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
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