Question

Difficulty: EasyPediatric Neurologic Disorders

An 8-year-old boy is evaluated in the clinic for unusual behaviors. His parents report that for the past 15 months, he has had intermittent episodes of rapid eye blinking, facial grimacing, and shoulder shrugging. Additionally, for the last 6 months, he has been frequently grunting and sniffing loudly. These actions occur many times a day and worsen when he is stressed about schoolwork. He is fully conscious during the episodes and says he feels a "build-up of tension" that is relieved only by performing the actions. Physical and neurologic examinations are otherwise unremarkable. Which of the following is the most likely diagnosis?

  1. Tourette syndromeAnswer
  2. B
    Provisional tic disorder
  3. C
    Normal developmental stereotypies
  4. D
    Sydenham chorea
  5. E
    Focal aware seizures

Answer

Tourette syndrome
The correct answer is Tourette syndrome. The diagnosis of Tourette syndrome requires the presence of multiple motor tics (e.g., rapid eye blinking, facial grimacing, shoulder shrugging) and at least one vocal tic (e.g., grunting, sniffing) manifesting before age 18. Furthermore, the tics must be present for more than 1 year since the onset of the first tic. This patient's symptoms are preceded by a premonitory urge ("build-up of tension") that is temporarily relieved by performing the action, which is a classic feature of tics.

Here is a summary of pediatric tic disorders:

DisorderCharacteristicsDuration
Tourette syndromeMultiple motor AND ≥1\geq 1 vocal tic>1> 1 year
Persistent (chronic) tic disorderMotor OR vocal tics (but not both)>1> 1 year
Provisional tic disorderMotor and/or vocal tics<1< 1 year

Step-by-Step Solution

1
Analyze the clinical presentation of the patient's movements.
The patient exhibits rapid eye blinking, grimacing, and shoulder shrugging (motor tics), as well as grunting and sniffing (vocal tics).
Identifying the specific types of movements is essential to differentiate tics from chorea, stereotypies, or seizures.
2
Evaluate the timeline and duration of the symptoms.
The motor tics have been present for 15 months, which is greater than the 1-year threshold.
Duration is a critical diagnostic criterion that separates provisional tic disorders from chronic tic disorders.
3
Combine the symptom types and timeline to establish the diagnosis.
The presence of multiple motor tics and at least one vocal tic for >1 year confirms the diagnosis of Tourette syndrome.
Strict application of DSM-5 diagnostic criteria is necessary for accurate diagnosis.

Key Concept

The diagnosis of Tourette syndrome requires the presence of both multiple motor tics and at least one vocal tic for a duration of greater than 1 year.
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