BG Nuerophys and Disorders

Question 1

What are the primary movement disorders of the BG

Answer 1

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

Question 2

What composes the BG

Answer 2

Caudate nucleus
Putamen
GP
w/some STN and substantia nigra

Question 3

T or F: BG has direct output to motor units

Answer 3

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

Question 4

What is SNc

Answer 4

Substantia nigra pars compacta

Question 5

What is SNr

Answer 5

Substantia nigra pars reticulata

Question 6

What is a flow through the cortex and BG?

Answer 6

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

Question 7

What composes the striatum

Answer 7

Caudate nucleus and the putamen

Question 8

__ is Faciliatory/Inhibitory from x?

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

Answer 8

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

Question 9

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

Answer 9

Faciliatory

Question 10

What drives the excitability of the cortex?

Answer 10

THE THALAMUS

Question 11

What could cause a hyperkinetic movement disorder?

Answer 11

[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

Question 12

What could cause a hypokinetic movement disorder

Answer 12

[Increased inhibition of thal]

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

Question 13

What is the prevalence of Huntington’s Disease?

Answer 13

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

Question 14

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

Answer 14

after 30 yrs dx

death 15-20 yr later

Question 15

What type of disease is HD

Answer 15

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

Question 16

What is the neurophys of HD?

Answer 16

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

Question 17

What are symptoms of HD (List)

Answer 17

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

Question 18

What are chorea/choreiform movements

Answer 18

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

Question 19

When in HD are akinesia and bradykinesia evident

Answer 19

Later stages or w/too high dose of med

Question 20

What vision changes occur w/HD

Answer 20

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

Question 21

What cognitive problems occur in HD

Answer 21

Poor memory and judgement
Impulsive
Slow processing

Question 22

What psychosocial problems occur in HD

Answer 22

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

Question 23

What is the Unified HD Rating Scale? (UHDRS)

Answer 23

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

Question 24

Define dystonia

Answer 24

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

Question 25

What is the duration of dystonia?

Answer 25

Few seconds, minutes, hours or longer

Question 26

T or F: Dystonia is not painful

Answer 26

False - it can be very painful

Question 27

What part of the body is influenced w/_ dystonia? a) Generalized b) Focal

Answer 27

a) Full body | b) One jt or few related joints

Question 28

List some types of Focal Dystonia

Answer 28

Pharyngeal Cervical Writer's Cramp Musician's Cramp

Question 29

Pharyngeal dystonia

Answer 29

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

Question 30

Cervical dystonia

Answer 30

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

Question 31

What is the most common dystonia

Answer 31

Cervical dystonia

Question 32

Writer's cramp

Answer 32

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

Question 33

Musician's cramp

Answer 33

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

Question 34

What is the onset and progression of Generalized dystonia

Answer 34

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

Question 35

What is the onset and progression of Focal dystonia

Answer 35

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

Question 36

What are some treatment of generalized dystonia?

Answer 36

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

Question 37

What is a treatment of focal distonia?

Answer 37

Botox that is effective for 3-4 mo

Question 38

Define Tourette's syndrome (TS)

Answer 38

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

Question 39

What s/s must be present for TS

Answer 39

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

Question 40

List some simple motor tics in TS

Answer 40

``` Eye blinking Facial grimacing Jaw mvmts Head mvmts Shldr shrugging Neck Stretching Arm jerking ```

Question 41

What are complex motor tics in TS

Answer 41

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

Question 42

What are simple vocal/phonic tics in TS

Answer 42

``` Sniffing Throat clearing Grunting Hooting Shouting ```

Question 43

What are complex vocal tics in TS

Answer 43

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

Question 44

What is the progression of TS

Answer 44

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

Question 45

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

Answer 45

True - a minority of people continually have tics into adulthood

Question 46

What is the prevalence of TS

Answer 46

1/160 kids b/t 5-17 in US | = 300,000 kids in US

Question 47

Is TS more common in boys or girls?

Answer 47

3-4x more likely in boys