Movement Disorders

Question 1

Criteria for Parkinson’s disease

Answer 1

Brady kinesia
resting tremor
rigidity

Question 2

Which parts of the brain are involved with movement disorders

Answer 2

Basal ganglia which includes caudate nucleus, putamen, Globus pallidus, subthalamic nucleus, substantial nigra, thalamus

Question 3

Is movement disorder related to perimeter or extra pyramidal system

Answer 3

It is related to extrapyramidal system

Question 4

What are the neurotransmitters of the basal ganglia that contribute to movement disorder symptoms

Answer 4

Dopamine inhibitory, GABA inhibitory, acetylcholine excitatory

DAG

Question 5

Parkinson’s disease is characterized by

Answer 5

Parkinsonism
Neuronal loss in the substantial nigra
Dopamine depletion in the stratum
Responsiveness to dopaminergic treatment
Presence of Lewy bodies
Front of critical circuitry dysfunction

Question 6

Neuropathology of Parkinson’s disease

Answer 6

Dopaminergic neuronal loss in the substantial nigra

Dopaminergic deneevation of the stratum

affects ventral lateral cells in the group

Lewy body and lewy neurites in the brainstem

Alpha sin nuclein pathology outside the nigrostriatal system affecting symptoms such as psychiatric, cognitive dysfunction, autonomic deficiencies, sensory disturbance, pain, sleep

Question 7

Most cases of Parkinson’s disease are

Answer 7

Sporadic and 90% of cases

10% of the cases are genetics

Question 8

Risk factors for Parkinson’s disease

Answer 8

Each increase, Hispanics, being male, viral encephelitis, drugs with dopamine antagonistic properties, neuroleptics, substances, herbicides, pesticides, heavy metals, iron levels, drinking contaminated water, gut dysfunction

Question 9

Medication for Parkinson’s disease

Answer 9

Treating early to middle stages include levodopa, dopamine agonist, MAO B inhibitors

Anticholinergic drugs useful for patients under 70 with tenors

Amantadine for younger patients or later when dyskinesia becomes problematic

DBS treating subthalmic nucleus and Globus pallidus

Question 10

Neuropsych assessment results in Parkinson’s disease

Answer 10

Poor attention, poor processing speed, dysarthria reduced speech output, hypophonia, micrographia, visual spatial constructional, learning and memory can benefit from cue recognition, difficulty coming up with strategies in remembering things, exocrine functions, flexibility, sensory motor functions, emotional functioning including depression and anxiety,

Question 11

Huntington’s disease

Answer 11

Fetal autosomal dominant Nuro degenerative disorder

CAG trinucleotide repeats on chromosome 4

Earlier onset is inversely associated with CAG repeats

Question 12

Is Huntington’s single or multi system

Answer 12

Huntington’s disease is characterized as a single focal or multi system degenerative disease

Question 13

motor symptoms of Huntington’s disease include the following

Answer 13

Chorea
Hypokinesia
Dystonia
Tic
Incoordination

Other features include
unintended weight loss
Sleep circadian rhythm disturbances
Autonomic nervous system dysfunction

Question 14

Neuropathology of Huntington’s disease

Answer 14

Loss of medium spiny cells seen in caudate nucleus and putamen

Indirect basal ganglia thalamiccortical circuitry

Chorea and motor inpersistence only in early in the disease course

Neurodegeneration in cerebral cortex, thalamus, pallidum, brainstem, cerebellum

With disease progress, further degeneration in striatum circuits, frontal and temporal neo cortical regions

At death, 25 %reduction in brain volume

Question 15

Neuro findings and Huntington’s

Answer 15

Atrophy in caudate and putamen

Box car ventricles

Question 16

Epidemiology of Huntington

Answer 16

Mean onset 30 to 50,
range from 2 to 85 years

gender no difference

Question 17

Morbidity of Huntington

Answer 17

Psychiatric problem
depression high suicide rate
Heart failure in 30%
Weight loss

Disease duration is 15 to 20 years

Pneumonia, heart disease, suicide common causes of death

Question 18

Amount of CAG repeats determine the severity of Huntington’s

Answer 18

Less than 27 of CAG repeats is normal

27 to 35 is intermediate
36 to 40 is reduced
more than 40 is full

Question 19

Presentation and symptoms of Huntington’s

Answer 19

Involuntary, unwanted movements
Initially they occur in distal extremities
Unstable gait
Unwanted movements spread from distal to proximal
Person look slightly intoxicated
Chorea movements present during waking hours
Hard to swallow and talk
Dysarthria and dysphasia prominent
Everyone develops hypokinesia, akinesia, rigidity eventually
Behavioral problems
Irritability, depression, anxiety, obsessive compulsive, psychosis, apathy, suicide
Cognitive symptoms with executive function impairment

Question 20

Why do people with Huntington’s disease have weight loss

Answer 20

Weight loss unintended because of chorea in longer CAG repeat

Question 21

Treatment for Huntington’s

Answer 21

No cure, no way to slow it down, treatment is focused on helping person live comfortably

Question 22

Cognitive changes in Huntington’s disease can be detected how many years prior to dx

Answer 22

15

Question 23

Most sensitive changes cognitively in prodromal period for Huntington’s

Answer 23

Psycho motor and attention issues

Question 24

Juvenile Huntington’s disease what is frequently observed

Answer 24

Seizures, beaver disturbances, learning difficulties, CAG repeats generally greater than 55, having an infected father

Question 25

Lewy body dementia

Answer 25

Characterized by parkinsonism and cognitive decline

Question 26

Lewy body dementia include three types of dementia

Answer 26

Parkinson’s disease with dementia
Diffuse Lewy body disease
Lewy body variant of Alzheimer’s disease

Question 27

What is the second most common form of dementia after Alzheimer’s disease

Answer 27

Lewy body related dementia

Question 28

 Symptoms of presentation of Lewy body dementia

Answer 28

Fluctuating cognition with variations in alertness and attention
Recurrent visual hallucinations
REM sleep behavior disorder
Features of parkinsonism

Question 29

Survival time is Lewy body dementia

Answer 29

Eight years