The motor neuron disease Madras is a disease that is essentially characterized by a pronounced weakness of the extremities of the affected patients. The disease usually begins during puberty. Atrophy of the limbs develops, in addition, there are paralysis of various nerves of the brain. In addition, the individuals suffer from sensorineural hearing loss.
What is Madras Motor Neuron Disease?
The motor neuron disease Madras is often referred to by the medical profession with the abbreviation MMND, which is derived from the English term disease. The disease is characterized by weak extremities that show atrophy. In addition, the sick patients show paralysis (medical term paresis) of the cranial nerves in the lower area. See AbbreviationFinder for abbreviations related to MMND.
Sensorineural hearing loss is also characteristic of Madras motor neuron disease. Basically, the motor neuron disease Madras occurs relatively rarely. Experience to date indicates that the disease is somewhat more common in male patients than in females.
Causes
The motor neuron disease Madras has not yet been sufficiently researched to make reliable statements about the pathogenesis of the disease. In principle, however, most researchers agree that the disease has a genetic component. Many medical professionals assume that anomalies in the DNA of the mitochondria are responsible for the development of the disease.
However, inflammatory processes are also discussed as potential causes. In addition, certain environmental factors may contribute to the development of Madras motor neuron disease. The lack of research studies on Madras motor neuron disease is also related to the low prevalence of the condition. The exact frequency of Madras motor neuron disease has not yet been established.
However, around 200 cases of illness are known to date. The majority of patients come from southern India. The disease also occurred occasionally in Italy and Thailand. The disease begins in most cases before patients reach the age of 15. Madras motor neuron disease is slightly more common in men than women. In some patients, the parents are consanguineous.
Symptoms, Ailments & Signs
People with Madras Motor Neuron Disease have a thin physique and weak extremities with atrophy. The weakness primarily affects the muscles of the arms and legs. The bulbar and facial muscles are also usually affected by the impairments. In addition, the sick people show so-called pyramid signs.
Another typical symptom of Madras motor neuron disease is paralysis of nerves in the brain. The nerves 7, 9 and 12 are particularly involved. Basically, all people with Madras motor neuron disease suffer from hearing loss. Some of the patients are also characterized by what is known as optic atrophy.
Diagnosis & course of disease
The diagnosis of Madras motor neuron disease is based on the clinical symptoms of the disease and is carried out by a medical specialist. The main complaints of the patient may already substantiate the suspicion of the motor neuron disease Madras by the treating doctor. In many cases, the diagnosis of Madras motor neuron disease actually extends over a long period of time, as the disease is relatively unknown due to its rarity.
After the patient consultation, the specialist conducts visual examinations, in particular examining the limbs of the person. The typical symptoms and key indicators for the presence of Madras motor neuron disease is the combination of benign atrophy of the extremities and sensorineural hearing loss.
These complaints are first identified by visual examinations and hearing tests. Imaging methods can also be used to differentiate the disease from other forms of motor neuron diseases. As part of an electromyography, there are usually indications of chronic, denervating processes.
Since the symptoms also occur with other diseases, the doctor must always carry out a thorough differential diagnosis. For example, progressive muscular atrophy, amyotrophic lateral sclerosis and spinal muscular atrophy are excluded. In addition, the doctor differentiates the present disease from Brown-Vialetto-Van Laere syndrome, spinocerebellar ataxia and post-polio progressive muscular atrophy.
Complications
Motor neuron disease causes those affected to suffer from severe paralysis and sensory disturbances that can affect the entire body. This paralysis severely restricts the patient’s everyday life, resulting in a severely reduced quality of life. In many cases, normal everyday activities can no longer be carried out due to motor neuron disease and the patients are then dependent on the help of other people in everyday life.
It can also lead to severe movement restrictions. The muscles in the face can also be affected by the paralysis, making it difficult for the patient to eat and drink. Furthermore, the motor neuron disease leads to a hearing loss, so that in the worst case the affected person is completely deaf.
Young people in particular suffer from hearing loss and as a result develop severe depression and other mental health problems. It is not uncommon for the parents and relatives of the patients to be affected by motor neuron disease and they also suffer from psychological problems and upsets. Motor neuron disease is usually treated with therapies, which cannot limit all symptoms. The life expectancy of patients is significantly reduced by this disease. However, there are no particular complications during the treatment itself.
When should you go to the doctor?
Madras motor neuron disease is a serious condition that requires prompt diagnosis and treatment. People who notice weakness in the arms and legs, paralysis of the facial nerves or other typical signs should consult their [[[family doctor]]. Persistent hearing loss and symptoms of optic atrophy that cannot be attributed to any other cause should also be clarified quickly. Depending on their expertise, the family doctor can make an initial suspected diagnosis or at least rule out obvious triggers.
The actual diagnosis of Madras motor neuron disease can only be made by a medical specialist. Due to the rarity of the disease, a more detailed examination in a specialist clinic for genetic diseases is usually necessary. Patients who notice an increase in symptoms or suddenly suffer from severe paralysis should call the emergency doctor . Depending on the symptoms, treatment is carried out by neurologists, ophthalmologists, ear specialists, orthopedists and physiotherapists. Most will also be a psychologistinvolved in the treatment, since the Madras motor neuron disease also represents a significant burden for the patient’s psyche.
Treatment & Therapy
The motoneuron disease Madras is neither causally treatable nor curable. However, treating the symptoms improves the patient’s quality of life. Ideally, physicians from different disciplines work in an interdisciplinary manner to treat people affected by Madras motor neuron disease. Physiotherapists, psychologists and neurologists, for example, are essential.
The hearing loss is usually alleviated with adequate hearing aids . In particular, the patient’s physiotherapy therapy plays an important role in strengthening the weakened muscles of the limbs. Basically, Madras motor neuron disease develops progressively over a longer period of time. However, it is basically a benign or noncancerous disease.
The majority of those affected live more than 30 years after the Madras motor neuron disease first manifested itself. This suggests that Madras motor neuron disease does not adversely affect the life expectancy of affected patients. Given their limitations, patients are able to lead largely normal lives.
Outlook & Forecast
The outlook for Madras motor neuron disease is mixed. Scientists suspect that the disease is due to genetic causes. Therefore, no cure is possible to this day. It remains to be seen to what extent research for Madras motor neuron disease will develop therapies in the future. The probability of an occurrence in Europe is very low. The typical symptoms have so far appeared mainly in southern India. The number of patients recorded has so far amounted to just 200 cases worldwide. The main problem seems to be that the motor neuron disease Madras is constantly evolving, which reduces the quality of life. According to the current state of research, however, life expectancy does not suffer.
Patients have to adjust to an increase in symptoms as they get older. These can be minimized in part with suitable therapies and aids. However, Madras motor neuron disease remains present. Those affected often have to complete treatments with neurologists and physiotherapists. Typically, hearing loss develops, which can be compensated for by a hearing aid. If symptoms appear at a young age, patients often suffer from psychological problems. The limitations experienced in life can be treated within the framework of psychotherapy.
Prevention
Targeted prevention of Madras motor neuron disease is not practical, as there is still insufficient knowledge about the causes of the disease. Various potential factors of pathogenesis are currently being discussed by researchers, but there is neither agreement on the causes nor are valid results from research studies available.
You can do that yourself
Since the disease is associated with hearing loss, the person affected should be careful in everyday life, especially in traffic or in other dangerous situations. Life-threatening events can occur due to the inability to hear adequately. In order not to put too much strain on interpersonal relationships, the people in the immediate vicinity should be sufficiently informed about the complaints. When running team sports, it’s imperative to educate your comrades about the adversities.
The genetic disease can lead to optical changes in the face. This often triggers psychological and emotional problems. It should be checked whether psychotherapeutic help should be sought. In addition, self-confidence should be strengthened and stabilized. In order to cope with the challenges of everyday life, it is necessary to have mental strength. In self-help groups, the affected person can get in touch with other sufferers. Experiences are exchanged and mutual help for self-help can take place. In many cases, communicating with other patients helps to develop solution strategies in dealing with the disease on a daily basis.
Relaxation and mental techniques have proven to be very effective in reducing stress in recent years. These can be carried out independently at any time. Yoga or meditation offer everyone who is interested different ways of doing it. They stabilize the mental powers.