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Deep brain stimulation at the MHH saves two sisters from a wheelchair
Nele (22) and Jette (18) R. are cheerful young women who go their own way with confidence. And they are sisters. But apart from, or perhaps because of, their close relationship, they have something else in common: both developed generalized dystonia in childhood. This is a rare, often genetic movement disorder. If left untreated, the condition eventually forces those affected into a wheelchair. But Nele and Jette were lucky. At the Department of Neurosurgery at Hannover Medical School (MHH), deep brain stimulation (DBS) saved them from this. During the surgical procedure, a neurostimulation system, a type of brain pacemaker, is implanted. For the sisters, this was more than ten years ago - but they still come to the MHH regularly and now have an almost friendly relationship with Clinic Director Professor Dr. Joachim Krauss.
Long search for help
Nele can hardly remember the beginnings of her illness. When the tension and internal rotation of her left foot first appeared, she was at elementary school. “I only remember taking part in PE lessons with crutches,” she says. Her mother's memories, on the other hand, are still very vivid. “It took a long time before anyone could really help us. The term 'dystonia' came up quickly, but we didn't get anywhere at first,” reports Michaela R. ”An orthopaedic surgeon even said we should accept the fact that our daughter would be dependent on a wheelchair.” A neurosurgeon finally put the family from near Bad Pyrmont in touch with MHH Neurosurgery. Its director, Professor Krauss, is regarded worldwide as one of the pioneers of deep brain stimulation. In 1997, he developed the surgical method for treating dystonia and then introduced it at the MHH in 2005.
First symptoms at primary school age
Generalized dystonia is also known as Oppenheim disease. The first years of life of those affected are usually quite normal until the first symptoms appear sometime between primary school and adolescence. At first, the involuntary muscle contractions are only noticeable intermittently and in one part of the body, but later they lead to permanent twisting and incorrect posture in the limbs, trunk and neck. The ability to speak and swallow can also be impaired. “The cause of these symptoms lies in the brain, leading to a malfunction of the basal ganglia, which are located deep in the cerebrum. In dystonia, neuronal activity in the so-called globus pallidus internus, which is involved in the control of movement sequences, is particularly disturbed,” explains Professor Krauss.
Electrical stimuli correct brain function
When Nele came to Professor Krauss in 2011, he had long since established deep brain stimulation at the MHH, but the procedure was still quite new in children - Nele was nine years old at the time. “We now know that DBS works just as well in children as it does in adults, and tends to work even better,” emphasizes Professor Krauss. It is generally a good idea to carry out the procedure as early as possible in the course of the disease. During the operation, the neurosurgeons implant a neurostimulation system with a battery that can be recharged through the skin. They place thin electrodes with millimeter precision at precisely localized positions in the globus pallidus internus and also at other target points in the brain. The brain function is modulated by constant weak electrical stimuli, whereby neuroplastic phenomena are also relevant in the case of dystonia. The pacemaker is implanted under the skin below the collarbone and connected to the electrodes. The neurosurgical team at the MHH has already performed more than 200 DBS for generalized dystonia. The procedure is also used for Parkinson's disease, epilepsy, pain syndromes, obsessive-compulsive disorder and essential tremor. The clinic works closely with the neurology and psychiatry departments at the MHH.
Same symptoms as the sister
After the implantation of the neurostimulation system in 2012, Nele's symptoms subsided within a few weeks.“ Fortunately, the deep brain stimulation took place before her ankle stiffened,” reports her mother Michaela with relief. When her younger daughter also began to show symptoms, she was forewarned. “We had also had genetic tests done in the meantime. After that, it was clear that the generalized dystonia was hereditary in both of them,” she says. The condition had never occurred in the family before.Jette showed the same symptoms as her older sister, but even earlier. That's why she was fitted with a brain pacemaker when she was seven. It took a little longer for her to get the device adjusted correctly. “Sometimes it's a matter of 0.1 volts. You need a little patience and have to observe the effect of the pacemaker for longer,” explains Professor Krauss.
Hip-hop and skiing
Once a year, the young women come to the MHH for a check-up. “We always combine this appointment with a nice day out for us,” reports Jette. “We go shopping or do something special in Hanover.” The whole family loves this ritual. Over the years, a friendly relationship has developed between the sisters and Professor Krauss as a result of the one- to two-hour appointments. Six years ago, Nele even did an internship with him in the neurosurgery department and was able to observe the deep brain stimulation procedure herself. She was interested in medicine and wanted to learn all about DBS. Professor Krauss is always up to date on the two former patients. He knows that Nele is now studying computer science instead of medicine and that Jette will be graduating from high school next year and would then like to study midwifery.And he is glad that movement disorders are no longer an issue for either of them: Jette loves to dance hip-hop and Nele does weight training, skiing and mountain biking.
SERVICE
For further information, please contact Professor Dr. Joachim Krauss, krauss.joachim@mh-hannover.de.
Have known each other for a long time: Jette, Professor Krauss and Nele (from left)
Copyright: Karin Kaiser/MHH
Professor Krauss looks at the image of a brain with Jette (left) and Nele. The electrodes of the neu ...
Copyright: Karin Kaiser/MHH
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Have known each other for a long time: Jette, Professor Krauss and Nele (from left)
Copyright: Karin Kaiser/MHH
Professor Krauss looks at the image of a brain with Jette (left) and Nele. The electrodes of the neu ...
Copyright: Karin Kaiser/MHH
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