What can a routine infection have to do with a child’s sudden outbursts and obsessiveness?

One day she repeated the word “puppy” over and over and then laughed hysterically. The next, she seemed afraid of her mother, whom she didn’t appear to recognize, and kept repeating the word “scared.”

The 5-year-old who arrived at the Norton Children’s Hospital emergency department in December was far different than the happy, healthy girl her parents had come to know.

A month earlier, it started with emotional outbursts.

One day she repeated the word “puppy” over and over and then laughed hysterically. The next, she seemed afraid of her mother, whom she didn’t appear to recognize, and kept repeating the word “scared.” In the subsequent days, outbursts became worse and more frequent — she’d bang her head and threaten to run away.

She had been diagnosed elsewhere as experiencing psychological issues. Her bloodwork and other labs were normal, and an electroencephalogram (EEG) revealed no unusual activity in her brain waves.

Some clues that could easily be overlooked

The girl’s family was healthy, and while the girl had been mostly healthy, in the previous year she’d had both a strep throat infection and a urinary tract infection.

That, and the sudden onset of the symptoms, were the clues that Vinay Puri, M.D., child neurologist with Norton Children’s Neuroscience Institute, affiliated with the UofL School of Medicine, needed.

“It is sometimes not the symptoms but the abruptness of symptoms or the quality of the symptoms that can point you in the right direction,” said Dr. Puri, a professor of neurology and pediatrics at the University of Louisville School of Medicine.

Dr. Puri suspected pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS). It is a type of pediatric acute-onset neuropsychiatric syndrome, an uncommon neurological disorder characterized by an abrupt onset of obsessive-compulsive behaviors or severely restricted food intake.

PANDAS also comes with at least two of the following symptoms:

  • Anxiety, depression or rapid extreme mood changes
  • Sudden school performance trouble
  • Motor or sensory abnormalities
  • Sleep issues including disturbed sleep, bed wetting or frequent need to urinate

The cause of PANDAS is not known, but it is thought to be triggered by infections, other inflammatory reactions or metabolic disturbances. Infection can result in antibodies attacking parts of a child’s brain. Dr. Puri estimated there are probably only five to 10 cases of PANDAS in Louisville each year.

Because of the behaviors associated with PANDAS, it can be misdiagnosed as obsessive-compulsive disorder or an eating disorder.

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Listening to parents

“It helps very much to listen to parents when they say, ‘This is a radical transformation. We don’t recognize this child,’” Dr. Puri said. “Children and parents can feel very misunderstood. They are scared and frustrated.”

Very mild cases of PANDAS can get better on their own. Treatments that address the immune system, such as steroids, IV immunoglobulin (IVIg) or plasmapheresis can help with symptoms. For aggression or agitation, PANDAS patients may benefit from medication such as guanfacine, which is used for attention deficit hyperactivity disorder, or stronger medications, according to Dr. Puri.

With the 5-year-old girl, Dr. Puri first put her on steroids. Her condition improved, but once weaned off the steroids, she got worse. Dr. Puri switched to monthly IVIg treatments.

“She is doing remarkably better now,” Dr. Puri said.