VCU news

By Joe Kuttenkuler

A deep brain stimulator that Virginia Commonwealth University Medical Center doctors implanted this summer in the brain of a 7-year old girl who has been wracked with disabling body spasms since infancy is giving her parents and physicians reason to be hopeful the therapy might work.

During a recent follow-up visit with neurosurgeon, Kathryn Holloway, M.D., Lexi Haas showed small signs the stimulator is functioning as intended by disrupting the abnormal signals causing her erratic body movements and stiffened muscles – a condition called dystonia which is a movement disorder like torticollis, essential tremor, and Parkinson’s disease.

Lexi, who lives with her family in North Carolina, is unable to walk and speak but communicates with her parents and siblings through eye and tongue movements.

Since the stimulator was activated, Lexi’s muscles have relaxed and she has been able to perform simple movements like touching her head and face – even holding objects – something she wasn’t able to do before.

Holloway along with a team of doctors implanted the stimulator in July and activated it five weeks later.

The procedure is believed to be the first documented use of deep brain stimulation in a patient definitively diagnosed with kernicterus – a rare type of brain damage caused by untreated jaundice at birth. The resulting brain damage caused Lexi’s dystonia.

“This little girl has been trapped in her body for seven years. If we can provide some relief for her and enable her to live a more normal life, it will be tremendous,” said Steven Shapiro, M.D., a pediatric neurologist at the VCU Medical Center, who has been treating Lexi since 2004 and conducting kernicterus research for more than 25 years. “These initial signs are encouraging but she still has a long way to go.”

During the surgical procedure to implant the stimulator, Holloway and a team of neurologists guided tiny electrodes into Lexi’s brain to the site where the abnormal signals causing her stiffness and uncontrollable movements originate. During a second procedure, the electrodes were connected to a stimulator.

“The entire medical team has been inspired by Lexi's courage and the courage and love of her family and we were not going to quit until we have done the absolute best we can do for Lexi,” Holloway said. “The full benefit of the stimulator may not be realized for sometime and we’re all looking forward to seeing how she progresses over the next six months.”

Deep brain stimulation has been used for years with good success to treat Parkinson’s disease, dystonia and other genetic movement disorders. Doctors are unsure how Lexi will progress since her dystonia resulted from brain damage.

Jaundice results from a build up of bilirubin – a toxin created during the normal process of red blood cells breaking down and dying. Under normal circumstances, bilirubin is processed by the liver and eliminated. But because a newborn's liver is still immature, the enzyme that converts bilirubin to a non-toxic substance sometimes does not begin functioning immediately, which can cause bilirubin levels to rise in the body. Left untreated, very high levels of bilirubin – called hyperbilirubinemia – can damage parts of the brain resulting in kernicterus.  

A routine, inexpensive test for excessive jaundice is a standard practice in most newborn care.