review part 1 (test 3)

Question 1

What are the neuclei of the basal ganglion?

Answer 1

Caudate

Putamen

Globus pallidus (external and internal)

Subthalamic nuclei

Substantia nigra (pars compacta and pars reticulata)

Question 2

What nuclei make up the striatum?

Answer 2

caudate and the putamen

Question 3

Describe the direct pathway of the basal ganglia

Answer 3

1) Cortex neurons excite neurons in the striatum via the release of glutamate
2) Striatum neurons inhibit neurons in the internal globus pallidus and substantia nigra pars reticulata
3) Inhibited neurons in GPi and SNR cannot inhibit thalamic neurons
4) Thalamic neurons excite neurons in the cortex to allow for movement

Question 4

Describe the indirect pathway of the basal ganglia

Answer 4

1) Cortex excite neurons in the striatum via glutamate
2) Neurons in the striatum inhibit neurons in the external globus pallidus via GABA
3) Inhibited GPe neurons cannot inhibit the subthalamic nucleus
4) Subthalamic nucleis excite the internal globus pallidus and substantia nigra reticulata nuclei va glutamate
5) GPi and SNR inhibit neurons in the thalamus via GABA
6) Inhibited thalamus cannot stimulate cortex
7) No movement

Question 5

What part of the substantia nigra synthesizes dopamine? How does dopamine levels influence the internal and external basal ganglia pathways?

Answer 5

Substantia nigra pars compacta synthesizes dopamine

direct pathway cells in the striatum have D1 (excitatory) receptors and indirect cells in the striatum have D2 (inhibitory). Increased levels of dopamine favors the direct pathway and inhibits the indirect pathway. Low levels of dopamine favors the indirect pathway

Question 6

Parkinson’s is the destruction of cells in the _______. This results in overactivation of the ________ (direct/indirect) pathway.

Answer 6

Parkinson’s is the destruction of cells in thesubstantia nigra pars compacta. This results in overactivation of the ________ indirect pathway.

Question 7

What are the cardinal motor features of parkinson’s disease?

Answer 7

TRAPS (First Aid mnemonic)

Tremor (usually resting tremor)

Rigidity (cog-wheel rigidity)

Akinesia/bradykinesia (hypophonia, hypomimia, micrographia)

Postural instability

Shuffling gait

Question 8

Are smoking and caffeine protective factors against parkinson’s?

Answer 8

yes

Question 9

How do you treat parkinson’s?

Answer 9

levadopa + carbadopa

pramipexole, ropinirole (D2 receptor agonists)

selegiline, rasagiline (MAO-B inhibitors)

Question 10

Huntington’s disease is the destruction of cells in the ________ that control the ________ (direct/indirect) pathway.

Answer 10

Huntington’s disease is the destruction of cells in the striatum (caudate) that control the indirect pathway.

Question 11

What is the clinical triad of Huntington’s disease?

Answer 11

Movement disorder

• Chorea

Psychiatric symptoms

• depression, irritability, psychosis

Cognitive decline

Question 12

What is huntington’s chorea typicillay treated with?

Answer 12

tetrabenazine

Question 13

how many trinucleotide repeats are required for huntington’s to be 100% penetrant?

Answer 13

40 CAG repeats

Question 14

What is hemiballismus caused by?

Answer 14

Damage to subthalamic nucleus → hemiballismus (too much unwanted movement)

Pathophysiology: not enough stimulation of the substantia nigra pars reticularis, which tells the thalamus to stop stimulating the cortex → too much cortical activation via direct pathway

Question 15

What symptoms would you get if you damaged the cerebellar vermis?

Answer 15

truncal ataxia

titubation (nodding movement of the head or body)

Question 16

What symptoms would you get if you damaged the cerebellar hemispheres?

Answer 16

appendicular ataxia

dysmetria

dysdiadochokinesia

Question 17

Define hyperkinetic and hypokinetic

Answer 17

hyperkinetic is characterized by excessive movement

hypokinetic is characterized by bradykinesia (slowness of movement)

Question 18

What is a resting tremor?

Answer 18

Tremor that occurs when affected body part fully supported and relaxed

Question 19

What is an essential tremor? What are the 3 types?

Answer 19

Broad term that encompasses a tremor with movement

postural tremor, kinetic tremor, and intention tremor

Question 20

What is a postural tremor?

Answer 20

essential tremor

Occurs when muscles activated against gravity w/o movement

Question 21

What is a kinetic tremor?

Answer 21

type of essential tremor

Tremor that occurs with movement of a body part (finger-to-nose test)

Question 22

What is an intention tremor?

Answer 22

type of essential tremor

tremor that occus near the target (tremor begins when the finger gets close to touching the nose or the finger in the finger-to-nose test)

Question 23

How do you treat an essential tremor?

Answer 23

propranolol, primidone (GABA agonist), topiramate, deep brain stimulation

EtOH improves the tremor

Question 24

How is dystonia characterized?

Answer 24

dystonia is sustained muscle contractions that frequently cause twisting and repetitive movements or abnormal postures

Question 25

What are tics?

Answer 25

recurrent, intermittent, stereotyped, semi-voluntary movements or sounds associated with an urge

Question 26

What is tourette syndrome? Are motor and vocal tics involved?

Answer 26

Defined as a tic disorder > 1year in duration

combination of both motor and vocal tics

Question 27

What is gene mutation in Wilson’s disease? What will serum copper, serum ceruloplasmin, 24 urine copper, and liver copper show (increase/decrease?

Answer 27

ATB7B gene mutation —> messed up copper transporter pump —> inability to put copper into bile —> copper builds up in brain, liver, corneas, kidneys

Question 28

How does Wilson’s disease present?

Answer 28

asteristics

dementia

hemolytic anemia

dyskinesia

Kayser-Fleischer rings

cirrhosis –> HCC

Question 29

How is Wilson’s disease treated?

Answer 29

D-peniclillamine and zinc (block copper uptake)

Question 30

What is multiple sclerosis?

Answer 30

demyelinating disease that affects the CNS

Question 31

What are the symptoms of multiple sclerosis

Answer 31

UNSULARO

INSULAR:
Intention tremor/internuclear ophtalmoplegia
Nystagmus
Scanning/slurred speech
Uthoff’s phenomenon=Uhtoff’s phenomenon describes the well known finding of a temporary production or worsening of neurological symptoms in MS patients exposed to heat, such as that from a hot shower, exercise, or a warm climate. This is thought to be due to the effect of heat on myelin (insulation sheath or covering of nerves) which results in abnormal transmission of nerve impulses
Lhermitte’s sign-flexing of neck produces electric shock sensation running down
the spine
Ataxia
Rebound/relapse

Optic neuritis

Question 32

Is optic neuritis a common presentation in patients with MS?

Answer 32

yeah

Question 33

What are the 4 different subtypes of MS?

Answer 33

Question 34

How is a diagnosis of MS made?

Answer 34

diagnosis is made through space and time

MRI shows 2 lesions affecting 2 different parts of the CNS that occured at different times

Question 35

What would you expect to find in the CSF of a MS patient?

Answer 35

oligoclonal IgG in CSF (on electrophoresis)

Question 36

How do you treat an acute MS relapse?

Answer 36

IV solu-methylprednisolone (corticosteroids)

Question 37

What are the MS drugs?

Answer 37

corticosteroids

interferon beta (Avonex and betaseron)

dimethyl fumerate

teriflunomide

fingolimod

mitoxantrone

natalizumab

glatiramir acetate

Question 38

Which MS drug is safe for pregnancy?

Answer 38

glatiramer acetate