Coprolalia

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]
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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]