Neuro Degenerative Diseases

Question 1

Define Parkinson

Answer 1

Complex motor disorder (both systemic non-motor and neurological symptoms

Question 2

Define Parkinson Disease with Dementia

Answer 2

When motor disturbances develop first, and then dementia.

Question 3

Primary PD

Answer 3

Onset >40, sporadic idiopathic, genetic (familial), autosomal dominant/autosomal recessive

Question 4

Secondary PD

Answer 4

• Other neurodegenerative diseases/“acquired” diseases
• drug induced is most common
• reversible (Ex: head Trauma/environmental)
• Neurodegeneraitve diseases are secondary to to the PRIMARY condition

Question 5

Neurotransmitter involved in PD

Answer 5

DOPAMINE

Question 6

Dopamine

Answer 6

Excitatory/inhibitory involved in reward, motivation, movement

Question 7

Dopamine (increases/decreases) with PD in the substantia nigra _____ ______.

Answer 7

Decreases; pars compacta

Question 8

where is dopamine made?

Answer 8

Neurons and adrenal medulla

Question 9

Which part of the brain affects people with PD?

Answer 9

Basal Ganglia

Question 10

Basal Ganglia is responsible for

Answer 10

Movement, cognition, learning, motivation

Question 11

Substantia Nigra is considered the “nucleus” of the _____. It produces most of the _____ in your brain.

Answer 11

Midbrain; Dopamine

Question 12

Substantia Nigra PARS COMPACTA contains what type of neurons?

Answer 12

Dopaminergic neurons (darker area of the brain where people with PD lose neurons)

Question 13

Substantia Nigra Pars RETICULATA is made up of what type of neurons?

Answer 13

GABAnergic Neurons

Question 14

Pathophysiology for PD

Answer 14

1) Oxidative Stress
2) Lewy Body containing synuclein accumulate in dopamine neurons
3) Mitochondria dysfunction
4) Lysosomal/Autophagy dysfunction
5) Neuroinflammation
6) Decrease of astrocytes
7) Decrease Subtype glial cells (homeostasis;neural metabolism)
8) MICROglia Increase: cell mediated immune response acts as macrophages in CNS clearing cellular debris, dead neurons from phagocytes

Question 15

Clinical Manifestations of PD

Answer 15

• 70-80% of neurons die before symptoms appear
• Starts out unilateral then affects entire body
• Rigidity
• bradykinesia/akinesia
• resting tremor, unilateral, (it can go away during sleep)
• Autonomic neuroendocrine symptoms
• cognitive affective symptoms and dementia (age 60-70)

Question 16

Define Huntington Disease

Answer 16

Rare, hereditary, HYPERKINETIC movement disorder involving the basal ganglia and cortex

Question 17

Huntington: Genetic/Risk Factors

Answer 17

-Autosomal Dominant
-High age related penetrance: Display phenotype depending on age
Ex: 5y/o no penetrance vs 40 y/o high penetrance

Question 18

Huntington Gene: Under ____ normal. Over 40 repeats is excessive.

Answer 18

Excessive CAG repeats UNDER 35 is normal ( the more CAG repeats the more the disease manifests)

Question 19

Excessive repeats leads to _______ which is toxic.

Answer 19

Protein misfolding

Question 20

Brain structures involved with Huntington’s disease:

Answer 20

Basal ganglia & Cortex

Question 21

Huntington’s Disease Patho

Answer 21

• Neuronal death is unknown
• Intracellular accumulation of abnormal Huntington protein
• Degeneration of STRIATUM and BASAL GANGLIA - ENLARGEMENT OF VENTRICLES (similar to schizo)

Question 22

Clinical Manifestations of Huntington’s Disease

Answer 22

• Slow progression of symptoms
• involuntary fragmented movements: Chorea (frigidity), athetosis (extremities), Ballism (involuntary movements)
• Leads to dementia!

Question 23

Multiple Sclerosis (CNS DISORDER)

Answer 23

Demyelination disorder:

• progressive
• chronic
• inflammation
• demyelination
• AUTOIMMUNE disorder of the CNS!!!

Question 24

Genetic/Risk Factors of MS

Answer 24

• Onset ages 20-40
• Women are most affected
• Men to have more severe disease
• Risk Factors:female, smoking, low vitamin D, EBV infection, living in high altitudes

Question 25

MS Pathophysiology

Answer 25

CNS inflammatory autoimmune disease:
T-cells
B-cells
Microglia: Act as macrophages 
Pro inflammatory cytokines