Neuro Degenerative Diseases Flashcards
Define Parkinson
Complex motor disorder (both systemic non-motor and neurological symptoms
Define Parkinson Disease with Dementia
When motor disturbances develop first, and then dementia.
Primary PD
Onset >40, sporadic idiopathic, genetic (familial), autosomal dominant/autosomal recessive
Secondary PD
- Other neurodegenerative diseases/“acquired” diseases
- drug induced is most common
- reversible (Ex: head Trauma/environmental)
- Neurodegeneraitve diseases are secondary to to the PRIMARY condition
Neurotransmitter involved in PD
DOPAMINE
Dopamine
Excitatory/inhibitory involved in reward, motivation, movement
Dopamine (increases/decreases) with PD in the substantia nigra _____ ______.
Decreases; pars compacta
where is dopamine made?
Neurons and adrenal medulla
Which part of the brain affects people with PD?
Basal Ganglia
Basal Ganglia is responsible for
Movement, cognition, learning, motivation
Substantia Nigra is considered the “nucleus” of the _____. It produces most of the _____ in your brain.
Midbrain; Dopamine
Substantia Nigra PARS COMPACTA contains what type of neurons?
Dopaminergic neurons (darker area of the brain where people with PD lose neurons)
Substantia Nigra Pars RETICULATA is made up of what type of neurons?
GABAnergic Neurons
Pathophysiology for PD
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
Clinical Manifestations of PD
- 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)
Define Huntington Disease
Rare, hereditary, HYPERKINETIC movement disorder involving the basal ganglia and cortex
Huntington: Genetic/Risk Factors
-Autosomal Dominant
-High age related penetrance: Display phenotype depending on age
Ex: 5y/o no penetrance vs 40 y/o high penetrance
Huntington Gene: Under ____ normal. Over 40 repeats is excessive.
Excessive CAG repeats UNDER 35 is normal ( the more CAG repeats the more the disease manifests)
Excessive repeats leads to _______ which is toxic.
Protein misfolding
Brain structures involved with Huntington’s disease:
Basal ganglia & Cortex
Huntington’s Disease Patho
- Neuronal death is unknown
- Intracellular accumulation of abnormal Huntington protein
- Degeneration of STRIATUM and BASAL GANGLIA - ENLARGEMENT OF VENTRICLES (similar to schizo)
Clinical Manifestations of Huntington’s Disease
- Slow progression of symptoms
- involuntary fragmented movements: Chorea (frigidity), athetosis (extremities), Ballism (involuntary movements)
- Leads to dementia!
Multiple Sclerosis (CNS DISORDER)
Demyelination disorder:
- progressive
- chronic
- inflammation
- demyelination
- AUTOIMMUNE disorder of the CNS!!!
Genetic/Risk Factors of MS
- 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
