8.3.1 Movement Disorders 2

Question 1

____ ____ of Huntington’s disease usually begin with arm jerks and facial twitches. Then tremors spread to other parts of the body and develop into writhing.

Answer 1

Motor symptoms

Question 2

Gradually, in Huntington’s the tremors interfere more and more with walking, speech, and other ____ ____. The ability to learn and improve new movements f is especially limited.

Answer 2

volunteering movements

Question 3

The Huntington’s disorder is associated with gradual, extensive ____ ____, especially in the caudate nucleus, putamen, and globus pallidus but also in the cerebral cortex.

Answer 3

brain damage

Question 4

People with Huntington’s disease also suffer ____ ____, including depression, sleep disorders, memory impairment, anxiety, hallucinations and delusions, poor judgment, alcoholism, drug abuse, and sexual disorders ranging from complete unresponsiveness to indiscriminate promiscuity.

Answer 4

psychological disorders

Question 5

Deficits in ____ and ____ usually proceed the motor symptoms in Huntington’s.

Answer 5

memory and reasoning

Question 6

Occasionally, individuals in the early stages of Huntington’s disease are misdiagnosed as having ____.

Answer 6

schizophrenia

Question 7

Huntingtons disease most often appears between the ages of __ and __, although onset can occur at any time from early childhood to old age.

Answer 7

30 and 50

Question 8

Once the symptoms of Huntington’s emerge, both the psychological and the motor symptoms grow ____ ____ and culminate in death.

Answer 8

progressively worse

Question 9

Huntington’s disease results from a dominant gene on ____ __.

Answer 9

chromosome #4

Question 10

As a rule, a mutant gene that causes the loss of a function is ____. The fact the the Huntington’s gene is dominant implies that it produces the gain of some undesirable function.

Answer 10

recessive

Question 11

The critical area of the gene chromosome #4 includes a sequence of bases C-A-G (cytosine, adenine, guanine), which is repeated __ to __ times in most people. That repetition produces a string of __ to __ glutamines in the resulting protein.

Answer 11

11 to 24

Question 12

People with up to __ C-A-G repetitions are considered safe from Huntington’s disease. People with __ or more repetitions are likely to get the disease. The more C-A-G repetitions someone has, the earlier the probable onset of the disease.

Answer 12

35 : 39

Question 13

A ____ ____ can predict not only whether a person will get Huntington’s disease but also approximately when.

Answer 13

chromosomal examination

Question 14

Variant forms of genes controlling glutamate receptors do not produce Huntington’s disease by themselves, but they influence the age of ____ of ____.

Answer 14

onset of symptoms

Question 15

For people with later onset of Huntington’s, the role of ____ is weaker or less certain.

Answer 15

genetics

Question 16

Indentification of the gene for Huntingtons disease led to the discovery of the protein that is codes, which has been designated ____.

Answer 16

huntingtin

Question 17

Huntingtin occurs ____ the human body, although it’s mutant form produces no known harm outside the brain.

Answer 17

throughout

Question 18

Within the brain huntingtin occurs ____ neurons, not on their membranes. The mutant form impairs neurons in several ways.

Answer 18

inside

Question 19

In the early stages of the disease, huntingtin increases ____ ____, sometimes causing overstimulation of the target cells. Later, the protein forms clusters that impair the neurons mitochondria. It also impairs the transport of chemicals down the axon.

Answer 19

neurotransmitter release

Question 20

Several drugs block the glutamine chains from ____.

Answer 20

clustering

Question 21

Another drug interferes with the RNA that enables ____ of the huntingtin gene.

Answer 21

expression

Question 22

_____ are effective if injected into the brain, although that is not an option for humans. (Neurotrophins do not cross the blood-brain barrier)

Answer 22

Neurotrophins

Question 23

A more promising option is drugs that ____ the brain to make more neurotrophins itself.

Answer 23

stimulate

Question 24

People with Huntington’s show disrupted circadian patterns and poor sleep as well as impairments in learning and memory. Giving them a daily ____ ____ improved their sleep, learning and memory.

Answer 24

sleeping pill