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Neuropysch > Neurodegen disorders (1) > Flashcards

Neurodegen disorders (1) Flashcards

1
Q

What ares of brain are most susceptible to stroke?

A

III: external pyramidal layer

V: interal pyramidal layer

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2
Q

Primary cortices of cerebral cortex

A

: somatosensory, motor, visual, and auditory

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3
Q

Association cortices:

– _______: Concerned with integration of function from a single area

–______: Higher order information processing: integration of function from multiple sensory and/or motor modalities

A

Unimodal

Heteromodal

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4
Q

What makes up the limbic system of the brain?

A

Papez’s circuit: Cingulate to hippocampus to fornix to mammillary bodies to thalamus

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5
Q

Outer Core Cortical Compoments:

A

Cingulate cortex

Orbital frontal lobe

Temporal lobe: hippocampus, parahippocampus, entorhinal cortex

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6
Q
A
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7
Q

Key for emotions and motor

this part for visual spatial and memory

A

Cingulate cortex

Rostal: emtions and motor

caudal = visual spatial and memory

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8
Q

in charge of personality, behavioral control adn self awareness (key for alzeihmers path)

A

Orbital frontal lobe

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9
Q

Key for memory

A

temporal lobe (hippo)

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10
Q

Key INNER core subcortical components

A

Hypothalamus

Amygdala

septum

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11
Q

Pleasure center, autonomic and endocrine integration

neurons from here project to pituitary to regulate ACTH and TSH secreation

*key for maternal behavior, BP, feeding, temp regulation and immune

A

HYpothalamus

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12
Q

“preservation of self” emotion, social behavior, aggressin and defense response, sexual behavior, affective visual stimuli, affect of faces, affective regulation

A

Amygdala (part of inner core)

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13
Q

Preservation of SPECIES, sexual behvior, personality

A

Septum

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14
Q

Lesion here can lead to amnestic states ro memory impairments and are key in declaritive memory

A

Hippocampus, dosral medial nucleas of thalamus, mammillary nuclie

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15
Q

Crucial for formaiton of episodice memories in humans (record of personal events)

A

HIppocampus

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16
Q

Difference of episodic and semantic memory

A
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17
Q

Explictic or delcaritive memory of facts(semantic) and events(episodic) are examples of long term memory stored in:

A

Medial temporal lobe

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18
Q

IN charge of highest cognitive functions adn emotional control

A

Frontal cortex

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19
Q

In charge of primitive emtional responses

A

Hypothalamus

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20
Q

Key for storage of emotional memories

A

Amygdala

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21
Q

Storage of emtional memories and actived and inhibited by emotionality

A

hippocampus

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22
Q

Structal organization of the basal ganglia

A

be familiar with structre

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23
Q

What makes up corpus STriatum?

A

Caudate nucleus, Putamen and Globus Pallidus

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24
Q

Putamen + Globus pallidus =

CN + putamen =

A

Lentiform/lenticulate

Striatum/neostritum

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25
Q

What structures are int he GP?

A

GPe = external

GPi= internal or medial segment

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26
Q

What is located in the substantia nigra

A

pars compacta (SNc; dopaminergic)

pars reticulata (SNr: GABAergic)

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27
Q

What affect does dopamine have on the D1 receptors in the striatum?

A

D1 = EXCITE direct to facilitate MOVEMENT

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28
Q

Neurons with D2 dopamine receptors will:

_______ indirect pathway with the goal to ______ movement

A

INHIBITS indirect path (which fnx to inhibit movement)

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29
Q

Understand Direct pathway in thalamus

A

Thalaus is DISinhibited or EXCITED

movement is facilitated

via G1 path

30
Q

Understand indirect pathways of thalamus

A

thalamu is Inhibited

movement is inhibitied

31
Q

Understand the selective vulnerability of specific neurons and systems that is common to neurodegenerative diseases

A

Gray matter diseases: progressive loss of neurons, groups of neurons, associated fiber tracts, usually functionally related (rather than physically contiguous) & relatively symmetric

– Leading to progressive decline in nervous system function

32
Q

List of more grey matter diseaes that lead to progressive decline of NS fnx

A

Parkinson’s disease: extrapyramidal system

Alzheimer’s disease: cerebral cortex (higher order association cortices and limbic system)

Huntington’s disease: extrapyramidal system

Amyotrophic lateral sclerosis: pyramidal system

33
Q

Understand how misfolded or aggregates of proteins lead to neurodegenerative diseases

A

• Misfolded and/or aggregated proteins
– Common cellular hallmark in many degenerative diseases

Resistant to normal degradation processes (ubiquitin- proteosome system)

Often form inclusions (traditionally used to diagnose disease at autopsy)

Cytotoxic to neuron +/or marker of disease presence

34
Q

Examples of neurodegenerative disease d/t misfolded proteins or aggregates

A

• Alzheimer’s disease: amyloid-B and tau

  • Parkinson’s disease: alpha-synuclein
  • Frontotemporal lobar degeneration: tau, ubiquitin, TDP-43

• Huntington’s disease: polyglutamine

35
Q

Forms of neurodegen diseases are sporadic and familial with ____ being more common especially in Alzeihmers, Parkinsons, and AML while ___ more common in Huntingtons

A

sporadic

familial

36
Q

What is useful about familial degenrateive disease when we study them?

A

amilial forms of disease
– Genetic linkage studies make candidate genes and proteins easier

to identify

– Aid in deciphering mechanisms (often applicable to both sporadic and familial disease types)

– Aid in development of animal models (transgenic)

37
Q

Etiology of Neurodegen disease

A

– Genetic mutations

– Geneticpolymorphisms(risk factors)

– Aging
– Environmental toxins

38
Q

Understand list of cellular mechanisms responsible for pathogenesis seen in neurodegenerateive disease

A

– Oxidative stress and generation of ROS

– Inflammation

– Disruption of axonal transport and synaptic function

– Dysfunctional waste clearance: Inhibition of ubiquitin proteosome system and Autophagy

– Mitochondrial dysfunction

– Programmed cell death (apoptosis)

39
Q

Some neurons are more vulnerable to toxic insults than others: _______

This is true even when the gene responsible is widespread (eg. Huntington’s Disease)

These basic observations along with other data have lead to the idea that neurodegeneration occurs because of :

A

“selective vulnerability”

a combination of events, not a single factor

40
Q

How do Envi toxins/neuronal metabolism/aging contribute to neurodegenerative disease?

A
41
Q

Insults that can induce neurodegeneration

A

** Environmental toxins**

– Reduce mitochondrial function; decrease ATP production, increased oxidative stress

• Neuronal metabolism– Is oxidative

• Aging
– Affects mitochondrial function
– L_oss of protective e_nzymes and molecules – _Progressive hits _

42
Q

Describe how free radical oxidation leads to neurodegenerative disease

A

Can arise from dysfunctional mitochondria

Toxins, aging lead to loss of mitochondrial function

Inefficient mitochondrial electron transport “leaks out” electrons; oxygen radicals

In particular, complex 1 is vulnerable to injury in response to free radicals

Free radicals cause **lipid peroxidation; loss of membrane integrity **

43
Q

Describe how oxidative stress plays a role in neurodegenerative disease

A
  • Hydrogen peroxide and superoxide
  • Oxygen is easily converted to these reactive molecules by the same enzymes that utilize oxygen as fuel; “leaky, inefficient”
  • A precipitating factor for both excitoxicity and mitochondrial dysfunction; also results from both excitotoxicity and mitochondrial dysfunction
44
Q

Excess GLUTAMATE and SUPEROXIDE both lead to persistant acitivation of _____

which resluts in _____

A

persistant activation of NMDA receptor

excess intracellular calcium

45
Q

Persistant activaiton of NMDA receptors lead to excess intracellular calcium, why is this an issue?

A

See ATP depletion and which leads to cell death which causes excess glutamate and cycle continues

46
Q

How do cells deal with oxyradicals?

A

•Ascorbate

  • Glutathione
  • Superoxide dismutase
  • Catalase (inactivates hydrogen peroxide)

***Degeneration occurs when the cells either produce too many radicals or l**ose the ability to detoxify them **

47
Q

Autosomal dominant disorder

Progressive motor, cognitive and behavioral domains

Prevalence 4 to 8 per 100, 000 person

Symptoms present in the 4th and 5th decade

Symptoms generally progress over several decades with complete motor disability and dementia approximately 20 years after symptom onset

A

Huntingtons disease

48
Q

– _______ are predominant at the onset of HD in 60% of cases

•_____ being the characteristic feature

A

Motor symptoms

Chorea

49
Q

– _____ symptoms may occur at or before the onset of

motor symptoms in pts with Huntingtons

• As the disease progresses patients eventually become______

–_______ symptoms are exhibited in 98% of patients

• Over 10% of patients with HD have attempted suicide

A

Cognitive

demented

Neuropsychiatric

50
Q

Huntingtons: Mutation on ______

– Expanded trinucleotide repeat in gene (CAG) causes structural abnormalities in huntingtin protein

A

huntingtin gene on chr.4

51
Q

• Higher number of repeats associated with early age of onset

_________: earlier age of onset in subsequent generations, found when mutated gene passed to offspring from father

A

• Anticipation

52
Q

Role of Huntingtin gene

A

– Function of Huntingtin protein is unknown

– Hypothesized that gene expansion causes a toxic gain of function in the huntingtin protein

53
Q

What happens when huntingtin gene is mutated?

A

Mutation causes protein aggregation
• Formation of intranuclear inclusions in basal ganglia

• Direct pathway to cellular injury is not understood

54
Q

What causes the motor and cognitive changes we see in Huntingtons

A

– Loss of medium striatal neurons in the caudate and putamen (these modulate motor activity): Loss results in increased motor output-chorea

– Neuronal loss in c_erebral cortex – cognitive changes _

55
Q

What will we see on MRI in pt with Huntingtons

A

Atrophy of caudate & putamen, ventricular enlargment Mild to moderate atrophy of gyri

56
Q

What is the reason for chorea in pt with Huntingtons

A

Increased inhibition of the subthalamic nucleus

results in decreased excitation to the Globus pallidus and SnPr

overall decrease inhibition to thalamus = OVER stimulation

57
Q

What is recommended for tx of Huntingtons

A

Treat the symptoms, not disease

Focus on depression, delusions and movement control

Fluoxetine for depression

antiphyschotics for delusion + paranoia

Terabenzine for mvmt control

58
Q

Etiology for Amyotropic Lateral sclerosis (ALS)

A

Amyotrophic lateral sclerosis (ALS)

Occurs in all parts of the world, 1-5 cases/100,000

About 23,000 cases in U.S.

Males>females

Typical age of ALS onset is 60 years, rare <35

Most cases are sporadic: only 5-10% of ALS cases are believed to be familial

59
Q

ALS is defined by Widespread degeneration of

A

upper and lower motor neurons

60
Q

Results of Lower motor neuron degeneration seen in ALS

A

Lower motor neuron degeneration:

– Muscle atrophy, weakness and fasciculations (“Amyotrophy”)

– Due to degeneration of the a**nterior horn cells and axons **

61
Q

Features seen in ALS dt

Upper motor neuron degeneration:

A

– Spastic tone, hyperreflexia and Babinski signs

– Degeneration of the cortico spinal tracts in lateral column of spinal

cord (“Lateral sclerosis”)

62
Q

Why do pts with ALS have issues with swallowing and speaking?

A

Bulbar dysfunction (involvement of the brainstem motor cranial nerves): dysarthria, dysphagia

63
Q

Weakness progresses, sensation intact

– Pt. loses ability to move, eat, speak and breathe

A

progression of ALS

64
Q

What do we see on

MRI

LP

in pt with ALS

A

CSF normal

MRI normal

Mean survival 3-5 years (death from respiratory failure)

65
Q

Familial ALS

Genetic mutations: 14 different genes have been described

Most often, ______ inheritance

  • Most common mutation: ________
  • TDP-43 accumulations and gene mutations are also found in a subset of ALS
A

autosomal dominant

superoxide dismutase 1 gene (SOD1)

66
Q

ALS pathogenesis

A

– SOD1 mutations

  • ?Decreased ability to detoxify free radicals
  • ?Misfolded proteins trigger injurious cellular reaction (called unfolded protein response)
  • Environmental agent? (higher incidence in Guam)
67
Q

Pathology of ALS

A

athology

– Anterior (motor) roots of spinal cord are atrophic

– Primary motor cortex (cerebrum) may show atrophy

– Reduced numbers of anterior horn cells

– Loss of corticospinal tract axons and myelin

– Brainstem motor cranial nerves may be affected (Hypoglossal, motor trigeminal, etc.)

68
Q

What would look different in gross pathology of pt with ALS in spinal cord?

A

ATROPHIC ANTERIOR SPINAL ROOTS (MOTOR ROOTS)

69
Q

What do we see on histology in pt with ALS in cross section of spinal cord?

A

Loss of anterior horn cells and degeneration of lateral corticospinal columns

(loss of anterior corticospinal tract)

70
Q

MOA for Riluzole

A

Riluzole

Mechanism: inhibitor of NMDA channels, inhibits glutamate release; increases uptake

Has modest but genuine effects on ALS Increases life span 2-3 months

71
Q

What drug inhibits NMDA channels to inhibit glutamate release and inhibit uptake to increase lifespan of ALS pts for up to 2-3 months

A

Riluzole

Neuropysch (18 decks)

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