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Chapter: Diseases of The Brain and Nervous System(A Health Education Guide): Motor Neuron Disease

Motor Neuron Disease: Types, Diagnosis, Treatment

This is a cruel disease in which there is gradual wasting – degeneration of the muscles.


This is a cruel disease in which there is gradual wasting – degeneration of the muscles. Even after numerous researches no accurate or effective treatment is yet available and the patient becomes weaker and thinner day-by-day and the doctor and relatives, helplessly watch him getting closer towards certain death. Unfortunately, the brain keeps on relatively functioning normal, till the end, so thoughts, emotions, consciousness, pain etc can still be felt.


Types of Motor Neuron Disease :

1. First of all, let us discuss about the primary (idiopathic) motor neuron disease, which occurs due to unknown reasons. In this, the main defect is in the motor neurons, and so the anterior horn cells from which the nerves of the spinal cord originate, and the brain stem or the bulbar neurons from which the cranial nerves emerge, are gradually destroyed due to some unknown reasons.


2. The muscles in the hands and legs start wasting and the movement becomes less. There is difficulty in holding things, writing or moving the hands up and down and gradually climbing stairs and even wearing footwear also becomes difficult. In 3 to 7 years of the onset of the disease the patient becomes completely bedridden and the body weight reduces drastically. This type is called Amyotrophic Lateral Sclerosis(A.L.S.). In this, the anterior horn cells of the spinalcord as well asthe pyramidal fibres, which control the spinal cord, are affected. In neurological language, in this disease signs of both upper and lower motor neuron dysfunction are seen (on examination).


3. Progressive Muscular Atrophy is a type of motorneuron disease in which the pyramidal fibres are not affected and thus the neurological signs of upper motor neuron are not seen (like brisk jerk, extensor plantar etc). This is comparatively a slow -evolving disease.


4. As mentioned earlier, in Bulbar Motor Neurondisease, the cells from which the cranial nerves emergeget afflicted and so important functions like swallowing food, and speech etc get affected and there is difficulty in respiration. In this, the death occurs quickly within 1 to 3 years.


5. Pseudobulbar Palsy is related to A.L.S. and is adisorder of the cranial nerves in which as mentioned earlier swallowing, speech and some other functions are affected. Involuntary laughter, crying etc. like peculiar symptoms may also be seen.


6. In some cases by chance the disease is limited to a single limb, which is known as Monomelic MotorNeuron Disease. Associated deafness, which is notpresent in other motor neuron diseases, is noted in Madras Motor Neuron disease.


Diagnosis :

The disease can be diagnosed accurately with E.M.G. test. It is essential to get a confirmed diagnosis of this disease by a Neurologist, as the patient has very less active functional lifetime left after being afflicted by this disorder. Therefore, it is always better to systematically arrangefinancial, medical, and social requirements as soon as possible. Many a times, the symptoms similar to motor neuron disease are also caused by diseases like deficiency of Parathyroid Hormone, spinal cord injury (whiplash injury), effects of metals on the body (like lead), radiation, side effects of chemicals, myeloma as well as other types of cancers or AIDS. This category is called SecondaryMotor Neuron Disease.


Treatment :

1.        The patient and the relatives should be timely informed about the seriousness of the disease, so that the patient can make arrangements for the rest of his life.

2.        Muscles can be kept functional, as far as possible by physiotherapy, muscle training, walking exercise etc. Along with that the patient should make use of instruments like crutches and calipers, to ease the functions like walking and moving hands.

3.        This agonizing disease has no specific treatment. After a lot of research, a recently discovered drug called Riluzole (Rilutec) is being used. Usually it is given for 3 months and the expenses come to about 60 to 70 thousand Indian Rupees. But the disease can be slowed down only for about 3 to 6 months, which only prolongs the torture. The general experience is that it has no magical role in the treatment, although it can be used in appropriate cases.

4.        If swallowing of food, speech etc gets affected; proper training can help to a certain extent. If that fails, Ryles tube can be used for feeding. Better still is gastrostomyfeeding, in which a tunnel is made under the skin, anda tube is passed through the stomach to give the necessary nutrition.

5.        Necessary medical treatment should be given in case of emotional lability, depression, involuntary laughing and crying etc.

6.        Exercises of the respiratory muscles should be started right from the beginning. Eventually the life span of the patient can be prolonged a little, by helping in respiration with the ventilator machine. Nursing care, love of the relatives, affectionate treatment from the doctor etc. can provide support to the patient in his fight against this incurable and painful disease in his remaining life.


7.        There is an association of patients of this disease called “Motor Neuron Disease Association” which provides relevant information to the patients.

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