MOTOR NEURONE DISEASE
The movements essential in fulfilling our day-to-day requirements are a result of the coordinated control of the downstream targets by the motor neurons present in our body. The identity of the targets innervated by these neurons are thoroughly matched. Thus, by transmitting the signals to the skeletal/smooth muscles of our body from the spinal cord, motor neurons are responsible for all our body’s muscle movements. When these motor neurons start to degenerate, a condition called Motor Neurone Disease is caused. Integral muscle movements such as walking, speaking, swallowing, breathing and gripping are impaired due to this condition. With the increasing severity of such a condition, the patient finds it difficult to keep up with its day-to-day activities and ultimately reaches a state of paralysis.
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In spite of the elaborate research on the disease, no links of the condition with an individual’s diet, lifestyle or race have been well-founded. However, certain genetic predisposition has been an element. It has been found out the 5% of the sufferers have an associate who has also suffered from the condition or a related illness termed frontotemporal dementia.
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Motor Neurone Disease has no cure that leads to its complete elimination. However, the various methods of treatment target towards the deceleration of the condition’s progression and thus slow down the certain types of MND. Most of the treatments employ various approaches that compensate for an individual’s loss of mobility. Drugs such as Riluzole open SK channels and come off as effective in preventing nerve damage caused by excessive release of the excitatory amino acid, glutamate. Baclofen, a derivative of GABA, is a muscle relaxant that can treat spasticity associated with MND. Spasticity can also be treated by Tizanidine whose effectiveness is due to the presynaptic inhibition of spinal motor neurons. Botulinum toxin is also approved for the treatment of spasticity. The latter causes a neuromuscular blockade by decreasing the release of a neurotransmitter, acetylcholine. Other possible treatments include Botox injections which hold the potential to be effective by blocking the transport of signals from the CNS to the muscles. In most of the cases, medication has been employed to mildly boost the patient’s survival as the progression cannot be put an end to. People who fall prey to this condition has an average survival period of 3-4 years. However, proper treatment can extend this time frame to 10 years or more.
In this condition, the symptoms are generally pain-free and typically develop gradually over the course of months. In most of the cases, the preliminary symptoms appear on one side of the body. Typical symptoms appear in the arms, legs, mouth and even in the respiratory system. In the primary stage, the patient might experience difficulty in picking up and holding things. Physically evident symptoms such as fatigue, slurred speech, muscle cramps/twitches, difficulty in swallowing (causes drooling), joint pain and shortness of breath are also included. Gradually the patient reaches a stage where he requires an external support in moving, eating and breathing. Considering that basic processes are impaired by this condition, it rather comes off as life threatening.
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In the early stages, the diagnosis of MND is almost difficult as it bears an underlying similarity with other conditions such as multiple sclerosis. No specific tests that can diagnose Motor Neurone Disease have been developed. Close clinical examinations fuelled by the basis of neurology are employed in the prediction of the diagnoses. Sometimes, specialised tests could also be advised to rule out disorders with similar features. This is when a relative diagnosis comes into the picture. Around 10-15% of the individuals with MND are patients of frontotemporal dementia. The latter is the reason for certain alterations in personality and behavioural approach that can be observed.