L11: Hypokinetic Dysarthria

Question 1

The basal ganglia includes…

Answer 1

the striatum, putamen, globus pallidus, substantia nigra, and subthalamic nuclei

Question 2

the BG has complex interconnections and loops w the _____ and ____

Answer 2

motor cortex and thalamus

Question 3

The BG are involved in 5 things…

Answer 3

regulation of muscle tone

scaling the force, amplitude, and duration of movements

adjusting movements to changes in the enviro/context

assisting in the learning, preparation, and initiation of movements

sensorimotor integration processes

Question 4

depending on which parts of the BG are damaged, the BG disorders can cause ______ or _______

Answer 4

excessive movements (hyperkinetic disorder) or reduced movement (hypokinetic disorder)

Question 5

in PD or parkinsonism, the movements are mostly…

Answer 5

reduced or hypokinetic

Question 6

in PD the _____ neurons are destroyed and this causes….. which disrupts…

Answer 6

substania nigra

causes a reduction in the supply of dopamine to the striatum

this disrupts the sensorimotor integration circuits and motor output of the BG

Question 7

A protein found in the NS, called ______ can start to miss fold and form ____ w/o neurons ….. which …

Answer 7

alpah-synuclein

lewy bodies

which damage the neurons and cause PD to develop

Question 8

certain regions of the NS such as the _____ are more susceptible to the formation of _____

Answer 8

substantia nigra

lewy bodies (possible link to large number of dendrites and mitochondira in the SN)

Question 9

the presence of lewy bodies is confirmed by…

Answer 9

postmorterm analysis - work on detecthing from spinal fluid is progressing

Question 10

PD is thought to involve of combo of

Answer 10

enviro and genetic factors

Question 11

substantia nigra is important in PD dev, but other structures….

Answer 11

are involved as the disease progresses from presymptomic (prodromal) to later stages

Question 12

PD = the traditional form of

Answer 12

idiopathic PD

Question 13

parkinsonism =

Answer 13

showing parkinson like symps (not necessarily idopathic PD)

Question 14

parkinsonism is caused 89% of the time by…..

67% of it being ____
22% of it being ____

other 3% is ____ and another 3% is ____

Answer 14

degenerative conditions

67% = idiopathic PD

22% = atypical parkinsonism diseases, parkinson plus syndromes (ex. multi system atrophy)

strokes and other conditions (postencephalitis, drugs/toxins)

Question 15

MPTP a drug …

Answer 15

was first observed in heroin users

will produce parkinsonism in monkeys

Question 16

ideopathic PD is of ____ etiology, probably…

Answer 16

unknown

prob combo of genetic and enviro factors

Question 17

Ideopathic PD is ____ - ___% of general population, about ____ % of population over 60 years

Answer 17

0.1-0.2%

2-3%

Question 18

onset of PD is usually after ____ but some younger, progression is ____ when diagnosed later in life

Answer 18

55 years

faster

Question 19

IPD is caused by…. which leads to…

Answer 19

loss of dopaminergic cells in sunstantia nigra, which leads to reduced amount of dopamine released in the putamen (striatum)

Question 20

___% of substantia nigra cells are destroyed before onset of motor symptoms

Answer 20

70-90%

Question 21

providing IPD patients w replacement for lost _____ significantly reduced symptoms

Answer 21

striatal dopamine

Question 22

levodopa (L-dopa) is

only about ___% of medication reaches the blood brain barrier

Answer 22

a precursor to dopamine

compound w carbidopa to inhibit breakdown of levadopa in the peripheral blood

5%

Question 23

_____% of patients will begin to develop periods of dyskinesia (chorea or dystonia) after 5 years of levodopa (eg. peak-dose dyskinesias)

Answer 23

80-90%

Question 24

after 10 years of levodopa some symptoms such as ____ are hypothesized to become _____ to levodopa

Answer 24

dysarthria

resistant

but recent work in Adam’s lab does not support this

Question 25

4 main symptoms of parkinsonism (TRAP acronym)

Answer 25

Rest tremor akinesia rigidity loss of postural reflexes

Question 26

describe rest tremor

Answer 26

4-6 per second present when limb is fully supported and at rest reduced or absent during purposeful movement may be related to abnormal central oscillators or alterations in peripheral feedback loops not always present in parkinsonism but very common

Question 27

describe akinesia

Answer 27

reduction in spontaneous movements movements are reduced in range, force, complexity or speed (slowness = bradykineasia) (delayed initiation of movement, simple manual tasks are diff and slow)

Question 28

describe rigidity

Answer 28

an inc resistance to passive movement approx equal in flexors in extensors present thru entire range of movement (lead pipe) cogwheel often present intermittent inc in resistance over the range of movement

Question 29

describe loss of postural reflexes

Answer 29

pts do not respond normally to a threat to their posture pull test causes retropulsion (many steps backward) or falling in advanced PD this leads to severe disability and bed or wheelchair bound

Question 30

name 2-3 other symptoms of PD

Answer 30

reduced arm swing during walking stooped or flexed posture shuffling gait motor freezing, i.e. in front of doorways festination of gait or other movements (rapid & accelerating movements) mask-like or reduced facial expression reptilian stare with reduced blinking micrographia (small writing) swallowing problems non-adapting glabellar reflex (forehead tap/eye blink) cognitive disturbances; typically in later stages (10-30%)

Question 31

what are the two ways IPD can be diagnosed? (3rd new way?)

Answer 31

based on clinical criteria and neuromotor exam (UPDRS) autopsy assessment of lewy body levels in BG new option --> DaT scan

Question 32

diagnosis of IPD bases on clinical criteria and neuromotor exam (UPDRS) involves...

Answer 32

presence of bradykinesia + rest tremor or rigidity at least 2 supportive criteria (i.e. response to levodopa, olfactory loss) absence of exclusion criteria or red flags (i.e. ataxia, vertcial gaze probs, rapid progression of gait, speech, swallow in first 5 years autonomic probs

Question 33

bradykinesia =

Answer 33

slowness of movement

Question 34

diagnosis of IPD via autopsy involves

Answer 34

pt must be deceased and brain donated for evaluation has been used to demonstrate that clinical diagnosis is only about 85% accurate

Question 35

describe DaT scan (dopamine transporter scan) as an IPD diagnosis tool

Answer 35

imaging procedure (SPECT) and radioactive material to detect dopamine transporters (DaT) on nigro-striatal cells supportive info (not typically used to diagnose PD) : somtimues used in support of a v mild PD - can be associated w false negs - does not distinguish bw PD and other parkinsonism -NOT being used in canada bc of false negs

Question 36

how many stages of Parkinsonism?

Answer 36

5

Question 37

stage 1 of parkinsonism? mean time?

Answer 37

unilateral involvement (eg. tremor and reduced arm swing on one side) 4.5 years

Question 38

stage 2 of parkinsonism? mean time?

Answer 38

bilateral involvement without posture or balance impairments (eg. bilateral tremor and rigidity) 6.5 years

Question 39

stage 3 of parkinsonism? mean time?

Answer 39

bilateral involvement and postural instability - mild to moderate impairment 8.5 years

Question 40

stage 4 of parkinsonism? mean time?

Answer 40

bilateral involvement and postural instability - severe impairment; still able to walk or stand unassisted 11.5 years

Question 41

stage 5 of parkinsonism? mean time?

Answer 41

wheelchair bound or bedridden unless aided 15 years

Question 42

the most common method of assessing motor symptoms in PD is the? how are things rated?

Answer 42

Unified parkinson's disease rating scale (UPDRS) 0 - 4 point scales with 27 items (4 * 27 = 108 max score) that rate the severity of PD symptoms (tremor, bradykinesia/hypokinesia, rigidity, posture/gait, speech)

Question 43

quite a bit of _____ in symptoms and rate of progression in PD

Answer 43

heterogeneity

Question 44

several subtypes of PD have been proposed but none are _____, some are:

Answer 44

universally accepted tremor dominant vs gait/posture subtypes; mild motor onset vs motor + non-motor symptom onset subtypes

Question 45

what are the 5 abnormal sensation and perception features in PD?

Answer 45

1. Sense of jaw position (proprioception) abnormal 2. Two-point lip and tongue discrimination (touch) abnormal 3. Sense of tongue force levels and levels of respiratory effort are abnormal 4. Abnormal sense of smell (olfaction) a very early symptom in PD 5. Abnormal perception of speech intensity, speech rate, emotional tone of speech, and facial expressions of emotion.

Question 46

has been hypothesized that there is a causal link bw impaired ______ and motor impairments in PD _____ has been hypothesized to be linked to an impairment in self-loudness perception

Answer 46

perceptual processes hypophonia

Question 47

7 most distinctive features of hypokinetic dysarthria are

Answer 47

monopitch reduced stress monoloudness inappropriate silences short rushes rapid rate reduced loudness level