review part 1 (test 3) Flashcards by John Goodman

What are the neuclei of the basal ganglion?

Caudate

Putamen

Globus pallidus (external and internal)

Subthalamic nuclei

Substantia nigra (pars compacta and pars reticulata)

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What nuclei make up the striatum?

caudate and the putamen

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Describe the direct pathway of the basal ganglia

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

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Describe the indirect pathway of the basal ganglia

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

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

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

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

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

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

TRAPS (First Aid mnemonic)

Tremor (usually resting tremor)

Rigidity (cog-wheel rigidity)

Akinesia/bradykinesia (hypophonia, hypomimia, micrographia)

Postural instability

Shuffling gait

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Are smoking and caffeine protective factors against parkinson’s?

yes

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How do you treat parkinson’s?

levadopa + carbadopa

pramipexole, ropinirole (D2 receptor agonists)

selegiline, rasagiline (MAO-B inhibitors)

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

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

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What is the clinical triad of Huntington’s disease?

Movement disorder

Chorea

Psychiatric symptoms

depression, irritability, psychosis

Cognitive decline

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What is huntington’s chorea typicillay treated with?

tetrabenazine

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

40 CAG repeats

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What is hemiballismus caused by?

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

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

truncal ataxia

titubation (nodding movement of the head or body)

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

appendicular ataxia

dysmetria

dysdiadochokinesia

Define hyperkinetic and hypokinetic

hyperkinetic is characterized by excessive movement

hypokinetic is characterized by bradykinesia (slowness of movement)

What is a resting tremor?

Tremor that occurs when affected body part fully supported and relaxed

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

Broad term that encompasses a tremor with movement

postural tremor, kinetic tremor, and intention tremor

What is a postural tremor?

essential tremor

Occurs when muscles activated against gravity w/o movement

What is a kinetic tremor?

type of essential tremor

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

What is an intention tremor?

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)

How do you treat an essential tremor?

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

EtOH improves the tremor

How is dystonia characterized?

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

What are tics?

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

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

Defined as a tic disorder \> 1year in duration combination of both motor and vocal tics

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

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

How does Wilson's disease present?

asteristics dementia hemolytic anemia dyskinesia Kayser-Fleischer rings cirrhosis --\> HCC

How is Wilson's disease treated?

D-peniclillamine and zinc (block copper uptake)

What is multiple sclerosis?

demyelinating disease that affects the CNS

What are the symptoms of multiple sclerosis

**UNSULARO** INSULAR: **I**ntention tremor/**i**nternuclear ophtalmoplegia **N**ystagmus **S**canning/**s**lurred speech **U**thoff'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 **L**hermitte's sign-flexing of neck produces electric shock sensation running down the spine **A**taxia **R**ebound/**r**elapse **O**ptic neuritis

Is optic neuritis a common presentation in patients with MS?

yeah

What are the 4 different subtypes of MS?

How is a diagnosis of MS made?

diagnosis is made through space and time MRI shows 2 lesions affecting 2 different parts of the CNS that occured at different times

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

oligoclonal IgG in CSF (on electrophoresis)

How do you treat an acute MS relapse?

IV solu-methylprednisolone (corticosteroids)

What are the MS drugs?

corticosteroids interferon beta (Avonex and betaseron) dimethyl fumerate teriflunomide fingolimod mitoxantrone natalizumab glatiramir acetate

Which MS drug is safe for pregnancy?

glatiramer acetate