It happens from one second to the next. Legs or arms are paralysed for minutes – perhaps even several hours. The attacks come and go. But over the years, muscle atrophy often develops, making walking impossible. Hypokalemic periodic paralysis (HypoPP) is the name of the disease, which only affects one person in a hundred thousand.
The Disease
Hypokalaemic periodic paralysis is inherited and often affects all four limbs simultaneously. Sometimes a carbohydrate-rich meal is enough to trigger the paralysing attacks. However, it can also happen with fear, cold, sport or alcohol consumption. Even if the paralysis passes, the patient’s condition worsens with age – so much so that sooner or later they are dependent on a wheelchair. For a long time, patients were helplessly at the mercy of their fate. But then a team of researchers made a groundbreaking discovery
The Research
Privatdozentin Dr Karin Jurkat-Rott from Ulm University Hospital and Prof Dr Marc-André Weber from Rostock University Hospital discovered that muscle paralysis is triggered by a low concentration of potassium in the blood. They also discovered sodium and water retention through MRI examinations. In order to dehydrate the tissue and increase the potassium in the blood, the scientists administered a drug that is actually used to treat heart failure to two patients as part of an individualised treatment trial. The results could hardly have been more impressive: Both patients’ muscle strength improved so much that they were no longer dependent on a wheelchair. Karin Jurkat-Rott and Marc-André Weber received the Eva Luise Köhler Research Award 2010 for their findings, and the drug is now part of the standard therapy for patients with heart failure. The researchers used the award money from the foundation to finance a highly specialised MRI scanner that enables even more precise measurements in the muscle.
The Future
And even today – more than ten years later – the research continues. Prof. Dr Marc-André Weber is working together with the University of Erlangen to investigate the importance of potassium in muscle diseases even better – again using highly specialised MRI. Marc-André Weber: ‘We hope to gain new insights that will enable us to help people with other rare muscle diseases as specifically as possible.’
