Motor Systems IV- Basal Ganglia Flashcards

1
Q

A twenty six year old white mail develops uncontrolled INVOLUNTARY movements of limbs, body and trunk that are progressively getting worse.

He also is losing cognitive function.

His fatehr died of a similar disease but the onset was later in life w/ slower progression of the dieseae.

What is the disease?

A

Huntingtons

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2
Q

What are the 4 major components of the basal ganglia?

A

Neostriatum (INPUTS)

Globus pallidus (OUTPUT)

Substantia nigra

Subthalmic nucleus

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3
Q

What does the neostriatum consist of and what does it do?

A

Consists of caudate and putamen

Receives the major INPUTS

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4
Q

What are the neurons associated with the caudate and putamen and what NT do they use?

It it excitatory or inhibitory?

A

1. Spiny neurons

Receive INPUT and are primary OUTPUT

Two groups of neurons

  • GABA/substance P (SP) group
  • GABA/enkephalin (ENK) group
    2. Aspiny neurons= interneurons

INhibitory

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5
Q

Afferent input to the striatum from OUTSIDE the basal ganglia comes from where?

What NT does it use?

It it excitatory or inhibitory?

A

Cerebral cortex to the straitum = corticostriate pathway

Originates from premotor and primary motor cortex

terminates on spiny neurons in neostriatum

NT: Glutamate

Excitatory projection

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6
Q

Describe the components of the DIRECT pathway projecting to the globus pallidus.

A
  1. Striatal (GABA/SP) neurons project to internal segment of Globus pallidus GPi
  2. Project to VA/VL of thlamus via lenticular fasciculus
  3. These areas project BACK to cortical areas and are the source of the corticostriate pathway (Cerebral cortex –> striatum)

Main OUTPUT projection

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7
Q

Describe the components of the DIRECT pathway projecting to the substantia pars reticulata.

What is the SNr used to control?

A

SNr- part of OUTPUT projection that ctronls eye movements and locomotion

Projects to thalamus (VA/VL)–>

Then to brainstem–>

Superior colliculus for eye movements

Peduncolopontine nuclei for locomotion

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8
Q

Describe the striatum projections via the INDIRECT pathway.

A

GABA/ENK spiniy neurons inhibit the external segment of the globous pallidus (GPe)–>

GPe inhibits (GABA) the subthalamic nucleus (STh)–>

STh excites GPI/SNr

(uses glutamate)

Source of major EXCITATORY input to GPi and SNr

Also recieves input from the cerebral cortex through the hyperdirect pathway (Glut +)

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9
Q

How is the SNc linked to the striatum?

What is it used for?

A

Striatum (GABA/SP) spiny neurons inhibits–>SNc/ SNR

SNc–> spiny neurons

Selection of movements and rewards

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10
Q

What are hte two effects of the SNc when it terminates on spiny neurons?

A
  1. INHIBIT GABA/ENK via D2 receptors
  2. EXCITE GABA/SP neurons via D1 receptors
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11
Q

What are the three major pathways from the cortex to striatum to GPi and what do they do?

A
  1. Direct pathway–facilitates movement

Cortex >+ striatum > - GPi–> - thalamus > + movement

  1. Indirect pathway–suppresses movement

Coretex > + striatum> - GPe > - STh > + GPi > - Thalamus > - movement

  1. Hyperdirect pathway–suppresses movement

Cortex > STh > GPi

GPi when activated is INHIBITORY to the thalamus and DECREASES motor activity.

Motor activity requires DECREASING Gpi activity.

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12
Q

Describe how the basal ganglia circuit is used in eye movements.

A
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13
Q

How does an increase in dopamine affect the striatum?

A
  1. Less excitation of STh on GPi
  2. Increase of inhibition on GPi via putamen

This leads to less GPI inhibitory output to VA/VL–>

MOTOR ACTIVITY

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14
Q

What do subthalamic lesions cause?

A

Hyperkinetic dyskinesia

  1. Ballismus- wild exaggerated movements of the limbs d/t loss of excitatory drive on GPi
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15
Q

A patient present with braykinesia, rigidity, a resting tremor and postural instability. What diesease do they have?

What is the treatment

A

Parkinson’s disease

Resotre dopamine using L-DOPA

This can lead to hyperkinetic movements (involuntary movements)

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16
Q

A lesion of the GPi would cause what symptoms?

A

Flexion dystonia

(sustained muscle contraction d/t an increase in motor activity)

Loss of normal tonic inhibitory activity of GPi on VA/VL–> uncontroled movements

17
Q

A lesion of the putamen (part of the striate) would cause what disorders?

A

Motoric in nature

stereotypic behavior–repeat actions over and over

18
Q

A lesion of the caudate would cause what movement disorders?

A

Caudate= prefrontal cortex/ pre-motor cortex/ SMA

Changes in “complex behvaiors”

Cognitive disorders:

vulgarity, impulsivity

increased apetite, polydipsia

hypersexuality

19
Q

What gene is associated with Huntingtons?

A

AUTOSOMAL DOMINANT

abnormal gene on short arm of chromosome 4

CGA repeat–> gain of function

25-40 years old

20
Q

What is the pathology of Huntingtons?

A

Death of spinyp neurons in neostraitum (pariculary GABA/ENK group)

Loss of cerebral cortical neurons

All of striatum eventually dies

21
Q

What are the symptoms of Huntingtons?

A

Involuntary movements

Dementia/personality changes

akinesia (impaired voluntary movement)

22
Q

How do you treat Huntingtons?

A

Dopamine receptor blockers