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Flashcards in Basal Ganglia Disorders Deck (51):
1

Most common basal ganglia diseases:

Parkinson’s disease, Huntington Chorea and dystonias (including drug induced dyskinesias)

2

What do basal ganglia involve impairments in:

Muscle tone
Movement coordination and motor control
Postural stability
Presence of extraneous movement

3

What are dorsal or sensorimotor basal ganglia composed of:

Caudate nucleus
Putamen
Globus pallidus

4

What are the two brainstem nuclei involved?

Substantia nigra
Subthalmic nucleus

5

Relationship of the BG to Movement and Posture:

Automatic Movement
Motor Problems
Movement initiation and preparation
Postural Adjustments
Perceptual and Cognitive Functions

6

What do basal ganglia diseases result in?

difficulty initiating, continuing, or stopping movement
difficulty with muscle tone (particularly rigidity), and increased involuntary movements (tremor, chorea

7

Symptoms of basal ganglia disease:

Movement changes, such as involuntary or slowed movements
Increased muscle tone
Muscle spasms and muscle rigidity
Memory loss
Problems finding words
Tremor
Uncontrollable, repeated movements, speech, or cries (tics)
Walking difficulty

8

Parkinson’s Disease

Chronic progressive disease of the CNS with degeneration of dopaminergic substantia nigra neurons and nigrostriatal pathways

9

What does the loss of inhibitory dopamine result in?

excessive excitatory output from cholinergic system (acetycholine) of BG

10

Earliest signs of PD:

occur in the enteric nervous system
Medulla: particularly the olfactory bulb
NON-motor symptoms can precede motor symptoms

11

Classic symptoms of PD:

Rigidity (leadpipe or cogwheel)
Bradykinesia (hypokinesia)
Resting tremor
Impaired postural reflexes

12

When does resting temor appear?

Appears when muscles are relaxed: when hands are in lap or arms loosely held at the side

13

Where is resting tremor most common?

unilateral hand or foot

14

What exacerbates resting tremor?

stress or excitement

15

Rigidity:

Inflexibility & stiffness of limb, neck & trunk

16

Where is rigidity most common in PD?

neck, shoulder, and leg

17

Rigidity can lead to:

decreased ROM
decreased arm swing
pain/discomfort

18

Secondary Motor Symptoms:

Freezing
Micrographia
Mask-Like Expression
Unwanted Accelerations
Stooped posture
Dystonia
Swolling difficulty
Slurred speech
Soft speech

19

Pre-Diagnostic Signs of PD

Olfactory function
PD patients prefer sweets!
Autonomic function
Incontinence, constipation, erectile dysfunction
Sleep function
REM sleep disorder
Vivid dreams/acting out dreams
Emotional/Cognition
Depression, Apathy

20

Hoehn and Yahr 1:

unilateral involvement, usually with minimal or no functional disability

21

Hoehn and Yahr 2:

bilateral or midline involvement without impairment of balance

22

Hoehn and Yahr 3:

bilateral disease: mild to moderate disability with impaired postural reflexes; physically independent

23

Hoehn and Yahr 4:

severely disabling disease; still able to walk or stand unassisted

24

Hoehn and Yahr 5:

confinement to bed or wheelchair unless aided

25

Best prognosis in PD:

non-demented & tremor predominant cases of PD

26

Predictive factors for more rapid motor progression, nursing home placement, and shorter survival of PD:

Older age of onset
Comorbidities
Rigidity/bradykinesiaphenotype
Decreased responsiveness to dopamine
Dementia

27

Cognitive/Behavioral Status of PD:

Intellectual impairments/Dementia occurs in advanced stages
Memory
Bradyphrenia (slowing of thought processes)
Depression

28

Communication in PD:

Dysarthria
Hypophonia (decreased volume)
Mutism in advanced stages
Mask like face with infrequent blinking and expression
Writing becomes progressively smaller

29

Oromotor Control/Nutritional Status

Dysphagia
Problems in chewing and swallowing

30

Respiratory Status

Breathing patterns
Vital capacity
Decreased chest expansion

31

Where are common contractures in PD?

flexors, adductors

32

Persistent posturing in PD:

kyphosis with forward head
Many patients osteoporotic with high risk of fracture

33

Sensation/Perceptual Function in PD:

Aching and stiffness
Abnormal sensations (cramp like sensations)
Problems in spatial organization
Perception of vertical
Extreme restlessness (akathisia)

34

Vision in PD:

Blurring
Cogwheeling eye pursuit
Eye irritation from decreased blinking
Decreased pupillary reflexes

35

PD drugs to control tremor:

Anticholinergic drugs

36

PD drug to enhance dopamine release:

amantadine

37

What drug is used during early disease to slow progression:

selegiline

38

Diagnostic Categories of Atypical PD

Progressive SupranuclearPalsy
Cortical Basal Degeneration
Multiple System Atrophy
LewyBody Dementia

39

Clues Suggesting Atypical PD

Eye movement dysfunction
Vertical saccade slowing
Vertical gaze palsy: Downgaze > upgaze
Upper motor neuron signs (DTR’s, Babinski)
Cerebellar signs: dysmetria, ataxia
Autonomic dysfunction
Orthostatic hypotension
Urinary incontinence
Early onset/Rapid Progression
Falls & dementia

40

Progressive Supramuclear Palsy:

Most common atypical parkinsonism
“tauopathy” due to accumulation of Tau protein in brain

41

Progressive Supramuclear Palsy signs:

Limited vertical eye movement: down > up
Eye palsy= limited blinking
Loss of righting reaction: fall backwards suddenly
Severe axial rigidity
“Surprised expression”
Growling/groaning
Increased extensor tone, wide BOS
Dysphagia & dysarthria
Cognitive dysfucntion& emotional lability

42

Which cranial nerve is tested in PSP?

CNIII

43

What area of brain is involved in multiple system atrophy?

alpha-synucleininclusions in the glial cells
cerebellar involvement

44

Mean onset of multiple system atrophy?

54 years of age

45

Multiple System Atrophy signs:

autonomic symptoms
Orthostatic hypotension, supine hypertension, urinary & sexual dysfunction, respiratory & breathing problems
▪**decrease >20 mm Hg systolic BP > 10 mm Hg diastolic
▪Impotence is often first symptom
▪Loud sighing
▪Gait & limb ataxia: irregular, jerky tremor,
▪Wide based gait vs. narrow in idiopathic
▪Head/oral dyskinesias
▪“Pisa” syndrome: frequent falls early

46

What is the second most frequent cause of dementia in elderly?

dementia with Lewy Body

47

Signs and symptoms of Lewy Body:

Rigidity, bradykinesia, tremor
Shuffling gait, instability, frequent falls
Depression, paranoid ideation
Hallucinations

48

Huntington's Disease:

A fatal autosomal dominant hereditary disorder, which occurs with an insidious onset, generally occurring in the mid-30s – 40s

49

Signs and Symptoms of Huntington's Disease:

Causes cognitive & emotional disturbances
Problems with voluntary a& involuntary movement
Degeneration of the caudate & putamen are most characteristic of HD.
Dystonia, Athetosis, akinesia, & bradykinesia develop
Other problems: executive function, STM, Visuospatial function (early) to dementia (late). Cause of death tends to be respiratory.

50

Stages I-III—MILD to Moderate of HD:

Increased concerns about cognitive issues
Irritability
Small coordination problems
Chorea: athetoid movements develop

51

Stage IV and V—Severe
of HD:

Lack of cognitive concern-dementia is late stages
Fatal stage-usually do to respiratory problems