Amyotrophic lateral sclerosis: Does the clinical picture of ALS differ between the sexes?

Amyotrophic lateral sclerosis: Does the clinical picture of ALS differ between the sexes?


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Are there differences in the symptoms and clinical picture between the sexes of ALS? And what do they look like? Do they affect therapy?

ALS – amyotrophic lateral sclerosis is a disease of the motor neurons that causes… Muscle paralysis leads. It is progressive and usually leads to death within a few years. There is currently no cure. Research suggests that it may Differences in the symptoms and in the course between men and women. Claudia Albreit from Nuremberg suffers from the disease. Her son can barely remember her voice.

ALS: How does it come about and what can you do?

The Amyotrophic lateral sclerosis (ALS) was first diagnosed around 150 years ago by the French doctor Jean Martin Charcot described. He is considered the founder of the modern neurology and he managed to match clinical symptoms with anatomical findings and thus differentiated ALS from multiple sclerosis. He described the changes in nerve tissue in great detail and predicted that it would be a long time before ALS could be successfully treated.

ALS is a disease of the central and peripheral nervous system. The causes are largely unknown. Every year, around one to two people per 100,000 develop ALS; it usually occurs between the ages of 50 and 70, younger people Adult are rarely affected. However, the frequency seems to be increasing somewhat worldwide. The most famous patient is probably the physicist Stephen Hawkingwho was diagnosed with the disease when he was just 21 years old. Men become ill slightly more often than women at a ratio of 1.2 to 1. The disease almost exclusively affects the motor nervous system. Sensations such as touch, pain and temperature.

Sight, smell, hearing, taste and the functions of the bladder and intestines remain largely normal. Our Muscles are from “motor system” controls, which also controls the movements. This disease occurs both in its central (1st motor neutron in the brain to the spinal cord) and in its peripheral parts (2nd motor neutron in the brain stem and spinal cord to the muscle). This leads to involuntary Muscle twitchesmuscle weakness and atrophy in the arms and legs as well as in the respiratory muscles. Speech, chewing and swallowing muscles can also be weakened. Likewise can Muscle paralysis and spasticity occurs.

Symptoms and diagnosis: This is how the disease progresses

The first symptoms can occur in those affected different places appear. Weaknesses may initially only be noticeable in individual muscles in the hand and forearm muscles, even on one side, before they spread to the opposite side or the legs. Less common The symptoms begin in the lower leg or foot muscles and in the upper arm or shoulder muscles. The disease sometimes begins with symptoms in the speech, chewing and swallowing muscles. However, spastic paralysis is rare at the beginning.

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But already in Early stage People often complain about involuntary muscle twitching or painful muscle paralysis. The disease usually progresses slowly and spreads to other areas of the body, resulting in increasing Weakness in breathing leads. Life expectancy is usually shortened to three to four years, although very slow progressions of ten years or more are also known.

Diagnosed The disease is diagnosed by the neurologist, who assesses the muscles with regard to muscle loss, strength and twitching. Speech, swallowing and breathing function as well as reflexes must also be checked. In addition, an assessment of the functions of the nervous system is essential in order to Misdiagnoses This is particularly necessary with regard to the prognosis and treatability.

Treatment of ALS

ALS is currently not curable. The course can be controlled with medication slowbut not stopping or undoing. The disease usually progresses very quickly; most of those affected die within two to five years, even if it is Exceptions like Stephen Hawking, who, despite all predictions, lived with ALS for more than 50 years.

Using medications and therapies as well as medical devices, it is possible to slow the progression and extend life expectancy. These are:

  • Drug therapy: Some medications can delay the progression of the disease and relieve symptoms.
  • Physiotherapy: Physiotherapy and occupational therapy can help maintain mobility and make everyday life easier.
  • Communication therapy: Speech therapists can help with swallowing and speaking problems and make it easier to use communication aids such as special computers.
  • Medical devices: Medical devices such as ventilators can help with breathing problems and improve quality of life.
  • Support: Open communication with doctors and self-help groups can support the sufferer and help them achieve a better quality of life.
  • Forecast: Because the disease is progressive and has no cure, treatment focuses on relieving symptoms and improving quality of life.

As a medication it is usually used Riluzole administered, which extends life expectancy by approximately 25 percent, although Side effects such as dizziness, gastrointestinal complaints and liver problems. Another drug, Edaravone, is not approved in the EU and only works in a subset of patients. In addition, supportive medications are prescribed to… Symptom Burden to reduce how Antidepressants or sedatives. To do this, you have to pay attention to a high-calorie diet in order to supply the muscles with enough energy.

Differences between the sexes

Even though there is currently no cure for ALS, research is ongoing Causes and possible treatment methods. Researchers at the Technical University of Munich have the molecular Backgrounds systematically examined and found that ALS can be found in Subtypes can be divided. Depending on the subtype, different medications could be effective. There are clear differences in molecular processes between men and women. The molecular processes that lead to a progressive loss of control over motor functions remain poorly understood. So far, studies have been limited to individual aspects of molecular processes. With the one now carried out study ALS was examined using a “multi-omics” approach, where the coded and non-coded RNA molecules and the entirety of the proteins were examined.

A central finding of the study led to the identification of overall four subtypes. These cannot be distinguished based on the clinical symptoms, only at the molecular level. However, very different things happen at the molecular level. An active ingredient that is helpful for one variant could have no effect at all on another. So far, only these have been used in clinical studies Effects considered across all types. As a result, potentially effective substances could not have been recognized. While one subtype affected genes associated with inflammatory processes and immune responses, in another case there were disruptions in the transcription of DNA into RNA molecules. Others let themselves different signs for oxidative stress in the cells. It is also possible that the ALS subtype could change over the course of the disease.

Men get sick, for example 1.2 times more common of ALS than women. The breakdown of the molecular Processes also showed significant differences between genders. The subtypes appear to occur equally frequently in both, although the researchers found a larger number of altered ones in men Gene products firmly. This could mean that men and women will have to be treated differently in the future. Through the multi-omics analysis a signaling pathway identified, which could be a particularly good target for new drugs against the disease. This signaling pathway, MAPK, has been well researched and plays a role in various processes in ALS. The researchers are certain that an already approved cancer drug that acts on MAPK can also be used against ALS. Further steps should now be taken to find a way to determine the ALS subtype in patients. That would make it Causes track down the disease and thus develop more targeted therapies.

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Source: German