Pediatric Annals

Firm Rounds 

A 7-year-old Boy with Behavioral Problems

Robert Listernick, MD

Abstract

This 7-year-old boy was in his usual state of good health until approximately 1 year prior to admission when he started having difficulty controlling his behavior in school. He had outbursts of aggressive behavior towards his peers. In addition, he apparently had trouble concentrating on his work and sitting still. As such, he was diagnosed as having attention-deficit/hyperactivity disorder (ADHD) and was started on Focalin (dexmethylphenidate), which had little effect. After 2 months, he was switched to Adderall (amphetamines). His parents felt that he was “not himself” while taking the medications and that he acted like a “zombie.” Over the past several weeks, he started having trouble with balance and difficulty climbing stairs. He often fell off his chair. Most recently, he started to drool and began having slurred speech and poor handwriting. The family began to have difficulty understanding his speech.

Key Learning Points

  1. As many as 70% of children who have attention-deficit/hyperactivity (ADHD) have a positive response to the stimulant class of medications, such as methylphenidate. An additional 20% respond to the alternative stimulant class of medications.
  2. The combination of behavioral changes and spasticity suggests bihemispheric white matter disease.
  3. X-linked adrenoleukodystrophy (XALD) is the result of disordered peroxisomal oxidation leading to the accumulation of very long chain fatty acids.
  4. Cerebral inflammatory XALD is the most common childhood form of XALD. The age of onset is between 4 and 8 years and starts with behavioral/academic concerns before the onset of overt neurologic disease.
  5. Approximately 70% of boys with XALD develop adrenal insufficiency and require cortisol supplementation.

ABOUT THE AUTHOR

Dr. Listernick is Professor of Pediatrics at Feinberg School of Medicine, Northwestern University, and Director of the Diagnostic and Consultation Service, Division of General Academic Pediatrics, Children’s Memorial Hospital, Chicago, IL.

Abstract

This 7-year-old boy was in his usual state of good health until approximately 1 year prior to admission when he started having difficulty controlling his behavior in school. He had outbursts of aggressive behavior towards his peers. In addition, he apparently had trouble concentrating on his work and sitting still. As such, he was diagnosed as having attention-deficit/hyperactivity disorder (ADHD) and was started on Focalin (dexmethylphenidate), which had little effect. After 2 months, he was switched to Adderall (amphetamines). His parents felt that he was “not himself” while taking the medications and that he acted like a “zombie.” Over the past several weeks, he started having trouble with balance and difficulty climbing stairs. He often fell off his chair. Most recently, he started to drool and began having slurred speech and poor handwriting. The family began to have difficulty understanding his speech.

Key Learning Points

  1. As many as 70% of children who have attention-deficit/hyperactivity (ADHD) have a positive response to the stimulant class of medications, such as methylphenidate. An additional 20% respond to the alternative stimulant class of medications.
  2. The combination of behavioral changes and spasticity suggests bihemispheric white matter disease.
  3. X-linked adrenoleukodystrophy (XALD) is the result of disordered peroxisomal oxidation leading to the accumulation of very long chain fatty acids.
  4. Cerebral inflammatory XALD is the most common childhood form of XALD. The age of onset is between 4 and 8 years and starts with behavioral/academic concerns before the onset of overt neurologic disease.
  5. Approximately 70% of boys with XALD develop adrenal insufficiency and require cortisol supplementation.

ABOUT THE AUTHOR

Dr. Listernick is Professor of Pediatrics at Feinberg School of Medicine, Northwestern University, and Director of the Diagnostic and Consultation Service, Division of General Academic Pediatrics, Children’s Memorial Hospital, Chicago, IL.

This 7-year-old boy was in his usual state of good health until approximately 1 year prior to admission when he started having difficulty controlling his behavior in school. He had outbursts of aggressive behavior towards his peers. In addition, he apparently had trouble concentrating on his work and sitting still. As such, he was diagnosed as having attention-deficit/hyperactivity disorder (ADHD) and was started on Focalin (dexmethylphenidate), which had little effect. After 2 months, he was switched to Adderall (amphetamines). His parents felt that he was “not himself” while taking the medications and that he acted like a “zombie.” Over the past several weeks, he started having trouble with balance and difficulty climbing stairs. He often fell off his chair. Most recently, he started to drool and began having slurred speech and poor handwriting. The family began to have difficulty understanding his speech.

Key Learning Points

  1. As many as 70% of children who have attention-deficit/hyperactivity (ADHD) have a positive response to the stimulant class of medications, such as methylphenidate. An additional 20% respond to the alternative stimulant class of medications.
  2. The combination of behavioral changes and spasticity suggests bihemispheric white matter disease.
  3. X-linked adrenoleukodystrophy (XALD) is the result of disordered peroxisomal oxidation leading to the accumulation of very long chain fatty acids.
  4. Cerebral inflammatory XALD is the most common childhood form of XALD. The age of onset is between 4 and 8 years and starts with behavioral/academic concerns before the onset of overt neurologic disease.
  5. Approximately 70% of boys with XALD develop adrenal insufficiency and require cortisol supplementation.

ABOUT THE AUTHOR

Dr. Listernick is Professor of Pediatrics at Feinberg School of Medicine, Northwestern University, and Director of the Diagnostic and Consultation Service, Division of General Academic Pediatrics, Children’s Memorial Hospital, Chicago, IL.

10.3928/00904481-20090201-04

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