are caused by respiratory failure and weakness of the respiratory muscles, eventually causing respiratory failure.
Presence of LMN features, old age, bulbar onset, low forced vital capacity (FVC) and low score on the revised ALS-functional rating scale (ALS-FRSr). Some factors that may be responsible for the poor prognosis have been identified.
Motor Neurone Disease Treatment
Currently, ALS cannot be cured. Multidisciplinary care is required to improve the quality of life and complications that develop due to long-term and progressive muscle weakness.
Generally, management includes pharmacological intervention, rehabilitation, nutritional recommendations, good nursing care, artificial ventilator support in later stages of the disease, and percutaneous endoscopic gastrotomy (PEG) to prevent complications due to dysphagia.
Since multiple body systems are involved. , the best approach is a multidisciplinary holistic management of the disease. Currently, ALS is treated with a combination of medical, surgical and rehabilitative treatments.
Neurorehabilitation
· Physiotherapy aims to increase muscle strength, normalize tone, improve quality of life by providing gait training and functionality.
· Occupational Therapy - The purpose of occupational therapy is to teach functional activities, transfers, bed mobility skills necessary for daily living activities. It also trains individuals with ALS to use a wheelchair or other mobility aid independently.
· Speech and Language Therapy – A speech and language therapist helps with two of the most common symptoms – slurred speech ( dysarthria ) and swallowing problems (difficulty swallowing).
· Some aids and devices, such as walking canes, modified eating utensils, speech aids, can also be used by individuals with ALS.
For Motor Neurone Disease. Cell Therapy
Cell transplantation is an attractive management strategy for ALS/MND. Preclinical animal studies have shown benefits in motor function. The safety of autologous stem cell transplantation has been established and allogeneic cell transplantation has been studied. Derived from peripheral blood using various transplantation modes such as intracerebral, intra-spinal and intrathecal Transplantation of n mononuclear cells has shown beneficial gains in survival, respiratory function, muscle strength, and bulbar impairment. It also slows down the progression of the disease and the rate of decline in respiratory function.
Marked axonal degeneration of motor neurons in the spinal cord and motor cortex is noted in ALS / MND. Currently, the goal of the transplantation process is to preserve existing motor neurons and attempt to provide regeneration and repair in damaged motor neurons. BMMNCs have neurogenic potential as well as neurotrophic effect on the nervous system. They also display immodulatory, anti-inflammatory and cytoprotective properties. Growth factors, such as vascular endothelial growth factor, secreted by these cells enable neoangiogenesis. These paracrine effects of transplantation of BMMNCs result in neuroprotection and subsequent alteration in disease course and progression.
Clinical Results of Stem Cell Therapy at NeuroGen Brain and Spine Institute: -
Intrathecal autologous compared the survival of ALS/MND patients treated with bone marrow mononuclear cell transplantation to patients who did not undergo intrathecal autologous BMMNC transplantation. There were a total of 46 patients in the intervention group and 20 patients in the control group. Both groups shared similar basic demographic characteristics. Comparison of survival time showed that patients treated with intrathecal autologous BMMNCs transplantation had longer median survival times than those untreated. The difference between the two was found to be statistically significant (p = 0.043). A clinically significant difference in survival time of 47 months demonstrates the potential of intrathecal autologous BMMNC transplantation in the treatment of ALS/MND. Some patients were given stem cell therapy along with Lithium for 6 weeks. It has been stated that younger patients given lithium experience better survival than others. This may be associated with better survival and growth of the transplanted cells.
In the majority of patients:
· Speaking more clearly and loudly,
· Improved tongue movements and
· ; Reduced speech fatigue.
· Reduced bulbar symptoms such as choking, swallowing, reduced salivation,
· Increased respiratory capacity.
· A few have shown better neck control.
· Extremity function was observed to be better in a few patients.
o Improvement in some patients,
o Lower extremity function,
o ambulation,
o Fine motor activities,
o Static and dynamic standing and sitting balance.
All these changes were noted in the early post-transplant period.
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