Motor Disorders (pg 774-783) Flashcards

1
Q

What brain structure is typically involved in motor disorders

A

the basal ganglia

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2
Q

What are the clinical symptoms of motor disorders?

A

abnormalities in movement and posture

cognitive symptoms

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3
Q

What are the two groups of motor symptoms?

A
  1. loss of movement, reffered to as a hypokinetic-rigid syndrome (Parkingson’s)
  2. increased motor activity aka hyperkinetic-dystonic syndrome (Tourette’s syndrome.)
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4
Q

What two hyperkinetic disorders are listed in the book?

A

Huntington’s disease

Tourette’s syndrome

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5
Q

What are the three results of Huntington’s disease listed in the text?

A

intellectual deterioration
personality changes
abnormal movements called choreas

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6
Q

What are the behavioral symptoms of Huntington’s?

A

impairment of recent memory
defective ability to manipulate acquired knowledge
slowed information processing
Emotional changes (anxiety, depression, manic, schizophrenic)

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7
Q

On average, what is the age range of initial diagnosis of Huntington’s?

A

30-50 years old (though can occur earlier)

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8
Q

On average, how long to patients live after their diagnosis?

A

12 years

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9
Q

What is the death rate of Huntington’s?

A

1.6 per million people per year, worldwide

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10
Q

Genetically, Huntington’s is transmitted as…

A

an autosomal dominant allele with full penetrance.

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11
Q

True or false. Half the offspring of a Huntington’s patient will develop the disease.

A

True.

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12
Q

True or false. Huntington’s disease is declining due to genetic counseling.

A

True.

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13
Q

What do we know about the gene that causes Huntington’s?

A

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).

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14
Q

What is generally found at autopsy of a patient with Huntington’s disease?

A

cortical shrinkage and thinning

grossly atrophied basal ganglia with marked neuronal loss

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15
Q

What is the dominant explanation for Huntington’s disease?

A
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.
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16
Q

What neurotransmitter is believed responsible for the appearance of choreas in Huntington’s disease?

A

increased dopamine levels, though how this happens is uncertain.

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17
Q

Fedio et al, 1979 and Wexler, 1979 ran neurological studies in which results showed that…

A

patients with Huntington’s disease have impairment in a broad range of memory and perceptual tasks.

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18
Q

Which 2 tests are suggested to measure poor performance in frontal lobe areas?

A

Chicago Word-Fluency test

stylus-maze test

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19
Q

Who described Tourette’s syndrome in 1885?

A

Georges Gilles de la Tourette

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20
Q

What were the three stages proposed by Georges Gilles de la Tourette?

A

1 multiple tics only

  1. inarticulate cries and multiple tics
  2. inarticulate cries, multiple tics, echolalia, coprolalia.
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21
Q

What is echolalia?

A

a symptom of Tourette’s; repetition of words others have said as well as repeating actions.

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22
Q

What is coprolalia?

A

obscene, lewd speech seen in Tourette’s syndrome.

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23
Q

Bitsko et al., 2014 showed the prevalence rate of Tourette’s is about…

A

1 in every 360 people.

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24
Q

What is the average age of onset of Tourette’s syndrome?

A

between 2 and 15 years with a median of 7

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25
Q

By age 11, what percentage of patients will show symptoms of Tourette’s?

A

97%

26
Q

What is the percentage of tics seen in the three listed body areas in the text?

A

tics of:
eye, head and face (97%)
upper limbs (81%)
lower limbs and body (55%)

27
Q

What percentage of patients have symptoms of complex movements including touching, hitting and jumping in Tourette’s?

A

30-40%

28
Q

What percentage of patients with Tourette’s experience coprolalia?

A

60%

29
Q

What brain areas are impacted by Tourette’s syndrome?

A

the basal ganglia and abnormalities in the frontoparietal circuitry

30
Q

What two medications prove most effective in reducing symptoms of Tourette’s?

A

haloperidol (antidopaminergic agent)

Clonidine (norepinephrine-receptor agonist)

31
Q

in 1982, Robert Sutherland and his colleagues determined, by using the Rey Complex Figure task, that…

A

the right hemisphere of Tourette’s patients in general show abnormalities in cognitive functions.

32
Q

What are the three positive symptoms of Parkinson’s Disease (TMI)?

A
  1. Tremor at rest
  2. Muscular rigidity
  3. involuntary movements
33
Q

What are the five negative symptoms of Parkinson’s disease (PRLSA)?

A
  1. Postural disorders
  2. Righting disorders
  3. Locomotive disorders
  4. Speech disturbances
  5. Akinesia
34
Q

In what year did James Parkinson publish an essay detailing the symptoms of what he called “the shaky palsy”?

A

1817 (Later, renamed after him to Parkinson’s disease

35
Q

How common is Parkinson’s disease?

A

Exists in 0.1 to 1% of the worldwide population, and incidence rises sharply in old age.

36
Q

True or False. Incidences of Parkinson’s disease are found more frequently in countries with lower life expectancy.

A

False. They are seen in countries with higher life expectancy.

37
Q

What are the four major symptoms of Parkinson’s disease?

A
  1. tremor
  2. muscular rigidity
  3. involuntary movement
  4. postural disturbance
38
Q

Positive symptoms of Parkinson’s represent new abnormal behaviors, while negative symptoms show…

A

loss of normal behaviors.

39
Q

what is akathesia?

A

(cruel restlessness) small movements and posture changes as a result of Parkinson’s disease.

40
Q

What happens during oculogyric crisis?

A

the head and eyes involuntarily turn, lasting minutes to hours.

41
Q

What two postural disorders are listed in the text as symptoms of Parkinson’s disease?

A

disorder of fixation (can’t maintain posture)

disorder of equillibrium (difficulty in movement, like being unable to walk without tipping)

42
Q

What is festination?

A

a symptom seen in Parkinson’s disease in which the patient, due to locomotor disorders, walks faster and faster with each step until they are practically running as to not fall over.

43
Q

What is prosody?

A

tone of voice, variation in stress, pitch, and rhythm of speech that conveys different shades of meaning. Lost in Parkinson’s.

44
Q

What is Akinesia?

A

poverty, slowness of movement seen in Parkinson’s disease. Patients may not blink, may swing arms when walking, make spontaneous speech and display typical fidgeting movements. May also make repetitive movements impossible (like tapping a finger)

45
Q

What are the genetic factors for Parkinson’s disease?

A

only 25% of patients have a relative with the disorder, but more than two dozen genome-wide variations exist (Nalls et al., 2014.) The more variants a patient has, the greater the risk of developing the disease.

46
Q

Explain the progression of Parkinson’s disease?

A

Insidious onset of positive and negative symptoms.
Slow movement, emotionless expression, shuffling gait, monotone speech and difficulty in swallowing
As it progresses, the rate is variable, usually resulting in debilitating symptoms 10-20 years following diagnosis, though some have degraded in only 5 years.
on-again-off-again quality (Oliver Sacks, 1973) Sacks recorded a dramatic case of a wheel-chair bound individual recovering from Parkinson’s enough to save a drowning man, then returning to a state of inactivity. These results are uncommon.

47
Q

What are the three major types of Parkinson’s disease?

A
  1. idiopathic
  2. postencephalitic
  3. drug-induced
48
Q

Parkinson’s can result from what diseases or conditions?

A

arteriosclerosis, syphilis, tumor development, carbon monoxide poisoning, manganese intoxication.

49
Q

the cause of idiopathic Parkinson’s is…

A

unknown, seen mostly in people over 50.

50
Q

The cause of postencephalitic Parkinson’s is believed to be…

A

a sleeping sickness (encephalitis lethargica) that appeared in 1916-1917 but was gone by 1927. The main change in the brain for this type is the death of cells in the substantia nigra.

51
Q

What drugs can induce Parkinson’s?

A

major tranquilizers, (reserpine and several phenothiazine and butyrophenone derivatives), MPTP in synthetic heroin, Toxins in the environment that react similarly to MPTP. (Increased incidence of Parkinson’s in people under 40)

52
Q

Which brain structure is associated with Parkinson’s disease?

A

the substantia nigra

53
Q

What percentage of dopamine is absent in patients with Parkinson’s disease?

A

90%- untraceable amounts.

54
Q

Is dopamine depletion solely responsible for Parkinson’s disease?

A

no, as decreases in norepinephrine have been reported as well as the degeneration of some cells in the basal ganglia.

55
Q

What two behavioral treatments are recommended for patients with Parkinson’s disease?

A

psychological counseling related to the disease and physical therapy

56
Q

What are the two main objectives for the Pharmacological treatment of Parkinson’s disease?

A
  1. increase dopamine production remaining

2. balance the brain so that the areas that are impacted by a lack of dopamine stay within normal ranges

57
Q

What drugs are prescribed to increase dopamine production in Parkinson’s pateints?

A

L-Dopa (creates dopamine), amantadine, amphetamine; monoamine oxidase (MAO) inhibitors and tricyclic antidepressants.

58
Q

What drugs reduce the levels of other neurotransmitters that increase as dopamine production decreases?

A

natural anticholinergic drugs (atropine and scopolamine) and synthetic anticholinergics (benztropine or Cogentin, and Trihexyphenidyl or Artane) block cholinergic activating systems that show heightened activity in the absence of adequate dopamine activity.

59
Q

What two “other” treatments exist for Parkinson’s that are being explored?

A

stem cells to regrow the cells in the substantia nigra (Hallett et al., 2014) and deep brain stimulation.

60
Q

What is the best studied similarity between Parkinson’s and Alzheimer’s disease?

A

the Lewy body, which forms most often in the substantia nigra, and are thought to signal abnormal neurofilament metabolism.