Basal Ganglia Diseases

Medial Motor Pool

Medial Motor Pool

Which neuron pool is responsible for

• Adjusting muscle tone in the postural/girdle muscles

• Stepping movement patterns of LE

• Contraction of neck muscles and extensor muscles

• Contraction of neck, shoulder and trunk muscles

Lateral Motor Pool

Which neuron pool is responsible for

• Fractionated movement

• UE limb extension

• Caudate

• Putamen

• Globus Pallidus

• Subthalamic Nucleus

• Substantia Nigra

Basal ganglia is composed of 5 nuclei, which are?

Huntington Disease

What is the medical diagnosis of a disease that damages the indirect pathway of the BG, is autosomal dominant, & onset is seen within the 3rd/4th decade? (No brakes)

Parkinson Disease

What is the medical diagnosis of a disease that damages the direct pathway of the basal ganglia and symptoms begin to develop after the age of 50? (No accelerator)

Glutamate

The cortical motor areas produce which neurotransmitter that excites the striatum?

Dopamine

The substantia nigra is correlated to the production of which neurotransmitter?

Parkinson’s

Which disease is due to the death of dopamine producing cells in the substantia nigra → loss of dopamine to the putamen (reducing voluntary movement) & death of ACh producing cells in the PPN → disinhibition of the reticulospinal tract (excessive contraction of postural muscles) ?

Toxic Parkinsonism & Drug Induced Parkinsonism

What are the disease producing secondary PD? ( usually there’s a correlation to older meds that we don’t see often)

Rigidity

What is the term for increased resistance to movement ( in both flexor & extensor muscles) in all directions? Seen even in their sleep, the reflex never turns off

Cogwheel Rigidity

What is the term for when passive movement has repeated catches and releases?

Festinating Gait

What is the term for decrease in step length and heel strike resulting in small steps/shuffling →increased step cadence?

Resting Tremor

What is the term for involuntary, rhythmic shaking? Tend to see pill-rolling behavior and tremor decreases during active movement

Isometric Hold

In terms of resting tremor what type of hold/contraction do we see elicit the tremor

Bradykinesia

What is the term for slow movement especially with initiation?

Hypokinesia

What the term for less movement often seen in facial expressions?

Akinesia

What is the for term absence of movement?

Postural Instability and Tremor-Dominant

What are the 2 clinical subgroups of Parkinson’s Disease?

Postural instability and Gait disturbance

What are the 2 main symptoms of PIGD phenotype?

Few problems with bradykinesia, lower risk of dementia/non-motor symptoms, less likely to have gene mutations

What are the 3 differences seen with TD phenotype?

80-90%

Diagnosis of PD peaks at ages 60-69, but by the time of detection individuals have lost about how much function of their substantia nigra?

Tremor, Rigidity, Akinesia (hypo- or brady-), and Postural instability

What are the 4 cardinal features of PD? ( diagnosis requires 2/4 features to be present)

Motor channel, Oculomotor channel, Emotional/Limbic channel, Prefrontal Channel

What are the 4 Basal Ganglia Loops?

Lesions in the olfactory bulb, dorsal 9/10 nucleus of brainstem→ decreased smell and taste

What is Stage 1 PD & what do we see as the signs of it?

Lesions in the pontine tegmentum → effects level of alertness, posture and awareness of environment

What is Stage 2 PD & what do we see as the signs of it?

Involvement of the nigrostriatal system (substantia nigra) → effects in voluntary movement

What is Stage 3 PD & what do we see as the signs of it?

Lesions in the hypothalamus, thalamus and cortex (temporal) → effects language, planning sensory integration

What is Stage 4 PD & what do we see as the signs of it?

Affects sensory associations of the neocortex & prefrontal neocortex → impacts risk-reward judgement, personality and emotional regulation

What is Stage 5 PD & what do we see as the signs of it?

Impacts first-order association areas of neocortex

What is Stage 6 PD & what do we see as the signs of it?

Motor Performance

In terms of clinical manifestation of PD, which category do the following fall under as effecting

• decreased torque production

• fatigue

• contractures/deformity

• micrographia

• resting tremor (70% of patients)

Motor Planning

In terms of clinical manifestation of PD, which category do the following fall under as effecting

• starting hesitation

• freezing

• “poverty” of movement

• visuomotor difficulties

Motor Learning

In terms of clinical manifestation of PD, which category do the following fall under as effecting

• slower, reduced efficiency

• increased context specificity ( cannot translates movements to new settings)

• Procedural learning deficits for complex tasks

Posture

In terms of clinical manifestation of PD, which category do the following fall under as effecting

• Kyphosis with forward head, scapular protraction

• Leaning to one side

• Increased fall risk

Sensation

In terms of clinical manifestation, which category do the following fall under as effecting

• Paresthesia

• Pain

• Akinesia

• Proprioceptive and kinesthetic deficits

Speech, Voice and Swallow

In terms of clinical manifestation, which category do the following fall under as effecting

• hypokinetic dysarthria

• dysphagia

• reduced vital capacity

Gait

In terms of clinical manifestation, which category do the following fall under as effecting

• Reduced step length

• Reduced step width and BOS

• Reduced speed

• Freezing of gait

• Difficulty side stepping, dual tasking

Cognition & Behavior

In terms of clinical manifestation, which category do the following fall under as effecting

• Dementia

• Bradyphrenia

• Visuospatial deficits

• Depression

• Dysphoric Mood

• Apathy

• Anxiety

Autonomic Nervous System

In terms of clinical manifestation, which category do the following fall under as effecting

• Excessive sweating

• Constipation

• Urinary bladder dysfunction

• Orthostatic Hypotension

Cardiopulmonary Function

In terms of clinical manifestation, which category do the following fall under as effecting

• Low resting blood pressure

• Compromised cardiovascular response to exercise

• Impaired respiratory function

Low BP, Dizziness, Dyskinesias, Weakness

What are the common and severe side effects of medication affecting dopamine to treat PD?

Postural Control

What is the ability to achieve, maintain and regain balance?

Postural Instability

• Abnormal response to controlling COM w/in BOS

• Smaller function LOS

• Narrowing BOS

• Competing attentional demands

• Difficulty during dynamic and self-initiated movements

• Abnormal coactivation response…inability to access postural synergies

• Difficulty regulating feed-forward or anticipatory responses (slowed responses)

• Impaired sensorimotor integration (muscle force is not proportional to demand)

These are all causes of what in terms of PD….

Huntington’s Disease

What disease is hereditary, progressive neurodegenerative disorder characterized by emotional, behavioral, psychiatric abnormalities, and movement abnormalities? ( no breaks)

Excessive motor output from cerebral cortex

HD is the degeneration of striatum and cerebral cortex, decreased signals from BG, disinhibition of motor thalamus and PPN →

Chorea, Dementia, Disorientation and Personality Disintegration

What are the 4 characteristics associated with HD?

Balance but does not reduce falls

Balance and Training exercise improve ______ __but does not ___ ___________

FOGS ( Family story, Orientation, General info, Spelling)

What is the cognitive screen done on our patients with HD or PD (any neurological condition)?

Cognitive impairments, communication impairments, poor balance, decreased motivation, Lack of caregiver support or ability, financial limitations

What are the barriers care that we face when it comes to HD and PD?