The Mysterious Case of the Boy who Barked

A 14-year-old boy has been seen by several physicians who have failed to treat a perioral rash successfully. He is acting out at home and school and mother and son are miserable

The patient blames his behavior on the discomfort of the facial rash; his mother blames it on poor attitude and complains about his twitching and “barking” like a dog.

Patient has been treated by several clinicians for lip licking, with steroids, moisturizers, without effect. Behavioral and family issues have not been addressed.

The 14-year-old is teased at school for his habit of rapid blinking and has developed a nervous cough that resembles a “bark.” Ritalin was prescribed but had a negative effect.

Patient relaxes during exam. Notable signs: Diffuse perioral dryness, erythema without exanthema/enanthema; subtle hand twitching and clearing of the throat.

Impression: lip licking is a problem but subtle motor and vocal tics suggest other underlying mechanisms.

Physical examination: Notice the dry, excoriated perioral erythema.

Physical examination: The tip of his tongue extend as far as the borders of the perioral dermatitis.

You have created a timeline for possible disease progression and have a presumptive diagnosis in mind; time for a consult with a neurologist who concurs with your impression.

What is your diagnosis: Temporal lobe epilepsy; petite mal seizures; ADHD/behavioral issues; Tourette syndrome; lip licking; Tourette and lip licking; none of these; need more information.

Diagnosis: Tourette syndrome and lip licking. Perioral dermatitis is best treated with oral tetracycline or topical metronidazole or erythromycin.

Tourette syndrome diagnostic criteria: onset before age 21y; recurrent, involuntary, rapid, purposeless motor movements; one+ vocal tics; periodic variation in symptom intensity; duration longer than 1y.

Tourette syndrome etiology: Evidence for dopaminergic involvement--dramatic response to neuroleptics (eg, haloperidol), exacerbations induced by stimulant medications; TS may be result of post-synaptic dopamine receptor hypersensitivity.

Tourette syndrome tics: motor, vocal, complex. May be initially very subtle, delaying diagnosis of tics and of Tourette

Tourette syndrome motor symptoms: Simple motor tics: fast, darting, and meaningless; eg, eye blinking, grimacing, nose twitching, lip pouting, shoulder shrugging, rapid jering of any part of the body; copropraxia, echopraxia.

Tourette syndrome complex motor symptoms: Hopping, clapping, touching objects arranging, gyrating, head banging, tongue thrusting, eye rolling, repetitive odd expressions, repetitive writing, tearing paper/books.

Tourette syndrome motor tics: summary.

Tourette syndrome vocal tics: meaningless sounds and noises; Complex vocal tics: linguistically meaningful utterances such as words and phrases (ie, coprolalia, echolalia, and palilalia).

Tourette syndrome vocal symptoms: Ritual vocal repetitions; atypical speech features; coprolalia

Tourette syndrome vocal tics: summary

Tourette syndrome complex symptoms: summary

Tourette syndrome behavioral/developmental symptoms: ADHD, obsessive/compulsive disorder, self injury

Tourette syndrome treatment highlights: Multidisciplinary: neurology, child psychiatry, social work/PhD psychologist, pediatrician; medication is helpful, not curative.

Tourette syndrome case conclusion: medication partially controlled tics; CBT for impulsivity; barking resolved; completed secondary education.

Lesson learned: First impressions most often deserve a second, longer look.