Movement Disorders Flashcards
- What are movement disorders caused by? 2
2. What modulates motor cortical activity but also the activity of the association cortex, in the frontal lobes?
- extrapyramidal (basal ganglia)
- cerebellar dysfunction.
- Basal ganglia
Huntington’s Disease
- How is it acquired?
- What does it affect? 3
- Mean onset is at what age?
- What is the mean duration of illness?
- Progressive autosomal dominant
- Affects
- motor function
- cognition
- behavior - Mean onset is age 40
- Mean duration of illness is 20 years
- Huntington’s Disease Pathophysiology?
2. Where is the disease at? 2
- brain cells are wasting away/localized cell death
- Decrease of GABA and GABA receptors in the basal ganglia
- cauda nucleus/putemen of basal ganglia
What is Huntingtons disease characterized by?
3
- Chronic progressive chorea
- Psychological changes
- Dementia
Decrease of GABA and GABA receptors in the basal ganglia is part of the pathophysiology of Huntington’s. What does this cause and why?
GABA is an inhibitory neurotransmitter. So without the GABA theres nothing to stop movement = chorea.
Huntington’s adult onset clinical presentations? 6
Juvenile onset? 4
- Chorea affects the limbs and trunk
- Dystonia- twisting and loss of movement
- Rigidity
- Postural instability
- Myoclonus
- Nystagmus
- Very rare
- Bradykinesia
- Rigidity
- Quicker progression
Early psychological manifestations of Huntington’s Disease?
10
1. Depression Personality changes 2. Memory loss 3. Impulsive behavior- too much 4. dopamine 5. Moodiness 6. Antisocial behavior 7. Emotional outbursts 8. Lack of initiative 9. Loss of spontaneity 10. Inability to concentrate
Early physical signs of Huntingtons? 2
Lateral physical signs of Huntingtons? 5
Early physical signs:
- Fidgeting
- Restlessness
Later physical signs:
- Chorea
- Dystonic posturing
- Progressive rigidity
- Akinesia
- Dementia
Diagnostic Studies for Huntingtons?
- MRI
- PET
- Genetic testing
Huntington’s Disease
What would the following show?
1. MRI?
2. PET?
MRI
1. Caudate atrophy
PET
2. Abnormal metabolic changes in the caudate
Huntington’s Treatment?
3
Treatment of symptoms only, no medication will change the course of Huntington’s
- Tetrabenazine (VAMT Inhibitor)
- Antidopaminergic agents (Phenothiazine, Haloperidol)
- Antipsychotics (Resperidone, Olanzapine)
What is tetrabenazine used to treat in parkinson’s?
dyskinesia
- In Huntingtons what are we mostly treating?
2. What other things are we treating?
- Chorea
- Postural instability
- Rigidity worsen
- Treatment of depression
- Psychosis
- What are the anticonvulsants used for Huntington’s? 2
- What are the antipsychotics used for Huntington’s? 2
- What are the antidepressants used for Huntington’s? 3
- Clonazepam (Klonopin)
- Valproic Acid (Depakote)
- Resperidone (Resperdal)
- Olanzapine (Zyprexa)
- Fluoxetine (Prozac)
- Sertraline (Zoloft)
- Nortriptyline (Aventyl, Pamelor)- Tricyclic antidepressant
What is the risk of TCAs?
2
suicide risk and also really dangerous in a overdose.
Side effects from many of the drugs used to treat the symptoms of Huntington’s disease may include what?
3
- hyper-excitability,
- fatigue
- restlessness.
Antipsychotic drugs may cause side effects that mimic the signs of what disease?
Parkinson’s disease, including involuntary twitching in the face and body (tardive dyskinesia).
What is the most common cause of tremors?
Essential tremors
Essential Tremors
- How is it acquired?
- What are the ranges of presentation?
- Unilateral or Bilateral?
- More prominent with action or with rest?
- Describe the frequency of the tremor?
- Inherited
- Ranges from cosmetic to disabling
- Affect both sides of the body symmetrically**
- More prominent with action than rest**
- Frequency of tremor is constant***
- Essential Tremor affects what parts of the body? 3
- Tremor (increased amplitude) aggravated by? 3
- What relieves essential tumor?
- Describe the onset compared to parkinson’s?
- Neck and head muscles
- Muscles of the voice
- Muscles of the arms and hands
- Stress
- Sleep deprivation
- Stimulants
- ETOH
- Usually starts earlier than parkinson’s
Treatment for essential tumor? 3
Treatment for essential tumor for pts with asthma or bronchospasm? 1
- Propranolol (Inderol) – Beta Blocker
- Mysoline (Primidone) – Anticonvulsant
- Gabapentin (Neurontin) - Anticonvulsant
- Atenolol (Tenormin) – Beta Blocker of choice for those with asthma or bronchospasm
Parkinson’s Disease
A progressive neurodegenerative disorder characterized by? 4
TRAP
- Resting and postural Tremor – T
- Rigidity – R
- Akinesia (Bradykinesia) – A
- Postural instability – P
Describe the Pathophysiology of Parkinson’s disease?
- Marked loss of dopaminergic nerve terminals in the substantia nigra***
- -Relative increase in cholinergic interneuron activity occurs due to degeneration of dopamine pathways, which contributes especially to tremor.
Possible causes and risk factors?
7
- Primary cause is unknown
- Genetics – 2004- Parkin gene found with early-onset Parkinson’s.
- Defective alpha-syneclein and Lewy bodies
- Complex l and oxygen free radicals**
- Immune factors
- Viral infections/Environmental exposures
- Aging