Other Neuro Topics Flashcards
What age does Tourette’s Syndrome symptoms typically start?
Most commonly 6 years of age (5-10 years)
How long does it typically take (duration) for Tourette’s Syndrome to be diagnosed?
About 2 years from onset of symptoms
When do tics increase in severity or frequency?
Under stress (new school, move)
What are the three tic disorders?
Tourette’s
Persistent
Provisional
How is Tourette’s defined?
Both motor and vocal tics persistent for at least 1 year
How is Persistent tic disorder defined?
Motor or vocal tics persistent for at least 1 year
How is Provisional tic disorder defined?
Motor or vocal or both
Present for at least 1 year
How is Tourette’s diagnosed?
Clinical
What is the diagnostic requirements for Tourette’s?
-2+ motor ticks
-1+ vocal tics
-Tics present for at least 1 year
-Tics onset prior to age 18
-Symptoms are not related to medication or other medical condition
What are the other top two diagnoses that Tourette’s presents with?
ADHD and Anxiety
What is the goal of Tourette’s treatment?
Tic suppression
What drug class is the only approved for Tourette’s syndrome?
Neuroleptics
(haloperidol, pimozidole, aripiprazole)
What are two off label drug classes used to treat Tourette’s?
Alpha adrenergic’s and stimulants
Why would you not prescribe a stimulant to a child with Tourette’s?
Although shown helpful at reducing tic frequency they are contraindicated for Tourette’s due to MOA
Do you typically treat Tourette’s with pharmacologic therapy?
No, behavioral therapy is more appropriate as a first line. Only severe tics are treated with neuroleptics.
What is the disease trajectory of Tourette’s Syndrome?
Typically tics decrease during adolescence/early adulthood, and sometimes disappear completely. Rarely worsens with age (10-15% of cases).
What does ALS stand for?
Amyotrophic Lateral Sclerosis
What is the Epidemiology of ALS?
Rare progressive neurological disease
Most common in men than women, diagnosed most commonly between 55-75 years of age (can strike at any age)
More in caucasians and non-Hispanics
What occupation is highly associated with ALS?
Military service
What is the life expectancy after diagnosis with ALS?
Most die from respiratory failure usually 3-5 years from symptom onset
What is the pathology of ALS?
Not well understood, thought to have a genetic predisposition with environmental triggers.
Degeneration of the upper and lower motor neurons
What are the two types of ALS?
Sporadic and Familial
What is the sporadic type of ALS?
90% of cases, diagnoses at random with no clear associated risk factors or family history
What is the familial (genetic) type of ALS?
5-10% of cases, inherited from one autosomal dominant parent
What is the initial presentation of ALS?
Early muscle weakness and stiffness (generally painless), gradual in onset
Begins with neck/head muscles, limb, respiratory muscles
What are the later symptoms of ALS?
Fasciculations in arm, leg, shoulder or tongue
Muscle cramps and spacicity
Muscle weakness (eventually diaphragm)
Slurred and nasal speech, uncontrollable laughing/crying
Difficulty chewing or swallowing