Executive Function Deficits,Attention-Deficit/Hyperactivity Disorder,Tics,and Obsessive-Compulsive Disorder in an Adolescent

Chief Complaint and Presenting Problem

R ., a 13-year-old boy, was referred by his home school district for a diagnostic evaluation. R. had been suspended from school for the last half of eighth grade due to aggressive behavior.

History of Present Illness

R.'s parents noted behavioral difficulties when R. was as young as 18 months of age. Mother reported she had a feeling that “something was not quite right” due to R.'s physical aggressiveness. One example of this was when R. threw a shoe at his mother while sitting in his car seat. Mother also reported that R. had several subsequent episodes of loss of behavioral control that included throwing a plant at her and biting his brother. During preschool R. was also difficult to manage.

R. began to develop repetitive behaviors at age 3 years such as lining up his cars and toys. He was initially evaluated by a psychologist who questioned a possible diagnosis of autism. By age 3 1/2 years, R. had developed motor tics such as eye blinking, squinting, eye rolling, nose twitching, nostril flaring, and more complex tics such as kicking. At age 5 years, R. was evaluated by a neurologist who diagnosed Tourette's disorder (TD) and attention-deficit/hyperactivity disorder (ADHD). Some obsessive-compulsive features were also noted. R. was subsequently referred to a specialty clinic, where a workup including MRI and EEG were within normal limits.

R.'s problems with aggressive behavior continued to escalate into his middle-school years. Loss of control was precipitated by a variety of innocuous things such as being asked to stop playing a video game. The school's decision to place R. out of school was reportedly made after several incidents of escalating aggressive behavior. R. reportedly hit a boy with a baseball during recess, got into fights with several classmates, and hit a teacher in the arm after hearing a remark that upset him.

Father describes R.'s aggressive outbursts as “like a seizure”; the outbursts occur approximately 2–5 times per day and last 15 minutes on average, with the longest episode lasting 45 minutes. Most often, the episodes resolve on their own if R. is left to calm down. R. is described as very remorseful after these episodes. Parents say that they are afraid to bring R. anywhere right now due to these rages. The outbursts have occurred in school, at home, and when R. plays sports but have not happened while with friends.

In terms of ADHD symptoms, the parents describe R. over the years as quite fidgety, restless, and very impulsive. The parents worried that R. would run out into traffic when he was younger. He continues to be restless and hyperactive. Although he is able to focus on topics that he likes, he has walked out of the classroom when he decides that a topic is “boring.” He has not been able to sit through classes and has been permitted to leave as needed accompanied by a paraprofessional.

With regard to obsessive-compulsive symptoms, R. continues to line up his animals around his bed. He has a history of worries about bad things happening to his brother, and he asks if he will die if he touches something and puts it in his mouth. He has requested that the family have a home security system at night so he can feel less worried about invaders. He is anxious and tense as well as generally concerned about contamination.

R. has talked about killing himself at times, not necessarily associated temporally with the explosive episodes. He feels he is a “burden to his parents” and says, “I don't like to live this way.” R. tried to choke himself last year and has had episodes of trying to hold his breath long enough to pass out. However, R. has not described periods of depressed mood or loss of interest lasting more than a few minutes up to a few hours. He has had no vegetative signs such as loss of appetite or change in sleeping habits.

Past Psychiatric Treatment History

R. has been evaluated in the past by several clinicians including psychiatrists and psychologists. One of the first interventions was a parent management program when R. was approximately six years of age. His parents also worked with a social worker and a psychologist at the therapeutic school program where R. was enrolled. R. had a therapist at school but, since returning to public school in Grade 6, is currently not in therapy. Parents report that no interventions thus far have been helpful in addressing R.'s aggressive behaviors.

Developmental History

R. was the product of an uncomplicated 40-week pregnancy, labor, and delivery. Apgar scores were described as excellent. There were no neonatal problems except that it was difficult for R. to sleep through the night. Developmental milestones were achieved within normal limits. Parents reported that although R. developed language as expected, it was hard to understand what he said when he spoke, as his speech articulation was not clear. As a result, R. received speech therapy from ages 5–8 years within the elementary school setting.

Educational History

R. attended public elementary school from kindergarten through third grade. He was asked to leave in the fourth grade due to aggressive behavior. R. transferred to a therapeutic school for the remainder of fourth grade and all of fifth and sixth grades. The structured behavioral approach for anger management was helpful. R. was taught a number of skill-based interventions including how to identify his feelings and relaxation techniques. However, since the focus was therapeutic, it seems that R. did not learn sufficient academic skills.

R. returned to a public, mainstream middle school for seventh grade. He did well academically and behaviorally until the end of the year when his aggressive behaviors increased, and he was suspended five times. The start of eighth grade was very positive, in that R. was making honor roll, playing on the soccer team, and had a girlfriend. A paraprofessional was assigned to R. during seventh and eighth grade, but no other individualized educational accommodations or related remedial services were implemented. The personality match with the paraprofessional was reportedly not a good one, and problems developed after the first marking period. R. would walk out of class when he felt frustrated. School work was becoming more difficult, and it was hard for R. to concentrate. Despite changing to a different paraprofessional, R.'s outbursts increased at home and at school.

The decision to place R. out of school was made after several incidents of escalating aggressive behavior. R. currently receives in-home instruction provided by the school district since he is not attending school.

Social History

R. is described as having longstanding friends who have maintained support of him despite his behavioral difficulties. He has a best friend and a girlfriend. R. participates in athletic activities including soccer, basketball, and baseball, and enjoys a variety of age-appropriate activities including video games and skateboarding. R. is described as respectful and not rageful for the most part.

Family History

R. lives at home with his parents and younger brother. Mother, age 43, earned a bachelor's degree and works as a physical education teacher. Father, age 49, has a degree from a post high school technical program and is self-employed in the heating, ventilation, and air conditioning business. Father may have had some ADHD symptoms, but no diagnosis was made. R.'s brother is 10 years old and attends public school.

Maternal relatives have had difficulty with mood, although no formal diagnoses of depression were made. Maternal grandfather and maternal cousin had thyroid problems.

R.'s half brother, age 25, by the father's first marriage, has a history of eye blinking. A paternal uncle had symptoms of TD but was not diagnosed. Paternal grandfather and paternal cousin were described as depressed.

Medical History

R. has been a healthy child with no hospitalizations and no history of head injury or seizures. He had several streptococcal pharyngeal infections at the age of five years but none associated with tics or obsessive-compulsive disorder (OCD) symptoms. R. has a history of mild asthma in the past. He is allergic to erythromycin.

Medication History

R. is not currently on any medication. For several weeks in the few months prior to evaluation, R. was on risperidone 1.5 mg daily, but an increase in his behavioral difficulties, OCD symptoms, tics, and inappropriate urination were noted.

Past medication trials included clonidine, 0.1 mg twice a day (bid) at age 8 years, which led to nausea, stomachaches and lethargy. R. was subsequently treated with dextroamphetamine salts 10 mg, which also resulted in stomachaches and headaches. At age 9 years, a trial of fluoxetine 20 mg, led to behavioral activation. A prior trial of risperidone, 0.5 mg, at age 9 years, led to increased OCD symptoms, tics, and behavioral problems. Most recently, in the month prior to evaluation, treatment with aripiprazole 2.5 mg, led to behavioral activation. Topiramate 25 mg added to aripiprazole was not helpful.

Mental Status Examination

R. is a small-for-age pre-pubertal boy. R. made good eye contact. He was very restless, moving about in his chair, and accidentally hit the chair against the wall. R. spoke quickly with mild disarticulation. Of note were multiple tics, 1–2+ frequency and intensity, including facial movements, eye blinks, shoulder shrugs, and occasional low-pitched guttural sounds.

R. showed no evidence of thought disorder and no evidence of current suicidal or homicidal suicidal ideation. He spoke of sports, particularly soccer, which he enjoys. He described his attachment to his younger brother and his “protectiveness” of him. When asked directly, R. acknowledged significant worries about something bad happening to his brother, to his parents, and to himself, especially at night. He reports that he worries about burglars and fires at night, and he has an escape plan for both.

R. showed a range of affect and a good sense of humor. There was limited insight into his current difficulties and limited judgment.

Behavioral Observations During Neuropsychological Testing

R. provided his best effort to accommodate the requests of the examiner over three sessions; he worked for lengthy periods of time and completed all tasks. There were brief instances in which he became frustrated and hit himself in the head, broke pencils, or repeatedly said curse words. However, he easily shifted his focus and transitioned to other tasks. One task session required R. to copy a complex design. Since drawing is a skill that is challenging for R., he quickly became frustrated; he initially perseverated by tracing over lines, but he then started to rip up his paper. He was unable to transition to another task so a break was suggested. R. ran out of the room for about 30 minutes, as he had significant trouble calming down. After a break, R. successfully completed the remaining tasks. He was sincerely apologetic for becoming upset. Together, behavioral observations highlighted that he was a hard-working teen who was motivated to do well. His ability to keep his thinking and focus on line and to effectively complete tasks was impacted by low frustration tolerance, perfectionistic qualities that led to frustration, and difficulty controlling his behavior.

Summary of Neuropsychological Assessments

Past psychometric testing when R. was 8 years old reflected a statistically significant discrepancy between his high average Verbal IQ and his low average Performance IQ on the Wechsler Intelligence Scale for Children-Third Edition (WISC-III). Results of a Wechsler Intelligence Scale for Children-Fourth Edition (WISC-IV) administered at age 12 reportedly indicated that the discrepancy between his verbal comprehension and perceptual reasoning had narrowed.

The current assessment highlighted consistency in R.'s abilities to use verbal and non-verbal reasoning skills to solve problems (WISC-IV, Verbal Comprehension = 106, Perceptual Reasoning = 104). He demonstrated strength in his facility with language, and he easily processed and mentally manipulated information that he heard (WISC-IV, Working Memory = 104).

R.'s overall performance on measures of academic achievement reflected well-developed basic skills in reading and math. However, he had difficulty with computations that required using paper and pencil to write down each step. His good reasoning skills were evident when he was reading for meaning or when he solved functional math problems. He struggled with the motor precision and control required for handwriting. In addition, he struggled to keep his thinking on track and to maintain and sustain his attention. He verbalized his analysis and his plan to solve problems or answer questions in a specific way but then was unable to carry out the plan. In several instances it was his lack of ability to write the results of each step that hindered his ability to complete a problem. The latter was a great source of frustration for R.

Specific areas of weakness on neuropsychological measures corroborated R.'s difficulties on academically based tasks, and were noted in the areas of fine motor functioning, executive functioning skills, and frustration tolerance. His difficulties with fine motor precision and speed truly made handwriting and drawing challenging and arduous for him.

Although R. demonstrated the ability to think and work quickly, performances on measures of executive functioning highlighted difficulties with keeping his thinking on track, with controlling his impulses, and with planning ahead. When he becomes frustrated, it is even more challenging for him to control impulsive behavior. A significant strength, as frequently noted during testing, is his self-awareness and his ability to independently correct his mistakes or apologize for misbehavior.

Case Formulation

In summary, R. is a 13-year-old boy who meets criteria for moderate TD and ADHD. Emerging are more significant features of OCD including aggressive and contamination obsessions. His explosive outbursts should be viewed in this context. Approximately 25% of youth with TD referred to a specialty clinic have explosive outbursts; those with the triad of TD, ADHD, and OCD are more at risk for the development of explosive outbursts than those with TD without the comorbid disorders (Budman et al. 2008). From the psychopharmacological perspective, R. has historically reportedly had a poor response to first-line interventions for his TD and has had a possible episode of activation on fluoxetine for OCD.

From the neuropsychological perspective, R. has the intellectual capability to progress as expected behaviorally, socially, and academically. However, his success has been impacted by significant difficulties with impulse and behavioral control, and low frustration tolerance. Results of the neuropsychological assessment suggest that R. has deficits in executive functions that have profound impact on his adjustment and learning, including management of attention and behavior control, impulse control, and coordination of motor planning. Reduced reading speed and difficulty with drawing and handwriting further impede his academic functioning. As a result, many academic and social experiences are highly frustrating for him and are exacerbated by poor ability to control his responses when emotionally aroused.

From the biological perspective, family history is very significant for diatheses for tic, mood, and anxiety disorders and ADHD.

Medical history contributes asthma, currently in remission. Most likely, the inappropriate urination mentioned in the medication history was an adverse effect of the risperidone.

R. has a number of strengths including solid cognitive abilities, excellent athletic ability, a sense of humor, and attachment to his brother to whom he is very protective. R. has had the benefit of resourceful and devoted parents who are seeking to optimize his care.

Multi-Axial Diagnoses

Discussion

R. is a complicated young adolescent whose symptoms and course have been challenging to manage. His clinical picture cannot be captured comprehensively by a multi-axial diagnostic system alone, as the neuropsychological deficits are playing an important role in his difficulty in attaining appropriate academic, social, and emotional functioning. His course has been further complicated by a lack of response or inability to tolerate multiple first-line psychopharmacological agents for tics, ADHD, and OCD. Unfortunately, his co-morbid picture of this triad coupled with the neuropsychological deficits may render him less likely to respond to first-line agents for each disorder. In addition, there are no systematic algorithms available to guide treatment in complex cases such as these to guide clinical practice.

It is clear that R. is a candidate for an individualized treatment plan of targeted combined therapy tailored to his specific needs. The first task is to create a hierarchy of symptom clusters/diagnostic categories to target for intervention. The explosive outbursts that led to his removal from school are the target symptom of highest priority, at the interface of his impulsivity, compulsivity, and obsessionality, reflecting the underlying core problems of disinhibition and executive functions deficits. Prefrontal cortex dysfunction, serving as the template for R.'s neuropsychiatric symptoms, regulates behavior and attentional functioning. Given R.'s poor response to medication in the past, but lack of a methylphenidate-based stimulant trial, he is a candidate for a cautious trial of a methylphenidate-based stimulant.

Low-dose methylphenidate produces increased norepinephrine (NE) and dopamine (DA) release in the prefrontal cortex. In animal studies, moderate levels of catecholamines improve prefrontal function through alpha 2 adrenergic and D1 receptors; high levels impair prefrontal function (Arnsten 2006). Administration of low-dose methylphenidate to rats improves prefrontal cortex functioning mediated through improved attentional functioning and working memory. Interestingly, high doses impair working memory. Human studies of stimulant medication show improvements on some neuropsychological function; Barnett et al. (2001) studied effects of stimulant on spatial memory and spatial working memory and found that medication-naïve children had deficits in executive functions related to working memory compared to a group treated with stimulants. Working spatial memory in those treated with stimulants could not be differentiated from those of controls (Barnett et al. 2001). In a study of stimulant effects on neuropsychological functioning in young adults ages 15–25 year with ADHD, the ADHD group who had not received stimulants for a month performed more poorly on total aggregate scores, working memory, interference control, processing speed, sustained attention, and verbal learning domains compared to those on active stimulant treatment. However, the active stimulant group scored more poorly on total aggregate, interference control, and processing speeds compared to controls. The ADHD active stimulant group scored higher on domains of sustained attention and verbal learning compared to the ADHD no stimulant group. The authors concluded that ADHD youth treated with stimulants had higher neuropsychological measures of attention compared to ADHD subjects who were not taking medication, but differences were not found for other neuropsychological measures (Biederman et al. 2008).

Although group data do not suggest a significant increase in tics with the use of methylphenidate-based stimulants (Tourette Syndrome Study Group 2002; Gadow et al. 2007; Spencer et al. 1999), some children may experience a transient increase in tics on stimulants. One study suggested that methylphenidate-based stimulants are better tolerated than dextroamphetamine stimulants in children with ADHD and tics, and families prefer the methylphenidate group (Castellanos 1997). If tics should increase during a stimulant trial, R. could benefit from the addition of a tic suppressing medication such as guanfacine, which may also produce anxiolytic effects. The stimulant is likely to be beneficial in increasing his frustration tolerance, reducing his outbursts, and improving his attentional functioning. Taken together, any risk of increase in tics would be outweighed by the potential benefits of a stimulant.

To address the OCD, another selective serotonin reuptake inhibitor (SSRI) trial may be indicated in the future; however, given R.'s history of activation and the family history of mood disorder, one would need to be cautious. Given the significant obsessive-compulsive symptomatology, and the potential risks of activation on another agent, cognitive behavioral therapy to address the OCD might be considered first; habit reversal therapy could be added to address his tics.

From the educational perspective, R. will require carefully planned treatment with the goal of learning coping and executive functioning skills so that he can meet his full potential. It is important that the environments in which he interacts are supportive and provide guidance to help him to develop these skills. A specialized educational setting could be considered to integrate educational, therapeutic, psychopharmacological, and family interventions. Comprehensive support for his family and his teachers is required so that everyone who is involved with R. has the patience and skill to guide him as he struggles to build necessary skills.

Disclosures

Ms. Schwartz and Dr. Sharma have no conflicts of interest or financial ties to disclose. Dr. Coffey has received research support from Eli Lilly Pharmaceutical, NIMH, NINDS, Tourette Syndrome Association, Bristol-Myers Squibb, and Boehringer Ingelheim.