Unit 7: 13-14 Disorders of the Motor System

Question 1

What is ALS/ Lous Gehrig’s disease?

Answer 1

*UPPER MOTOR DISEASE
(stephen hawking)
(Amyotrophic lateral sclerosis)
a disease affecting motor neurons of the spinal cord, which causes progressive weakness and atrophy of muscles.

(Amyotrophic lateral sclerosis (ALS), formerly known as Lou Gehrig’s disease, is a neurological disorder that affects motor neurons, the nerve cells in the brain and spinal cord that control voluntary muscle movement and breathing. As motor neurons degenerate and die, they stop sending messages to the muscles, which causes the muscles to weaken, start to twitch (fasciculations), and waste away (atrophy). Eventually, in people with ALS, the brain loses its ability to initiate and control voluntary movements such as walking, talking, chewing and other functions, as well as breathing. ALS is progressive, meaning the symptoms get worse over time.)

Question 2

What are the symptoms of ALS/ Lou Gehrig’s disease?

Answer 2

Early symptoms include:
Muscle twitches in the arm, leg, shoulder, or tongue
Muscle cramps
Tight and stiff muscles (spasticity)
Muscle weakness affecting an arm, a leg, or the neck
Slurred and nasal speech
Difficulty chewing or swallowing

As the disease progresses, muscle weakness and atrophy spread to other parts of your body. People with ALS may develop problems with:
Chewing food and swallowing (dysphagia)
Drooling (sialorrhea)
Speaking or forming words (dysarthria)
Breathing (dyspnea)
Unintended crying, laughing, or other emotional displays (pseudobulbar symptoms)
Constipation
Maintaining weight and getting enough nutrients

Question 3

What is Babinski Sign?

Answer 3

*Upper motor neuron disease
*Dorsal Flexor
*Normal in infant, upper motor neuron lesion is > 24 month old
*Pathological adult: Positive (+) Babinski sign (DORSIFLECION of big toe)

(Reflexes are responses that occur when the body receives a certain stimulus. The Babinski reflex occurs after the sole of the foot has been firmly stroked. The big toe then moves upward or toward the top surface of the foot. The other toes fan out.
Babinski reflex is one of the normal reflexes in infants and in children up to 2 years old. It disappears as the child gets older. It may disappear as early as 12 months. In adults, this reflex is not normal and often indicates a problem with the brain or spinal cord.)

Question 4

What are the Symptoms of an Upper Motor neuronal disease?

Answer 4

*Hyperreflexia: Exaggerated stretch reflexes
*Hypertonia: Increased muscle tones
*(Later Stages): Progressive muscle weakness and atrophy

Question 5

What are the causes of Upper Motor neuronal diseases?

Answer 5

*15% of cases are casued by Inherited mutations (GLUTAMATE, TRANSPORTER, ENZYME MUTATION)

*other factors:
-free radicals
-autoimmune responses
-viral infections
-deficiency of nerve growth factor
-environmental toxins
-trauma

Question 6

What are the treatments for Upper motor neuronal diseases?

Answer 6

*Riluzole: NA+ channel INHIBITOR, decreasing GLUTAMATE
*Drugs that relieve fatigue, muscle pain and spasticity, difficulty sleeping, etc.

Question 7

What are the symptoms of Lower Motor neuronal diseases?

Answer 7

*PARALYSIS (loss of movement)
*PARESIS (weakness)
*Hyporeflexia (or areflexia): loss of reflexes
*Hypotonia: loss of muscle tones
*Atrophy of the affected muscles

Question 8

What are the causes of Lower Motor neuronal diseases?

Answer 8

*Certain diseases or localized lesions near the spinal cord
*Trauma
*Tumor
*Viral infection
*Inflammation

Question 9

What is the treatment for Lower Motor neuronal diseases?

Answer 9

*Intravenous immunoglobulin
*Physical therapy
*antibiotics

Question 10

What is Basal Nuclei?

Answer 10

*Three nuclei deep within each cerebral hemisphere make up basal ganglia

*They are GLOBUS PALLIDUS, PUTAMEN, and CAUDATE nucleus

Helps…
*(initiate) and (terminate) movements
*(suppress) unwanted movements
*(regulate) muscle tone

Question 11

What is the Striatum?

Answer 11

*Basal Nuclei associated structure
*made up of Caudate nucleus and Putamen

(The striatum is a deep-brain nucleus that links motivation to motor movements involved in the execution of simple motor tasks as well as more complex cognitive tasks, such as reward processing, decision-making, and social interactions.)
The striatum is involved in other social behaviors besides social action, social reward and reward inequity.

Question 12

Basal Nuclei Direct and Indirect pathway:

** refer to slides to review

Question 13

Basal Nuclei Direct and Indirect pathway:

What are the areas of the brain and distinct symptoms of the neuronal degenerative disease Alzheimer’s?

Answer 13

Alzheimer’s disease: Primarily affects memory and cognitive function, often leading to progressive impairment in daily activities.

Question 14

Basal Nuclei Direct and Indirect pathway:

What are the areas of the brain and distinct symptoms of the neuronal degenerative disease Frontotemporal dementia?

Answer 14

Frontotemporal dementia: Affects the frontal and temporal lobes of the brain, leading to changes in personality, behavior, and language.

Question 15

Basal Nuclei Direct and Indirect pathway:

What are the areas of the brain and distinct symptoms of the neuronal degenerative disease Parkinson’s?

Answer 15

Parkinson’s disease: Affects movement, often causing tremors, stiffness, and difficulty with balance and coordination.

Question 16

Basal Nuclei Direct and Indirect pathway:

What are the areas of the brain and distinct symptoms of the neuronal degenerative disease Creutzfeldt-Jackob?

Answer 16

Creutzfeldt-Jakob disease: A rare degenerative brain disorder that leads to rapidly progressive dementia and neurological symptoms.

Question 17

Basal Nuclei Direct and Indirect pathway:

Inhibitory signals in sequence will “cancel
out” and allow activation if there are ______ in sequence:
A) An odd number
B) An even number
C) More than one

Answer 17

A) An odd number

(In the basal ganglia, the direct and indirect pathways work in parallel to modulate movement. The direct pathway facilitates movement, while the indirect pathway inhibits movement. Both pathways involve inhibitory signals, but they have opposite effects on movement.

To allow activation, the direct pathway requires an odd number of inhibitory signals in sequence. This is because the direct pathway promotes movement, so inhibition must be overcome to allow activation. Conversely, the indirect pathway requires an even number of inhibitory signals in sequence to inhibit movement.)

Question 18

What are the disorders of the Bascal Nuclei?

Answer 18

“can affect body movements, cognition, and behavior”
*Parkinsons diseases (PD), Huntington disease (HD), Tourette syndrome, psychiatric disorders, Schizophrenia or OCD

Question 19

Disorders of the Basal Nuclei:

Describe Parkinson Diseases (PD)

Answer 19

Parkinson diseases (PD):DA neurons from substantia nigra to the putamen and caudate nucleus degenerate.

Question 20

Disorders of the Basal Nuclei:

Describe Huntington Disease (HD)

Answer 20

Huntington disease (HD): caudate nucleus and putamen degenerate, loss of GABA or ACh neurons

Question 21

Disorders of the Basal Nuclei:

Describe Tourette Syndrome

Answer 21

Tourette syndrome: dysfunction of cognitive neural circuits between the basal nuclei and the prefrontal cortex

Question 22

Disorders of the Basal Nuclei:

Describe Psychiatric disorders

Answer 22

Psychiatric disorders: dysfunction of neural circuits between the basal nuclei and the limbic system

(Schizophrenia or OCD: excess dopamine)

Question 23

Disorders of the Basal Nuclei:

Describe Schizophrenia or OCD

Answer 23

Schizophrenia or OCD: excess dopamine

Question 24

What is the function of the Cerebellum?

Answer 24

Function: is to evaluate how well movements
initiated by the cerebral motor cortex

Help correct the errors, smooth the movements, and coordinate complex skeletal muscles contractions

Question 25

What disorder is related to Cerebellum function?

Answer 25

Ataxia

often require the use of wheelchairs

(Ataxia is a degenerative disease of the nervous system. Many symptoms of Ataxia mimic those of being drunk, such as slurred speech, stumbling, falling, and incoordination. These symptoms are caused by damage to the cerebellum, the part of the brain that is responsible for coordinating movement. Ataxia treatment involves a combination of medication to treat symptoms and therapy to improve quality of life.

People affected by Ataxia may experience problems with using their fingers and hands, arms, legs, walking, speaking or moving their eyes. Ataxia affects people of all ages. Age of symptom-onset can vary widely, from childhood to late-adulthood. Complications from the disease are serious and oftentimes debilitating. Some types of Ataxia can lead to an early death.)

Question 26

Symptoms of Ataxia:

Answer 26

Lack of coordination
Slurred speech
Trouble eating and swallowing
Deterioration of fine motor skills
Difficulty walking
Gait abnormalities
Eye movement abnormalities
Tremors
Heart problems

Question 27

*** Review Last slide

Question 28

** Make questions for objective and summary