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Flashcards in Basal Ganglia Clinical Correlates Deck (56)
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1

BG impairments appear ______ to the lesion because:

contralateral
crossing of C-S tract through which deficits are manifested

2

PD is due to loss of dopaminergic cells in the ____. What % loss before symptomatic?

SNc
70-80% loss

3

Severely impaired PD patients have lost > __% of striatal dopamine.

90%

4

What is the most likely etiology for PD?

combo of accelerated aging, genetic predisposition, exposure to toxins and abnormality in oxidate mechanism

5

Oxygen free radical damage results in accumulation of:

lewy bodies in dopamine neurons in SNc

6

PD epidemiology:

onset 40-70 yoa
infrequent before 30yoa
men>women
black 1/4 that of whites
1 million in north ameraica
60,000 new cases/year

7

What are the key clinical features of PD?
How many required to receive clinical diagnosis of PD?

Must have 2 of the first 3 below AND consistent response to L-dopa replacement therapy:
1. pill rolling tremor
2. bradykinesia and poverty of mvmnt
3. cog-wheel rigidity
4. loss of equilibrium and postural reflexes

8

What is micrographia?

small handwriting

9

What is akinesia?

complete loss of mvmnt

10

Bradykinesia presents as:

dec. amp and speed of mvmnt
difficulty initiating
increased latency to onset of voluntary mvmnt
loss of reciprocal mvmnt
loss of facial expression
akinesia
micrographia

11

PD motor control impairments include:

1. difficulty generating internally triggered mvmnt
2. freezing
3. festinating gait
4. difficulty with sensory organization
5. rigidity
6. impaired postural control
7. impaired eye mvmnts

12

What might a PD patient freeze?

difficulty in switching motor programs
inflexible program selection
challenge with divided attention
difficulty processing sensory info

13

What is the major contributor to falls with PD patients?

freezing
*PD has the highest fall rate of any common neurological disorder

14

PD patients have trouble with sensory organization, therefor rely more heavily on:

vision
can't filter relevant from irrelevant sensory cues in the environment but vision tends to be more accurate

15

What does rigidity look like in PD?

flexed posture
dec. trunk rotation

16

How do impaired eye mvmnts present in PD?

hypometric saccades
breakdown of smooth pursuit (insufficient activation of agonist muscles)

17

What influences postural control in PD?

dec. LOS (esp. backwards)
dec. / ineffective strategies in response to perturbations
trunk rigidity
impaired anticipatory responses

18

What cognitive/limbic impairments are expected in PD?

difficulty in divided attention situations (freeze or unsteady)
concurrent depression and/or dementia as disease progresses

19

Later PD impairments include:

autonomic issues: orthostasis, dec. bowel motility, urinary frequency
sleep disturbances: periodic leg mvmnts, no muscle relaxation, sleep apnea

20

What secondary PD impairments might contribute to mobility difficulties?

contractures, dec. respiratory excursion, etc

21

What are two common PD rating scales?

Hoehn and Yahr
UPDRS = united PD rating scale

22

What meds are used in PD?

dopamine agonist (mirapex)
L-dopa replacement therapy (sinemet)

23

Why do you want to delay drug treatment as long as possible in PD?

side effects develop over time with tx, dyskinesias, on/off phenomenon

24

What non-pharmacological medical management is avail for PD?

DBS
can have dramatic effects
most common side is STN

25

Parkinson's plus syndrome are ddx of PD vs. other degenerative disorders. What are some similarities and differences?

early on may present with one or more PD-related signs like tremor or bradykinesia
these diseases are NOT dopamine responsive and progress more RAPIDLY than PD

26

Name some parkinson's plus syndromes:

MSA = multiple system atrophy
PSP = progressive supranuclear palsy
corticobasal degeneration
lewy body dementia
olivopontocerebellar atrophy

27

Chorea is a type of _____ due to damage of the _____.

dyskinesia
damage to striatum

28

Describe sxs of chorea:

Series of continuous rapid mbmtns of face, tongue, and limbs (esp fingers/toes).
Usually resemble fragments of fxnal mvmnts but are involuntary.
(greek word for "dance")

29

Athetosis is a type of _____ due to damage of the _____.

dyskinesia
damage to striatum

30

Describe sxs of athetosis:

(greek word for "without position")
slow writhing mvmnts, snakelike, mostly limb involvement

31

What distinguishes chorea vs. athetosis?

athetotis involves more of limbs than chorea

32

What is an intermediate form b/w chorea and athetosis?

choreoathetosis

33

Hemiballismus is a type of _____ due to damage of the _____.

dyskineisa
damage to STN (rarely result from stroke involving sm. branch of post. cerebral artery)

34

Describe sxs of hemiballsismusL

(greek word for "jumping about")
violent flailing / ballistic mvmnts of usually one arm or one leg

35

What tis the etiology of huntington's chorea?

autosomal dominant genetic disorder (50% chance of inheritance)
huntingtons allele on chromosome 4
genetic testing available
consists of extended CAG trinucleotide repeat > 40 repays causes huntington's disease

36

Describe the pathological changes in huntington's chorea:

profound loss of interneurons in caudate and putamen
reactive gliosis (inflammatory response) in striatum
other neuron loss in Cx and Cb
enlarged ventricles
cell death though to be due to accumulation of oxygen free radicals or failure of mitochondrial energy metabolism

37

Describe the epidemiology of huntington's chorea:

rare: 6.5/100,000 (all races)
mean are onset 35-42 (small percentage in young and old age)

38

What diagnostic testing avail for huntington's chorea?

genetic testing
MRI/CT: reduced volume in striatum
PET scan: reduced D1 and D2 binding
SPECT: reduced blood flow caudate > putamen

39

Hungtington's chorea usually presents initially with:

cognitive changes
depression, irriability
slowing cognition, decreased ability to problem solve
subtle changes in coordination, choreoathetotic mvmnts in fingers

40

Later huntington's chorea sxs include:

chorea advancing distal to proximal
ataxic gait with chorea
choreoathetotic mvmnts of face and tongue
depression and dementia

41

Dystonia is defined as:

involuntary muscle contractions causing abnormal twisting mvmnts or postures with contractions that can be sustained, intermittent, rhythmic, or tremulous

42

What is the physiology behind dystonia?

cortical disinhibition
indirect BG circuit does not inhibit the surround
sensory gating affected so abnormal links between sensory input and motor output
abnormal body schema representations in the cortex

43

What is the age of onset for dystonia?

infantile < 2 yrs
childhood 2-26 yrs
adult > 26 years

44

What is the etiology of dystonia?

primary: genetic
secondary: iatrogenic, tumor, stroke, trauma, CRPS
plrimary +: dystonia plus other neuro signs
part of another degenerative disorder like huntington's disease

45

What are some distributions of dystonia?

focal: bletherospasm (eyelid), torticollis (cervical), writers cramp, musicians
segmental (whole limb)
generalized (whole body)

46

How is dystonia treated?

some can be sensitive to dopamine replacement
botox injections (useful for focal)
maintain mobility/fitness
carefully evaluate the benefit of bracing as a way to alter sensory input
DO NOT stretch
DBS not as reliably effective as in PD

47

What are 4 subclassifications of CP?

1. spastic
2. extrapyramidal
3. hypotonic
4. mixed and atypical types

48

Common factors in CP?

1. abnormality in motor control
2. early onset (pre/peri-natal)
2. absence of recognized underlying progressive disease

49

What are common associated features in CP?

seizures, cognitive impairment, visual and visuo-motor abnormalitites, deafness, speech and learning impairments

50

Spastic CP signs and sxs:

UMN with hyperreflexia
abnormal postural reflexes
sustained clonus, adductor tone
dominiant tonic neck reflexes

51

What are topographic subclassifications of spastic CP?

hemiplegia
spastic diplegia
spastic quadriplegia
monoplegia
triplegia

52

What is the most common spastic CP sub classification?

spastic diplegia
due to damage of medial portion of internal capsule

53

What are sxs of extrapyramidal CP?

athetosis, chorea, choreo-athetotic

54

CP in low birth weight infants

spastic diplegia most common
predisposing factors to both CP and prematurity/low birth weight (i.e.: congenital malformation)

55

CP can influence full term infants as well:

infection
majority (80%) do not have serious perinatal asphyxia
can develop CP without obvious postnatal insult as well

56

What are predisposing factors for CP?

1/3 have cortical dysgenesis with abnormal neuronal migration
hyperbilirubinemia with athetotic CP
exposure to toxins
in utero infection
perinatal maternal infection/fever