Many neurological diseases lead to a weakening of the respiratory muscles. This is often experienced as breathlessness and functional impairment, or can lead to sleep disturbances and other symptoms.
By supporting breathing with ventilator therapy, the symptoms can often be alleviated and quality of life improved. This is illustrated in the following two examples of specific neuromuscular diseases:
Amyotrophic lateral sclerosis (ALS)
Amyotrophic lateral sclerosis (ALS) is a disease that causes muscle weakness due to the degeneration of nerve cells in the spinal cord and brain. Depending on the form of the disease, the arms, legs, throat muscles and predominantly the respiratory muscles can be affected. The disease typically progresses slowly but may also manifest sporadically.
Many symptoms such as muscle cramps and drooling as well as vocal and swallowing disorders can be alleviated by the appropriate treatment. A weakness of the respiratory muscles may become evident through decreased physical capacity, fatigue, headaches, shortness of breath (especially when lying down) and recurrent respiratory infections. Special lung function tests and studies are performed in the sleep laboratory for the early detection of respiratory muscle weakness. A ventilation mask aids respiration and can significantly improve breathing and quality of sleep as well as promote an improved physical condition.
Duchenne muscular dystrophy (DMD)
While ALS mostly affects elderly people, Duchenne muscular dystrophy (DMD) is usually diagnosed in childhood. DMD is a genetic disorder that only affects males (one in 3,300 male births), while females are carriers. The disease begins with difficulties in walking in early childhood, necessitates the use of a wheelchair at 10 to 14 years of age and requires full care and dependence on mechanical ventilation at 18 to 22 years of age. The Department of Pulmonology cares for a growing number of DMD patients, the majority of whom live and receive their education in the Mathilde Escher Home (MEH), Zurich. Thanks to optimal care and long-term ventilation, the life expectancy for those affected by DMD has increased significantly in recent years. In a widely acclaimed study, we showed that despite their physical disabilities, DMD patients perceive their quality of life as good and have a strong will to live.
Through optimal interdisciplinary care, complications such as pneumonia, life-threatening constipation with paralysis of the intestine (ileus) and heart failure can be detected and treated at an early stage.
The University Hospital Zurich Departments of Neurology and Pulmonology and the Zurich Children's Hospital (Kinderspital Zürich) have consolidated their expertise in a Neuromuscular Center to guarantee continuous, comprehensive diagnosis, treatment and support for children and adults with muscle and nerve disorders.
Furthermore, we collaborate closely with the Mathilde Escher Home in Zurich, which is specialized in caring for people with progressive muscle diseases.