Continuing Education Activity

Coprolalia comes from the greek "kopros," which means "dung, feces" and "lalein," which means "to babble." It's a tic-like occurrence that involves non-intentional obscene and socially inappropriate vocalizations. In 10 to 33% of cases, it may correlate with tic disorders, in particular with Tourette syndrome (also known as Gilles de la Tourette syndrome). Gilles the la Tourette is believed to occur in approximately 1% of the population worldwide, in the range between 0.4% to 3.8%. Coprolalia also occurs in patients with brain lesions, "senility" and in those with neurodegenerative and autoimmune disorders. It has can also occur in association with seizure disorders, in ictal or post-ictal status. This activity outlines the identification, evaluation, and management of coprolalia. It highlights the role of the interprofessional healthcare team in evaluating, treating, and improving care for patients with this condition.

Objectives:

• Summarize the etiology of patients with coprolalia.
• Identify the history associated with coprolalia and explain the most common complications of this condition.
• Outline the management considerations as well as the psychosocial considerations that healthcare professionals have to keep in mind when counseling patient's with coprolalia.
• Review the importance of interprofessional team members to improve outcomes for patients with coprolalia.

Introduction

Coprolalia comes from the greek "kopros," which means "dung, feces" and "lalein," which means "to babble." It's a tic-like occurrence that involves non-intentional obscene and socially inappropriate vocalizations. In 10-33% of cases, it may correlate with tic disorders, in particular with Tourette syndrome (also known as Gilles de la Tourette Syndrome). Gilles the la Tourette is believed to occur in approximately 1% of the population worldwide, in the range between 0.4% to 3.8%.[1]

Coprolalia also occurs in patients with brain lesions, "senility" and in those with neurodegenerative and autoimmune disorders. It can also appear in association with seizure disorders, in ictal or post-ictal status. 

There are also other vocal tic behaviors such as palilalia (involuntary repetition of words, phrases, or sentences), echolalia (repetition of another person's spoken words in a meaningless form), and klazomania (compulsive shouting) that can also be associated with coprolalia.[2]][3]

Coprolalia is the most common of the coprophenomena, which includes copropraxia (the urge to perform obscene gestures without control), mental coprolalia (obscenities thought obsessively), and coprographia (the urge to write down those expressions or obscenities).[3][4]

There is very little information about coprolalia in the absence of Tourette syndrome. Therefore, much of the information known about coprolalia is in the context of Tourette's and occasionally other tic disorders.

Etiology

The functional neuroanatomical basis of coprolalia is unclear. Among the different hypotheses to explain it, is the belief that the limbic circuit could have an important role in the pathophysiology of coprolalic behaviors.[2]

Other hypotheses have been constructed to explain the pathophysiology of Tourette's that describe the development of tic (whether motor or phonatory) and coprolalia. One theory is that the brain regions that are involved in Tourette, which are the limbic system and basal ganglia, are equivalent to those of regions involved in reproductive behavior/sex control in primitive animals. Therefore tics and coprolalia could result from dysfunction in these regions and appear as primitive vocal and motor fragments. 

Another hypothesis suggests that coprolalia is a part of the obsessive-compulsive disorder spectrum, which results from a failure to inhibit a part of the cortico-striato-thalamo-cortical pathway (which composes the basal ganglia).[3]

Epidemiology

The prevalence of coprolalia is unclear, and it ranges from very low to very high numbers in different series, but precise percentages are difficult to determine. Most studies have shown a higher male-to-female ratio.[3] The same higher male to female ratio is also present for patients with Tourette, whose pathognomonic sign is coprolalia.[5][6] In Japan, the number of reported cases in tertiary university centers ranges from 27% to 39% compared to primary care practices, whose prevalence approaches approximately 8%.[3] 

In terms of tics, which coprolalia is a part of, they start to arise between the ages of 4 to 6 years old and increase in severity when closer to pre-adolescence (10 to 12 years old). The rates are also higher in Whites compared to Hispanics and African-Americans.

History and Physical

Patients undergo evaluation mostly by a detailed history. 

Evaluation

The patient evaluation is through an interview with a detailed clinical history and assessment of the types of tic disorders. The non-controllable uttering may become apparent during the interview, as well.

For the diagnosis of Tourette syndrome, they must meet DSM-5 criteria, which are[7]:

• Having multiple motor tics and vocal tics (can not happen at the same time)
• Tics must be present for at least 1-year minimum, multiple times per day, and almost every day
• Tics began before the age of 18 years old
• Symptoms are not due to drugs or other medical conditions

Treatment / Management

In general, the decision to treat tics or coprolalia depends on the level of impairment and how much distress it may cause to the child, and requires the cooperation of the family. After education about the condition peer-reviewed, sometimes, the decision may be to monitor it clinically, without the need for active intervention, which can be non-pharmacological or pharmacological.

Non-Pharmacologic Intervention

Behavioral therapy has demonstrated success in helping the patient manage their compulsive behaviors and is thought to help patients with coprolalia.[3]

The treatment with the most robust empirical support that exists is habit reversal therapy (HRT), in which the patient can learn to recognize the signs that the urge is coming and become aware of it, as well as creating a response to these signs or the tic. Other behavioral treatments that have been found efficacious in clinical trials include comprehensive behavioral intervention for tics (CBIT), and exposure-response prevention (ERP).[8]

Pharmacologic Interventions

The pharmacologic treatment of coprolalia in itself is rarely addressed directly in the literature. It is almost always found together with the treatment of tics (whether motor or phonatory), obsessive-compulsive disorder, impulsivity, and other conditions, including Tourette syndrome. 

• Pimozide was found to decrease symptoms by 90% after one year of treatment in a series of 34 patients in which 50% had coprolalia, but coprolalia per se was not the addressed symptom.[3]
• Haloperidol was found to provide 79% improvement of symptoms when talking about tic frequency, but non-specific to coprolalia.
  • In these two medications, the side effects (e.g., extrapyramidal symptoms) have put a limit to their use as 1st-line treatment.
• Clonidine and clomipramine have been found to affect behavioral symptoms but again without specifically mentioning coprolalia.[3][9]
• Risperidone, and more recently, aripiprazole, is often chosen before other agents because of a safer profile and fewer side-effect. They have been approved by the Food and Drug Administration (FDA) for the treatment of tics.[10]
• In the cases of comorbid OCD, anxiety, or depressive disorders, it appears that children with tic disorders could benefit from selective serotonin reuptake inhibitors (SSRIs).[5]
• There have been attempts made with botulinum toxin injections in the vocal cords, but the benefits remain unclear.[11]

Differential Diagnosis

Coprolalia is most often associated with tic disorders, specifically Tourrette's, thus the healthcare provider is encouraged to identify the existence or absence of an underlying tic disorder. 

Other conditions that merit consideration in the differential diagnosis are Sydenham chorea and Hemiballismus. Both could have coprolalia involved but the motor tics and the ballistic/choreiform movements are pretty clear.[3] Seizures have also been noted to produce coprolalia. Ictal coprolalia associated with temporal or orbitofrontal epilepsy.[12]  Startle syndromes (latah, miryachit): more common in women, often secondary to trauma, and consist of startle myoclonus associated with echolalia, coprolalia, often uttering sexually-oriented words, may imitate people around them or things they see/hear, as well as automatic obedience.[3]  Another manifestation is klazomania, also known as compulsive shouting.[3]

Prognosis

Symptoms of coprolalia wax and wane over time and for no particular evident reason. A relationship with stress, whether emotional or physical, fatigue, caffeine, excitement (positive or negative), some medications, and hormonal changes (menstrual cycles) may be associated with exacerbating or worsening coprolalia and tics.[3][13] Relaxation, on the contrary, lessens the symptoms.[14]

A small percentage will have symptoms grow into adulthood with increased severity.[7]

Complications

• Impact on school performance due to several factors (difficulty studying, paying attention) 
• Inability to go to school
• Social isolation due to bullying and non-acceptance by the community
• Interpersonal and family conflict
• Peer victimization[15]

Deterrence and Patient Education

While educating the families about coprolalia, its common presentations, comorbid conditions, prognosis, as well as course and treatment options, the discussion should include no active treatment (bur monitoring). Exacerbating and decreasing factors also require a review with the affected individual. The clinician should also bring informative websites to the patient's attention for more information and to connect with support groups.

• Tourette Syndrome Association
• Tourette Syndrome “Plus”

Pearls and Other Issues

• Coprolalia is the most common of the copro phenomena.
• It is usually associated with tic disorders, in particular, Tourette syndrome.
• The distress it produces can lead to a decrease in academic performance and social isolation.
• Medications such as risperidone and aripiprazole, have been shown to improve tics, as well as habit reversal therapy and other specific behavioral treatments.
• Individuals with coprolalia may need special education services, or given classroom accommodations (leave the classroom as necessary or ignoring their tics).
• More studies and research are needed to determine the cause and management of coprolalia.

Enhancing Healthcare Team Outcomes

• Determining accurate numbers and statistics on coprolalia is not available at this point. There is a need for more studies that focus on tics solely or coprolalia, specifically since the majority of evidence is based on Tourette syndrome or other disorders that are accompanied by tics. 
• No studies have been done yet to determine the efficacity of habit reversal therapy in combination with medications.
• It is crucial to keep in mind that coprolalia (as well as other tics) can negatively influence a child's life, impacting their development and potential as a functional adult. It is essential to recognize coprolalia to provide the appropriate support to the affected individuals, both in the behavioral area, as well as in the education aspect, so that they can be understood and reach their highest potential. 
• It is essential to educate parents, coaches, teachers, other health professionals, and educators who interact with individuals with coprolalia to give them adequate support on how to be supportive rather than a reprimand or punishment, as to not cause more stress and worsen the condition.

Educational Aspect

Tourette syndrome and associated tics (such as coprolalia) can impact school performance in children in different ways, whether it is by interfering with studying, paying attention in class, and making it stressful for the child to be in the class. The stress of the classroom can make tics worse. The teachers may wrongly discipline them, or they may experience bullying by their classmates. All of which can lead to a decrease in academic performance and social isolation.[15]

Special education services are often recommended. Educational support is available with a 504 modification plan (75% of kids with Tourette syndrome are given classroom accommodations, like being able to leave the classroom as needed, or ignoring their tics). And when the students have educational needs, these recommendations are included in the individualized education plan.

Primary care providers should offer referring individuals with coprolalia or Tourette to a behavioral or developmental specialist for further support and management.

Diagnosing and managing coprolalia requires the efforts of an interprofessional team. When the clinician suspects a condition associated with coprolalia, they should immediately enlist a psychiatric specialist. In the event pharmaceutical treatment is part of the treatment plan, and board-certified psychiatric pharmacist can provide additional direction on pharmaceutical care, including agent selection, dosing, and medication reconciliation. Psychiatric health nurses can assist in monitoring, counseling the patient and family, and assessing treatment progress and effectiveness. Social workers can interact with school officials and teachers to ensure the provision of proper accommodations there. In this manner, an open, communicating, interprofessional healthcare team can direct patient outcomes to optimal results. [Level V]