Flashcards in Basal Ganglia Disorders Deck (51):
1
Most common basal ganglia diseases:
Parkinson’s disease, Huntington Chorea and dystonias (including drug induced dyskinesias)
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What do basal ganglia involve impairments in:
Muscle tone
Movement coordination and motor control
Postural stability
Presence of extraneous movement
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What are dorsal or sensorimotor basal ganglia composed of:
Caudate nucleus
Putamen
Globus pallidus
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What are the two brainstem nuclei involved?
Substantia nigra
Subthalmic nucleus
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Relationship of the BG to Movement and Posture:
Automatic Movement
Motor Problems
Movement initiation and preparation
Postural Adjustments
Perceptual and Cognitive Functions
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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
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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
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Parkinson’s Disease
Chronic progressive disease of the CNS with degeneration of dopaminergic substantia nigra neurons and nigrostriatal pathways
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What does the loss of inhibitory dopamine result in?
excessive excitatory output from cholinergic system (acetycholine) of BG
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Earliest signs of PD:
occur in the enteric nervous system
Medulla: particularly the olfactory bulb
NON-motor symptoms can precede motor symptoms
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Classic symptoms of PD:
Rigidity (leadpipe or cogwheel)
Bradykinesia (hypokinesia)
Resting tremor
Impaired postural reflexes
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When does resting temor appear?
Appears when muscles are relaxed: when hands are in lap or arms loosely held at the side
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Where is resting tremor most common?
unilateral hand or foot
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What exacerbates resting tremor?
stress or excitement
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Rigidity:
Inflexibility & stiffness of limb, neck & trunk
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Where is rigidity most common in PD?
neck, shoulder, and leg
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Rigidity can lead to:
decreased ROM
decreased arm swing
pain/discomfort
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Secondary Motor Symptoms:
Freezing
Micrographia
Mask-Like Expression
Unwanted Accelerations
Stooped posture
Dystonia
Swolling difficulty
Slurred speech
Soft speech
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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
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Hoehn and Yahr 1:
unilateral involvement, usually with minimal or no functional disability
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Hoehn and Yahr 2:
bilateral or midline involvement without impairment of balance
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Hoehn and Yahr 3:
bilateral disease: mild to moderate disability with impaired postural reflexes; physically independent
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Hoehn and Yahr 4:
severely disabling disease; still able to walk or stand unassisted
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Hoehn and Yahr 5:
confinement to bed or wheelchair unless aided
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Best prognosis in PD:
non-demented & tremor predominant cases of PD
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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
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Cognitive/Behavioral Status of PD:
Intellectual impairments/Dementia occurs in advanced stages
Memory
Bradyphrenia (slowing of thought processes)
Depression
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Communication in PD:
Dysarthria
Hypophonia (decreased volume)
Mutism in advanced stages
Mask like face with infrequent blinking and expression
Writing becomes progressively smaller
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Oromotor Control/Nutritional Status
Dysphagia
Problems in chewing and swallowing
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Respiratory Status
Breathing patterns
Vital capacity
Decreased chest expansion
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Where are common contractures in PD?
flexors, adductors
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Persistent posturing in PD:
kyphosis with forward head
Many patients osteoporotic with high risk of fracture
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Sensation/Perceptual Function in PD:
Aching and stiffness
Abnormal sensations (cramp like sensations)
Problems in spatial organization
Perception of vertical
Extreme restlessness (akathisia)
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Vision in PD:
Blurring
Cogwheeling eye pursuit
Eye irritation from decreased blinking
Decreased pupillary reflexes
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PD drugs to control tremor:
Anticholinergic drugs
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PD drug to enhance dopamine release:
amantadine
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What drug is used during early disease to slow progression:
selegiline
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Diagnostic Categories of Atypical PD
Progressive SupranuclearPalsy
Cortical Basal Degeneration
Multiple System Atrophy
LewyBody Dementia
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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
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Progressive Supramuclear Palsy:
Most common atypical parkinsonism
“tauopathy” due to accumulation of Tau protein in brain
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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
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Which cranial nerve is tested in PSP?
CNIII
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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
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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
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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.
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Stages I-III—MILD to Moderate of HD:
Increased concerns about cognitive issues
Irritability
Small coordination problems
Chorea: athetoid movements develop
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