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UM - Neuro Conditions > BG Nuerophys and Disorders > Flashcards

BG Nuerophys and Disorders Flashcards

BG Neurophysiology, disease, and disorders [Huntington's, dystonia, Tourette's]

1
Q

What are the primary movement disorders of the BG

A

Hypokinesia (PD)
Hyperkinesia (Huntington’s disease, tourette’s, dystonia)
Dyskinesia

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2
Q

What composes the BG

A

Caudate nucleus
Putamen
GP
w/some STN and substantia nigra

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3
Q

T or F: BG has direct output to motor units

A

FALSE ! It is always through the VL of the thalamus then to the the motor cerebral cortex

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4
Q

What is SNc

A

Substantia nigra pars compacta

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5
Q

What is SNr

A

Substantia nigra pars reticulata

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6
Q

What is a flow through the cortex and BG?

A

Cortex –> Striatum (Caudate and Putamen) –> [STN, LGP/EGP, MGP/IGP, SNr, SNc) –> VL of thal –> Cortex

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7
Q

What composes the striatum

A

Caudate nucleus and the putamen

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8
Q

__ is Faciliatory/Inhibitory from x?

a) GLU (glutamate)
b) GABA
c) dopamine (DA)

A

a) Faciliatory input from STN and cortex
b) Inhibitory to Thalamus
c) can be either

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9
Q

Output from the thalamus is (inhibitory/faciliatory) to the cortex

A

Faciliatory

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10
Q

What drives the excitability of the cortex?

A

THE THALAMUS

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11
Q

What could cause a hyperkinetic movement disorder?

A

[Decreased inhibition of thal]

  • Too little GABA to inhibit the thalamus causing over facilitation of the cortex
  • Too little glutamate to cause the release of GABA
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12
Q

What could cause a hypokinetic movement disorder

A

[Increased inhibition of thal]

- Too much GABA causing inhibition of the VL thalamus to dec facilitation of the cortex

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13
Q

What is the prevalence of Huntington’s Disease?

A

6.5/100,000 but highest in Mauritius at 1/2100

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14
Q

What is the typical onset and course of Huntington’s?

A

after 30 yrs dx

death 15-20 yr later

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15
Q

What type of disease is HD

A

Hyperkinetic Autosomal dominant disease on the short arm of chromosome 4 (too many CAG repeats)

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16
Q

What is the neurophys of HD?

A

Degeneration of SNr and LGP leading to dec GABA (underinhibition of thal-> overfacil of cortex) and increased DA and norepinephrine

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17
Q

What are symptoms of HD (List)

A 
Chorea/Choriform mvmts
Akinesia & Bradykinesia
Gait variations
Dysarthria
Dysphagia
Vision problems
Cognitive problems
Psychosocil problems
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18
Q

What are chorea/choreiform movements

A

Involuntary, purposeless, non-repetitive, small and/or large A mvmts

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19
Q

When in HD are akinesia and bradykinesia evident

A

Later stages or w/too high dose of med

20
Q

What vision changes occur w/HD

A

Poor control of saccade mvmts, target undershooting, latency in mvmt initiation

21
Q

What cognitive problems occur in HD

A

Poor memory and judgement
Impulsive
Slow processing

22
Q

What psychosocial problems occur in HD

A

Personality changes, mood swings, aggression, irritability, depression, apathy, suicidal ideation

23
Q

What is the Unified HD Rating Scale? (UHDRS)

A 
A multicomponent way to stage HD that includes:
Motor
Cognition
Behaviour
Independence
Functional activities
Total Functional capacity
24
Q

Define dystonia

A

Hyperkinetic disorder w/Abnormal mm tone w/simultaneous contraction of agonist and antagonist to cause contorted or twisted postures and can occur w/task-specific activities

25
Q

What is the duration of dystonia?

A

Few seconds, minutes, hours or longer

26
Q

T or F: Dystonia is not painful

A

False - it can be very painful

27
Q

What part of the body is influenced w/_ dystonia?

a) Generalized
b) Focal

A

a) Full body

b) One jt or few related joints

28
Q

List some types of Focal Dystonia

A

Pharyngeal
Cervical
Writer’s Cramp
Musician’s Cramp

29
Q

Pharyngeal dystonia

A

Distorts speech via involuntary contract of vocal chords and can be treated w/botox

30
Q

Cervical dystonia

A

MOST COMMON!

Hypertrophy of SCM to cause invol contraction of neck causing Lat fwd flex w/rot away

31
Q

What is the most common dystonia

A

Cervical dystonia

32
Q

Writer’s cramp

A

A task-specific focal dystonia that causes a spasm to affect certain mm of the hand/fingers

33
Q

Musician’s cramp

A

A task-specific focal dystonia that causes spasms of certain mm of hand/fingers

34
Q

What is the onset and progression of Generalized dystonia

A

About 8 yo that is a chronic progressive, and debilitating disease

35
Q

What is the onset and progression of Focal dystonia

A

About 30-50 yo for onset that is also chronic, progressive and may plateau after 5 years

36
Q

What are some treatment of generalized dystonia?

A

Meds: Baclofen, Artane, Sinemet, Klonopin
Sx: DBS of GP or Rhizotomy

37
Q

What is a treatment of focal distonia?

A

Botox that is effective for 3-4 mo

38
Q

Define Tourette’s syndrome (TS)

A

Hyperkinetic disorder w/Impulsions and compulsions to perform fragments of motor programs

39
Q

What s/s must be present for TS

A

> = 2 motor ticks
= 1 vocal/phonic tic
In some combo over the course of more than a year

40
Q

List some simple motor tics in TS

A 
Eye blinking
Facial grimacing
Jaw mvmts
Head mvmts
Shldr shrugging
Neck Stretching
Arm jerking
41
Q

What are complex motor tics in TS

A

Involving multiple mm groups or combo of mvmts and are more purposeful (hopping, twirling, jumping, skipping)

42
Q

What are simple vocal/phonic tics in TS

A 
Sniffing
Throat clearing
Grunting
Hooting
Shouting
43
Q

What are complex vocal tics in TS

A

Words or phrases that are not recognizable but occur out of context
Words could be inappropriate

44
Q

What is the progression of TS

A

Tics emerge b/t 5-7 yo most often as a motor tic
Severity inc at 8-12 yo
Late adolescence is noticeable improvement

45
Q

T or F: Tics can disappear in adulthood w/TS

A

True - a minority of people continually have tics into adulthood

46
Q

What is the prevalence of TS

A

1/160 kids b/t 5-17 in US

= 300,000 kids in US

47
Q

Is TS more common in boys or girls?

A

3-4x more likely in boys

UM - Neuro Conditions (12 decks)

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