Movement Disorders

Question 1

Movement disorders

Answer 1

group of diseases that primarily involve subcortical brain structures that are part of the extrapyramidal motor system, including the basal ganglia (caudate, putamen, and globus pallidus), thalamic and subthalamic nucleus, and substantia nigra, and their interconnections to each other and cortex

extrapyramidal motor system regulates movement and maintains muscle tone and posture.

Question 2

Three primary neurotransmitters of the basal ganglia that contribute to symptomatology of movement disorders

Answer 2

Dopamine (inhibitory)
acetylcholine (excitatory)
gamma-aminobutyric acid (GABA; inhibitory)

Question 3

Parkinson’s Disease

Answer 3

neurodegenerative disorder characterized by:

parkinsonism (rest tremors-most common and generally asymmetrical, slowness/bradykinesia, rigidity)

neuronal loss in the substantia nigra,

dopamine depletion in the striatum (and a clear and dramatic beneficial response to dopaminergic therapy)

frontal subcortical circuitry dysfunction.

Question 4

Risk factors for Parkinson’s Disease

Answer 4

viral encephalitis,
drugs with dopamine antagonistic properties (neuroleptics),
toxic substances, exposure to herbicides, pesticides, and heavy metals drinking of contaminated well water

Question 5

Medication for Parkinson’s Disease

Answer 5

levodopa, dopamine agonists, and
MAO-B inhibitors.

Question 6

Nonpharmacological Interventions for Parkinson’s disease

Answer 6

Physical exercise (especially exercises designed to improve balance,
flexibility, and strength),

cued exercises with visual (mirror),
voice therapy,
speech/swallowing therapy,

Deep brain stimulation (DBS) -
Best candidates have severe motor symptoms in the off-medication condition; targets include subthalamic nucleus or globus pallidus as the most common sites, although
ventrointermedius (VIM) thalamic nucleus has clear effect on tremor; dementia is usually a
contraindication for DBS.

Question 7

neuropsychological expectations for Parkinson’s disease

Answer 7

Impaired: complex attention, working memory, processing speed/EF, initial memory

Language: dysarthria and reduced speech output; comprehension, syntax, and grammar intact

Visuospatial: micrographia, difficulties with constructional praxis

Depression and anxiety common

Question 8

Huntington’s Disease

Answer 8

fatal autosomal dominant neurodegenerative disorder

CAG repeats on chromosome 4 - can be diagnosed by genetic testing

Symptoms: chorea (jerky involuntary movements), dystonia, tic, incoordination, hypokinesia, saccades

Mean age at onset between 30–50

apathy

Disease duration is 15–20 years. Causes of death include pneumonia (related to dysphagia), heart disease, and suicide

Question 9

Neuropathology of Huntington’s disease

Answer 9

loss of cells caudate nucleus and putamen.

As the disease progresses, further degeneration is observed in erebral cortex, thalamus, pallidum, brainstem, and cerebellum.

Question 10

Medication for Huntington’s disease

Answer 10

Chorea may be treated with tetrabenazine (TBZ), a dopamine-depleting agent.

botulinum toxin injections for muscle spasms/spasticity in later stage of HD

SSRIs may be helpful for depression and anxiety but increase for suicide

Question 11

Huntington’s disease neuropsychological outcomes

Answer 11

Impaired early:
visual attention,
processing speed,
visuospatial integration,
deficits in learning but recognition intact until middle/late stages,
working memory/EF severely impaired

Depression, anxiety, psychosis, OCD

Question 12

Lewy body disease (LBD)

Answer 12

progressive neurodegenerative disorder that is characterized by parkinsonism and cognitive decline/dementia.

Mean age of onset 50s-80s

Responsible for 20% or more of all dementias, second to AD.

Question 13

Lewy body disease (LBD) neuropathology

Answer 13

Lewy bodies - abnormal aggregates of protein that form within nerve cells and displace other cell components;
alpha-synuclein is the primary component)

Lewy neuritis (LN; alpha-synuclein cytoplasmic inclusion
bodies) in the cortex and brainstem

Question 14

Medication for Lewy body disease (LBD)

Answer 14

• Treatment of motor symptoms with levodopa can aggravate hallucinations.
• Treatment with dopamine agonists can result in compulsive behaviors

Question 15

Lewy body disease/dementia course

Answer 15

slow progression, but with frequent fluctuations whereby the patient may have episodes of marked confusion/attention/alertness interspersed with periods of complete lucidity.

Question 16

Lewy body disease/dementia neuropsychological expectations

Answer 16

Impaired early: attention, processing speed, EF, migrographia

marked impairment in visuospatial and constructional abilities

Well-formed visual hallucinations, delusions (e.g., Capgras syndrome)

Question 17

Progressive Supranuclear Palsy

Answer 17

progressive neurodegenerative disorder that represents the most common Parkinson’s plus syndrome

• Onset typically in the 60s, can occur in 40s.
• Dementia occurs in 50–80% of cases.

involves erosion of subcortical structures, as well as of subcortical-cortical connections (primarily to the prefrontal cortex) leading to:

** vertical gaze palsy (particularly downward gaze)** (hallmark)
* axial rigidity (neck and trunk - this is different from Parkinsons that affects limbs)
* postural instability with falls (often backward)
* wide-eye stare

other common symptoms:

• dysarthria
• dysphagia
• bradykinesia
• gait disorder (apraxia)
• cognitive impairment
• behavioral changes (apathy, depression, anxiety, pseudobulbar affect, neurobehavioral symptoms)

Question 18

Neuropathology of Progressive Supranuclear Palsy

Answer 18

• tau protein deposits.
• Dopamine depletion in the substantia nigra, caudate, and putamen.
• Neuronal loss and gliosis in the globus pallidus, subthalamic nuclei, red nuclei, dentate nucleus, superior colliculi, and periaqueductal gray matter.
• Neurofibrillary tangles and neuropil threads in the basal ganglia, brainstem

Question 19

Progressive Supranuclear Palsy neuropsychological outcomes

Answer 19

Impairment early:

attention, processing speed, EF, word list generation

depression, anxiety, pseudobulbar affect, wide-eye stare

Question 20

Tourette Syndrome

Answer 20

characterized by repetitive, stereotyped involuntary movements and vocalizations called tics, generally proceeded by a premonitory urge or sensation.

Age of onset is usually between 5 to 7 years, with symptoms peaking around 10–12 years of age

prevalence three to four times higher in males than females.

Tics usually progress from simple to complex and worsen during the teenage years, followed by improvement in adulthood,

Tics are thought to be a disorder of the basal ganglia/cortical brain circuitry, though the exact pathophysiology is unknown.

Question 21

Torette’s syndrome comorbidities

Answer 21

ADHD
learning disabilities
obsessive-compulsive disorder
rage attacks
depression
generalized anxiety
panic attacks
sleep disorders
migraines

Question 22

Onset of Parkinsonism and cognitive dysfunction in close proximity occurs in which disorder?

Answer 22

Lewy body dementia

Co-occurring (within 1 year) cognitive and motor symptoms are most common in LBD and are, in fact, part of the diagnostic criteria. Dementia is inevitable in LBD.

Question 23

What is a core psychiatric symptom in individuals with Huntington’s disease?

Answer 23

Apathy is the most prevalent neuropsychiatric feature of HD, and is primarily associated frontal- striatal circuit damage.

Question 24

Cognitive dysfunction in Parkinson’s disease tends to be higher in individuals who

Answer 24

do not have tremor at initial presentation

Older age of onset and initial symptoms other than tremor are risk factors for dementia in PD

Question 25

Function of cerebellum

Answer 25

Balance and posture, and accuracy of movement

Question 26

Motor system components and functions

Answer 26

Motor cortex - commands
Brainstorm and spinal cord - movements
Basal ganglia - force
Cerebellum - accuracy

Question 27

asymmetric motor dysfunction in older adults can be contributed to which diseases?

Answer 27

Motor symptoms are generally greater on one side for both Parkinson’ s disease and corticobasal syndrome; however, patients with corticobasal syndrome do not have a strong immediate response to levodopa.

Question 28

Neuroanatomy and neurotransmitter involved in Parkinson’s

Answer 28

substantia nigra – dopamine

Question 29

Neuroanatomy and neurotransmitter involved in Huntington’s

Answer 29

caudate nucleus and putamen – GABA

Question 30

Time from diagnosis to death is longest of the movement disorders?

Answer 30

Parkinson’s disease

Question 31

Which movement disorder/disease is most likely to result in dementia?

Answer 31

Huntington’s

Question 32

Which type of dementia is most likely to show retrieval rather than retention deficits?

Answer 32

Parkinson’s

Question 33

Which tremor is associated with Parkinsonism?

Answer 33

resting

Question 34

Patient’s with Parkinson’s disease do not show symptoms until they have lost most of the dopamine-containing axons from the substantial nigra is that

Answer 34

the receptors on the postsynaptic membranes develop denervation super sensitivity (the sharp increase of sensitivity of postsynaptic membranes to a chemical transmitter after denervation. It is a compensatory change)

Question 35

Deep brain stimulation for Parkinson’s disease

Answer 35

Highly effective in treating rigidity, but can negatively impact processing speed and EF

Question 36

A child born to a parent with Huntington has a ??% chance of inheriting the disease

Answer 36

50%

Question 37

What distinguishes Parkinson’s disease from atypical Parkinsonism?

Answer 37

PD responds to levodopa whereas atypical Parkinsonism does not

Question 38

Difference in symptoms between Progressive Supranuclear Palsy and Parkinson’s

Answer 38

PSP - more neck & trunk rigidity than limbs (Parkinson’s)

Question 39

Difference between essential tremor and Parkinson’s

Answer 39

Essential tremors
- tremors occur when you use your hand
- not usually associated with other neurological signs
- affects mainly hands, head, and voice

Parkinson’s
- tremors occur most when your hands are at your sides or resting in your lap
- associated with stooped postures, slow movement, and dragging feet when walking
- affects hands, legs, and other body parts