Motor Disorders (pg 774-783) Flashcards
What brain structure is typically involved in motor disorders
the basal ganglia
What are the clinical symptoms of motor disorders?
abnormalities in movement and posture
cognitive symptoms
What are the two groups of motor symptoms?
- loss of movement, reffered to as a hypokinetic-rigid syndrome (Parkingson’s)
- increased motor activity aka hyperkinetic-dystonic syndrome (Tourette’s syndrome.)
What two hyperkinetic disorders are listed in the book?
Huntington’s disease
Tourette’s syndrome
What are the three results of Huntington’s disease listed in the text?
intellectual deterioration
personality changes
abnormal movements called choreas
What are the behavioral symptoms of Huntington’s?
impairment of recent memory
defective ability to manipulate acquired knowledge
slowed information processing
Emotional changes (anxiety, depression, manic, schizophrenic)
On average, what is the age range of initial diagnosis of Huntington’s?
30-50 years old (though can occur earlier)
On average, how long to patients live after their diagnosis?
12 years
What is the death rate of Huntington’s?
1.6 per million people per year, worldwide
Genetically, Huntington’s is transmitted as…
an autosomal dominant allele with full penetrance.
True or false. Half the offspring of a Huntington’s patient will develop the disease.
True.
True or false. Huntington’s disease is declining due to genetic counseling.
True.
What do we know about the gene that causes Huntington’s?
the location of the gene is now know, and a marker can be used before symptoms appear (even in utero) to determine whether a family member will develop Huntington’s (Gusella et al., 1993).
What is generally found at autopsy of a patient with Huntington’s disease?
cortical shrinkage and thinning
grossly atrophied basal ganglia with marked neuronal loss
What is the dominant explanation for Huntington’s disease?
imbalance of neurotransmitter systems of the basal ganglia. intrinsic neurons (ACh and GABA) of the basal ganglia die leaving a largely intact nigrostriatal dopamine pathway. Decreased inhibition of dopamine by the now dead GABA pathway result in increased dopamine release into the basal ganglia.
What neurotransmitter is believed responsible for the appearance of choreas in Huntington’s disease?
increased dopamine levels, though how this happens is uncertain.
Fedio et al, 1979 and Wexler, 1979 ran neurological studies in which results showed that…
patients with Huntington’s disease have impairment in a broad range of memory and perceptual tasks.
Which 2 tests are suggested to measure poor performance in frontal lobe areas?
Chicago Word-Fluency test
stylus-maze test
Who described Tourette’s syndrome in 1885?
Georges Gilles de la Tourette
What were the three stages proposed by Georges Gilles de la Tourette?
1 multiple tics only
- inarticulate cries and multiple tics
- inarticulate cries, multiple tics, echolalia, coprolalia.
What is echolalia?
a symptom of Tourette’s; repetition of words others have said as well as repeating actions.
What is coprolalia?
obscene, lewd speech seen in Tourette’s syndrome.
Bitsko et al., 2014 showed the prevalence rate of Tourette’s is about…
1 in every 360 people.
What is the average age of onset of Tourette’s syndrome?
between 2 and 15 years with a median of 7