Two-year-old girl becomes the youngest ever to ever receive deep brain stimulation



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A girl from Scotland became the youngest patient in the world to have undergone revolutionary brain surgery at the age of two and a half years.

Viktoria Kaftanikaite had deep brain stimulation (DBS), which sends electrical impulses through the brain to repair abnormal nerve signals.

A diagnosis of dystonia was diagnosed shortly before his operation. A pathological condition would send her into uncontrollable spasms and let her scream with unbearable pain.

Her parents, Patrycja Majewska and Martinas Kaftanikaite, said that they felt powerless to want to take care of their daughter, who was having trouble eating and breathing.

The doctors said they had operated on Viktoria to save his life because, in a very small number of cases, dystonia can be fatal.

They were extremely cautious, but everything "worked like clockwork", offering promising results for future patients with movement disorders.

After a four-hour operation at Evelina Hospital in London in May, Viktoria is recovering in intensive care in her hometown, Glasgow.

Viktoria Kaftanikaite became the youngest person in the world to have undergone revolutionary brain surgery at the age of two and a half years. On the picture with his mother after

Viktoria Kaftanikaite became the youngest person in the world to have undergone revolutionary brain surgery at the age of two and a half years. On the picture with his mother after

Viktoria was the youngest patient to have undergone deep brain stimulation (DBS), which sends electrical impulses into the brain to correct abnormal communication. In the photo, during the surgery

Viktoria was the youngest patient to have undergone deep brain stimulation (DBS), which sends electrical impulses into the brain to correct abnormal communication. In the photo, during the surgery

Viktoria's muscles became extremely painful, leaving her screaming in unbearable pain. She spent five months in intensive care at the Royal Children's Hospital in Glasgow (photo), while the doctors were trying to understand why.

Viktoria's muscles became extremely painful, leaving her screaming in unbearable pain. She spent five months in intensive care at the Royal Children's Hospital in Glasgow (photo), while the doctors were trying to understand why.

According to the Dystonia Society, PCS can reduce symptoms by up to 80%. However, one in five patients does not receive much relief.

Ideally, the CPS should be offered to the children as soon as possible because its effects will subside longer than a patient is living with dystonia.

But before Viktoria, the youngest person to receive DBS treatment was a three-year-old boy, as surgeons gradually used treatment for young people.

Dr. Jean-Pierre Lin, a consultant pediatric neurologist who coordinated the treatment of Viktoria, told the Guardian that the operation on Viktoria had "crossed the wall of sound" with regard to age.

According to the Dystonia Society, about 70,000 people in the UK are suffering from dystonia and may be caused by other factors, such as cerebral palsy or stroke.

It took two years before Viktoria's dystonia was diagnosed and the doctors discovered that it was due to a rare mutation in the GNA01 gene.

The most severe form of dystonia has a death rate of 10%, and Dr. Lin said that Viktoria would have died without surgery.

Viktoria's arms, legs, eyes and mouth contracted constantly, which prevented her from eating and also impaired her ability to breathe on her own.

Her muscles became extremely painful, leaving her screaming with unbearable pain.

Viktoria's parents, Patrycja Majewska and Martinas Kaftanikaite, said that they felt helpless in wanting to take care of their daughter. In the photo after the surgery

Viktoria's parents, Patrycja Majewska and Martinas Kaftanikaite, said that they felt helpless in wanting to take care of their daughter. In the photo after the surgery

The doctors said they had operated on Viktoria to save his life. She has trouble eating or breathing without help because of constant contractions of her mouth and arms

The doctors said they had operated on Viktoria to save his life. She has trouble eating or breathing without help because of constant contractions of her mouth and arms

DBS involves implanting very fine wires with electrodes at their ends into the brain by piercing two 5p holes in the top of the skull. In the photo, the current operation

DBS involves implanting very fine wires with electrodes at their ends into the brain by piercing two 5p holes in the top of the skull. In the photo, the current operation

The four-hour operation was held at Evelina Hospital in London in May.

The four-hour operation was held at Evelina Hospital in London in May.

Ms Majewska said: "Viktoria had uncontrollable movements all the time. Her arms and legs would not move normally and she lowered her head and belly in the air.

WHAT IS DYSTONIA?

Dystonia is the name of uncontrolled and sometimes painful muscle spasms.

The symptoms vary from one patient to another, but more often than not, a part of the body, or several, tremble, contract or twist.

It is thought that dystonia, which exists under different types, is due to a problem with the part of the brain that controls the movements. The cause is often unknown, but sometimes it can be due to inherited genetic problems, Parkinson's disease, stroke, cerebral palsy, or multiple sclerosis.

Dystonia that begins in adulthood is usually focused on one part of the body. If dystonia begins in childhood, it tends to spread to many parts of the body.

It's usually a problem that lasts a lifetime, but a treatment can help relieve the symptoms.

It is estimated that this will affect at least 70,000 people in the UK and up to 300,000 people in North America.

However, as many cases are not diagnosed, it is difficult to determine the actual number.

"We noticed that from an early age she could not hold anything in her hands, like toys, and had trouble keeping her head up because she was falling from one side to the other. "

Ms. Majewska and her partner, Mr. Kaftanikaite, struggled to take care of their daughter while the doctors were trying to figure out what was wrong with her during a five-month stay in care at the Royal Children's Hospital in Glasgow.

For the vast majority of people, dystonia does not reduce life expectancy and is not fatal. But it can cause secondary complications that can be life threatening.

Shortly after her diagnosis, at two years and eight months, Viktoria and her family were flown to London where they waited for the procedure.

The DBS, which can be offered to NHS patients if other treatments do not work, involves implanting very fine wires with electrodes at their ends into the brain by drilling two holes the size of a 5p into the upper part of the skull.

The leads are connected to extensions under the skin behind the ear and in the neck, which then connect to a pulse generator.

This allows electrical impulses to be sent to the brain, regulating the abnormal communications responsible for the symptoms of dystonia.

Ms. Majewska said, "She could not do anything so far."

Viktoria is already seeing improvements. In the picture with his father since the operation

Viktoria is already seeing improvements. In the picture with his father since the operation

Dr. Jean-Pierre Lin, pediatric consultant neurologist who coordinated the treatment of Viktoria, said the operation "worked like a clock".

Dr. Jean-Pierre Lin, pediatric consultant neurologist who coordinated the treatment of Viktoria, said the operation "worked like a clock".

Viktoria's chances of getting rid of various disabling and painful movements have been greatly improved.

Dr. Lin said the doctors now knew that they could safely administer DBS at the age of two and a half years, which is promising for the future.

Young children with cerebral palsy, born premature or have had a birth injury now have better hopes of being treated with a CPS.

Dr. Lin stated that they had been offering DBS to a growing number of young children in the last 15 years and that they had seen a five-year-old boy with cerebral palsy walk completely unaided after having had a DBS.

"The case of Viktoria is exciting and potentially very important because it could offer an opportunity for children with early movement disorders to benefit from the CPS and have a better future," he said.

WHAT IS DEEP CEREBAL STIMULATION OF DYSTONIA?

Deep brain stimulation (DBS) helps control movement problems and is the main type of surgery used to treat Parkinson's disease. It is also approved to treat epilepsy, essential tremors, OCD and dystonia.

DBS is a relatively new procedure that uses electrical communication between two components implanted in the body.

There are two pieces of material:

  • A four-point electrode implanted in the brain.
  • A pulse generator, or neurostimulator, implanted under the skin, just below the clavicle or in the abdomen in the woman. It is programmable.

Both are connected with cables that pass under the skin.

When the generator is turned on, the electrodes deliver high frequency stimulation into the targeted area of ​​the brain, blocking the signals that cause the symptoms of dystonia.

Abnormal movements and postures and / or dystonic tremors can be alleviated as well as pain caused by dystonia.

DBS works most effectively for people who have genetic or idiopathic dystonia – when there is no known cause.

The treatment is for people with hereditary or idiopathic dystonia with severe generalized dystonia, cervical dystonia or dystonic tremor when other treatment options are not working well.

It is thought that the effectiveness of PCS decreases as the proportion of life experienced with dystonia increases.

Therefore, for children, the ideal would be that the DBS be administered early, preferably a few years after the onset of symptoms.

For hereditary and idiopathic dystonia, DBS on average reduces the severity of symptoms by just over 50%, according to Dystonia, but the experience varies widely.

About one in five patients receiving DBS gets very little benefit. At the other extreme, some people may experience a reduction in symptoms of 80% or more.

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