What is motor neuron disease ALS

What are motor neuron diseases?

Motor neuron diseases are diseases of the motor nervous system. The leading symptoms are progressive Muscle weakness ("Paresis") and Muscle wasting ("Atrophy") often with Muscle twitching ("Fasciculation") or one Muscle stiffness ("Spasticity").

Sensitive symptoms, i.e. a disorder of "feeling", are not typical. This is often important to differentiate it from other neurological diseases such as multiple sclerosis and a polyneuropathy.

Depending on the severity of the disease, damage to the motor nerve cells in the brain and spinal cord ('1st motor neuron') with resulting spastic paralysis and / or the motor nerve cell that pulls from the spinal cord to the muscles ('2nd motor neuron') can result come flaccid paralysis.

The following clinical pictures are counted among the motor neuron diseases:

  • Amyotrophic lateral sclerosis (ALS)
  • Spinal Muscular Atrophy (SMA)
  • Kennedy type spinobulbar muscular atrophy (SBMA)
  • Hereditary Spastic Paraplegia (HSP)
  • Post Polio Syndrome (PPS)

What are the typical symptoms of motor neuron disease?

The most common motor neuron disease is theAmyotrophic lateral sclerosis (ALS). The main symptoms of ALS are increasing muscle weakness in the arms and legs, and difficulty speaking and swallowing. These symptoms often progress relatively quickly within a few years. The drug riluzole is therapeutically approved in Germany, and since 2017 there has been approval for the drug Edaravone in the USA, Switzerland and Korea (not in the EU). There are also a variety of therapy options to alleviate individual symptoms of the disease, such as muscle cramps or increased salivation. New therapies are currently being investigated in clinical studies. The care of patients with ALS should be carried out in a multidisciplinary team with the involvement of a special outpatient department.

Thespinal muscular atrophy (SMA)is a hereditary motor neuron disease that often begins in early childhood. However, there are also forms that only lead to the first symptoms in early adulthood. This leads to progressive flaccid muscle weakness in the arms and legs. With the classic form of SMA (deletion of the SMN1 gene) there is now the possibility of treating the disease causally with specific drugs with the hope of slowing down the progression of the disease. In addition, physiotherapy continues to play an important role.

How are motor neuron diseases diagnosed?

The diagnosis of motor neuron diseases requires good delineation from other diseases with a similar appearance. A careful clinical-neurological examination by an expert is particularly important. In addition, electrophysiological examinations such as electromyography (EMG) and neurography (ENG), imaging methods such as MRI and sonography, CSF examination and laboratory diagnostics including genetic examinations are used.

How are motor neuron diseases treated?

Therapeutic So far, there has only been a causal treatment for very few motor neuron diseases. Therefore, the reliable diagnosis is crucial. There are also numerous effective symptomatic therapies to alleviate symptoms such as pain, muscle cramps and restricted mobility. It is also important to recognize and treat nocturnal breathing disorders and swallowing disorders at an early stage. In addition to medication and physiotherapy / speech therapy, aids such as nocturnal home ventilation, feeding tubes, orthotics and robots are used. We also regularly offer participation in innovative new clinical studies.

The UMG Neurology Clinic offers complete diagnostics and all treatment modalities for all motor neuron diseases, including clinical studies, in its special outpatient department.

Support groups

ALS (ALS-mobil e.V., Berlin)
SMA (Initiative SMA, Langenau / Berlin)
DGM (German Society for Muscle Sick People)

Ice Bucket Challenge of Neurology (2014)

Appeal for donations for ALS research

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