Basal Ganglia Disorders

Question 1

Most common basal ganglia diseases:

Answer 1

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

Question 2

What do basal ganglia involve impairments in:

Answer 2

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

Question 3

What are dorsal or sensorimotor basal ganglia composed of:

Answer 3

Caudate nucleus
Putamen
Globus pallidus

Question 4

What are the two brainstem nuclei involved?

Answer 4

Substantia nigra

Subthalmic nucleus

Question 5

Relationship of the BG to Movement and Posture:

Answer 5

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

Question 6

What do basal ganglia diseases result in?

Answer 6

difficulty initiating, continuing, or stopping movement

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

Question 7

Symptoms of basal ganglia disease:

Answer 7

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

Question 8

Parkinson’s Disease

Answer 8

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

Question 9

What does the loss of inhibitory dopamine result in?

Answer 9

excessive excitatory output from cholinergic system (acetycholine) of BG

Question 10

Earliest signs of PD:

Answer 10

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

Question 11

Classic symptoms of PD:

Answer 11

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

Question 12

When does resting temor appear?

Answer 12

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

Question 13

Where is resting tremor most common?

Answer 13

unilateral hand or foot

Question 14

What exacerbates resting tremor?

Answer 14

stress or excitement

Question 15

Rigidity:

Answer 15

Inflexibility & stiffness of limb, neck & trunk

Question 16

Where is rigidity most common in PD?

Answer 16

neck, shoulder, and leg

Question 17

Rigidity can lead to:

Answer 17

decreased ROM
decreased arm swing
pain/discomfort

Question 18

Secondary Motor Symptoms:

Answer 18

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

Question 19

Pre-Diagnostic Signs of PD

Answer 19

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

Question 20

Hoehn and Yahr 1:

Answer 20

unilateral involvement, usually with minimal or no functional disability

Question 21

Hoehn and Yahr 2:

Answer 21

bilateral or midline involvement without impairment of balance

Question 22

Hoehn and Yahr 3:

Answer 22

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

Question 23

Hoehn and Yahr 4:

Answer 23

severely disabling disease; still able to walk or stand unassisted

Question 24

Hoehn and Yahr 5:

Answer 24

confinement to bed or wheelchair unless aided

Question 25

Best prognosis in PD:

Answer 25

non-demented & tremor predominant cases of PD

Question 26

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

Answer 26

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

Question 27

Cognitive/Behavioral Status of PD:

Answer 27

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

Question 28

Communication in PD:

Answer 28

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

Question 29

Oromotor Control/Nutritional Status

Answer 29

Dysphagia | Problems in chewing and swallowing

Question 30

Respiratory Status

Answer 30

Breathing patterns Vital capacity Decreased chest expansion

Question 31

Where are common contractures in PD?

Answer 31

flexors, adductors

Question 32

Persistent posturing in PD:

Answer 32

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

Question 33

Sensation/Perceptual Function in PD:

Answer 33

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

Question 34

Vision in PD:

Answer 34

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

Question 35

PD drugs to control tremor:

Answer 35

Anticholinergic drugs

Question 36

PD drug to enhance dopamine release:

Answer 36

amantadine

Question 37

What drug is used during early disease to slow progression:

Answer 37

selegiline

Question 38

Diagnostic Categories of Atypical PD

Answer 38

Progressive SupranuclearPalsy Cortical Basal Degeneration Multiple System Atrophy LewyBody Dementia

Question 39

Clues Suggesting Atypical PD

Answer 39

``` 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 ```

Question 40

Progressive Supramuclear Palsy:

Answer 40

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

Question 41

Progressive Supramuclear Palsy signs:

Answer 41

``` 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 ```

Question 42

Which cranial nerve is tested in PSP?

Answer 42

CNIII

Question 43

What area of brain is involved in multiple system atrophy?

Answer 43

alpha-synucleininclusions in the glial cells | cerebellar involvement

Question 44

Mean onset of multiple system atrophy?

Answer 44

54 years of age

Question 45

Multiple System Atrophy signs:

Answer 45

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

Question 46

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

Answer 46

dementia with Lewy Body

Question 47

Signs and symptoms of Lewy Body:

Answer 47

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

Question 48

Huntington's Disease:

Answer 48

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

Question 49

Signs and Symptoms of Huntington's Disease:

Answer 49

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.

Question 50

Stages I-III—MILD to Moderate of HD:

Answer 50

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

Question 51

Stage IV and V—Severe | of HD:

Answer 51

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