Thursday, February 7, 2008

Tourette’s syndrome (TS)

Tourette’s syndrome (TS), a neurologic disorder is characterized by persistent tics—involuntary movements and vocalizations.


TS affects 1:10 children/1,000 (estimated)
3 boys:1 girl are affected

Onset of symptoms: 7-year-old
Peak of symptoms: 8 to 12-year-old
Decline of symptoms:
steadily at 20-year-old


Distinguishing Characteristics:
1. Repetitive motor
2. Vocal tics - differ in severity & duration
*grunting
*barking
*sniffing
*throat clearing

(other common kinds of tics: eye blinking, facial grimacing, shoulder shrugging, and head or shoulder jerking)


Extreme forms:
1. Tics that result in self-harm (punching oneself in the face)
2. Vocal tics
*coprolalia (uttering swear words)
*echolalia (repeating others’ words or phrases)


Coexisting Neurobehavioral Conditions:
1. Attention-deficit hyperactivity disorder
2. Obsessive-compulsive behaviors
*This may exacerbate the symptoms of TS and impair a child’s ability to learn. If successfully treated, these may diminish tic severity.
*TS doesn’t impair a person's intelligence.


A diagnosis:
1. based on patient history
2. based on clinical assessment
*no tests are diagnostic for TS

Formal criteria for a diagnosis of TS:
* multiple motor and at least one vocal tic
* a waxing and waning course with decreasing severity over time
* tic symptoms persisting for at least 1 year
* symptom onset before age 21
* no precipitating illnesses (such as encephalitis or stroke) or precipitating drugs (such as stimulants, antiepileptic drugs, levodopa, or haloperidol)
* observation of tics by a medical professional.


Patients with TS:

*Aren’t significantly disabled by their symptoms
*Don’t require medication
-may be indicated if the symptoms affect the persons functioning
-They only reduce the severity and do not eliminate symptoms
-must be monitored closely for adverse reactions


Management of mild symptoms:

1. Education
2. Counseling
3. Behavioral techniques

*MOST SUCCESSFUL BEHAVIORAL INTERVENTION: Habit reversal (focuses on techniques to replace the tic with an alternate behavior)

Management for severely disabled patients:
*experimental surgical interventions (deep brain stimulation)


Tier One Drugs (for mild to moderate symptoms):
1.Clonidine
2 Clonazepam
3 Guanfacine

Main Adverse Reactions:

1. Sedation
2. Irritability.


Tier Two Drugs (for severe symptoms):
1. Haloperidol
2. Pimozide
3. Risperidone
4. Fluphenazine

Main Adverse Reactions:
1. Sedation
2. Weight gain
3. Extrapyramidal symptoms
* tardive dyskinesia (involuntary movements, usually of the mouth, lips, and tongue)
* tremors
* rigidity
* akathisia (physical restlessness)
* acute dystonia (muscle contractions)


*Refer patients and their families to support groups and Web sites because symptoms can make patients feel socially isolated and self-conscious. TS can strain family relationships knowing that other families are having the same experiences.

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