Basal Ganglia Disorders Flashcards

1
Q

Most common basal ganglia diseases:

A

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

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2
Q

What do basal ganglia involve impairments in:

A

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

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3
Q

What are dorsal or sensorimotor basal ganglia composed of:

A

Caudate nucleus
Putamen
Globus pallidus

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4
Q

What are the two brainstem nuclei involved?

A

Substantia nigra

Subthalmic nucleus

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5
Q

Relationship of the BG to Movement and Posture:

A
Automatic Movement
Motor Problems
Movement initiation and preparation
Postural Adjustments
Perceptual and Cognitive Functions
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6
Q

What do basal ganglia diseases result in?

A

difficulty initiating, continuing, or stopping movement

difficulty with muscle tone (particularly rigidity), and increased involuntary movements (tremor, chorea

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7
Q

Symptoms of basal ganglia disease:

A
Movement changes, such as involuntary or slowedmovements
Increased muscle tone
Muscle spasms and muscle rigidity
Memory loss
Problems finding words
Tremor
Uncontrollable, repeated movements, speech, or cries (tics)
Walking difficulty
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8
Q

Parkinson’s Disease

A

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

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9
Q

What does the loss of inhibitory dopamine result in?

A

excessive excitatory output from cholinergic system (acetycholine) of BG

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10
Q

Earliest signs of PD:

A

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

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11
Q

Classic symptoms of PD:

A

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

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12
Q

When does resting temor appear?

A

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

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13
Q

Where is resting tremor most common?

A

unilateral hand or foot

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14
Q

What exacerbates resting tremor?

A

stress or excitement

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15
Q

Rigidity:

A

Inflexibility & stiffness of limb, neck & trunk

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16
Q

Where is rigidity most common in PD?

A

neck, shoulder, and leg

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17
Q

Rigidity can lead to:

A

decreased ROM
decreased arm swing
pain/discomfort

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18
Q

Secondary Motor Symptoms:

A
Freezing
Micrographia
Mask-Like Expression
Unwanted Accelerations
Stooped posture
Dystonia
Swolling difficulty 
Slurred speech
Soft speech
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19
Q

Pre-Diagnostic Signs of PD

A
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
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20
Q

Hoehn and Yahr 1:

A

unilateral involvement, usually with minimal or no functional disability

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21
Q

Hoehn and Yahr 2:

A

bilateral or midline involvement without impairment of balance

22
Q

Hoehn and Yahr 3:

A

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

23
Q

Hoehn and Yahr 4:

A

severely disabling disease; still able to walk or stand unassisted

24
Q

Hoehn and Yahr 5:

A

confinement to bed or wheelchair unless aided

25
Q

Best prognosis in PD:

A

non-demented & tremor predominant cases of PD

26
Q

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

A
Older age of onset
Comorbidities
Rigidity/bradykinesiaphenotype
Decreased responsiveness to dopamine
Dementia
27
Q

Cognitive/Behavioral Status of PD:

A

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

28
Q

Communication in PD:

A
Dysarthria
Hypophonia (decreased volume)
Mutism in advanced stages
Mask like face with infrequent blinking and expression
Writing becomes progressively smaller
29
Q

Oromotor Control/Nutritional Status

A

Dysphagia

Problems in chewing and swallowing

30
Q

Respiratory Status

A

Breathing patterns
Vital capacity
Decreased chest expansion

31
Q

Where are common contractures in PD?

A

flexors, adductors

32
Q

Persistent posturing in PD:

A

kyphosis with forward head

Many patients osteoporotic with high risk of fracture

33
Q

Sensation/Perceptual Function in PD:

A
Aching and stiffness
Abnormal sensations (cramp like sensations)
Problems in spatial organization
Perception of vertical
Extreme restlessness (akathisia)
34
Q

Vision in PD:

A

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

35
Q

PD drugs to control tremor:

A

Anticholinergic drugs

36
Q

PD drug to enhance dopamine release:

A

amantadine

37
Q

What drug is used during early disease to slow progression:

A

selegiline

38
Q

Diagnostic Categories of Atypical PD

A

Progressive SupranuclearPalsy
Cortical Basal Degeneration
Multiple System Atrophy
LewyBody Dementia

39
Q

Clues Suggesting Atypical PD

A
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
Q

Progressive Supramuclear Palsy:

A

Most common atypical parkinsonism

“tauopathy” due to accumulation of Tau protein in brain

41
Q

Progressive Supramuclear Palsy signs:

A
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
Q

Which cranial nerve is tested in PSP?

A

CNIII

43
Q

What area of brain is involved in multiple system atrophy?

A

alpha-synucleininclusions in the glial cells

cerebellar involvement

44
Q

Mean onset of multiple system atrophy?

A

54 years of age

45
Q

Multiple System Atrophy signs:

A

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
Q

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

A

dementia with Lewy Body

47
Q

Signs and symptoms of Lewy Body:

A

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

48
Q

Huntington’s Disease:

A

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

49
Q

Signs and Symptoms of Huntington’s Disease:

A

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
Q

Stages I-III—MILD to Moderate of HD:

A

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

51
Q

Stage IV and V—Severe

of HD:

A

Lack of cognitive concern-dementia is late stages

Fatal stage-usually do to respiratory problems