Motoneuron disease Madras is a disorder that is essentially characterized by marked limb weakness in affected patients. The disease usually takes its beginning in the phase of puberty. Atrophy of the limbs develops, and paralysis of various nerves of the brain also occurs. In addition, individuals suffer from sensorineural hearing loss.
What is Madras motor neuron disease?
Motoneuron disease Madras is often referred to among medical professionals by the abbreviation MMND, which is derived from the English term for the disease. Typical of the disease are weak extremities that show atrophy. In addition, the affected patients show paralysis (medical term paresis) of the cranial nerves in the lower region. A characteristic feature of Madras motor neuron disease is also sensorineural hearing loss. In principle, motor neuron disease Madras occurs relatively rarely. Previous empirical data suggest that the disease is slightly more common in male patients than in females.
Causes
To date, Madras motor neuron disease has not been adequately studied to draw firm conclusions about its pathogenesis. In principle, however, most researchers agree that the disease has a genetic component. Many physicians assume that abnormalities in the DNA of the mitochondria are responsible for the development of the disease. However, inflammatory processes are also under discussion as potential causes. In addition, certain environmental factors may contribute to the development of Madras motor neuron disease. The lack of research studies on motor neuron disease Madras is also related to the low prevalence of the disease. The exact prevalence of motor neuron disease Madras has not yet been researched. However, approximately 200 cases of the disease have been reported to date. The majority of patients originate from southern India. Isolated cases have also occurred in Italy and Thailand. In most cases, the disease begins before the patients reach 15 years of age. Madras motor neuron disease presents slightly more often in males than in females. In some patients, the parents are consanguineous.
Symptoms, complaints, and signs
People with motoneuron disease Madras have a gaunt physique and weak extremities with atrophy. The weakness primarily affects the muscles of the arms and legs. The bulbar as well as the facial muscles are also usually affected by the impairments. In addition, the diseased individuals exhibit so-called pyramidal signs. Another typical symptom of Madras motor neuron disease is paralysis of nerves of the brain. Involved are mainly nerves 7, 9 and 12. Basically all persons with motor neuron disease Madras suffer from hearing loss. Some of those with the disease are also characterized by what is known as optic atrophy.
Diagnosis and course of the disease
The diagnosis of motor neuron disease Madras is made on the basis of the clinical symptoms of the disease and is performed by a medical specialist. The patient’s chief complaints may already substantiate the treating physician’s suspicion of motor neuron disease Madras. In many cases, the diagnosis of motor neuron disease Madras real extends over a long period of time, because the disease is relatively unknown due to its rarity. After the patient interview, the specialist performs visual examinations, especially looking at the limbs of the persons. The typical complaints and decisive indications for the presence of Madras motor neuron disease is the combination of benign atrophy of the extremities and sensorineural hearing loss. These complaints are initially detected by visual examinations and hearing tests. The condition can also be differentiated from other forms of motor neuron disease by imaging methods. Electromyography usually reveals evidence of chronic denervating processes. Since the symptoms also occur in other diseases, the physician must always perform a thorough differential diagnosis. For example, progressive muscular atrophy, amyotrophic lateral sclerosis and spinal muscular atrophy are excluded.In addition, the physician differentiates the present disease from Brown-Vialetto-Van Laere syndrome, spinocerebellar ataxia, and post-polio progressive muscular atrophy.
Complications
As a result of motor neuron disease, affected individuals suffer from severe paralysis and disturbances in sensibility that can occur throughout the body. These paralyses severely limit the patient’s daily life, resulting in a greatly diminished quality of life. In many cases, ordinary activities of daily living can no longer be performed due to motor neuron disease and patients are then dependent on the help of other people in their daily lives. Likewise, there may be severe restrictions in movement. The muscles in the face can also be affected by the paralysis, making it difficult for patients to take in food and liquids. Furthermore, motor neuron disease leads to hearing loss, so that in the worst case the affected person is completely deaf. Young people in particular suffer from hearing loss and develop severe depression and other psychological symptoms as a result. It is not uncommon for the parents and relatives of the patients to be affected by motor neuron disease and also suffer from psychological discomfort and upset. Treatment of motor neuron disease is usually through therapies, which may not limit all symptoms. The life expectancy of patients is considerably reduced by this disease. However, no particular complications occur during the treatment itself.
When should one go to the doctor?
Madras motor neuron disease is a serious condition that must be diagnosed and treated promptly. Individuals who notice weakness of the arms and legs, paralysis of the facial nerves, or other typical signs are best to consult their [[[family doctor]]]. Persistent hearing loss and symptoms of optic atrophy that are not due to any other cause should also be promptly evaluated. The primary care physician may be able to make an initial tentative diagnosis or at least rule out obvious triggers, depending on his or her expertise. The actual diagnosis of Madras motor neuron disease can only be made by a medical specialist. Due to the rarity of the disease, further 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 an emergency physician. Depending on the symptoms, treatment is provided by neurologists, ophthalmologists, otologists, orthopedists and physiotherapists. In most cases, a psychologist is also involved in the treatment, since Madras motor neuron disease also places a considerable burden on the patient’s psyche.
Treatment and therapy
Motoneuron disease Madras is neither causally treatable nor curable. However, treating the symptoms will improve the patient’s quality of life. Ideally, medical professionals from various specialties work together in an interdisciplinary manner to treat individuals affected by motor neuron disease Madras. For example, physiotherapists, psychologists and neurologists are essential. Hearing loss is usually alleviated with adequate hearing aids. In particular, physiotherapy for patients plays an important role in strengthening the weakened muscles of the limbs. Basically, Madras motor neuron disease develops progressively over a long period of time. However, it is basically a benign or benign disease. The majority of affected individuals live for more than 30 years after the first manifestation of Madras motor neuron disease. This indicates that motor neuron disease Madras does not negatively affect the life expectancy of affected patients. Considering their limitations, patients are able to lead a largely normal life.
Outlook and prognosis
The outlook for Madras motor neuron disease is considered mixed. Scientists suspect that the disease results from genetic causes. As a result, no cure has been found to date. It remains to be seen to what extent research will develop therapies for Madras motor neuron disease in the future. The probability of occurrence in Europe is very low. The typical symptoms have so far appeared mainly in southern India. The recorded number of patients so far amounted to just 200 cases worldwide.The main problem appears to be that Madras motor neuron disease always develops further, which reduces the quality of life. However, according to the current state of research, life expectancy does not suffer. Patients must be prepared for an increase in symptoms with the years of life. These can be minimized to some extent by suitable therapies and aids. However, Madras motor neuron disease remains present. Affected persons have to undergo treatments by neurologists and physiotherapists on a frequent basis. Typically, hearing loss develops and can be compensated for with a hearing aid. If symptoms occur at a young age, patients often suffer from psychological problems. The limitations experienced in life can be treated in the course of psychotherapy.
Prevention
Targeted prevention of Madras motor neuron disease is not practical because sufficient knowledge regarding the causes of the disease is not yet available. Currently, researchers are discussing various potential factors of pathogenesis; however, there is neither agreement on the causes of development nor valid results from research studies.
Here’s what you can do yourself
Because the condition is associated with hearing loss, the affected person should be careful in everyday life, especially in traffic or other dangerous situations. Due to the inability to hear sufficiently, life-threatening events may occur. In order not to put too much strain on interpersonal relationships, people in the immediate vicinity should be sufficiently informed about the symptoms. When performing team sports, it is essential to inform one’s comrades about the adversity. The genetic condition can lead to visual changes in the face. This often triggers psychological and emotional problems. It should be considered whether psychotherapeutic help should be sought. In addition, self-confidence should be strengthened and stabilized. To cope with the challenges in everyday life, it is necessary to have a mental strength. In self-help groups, the affected person can get into contact with other sufferers. Experiences are exchanged and mutual help for self-help can take place. Communication with other sufferers helps in many cases to develop solution strategies in the daily handling of the disease. In recent years, relaxation and mental techniques have proved very effective in reducing stress. These can be carried out on one’s own responsibility at any time. Yoga or meditation offer every interested person different possibilities of implementation. They stabilize the mental forces.
