Multiple Tics in a Patient With Adams-Oliver Syndrome

Mr. “A.B.” was first referred to the Tourette Syndrome Clinic at the age of 28 years for the assessment and management of his motor and vocal tics. He reported a longstanding history of tic symptoms, dating back to early childhood. The age at tic onset was 6 years, and the first tics to be noticed consisted of head movements. He subsequently developed multiple motor tics mainly affecting his head and neck, including lip biting, mouth opening, smiling, spitting, licking, blowing, head turning, neck stretching, and shoulder shrugging. His motor tic repertoire also included arm and leg movements, plus complex whole body movements, such as jumping, stamping, hopping, and squatting. In terms of phonic tics, he reported grunting, throat clearing, coughing, barking, snorting, sniffing, and humming, plus complex vocalizations including animal sounds. The tics showed a typical waxing and waning course over time. Moreover, A.B.’s tics were characteristically preceded by premonitory urges and could be voluntarily suppressed for a few seconds at the expense of mounting inner tension. These involuntary symptoms were exacerbated by psychological stress and alleviated by physical activity and active concentration. With regard to complex tics and tic-related symptoms, A.B. reported coprophenomena (including mental coprolalia), paliphenomena, and echolalia. He scored 79% on the Tourette Syndrome Diagnostic Confidence Index³ and 58% on the Yale Global Tic Severity Scale,⁴ indicating moderate-to-marked tic severity. A.B. fulfilled the current diagnostic criteria for Tourette’s syndrome. With regard to behavioral comorbidities, there was evidence of specific obsessive-compulsive symptoms, which are considered part of the clinical phenotype of Tourette’s syndrome, including checking, counting (arithmomania), and excessive hand washing.^5,6 There was also a history of childhood attention deficit, which had improved to some extent with age, plus anxiety and affective symptoms.

Interestingly, A.B. had been diagnosed with Adams–Oliver syndrome based on the presence of both cranial aplasia cutis congenita and terminal transverse limb defects. His two brothers were both diagnosed with the same genetic condition, and one died at the age of 3 months. In terms of neurodevelopmental problems, dyslexia and oculomotor dyspraxia were noted since an early age, whereas tics prompted a diagnosis of Tourette’s syndrome only in early adulthood. Tourette’s syndrome has been reported in the context of other genetic conditions,⁷ and tic symptoms are proposed as a possible addendum to the neurodevelopmental spectrum of Adams–Oliver syndrome.