WBR0617

Author	[[PageAuthor::Ayokunle Olubaniyi, M.B,B.S [1] (Reviewed by Serge Korjian)]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Genetics, MainCategory::Pathology
Sub Category	SubCategory::Neurology
Prompt	[[Prompt::A 7-year-old girl is brought to the outpatient neurology clinic by her parents for the evaluation of a learning disability. The parents report that the child has been healthy and developing normally. She attended primary school with no problems. At the age 5, her teachers started noting some learning difficulties when the child was compared to her classmates. The parents also noticed that their daughter started having repetitive motor tics particularly jerking of the shoulder and hands and repetitive squinting. They also noticed episodes where the child would shout out her name repetitively and would not respond to their demands to stop. These symptoms were initially mild but have been worsening as the girl develops. What features are not likely to be found in this patient?]]
Answer A	AnswerA::Attention deficit hyperactive disorder
Answer A Explanation	AnswerAExp::Patients with Tourette's syndrome are at an increased risk of developing attention deficit hyperactive disorder (ADHD).
Answer B	AnswerB::Insomnia
Answer B Explanation	AnswerBExp::Sleep disorders such as longer sleep period time, longer sleep latency, reduced sleep efficiency, and prolonged wakefulness after sleep onset are common in patients with Tourette's syndrome.
Answer C	AnswerC::Intellectual disability
Answer C Explanation	AnswerCExp::Patients with Tourette's have normal intelligence despite some learning difficulties.
Answer D	AnswerD::Obsessive-compulsive behavior
Answer D Explanation	AnswerDExp::Patients with Tourette's syndrome are at an increased risk of obsessive-compulsive disorder (OCD).
Answer E	AnswerE::Autism
Answer E Explanation	AnswerEExp::Patients with Tourette's syndrome are at an increased risk of autistic spectrum disorders.
Right Answer	RightAnswer::C
Explanation	[[Explanation::Tourette's syndrome is a neuropsychiatric disorder characterized by multiple mannerisms known as tics. A tic is an unusual involuntary movement or sound that tends to be repetitive. Tics can start at any age, but are more common between the ages of 6 and 18 years. These tics characteristically wax and wane, but can worsen as the patient ages. Tics are typically worsened by anxiety, fatigue, excitement, and head injury. There are two main types of tics: Motor tics – e.g. blinking, eye-rolling, grinding of teeth, head jerking, neck twisting. Vocal tics – Grunts, squeaks, coughing, barking, hiccuping, words or phrases. The main etiology of tourette's syndrome is unknown but observational data suggest a dysfunction at the level of the basal ganglia. Tourette's syndrome patients are at higher risk of developing other psychiatric disorders including attention deficit hyperactive disorder (ADHD), learning difficulties, obsessive-compulsive disorder (OCD), depression, anxiety disorders, and sleep disorders. Despite this added risk, the majority of patients with Tourette syndrome have normal intelligence and no intellectual disability. Educational Objective: Tourette's syndrome is a neuropsychiatric disorder characterized by tics (motor or vocal) and is associated with an increased risk of with ADHD, OCD, autistic spectrum disorders, and sleep disorders. Although patients have learning difficulties, majority of them have normal intelligence and no intellectual disability. References: Kurlan R. Clinical practice. Tourette's Syndrome. N Engl J Med. 2010;363(24):2332-8.]]
Approved	Approved::Yes
Keyword	WBRKeyword::Tourette syndrome, WBRKeyword::Tourette's syndrome, WBRKeyword::Intellectual disability, WBRKeyword::Mental retardation, WBRKeyword::ADHD, WBRKeyword::OCD
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