Movement disorders Flashcards
1
Q
Cause of movement disorders?
A
- extrapyramidal (basal ganglia) or cerebellar dysfxn
2
Q
Fxn of basal ganglia?
A
- modulate motor cortical activity but also the activity of the association cortex in the frontal lobes
3
Q
Characteristics of Huntington’s?
A
- progressive autosomal dom
- affects motor fxn, cognition, and behavior - pathophys causes brain cells to waste away
- mean onset is age 40, mean duration is 20 years
- characterized by: chronic progressive chorea, psych changes, dementia
4
Q
GABA involvement in Huntington’s?
A
- decrease in GABA and GABA receptors in basal ganglia
- GABA is inhibitory NT: this leads to jerky movements because of decreased GABA
- Also reducing acetylcholine
- Dopamine not affected - lead toimbalance of dopamine and acetylcholine
5
Q
signs of adult onset of huntington’s?
A
- chorea affects limb and trunk
- dystonia
- rigidity
- postural instability
- myoclonus
- nystagmus
6
Q
Signs of juvenile onset of Huntington’s disease?
A
- very rare
- bradykinesia
- rigidity
- quicker progression
7
Q
Early psych manifestations of Huntington’s?
A
- depression
- personality changes: memory loss, impulsive behavior, moodiness, antisocial behavior (sociopath)
- emotional outbursts
- depression: lack of initiative, loss of spontaneity, inability to concentrate
8
Q
Early and late physical signs of Huntington’s?
A
early:
fidgeting, and restlessness
later: chorea*** dystonic posturing akinesia dementia
9
Q
Dx studies for Huntingtons?
A
- MRI: caudate atrophy
- PET: abnorm metabolic changes in caudate
- genetic testing: sensitive and specific, easy confirmation of clinical dx, dx of atypical pts, and presymptomatic testing in at risk individuals (raises ethical questions)
10
Q
Tx of Huntington’s?
A
- tx sxs only, no med will change course of Huntingtons:
downreg of dopaminergic neurotransmission
suppress chorea: *Tetrabenazine (VAMT inhibitor)
*Neuroleptics (antpsychotics) - deplete dopamine - underlying postural instability and rigidity will worsen
- tx of depression and psychosis
other meds:
anticonvulsants, antipsychotics, antidepressants
11
Q
Anticonvulsants used in Huntingtons?
A
- Clonazepam (klonopin)
- Valproic acid (depakote)
12
Q
Antipsychotic meds used in huntington’s?
A
- Resperidone (resperdal)
- olanzapine (zyprexa)
13
Q
Antidepressants used in Huntingon’s?
A
- Fluoxetine (Prozac)
- sertraline (zoloft)
- nortriptyline (Aventyl, pamelor) - TCAs dangerous in overdose -QT prolong and torsades
14
Q
SEs of Huntington’s meds?
A
- Hyper-excitability, fatigue and restlessness
- antipsychotic drugs may cause SEs that mimic signs of Parkinson’s disease, including involuntary twitching in face and body (tardive dyskinesia)
15
Q
Characteristics of an essential tremor?
A
- most common cause of tremors
- ranges from cosmetic to disabling
- affect both sides of body symmetrically
- more prom with ACTION than rest
- frequency of tremor is constant
- amplitude can vary - stress or caffeine (stimulants), sleep deprivation - amplify it, alcohol will depress it in small amounts
(tremor is most common sx of parkinsons - Ddx)
- tremor usually starts earlier than Parkinsons (have tremor - 4x more likely to develop parkinsons)
16
Q
What muscles do essential tremors affect?
A
- neck and head muscles
- muscles of the voice
- muscles of the arms and hands
17
Q
Tx of essential tremor?
A
- propanolol (inderol): BBlocker
- atenolol (tenormin) - BB of choice for those with asthma or bronchospasm
- mysoline (primidone): anticonvulsant
- gabapentin (neurontin): anticonvulsant
- botulinum toxin injections
18
Q
What is Parkinson’s Disease? TRAP - cardinal manifestations?
A
- progressive neurodegenerative disorder characterized by: TRAP
- Resting and postural Tremor-T
- Rigidity - R
- akinesia (bradykinesia) - A
- postural instability - P
19
Q
How many people affected in US, when does it manifest?
A
- 750,000 to 1 mill people in US
- leading cause of neuro disease in pts over 65
- usually begins after 50, but can occur earlier (earlier onset - worse prognosis)
20
Q
Pathophys of Parkinson’s?
A
- marked loss of dopaminergic nerve terminals in substantia nigra
- relative increase in cholinergic interneuron activity occurs due to degeneration of dopamine pathways, which contributes especially to tremor
21
Q
possible causes and RFs of parkinsons?
A
- primary cause is unknown
- genetics: parkin gene found with early onset
- defective alpha syneclein and Lewy bodies
- complex 1 and oxygen free radicals
- immune factors?
- viral infections/enviro exposures
- aging
22
Q
Cardinal manifestations of Parkinson’s? Hallmark?
A
tremor:
- 75% of persons with disease
- most visible manifestation
- affects distal segment of limbs
- mainly hands and feet
- pill rolling
- usually UNILATERAL
- appears at REST
- disappears with movement and sleep
23
Q
Cardinal manifestations - Rigidity?
A
- resistance to movement
- cog wheel movements
- ratchet like movements
- starts unilateral, and progresses to involve both sides of the body
24
Q
Cardinal manifestations - akinesia (bradykinesia)
A
- MOST DEBILITATING sx
- slowness in initiating and performing movements
- difficulty in sudden, unexpected stopping of voluntary muscles
- difficulty turning
- feel frozen in place, especially when moving through a doorway or preparing to turn
25
Q
Cardinal manifestations - postural instability?
A
- lean forward to maintain center of gravity
- take small shuffling steps w/o swinging the arms
- prone to fall, esp backwards
26
Q
other sxs of Parkinson’s?
A
- emotional and voluntary facial movements become limited and slow leading to “mask like” facies
- loss of blinking reflex
- tongue, palate, and throat muscles become rigid l/t drooling
- uncontrolled sweating
- sebaceous gland secretion
- seborrhea
- micrographia
- hypophonia
- depression
- orthostatic hypotension
- constipation
- impotence
- urinary incontinence
- dementia
27
Q
Dx of Parkinson’s?
A
- dx of PD during life is based on clinical impression
- no physiologic test or blood test that will confirm dx
- neurodx testing with computerized imaging is usually unrevealing
- GOLD STD: neuropathologic exam
- 2/3 cardinal manifestations (tremor, bradykinesia, and rigidity) must be present in order to make dx of idiopathic PD
28
Q
Rating scale of UPDRS?
A
- mentation/behavior/mood
- ADLs (subjective)
- motor exam (objective)
- complications of therapy
29
Q
Tx of Parkinson’s?
A
- levadopa/carbidopa (sinemet): give in combo - half life of levodopa is increased and more levodopa is available for entry into the brain, smaller dose needed.
- MAO-B inhibitors
- Dopamine agonists (watch for impulsive behaviors)
- COMT inhibitors
- amantadine (used for flu)
- acetylcholine blocking agents
30
Q
What are common complications in parkinson’s that you have to tx?
A
- depression: SSRIs
- hallucinations: decrease levadopa/dopamine agonists, zyprexa (Olanzapine)
- orthostatic hypotension: TED hose and slow rising
- sexual disturbances: Viagra/dopamine agonist
- constipation: stop offending med, bulking agents, NO REGLAN (dopamine receptor antagonist)
- freszing/difficulty initiating: tape through a doorway, mark on floor at turning pt
31
Q
What is DBS?
A
current TOC for best results (no SEs of meds)
- uses electric pulse generators to control sxs attached to STN or thalamus
- generators must be replaced q 5 yrs
- candidates usually under 70, who have motor fluctuations, drug induced dyskinesias, and no other serious medical or psych conditions
- complications occur in 2-4%: bleeding in brain and infection
32
Q
When should DBS be considered?
A
- when despite optimal pharmacotherapy, PD pt still is experiencing troubling motor sxs, such as:
- frequent motor fluctuations
- wearing off periods with severe bradykinesia, rigidity, tremor, and/or freezing gait
- severe on dyskinesia
- severe, tx resistant tremor
- think of DBS as electrical dopamine, best result with DBS will not be better than that of L-dopa at its best
33
Q
Eductating pt on parkinson’s?
A
- Impt to provide pt and family with some understanding and control over disorder
- Don’t overexpose material on disease to newly dx pts with mild sxs, scare them!
- Give them resources on disease (pamphlets and internet)
34
Q
support in parkinsons
A
- pt and family: normal rxns of anger, depression, anxiety concerns begin with onset of disease
- caregiver: learn to cope with increasing needs of family member
- support groups: small groups, natl support organizations
- referral to psychologist or psych social worker
35
Q
Why is exercise and physical therapy impt in PD?
A
- regular exercise promotes a feeling of physical and mental well-being. It is especially valuable due to chronic nature of PD and assoc prog. motor limitations
- exericse won’t slow progression of akinesia, rigidity, or gait disturbance, but it can prevent or alleviate some secondary orthopedic effects of rigidity and flexed posture such as shoulder, hip, and back pain, and may also improve fxn in some motor tasks
- stretching and strengthening - improve flexibility and strength, strengthening of extensor msucles can help counteract flexed posture of PD
- brisk walks, swimming, water aerobics good
- referral to PT or exercise group
- treadmill training with support
- balance training, High intensity resistance training
- cued exercises with visual, auditory and tactile feedbacl
- active music therapy
36
Q
Why is speech therapy impt in PD?
A
- dysarthria and hypophonia are common in PD
- help in improving speech volume (talk so quietly)
37
Q
Why is nutrition so impt in PD?
A
certain recommendations:
- a high fiber diet and adequate hydration help manage constipation
- large, high fat meals that slow gastric emptying and interfere with med absorption should be avoided
- dietary protein restriction not necessary except in some pts with advanced disease - AAs interfere with L-dopa absorption
38
Q
What is RLS? (Wittmaack-Ekbom’s syndrome)
- brought on by?
A
- uncontrollable urges to move limbs in order to stop uncomfortable, painful or odd sensation in body, most commonly in legs
- movement brings brief relief
- brough on by inactivity involving sitting or lying:
reading, plane ride, movie, napping - sxs may be worse in evening or early in night
39
Q
Unusual sensations of RLS?
A
- uncomfortable
- antsy
- electrical
- creeping
- painful
- itching
- pins and needles
40
Q
RLS occurs more commonly where? What ages?
A
- 2.7% pop experience RLS
- NA, europe more so
- Japan, India, and Singapore not as much (maybe ethnic or dietary factor?)
- 40-45 yoa most common, but can occur at any age, RLS in children can be misdx as growing pains
- onset usually slow, could be familial, cause is unknown (substantia nigra origin?)
41
Q
What has RLS been assoc with?
A
- pregnancy
- obesity
- smoking
- Fe def
- anemia
- nerve disease
- polyneuropathy (hypothyroidism, heavy metal toxicity, toxins, other conditions)
- diabetes
- kidney failure (can be assoc with Vitamin and mineral deficiency)
- varicose veins
- meds: anti-nausea, H2 blockers, antihistamines, TCAs (Elavil), SSRIs, antipsychotics
- foods: diet soda (aspartame), ETOH
42
Q
Tx of RLS?
A
- correct underlying cause (Fe def)
- take pain relievers (OTC ibuprofen)
- try baths, massages
- apply warm or cool packs, or alt 2
- try relaxation techniques: meditation or yoga
- est good sleep hygiene
- exercise
- avoid caffeine
- cut back on alcohol and tobacco (may aggravate or trigger sxs of RLS)
- stay mentally alert in evening ( don’t fall asleep until you actually go to bed)
43
Q
Meds for Parkinsons that also tx RLS?
A
- pramipexole (Mirapex)
- ropinirole (requip)
- Carbidopa/levodopa (Sinemet)
- lyrica: most common
SEs: mild - include nausea, lightheadedness, and fatigue
people with RLS at no greater risk of developing PD than those w/o RLS
44
Q
Tx for RLS?
A
- opiods: codeine, oxycodone and acetaminophen (percocet, roxicet), hydrocodone and acetaminophen (lortab, vicodin)
- meds for epilepsy: gabapentin (neurontin)
