Emma Horton is a little girl with a big personality.
“She lights up a room when she goes into it,” Cheryl Horton, Emma’s mom, said.
Even though the seven-year-old can’t talk –she can communicate with her iPad.
“My favorite movie is Frozen,” Emma said.
Emma has dystonia, a condition that causes her muscles to contract and spasm. Even the simplest tasks are a challenge.
“Just little things that you take for granted that a 7-year-old should be able to do, Emma was never able to do it,” Colby Horton, Emma’s dad, said.
But today Emma is doing better than ever – thanks to deep brain stimulation, DBS for short.
“We’ve been very pleasantly surprised by her,” John Honeycutt, MD, Neurosurgeon, Cook Children’s Medical Center, said.
Here’s how it works: electrodes are placed in the brain and wires connect them to batteries implanted in the chest. The device sends electrical pulses to affected parts of the brain – and “resets” them. The best part is Emma was able to have anesthesia and be asleep during the surgery.
“We used to keep the kids awake. They don’t have to be awake anymore,” Dr. Honeycutt said. “We saw immediately some of that undesired movement stop.”
Also, three months after DBS, Emma rode her bike for the first time.
Dr. Honeycutt says it typically takes six months or longer for patients with dystonia to see very good results after DBS. Emma is expected to improve even more. Although DBS is a promising treatment, it isn’t a cure. If patients have their device removed – their symptoms will likely return.
BACKGROUND: Dystonia is a movement disorder characterized by involuntary contractions and spasms of muscles. These actions force the body into repetitive, often twisting, movements and awkward, irregular postures. Dystonia, which may affect a single body area or be generalized through multiple muscle groups, affects men, women, and children of all ages and backgrounds. (Source: http://www.mayoclinic.com)
SYMPTOMS: Dystonia typically develops in a slow and gradual fashion, with mild symptoms. It affects muscles that may be controlled voluntarily in normal instance; it does not affect smooth muscle, as is found in the heart and bladder. Patients may begin to experience cramps, jerky or spasmodic muscle actions and loss of control of parts or areas of their body. These may grow more severe and result in the distinctive twisting and awkward postures that most people associate with this condition. (Source: www.cedars-sinai.edu)
TREATMENT: Dystonia symptoms may be managed or alleviated by medication, Botulinum Toxin therapy, or surgery. Treatment has improved in recent years, due to successes with botulinum toxin (Botox, Myobloc) injections. Some forms of early-onset dystonia respond to levodopa and carbidopa (Parcopa, Sinemet) — a medication combination that increases brain dopamine, a neurotransmitter involved with muscle movement. Surgery is considered only in certain types of dystonia and when other treatments have not worked. With certain types of dystonia, surgeons can sever or remove the nerves controlling the contracted muscle. This may be done for eyelid dystonia (blepharospasm) or neck (cervical) dystonia. (Source: http://www.mayoclinic.com, www.cedars-sinai.edu)
NEW TECHNOLOGY: Dystonia may also be treated with a range of surgical options, specifically deep brain stimulation (DBS). In DBS, leads are implanted deep in the brain and electrical stimulation is targeted at key sites to try to control shaking, stiffness and loss of muscle control. To modulate the effect of the treatment, doctors can adjust the frequency and intensity of electrical pulses. Risks include infection, stroke-like problems, such as weakness or paralysis, and possible speech difficulties. Now, doctors can perform the surgery to implant the stimulator when the patient is asleep. (Source: http://www.mayoclinic.com, Dr. John Honeycutt)
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