Lecture 6: Movement Disorders Flashcards
These are classified as conditions that produce inadequate or excessive movement
Movement disorders
This is termed as Paucity, Slowness, Or Too Little Movement
Hypokinetic
This is termed as Excessive, Involuntary, or Too Much Movement
Hyperkinetic
This is termed as Abnormal movement
Dyskinesia
Parkinson’s disease and Atypical Parkinsonian Syndromes are classified as what type of movement disorders?
HypoKinetic
Essential Tremors, Huntington’s disease, And Dystonia are classified as what type of movement disorders?
Hyperkinetic
Movement disorders impair the regulation of voluntary activity without directly affecting what 3 things?
- Strengths
- Sensation
- Cerebellar Function
Movement disorders usually involve what structure in the brain?
The Basal Ganglia
Inputs to the motor system
Descending systems from the cortex are classified as?
Upper Motor Neurons
Inputs to the motor system
The motor cortex is responsible for what 3 tasks?
- Planning
- Initiating
- Directing Voluntary Movements
Inputs to the motor system
The brainstem centers are responsible for what 2 things?
- Basic Movements
2. Postural control
Inputs to the motor system
What is the responsibility of the Basal Ganglia?
Gating proper initiation of movement
Inputs to the motor system
What is the responsibility of the Cerebellum?
Sensory motor coordination of ongoing movement
Inputs to the motor system
The Basal Ganglia and the Cerebellum directly affect what aspects of the descending systems (UMN)?
Motor cortex and Brainstem Centers
Inputs to the motor system
The Local Circuit neurons (LMN) receives information from what 3 regions?
- Motor Cortex (UMN)
- Brainstem Centers (UMN)
- Sensory Input (LMN)
Inputs to the motor system
The spinal cord and brainstem circuits consist of? (2)
- Local Circuit Neurons (LMN Integration)
2. Motor Neuron Pools (LMN)
Inputs to the motor system
Sensory inputs send information to what region of the body?
Local Circuit neurons (LMN Integration)
Motor Neuron pools directly affect what aspect of the body?
Skeletal Muscle
This is a collection of Grey Matter Nuclei located deep within the white matter of the cerebral hemispheres
Basal Ganglia
What are the main components of the Basal Ganglia? (5)
- Caudate Nucleus
- Putamen
- Globes Pallidus
- Subthalamic Nucleus
- Substantial Nigra
What 3 regions of the Basal Ganglia make up the Striatum?
- Caudate Nucleus
- Putamen
- Globus Pallidus
What 2 parts of the Basal Ganglia make up the Lenticular Nucleus?
- Putamen
2. Globus Pallidus
The Basal Ganglia has what 2 types of Pathways?
- Direct
2. Indirect
Movement disorders associated with the Basal Ganglia are often explained by an (Blank) in the direct and indirect pathways.
Imbalance
-between the direct and indirect pathways in the basal ganglia
What are the Functional Attributes of the Basal Ganglia? (4)
- Entire Cerebral Cortex Provides input to the BG
- There is no direct sensory input to the BG
- BG does not reject directly to UMN/LMN
- BG lesions produce
- Motor, behavior, and/or cognitive impairments
Basal Ganglia Lesions produce what 3 impairments?
- Motor Impairments
- Behavior Impairments
- Cognitive Impairments
Lesions of the Basal Ganglia motor circuits either produce? (2)
- Poverty of movement/Hypokinesia
2. Unwanted movement/Hyperkinesias
What are the 3 types of Hypokinesia?
- Akinesia
- Bradykinesia
- Rigidity
What are the different types of Hyperkinesias? (6)
- Resting Tremors
- Chorea
- Athetosis
- Tics
- Hemiballism
- Diatonic
Define: slowness of movement
Bradykinesia
Define: Loss or absence of movement
Akinesia
Define: Stiffness of muscle tone with passive movements
Rigidity
What are the 3 common signs seen in a patient with Parkinson’s Disease?
- Bradykinesia
- Akinesia
- Rigidity
What are the 4 main symptoms of Hyperkinesia movement Disorders?
- Tremors
- Chorea
- Dystonia
- Myoclonus
Define: Oscillatory, usually rhythmical and regular movement affecting one or more body parts
Tremor
Define: Random, quick, unsustained, purposeless movements that have an unpredictable, flowing pattern
Chorea
Tremors can be seen in what 2 disorders?
- Parkinson’s disease
2. Essential Tremors
Chorea can be seen in what 2 disorders?
- Huntington’s disease
2. Cerebral Palsy
Define: Torsional movements that are partially sustained and produce twisting postures
Dystonia
Define: Sudden, Brief, shock-like, involuntary movements usually caused by muscular contraction
Myoclonus
What is the prevalence of Parkinson Disease?
Cases per year?
160 cases per 100,000 people
6-7 million worldwide cases
1-2 million in US
At age 70, Increases to 550 cases per 100,000 people
What is the incidence of Parkinson’s Disease
Male to Female Ratio?
Mean age of symptoms onset?
Male 2:1 Female
Mean age of symptom onset is 56 in both sexes
With Parkinson’s disease, What is the etiology?
- Most cases are sporadic-Unknown cause
- Idopathic PD - Genetic Contributions 5-40% of cases
- Environmental Contributions
What is the phrase for Parkinson’s disease etiology?
“Genes load the gun”
“The Environment pulls the trigger”
Parkinson’s Disease is the degeneration of (Blank) neurons within the (Blank) and the (blank) in the brainstem
- Dopamine-Producing Neurons
- Substantial Nigra Pars Compacta
- Locus Ceruleus
Where does Parkinson’s disease effect the brain? (2)
Substantial Nigra Pars Compacta
Locus Ceruleus in the brainstem
With Parkinson’s Disease, there is an accumulation of mis-folded protein alpha-synuclien that spreads through brain areas forming _______________
Lewy BOdies
With Parkinson’s Disease, when symptoms become clinically evident, there is a 60% decrease in …
Dopaminergic Neurons of the Substantia Nigra
With Parkinson’s disease, dopamine levels have decreased by ____%
80% Decrease in Dopamine
What is the typical age of onset for Parkinson’s Disease?
Young Onset?
Late Onset?
Typical Onset: 55-60 years
Young Onset: < 40 years
Late Onset: >78 years
Describe the timeline of Parkinson’s Disease
- Death of Dopamine Cells
- Non motor symptoms (Loss of smell, constipation) and motor symptoms
- Dx
- Progression of all symptoms leading to probs with mobility
- Bed Bound: Dementia
These two created the stages for Parkinson’s Disease and there stage progression is called?
Hoehn and Yahr Stages
How long does it generally take to progression from one stage of Parkinson’s to the next?
Several Years
Hoeing and Yahr Stages of Parkinson’s Disease
Stage 1?
Stage 1: Symptoms 1 side of body
Hoeing and Yahr Stages of Parkinson’s Disease
Stage 2?
Stage 2: Bilateral/Axial
-No Balance Impairments
Hoeing and Yahr Stages of Parkinson’s Disease
Stage 3?
Stage 3: Balance Impairments
-Physically Independent
Hoeing and Yahr Stages of Parkinson’s Disease
Stage 4?
Stage 4: Severe Disability
-Able to stand or walk
Hoeing and Yahr Stages of Parkinson’s Disease
Stage 5?
Stage 5: W/C Bound or bed ridden
In order to be diagnosed with Parkinson’s Disease, what 2 things must be present?
- Bradykinesia and/or Resting Tremors
- 1 other disorder related to Parkinson’s
- Non motor Symptoms
- Freezing
- Flexed Posture
- Loss of postural reflexes
- Rigidity
- Bradykinesia
- Resting Tremors
What are the 7 clinical findings related to Parkinson’s disease?
- Resting Tremors
- Bradykinesia
- Freezing
- Nonmotor Symptoms
- Flexed Posture
- Loss of Postural Reflexes
- Rigidity
What are the 6 Non-Motor Symptoms associated with Parkinson’s Disease?
- Fatigue
- Sensory awareness
- Sleep Disorders
- Neuro-psychiatric & Cognitive
- Autonomic Dysfunction
- Bradyphrenia
What are the other clinical findings associated with Parkinson’s disease.
Other Associated motor features? (5)
- Hypophonia (Soft Voice)
- Hypomimia (Masked Face
- Micrographia (Small Writing)
- Gait Abnormality (Shiffling, Freezing, En bloc turns (moving non-segmentally), Small Stride)
- Stooped, Flexed (Simian) Posture
How do resting tremors appear?
At rest, the tremor is occuring
How does Bradykinesia Present?
Tested with finger tapping test
Can someone close their index and thumb as quickly and big as possible
-The sped of the movement slows down and the size of the opening decreases
What does a masked face look like?
The patient has no expression at all during any movement. Robot face if you will
How does postural instability present with Parkinson’s Disease?
Patient is pulled backwards and the patient should be able to maintain balance.
-Parkinson’s patients will fall backwards with no reflexive step. Looks like a trust fall
how does freezing and festinating gait present with Parkinson’s disease?
Very Short Steps
Shuffling and freezing are one in the same
Festering means quick little short steps
What test is used to diagnose Parkinson’s disease?
NO TEST
How does an MRI appear for a patient with Parkinson’s disease
NORMAL
How is Parkinson’s Diagnosed?
- Clinically based on History
- Clinical Exam
- absence of incompatible clinical
- Laboratory
- Radiologic abnormalities.
- Symptomatic Improvement with LEVADOPA helps to confirm Idiopathic PD
Symptomatic Improvement with Levodopa helps to confirm what diagnosis?
Idopathic Parkinson’s Disease
How can a PT clinically diagnose a patient with Parkinson’s Disease?
- Asymmetric Onset
- Gradual onset and progression
- Tremor is not required
- Two out of the following 6 symptoms
- Resting Tremor, Bradykinesia, Freezing, Flexed Posture, Rigidity, Loss of postural responses
* **At least one of these must be RESTING TREMOR or BRADYKINESIA
What are the 3 types of Parkinsonism?
- Primary Idiopathic Parkinson’s Disease
- Secondary Parkinsonism
- Atypical Parkinsonism syndromes
% of patients with…
- Primary Idiopathic Parkinson Disease
- Secondary Parkinsonism
- Atypical Parkinson’s Syndromes
- 80%
- 10-12%
- 3-5%
With secondary Parkinsonism, what are the 7 causes?
- Drug Induced (7-9%)
- Vascular (1%)
- Hydrochephalus (NPH)
- Trauma
- Metabolic
- Tremors
- Toxins
With Atypical Parkinsonian Syndromes, what are the causes?
- MSA
- PSA
- Dementia with Lewy BOdies
- CBD
How is Parkinson’s Disease Prevented? (5)
- Diet
- Exercise
- Environmental Agents
- Neuro protection
- early Detection and Treatment
With Parkinson’s Disease, how is it treatment medically? (4)
- Restore Dopamine producing cells
- Restore Dopamine
- Exercise
- Drugs
For Symptomatic Parkinson’s Disease, what is this treated? (5)
- Drugs
- Deep Brain Stimulation
- Rehab Therapies
- Exercise
- Treat Symptoms
What is the Gold Standard for Treating Parkinson’s Disease?
LEVADOPA
This drug converts Dopamine once it enters the brain
Levadopa
Dopamine alone does not cross what?
The blood brain barrier
What is the prognosis or Parkinson’s Disease? (5)
- Lifespan is a few years less than normal
- Disease progression can be for 20 years or more
- Currently, there is no cure
- Usually slowly progressive, but with individual variability
- death from other causes such as Heart Disease, Cancer, Secondary Complications like Pneumonia
What is the typical progression of Mild Parkinson’s Disease (5-7 Years post DX)? (4)
- Movement symptoms inconvenient, but do not affect daily activities
- changes in posture, walking ability or facial expression
- Parkinson’s Medication Effective
- Regular Exercise is important
What is the typical progression of Moderate Parkinson’s Disease (7-15 years post DX)? (5)
- Motor Fluctuations and Dyskinesias
- Freezing Episodes occasional
- Regular exercise and skilled physical therapy very important for good mobility and balance
- Goal to keep them moving as often and safely as possible
- Gait and Balance duel task difficulty
What is the Typical Progression of Advanced Parkinson’s Disease (15-20 years post DX)? (6)
- Cognitive problems may be prominent
- Balance and Gait dual task difficulty - Medication Less Effective
- May have frequent Freezing or Falls
- Swallowing - Dysphagia
- Autonomic dysfunction
- Severe Orthostatic Hypotension
- Severe Constipation causing impaction - Weight loss
For PT’s what is the clinical management of a patient with Parkinson’s disease? (6)
- Meaningful exercises the encourage power, extensor strengthening, body awareness, timing, coordination, and agility
- Dual task training
- Functional training with repetition, progression, and variability
- Vigorous aerobic exercise
- Skill-Specific practice
- Stress Reduction
For Clinical management of the patient with Parkinson’s Disease, what are the key things we need to identify in these patients?
- Posture
- Gait Difficulties (Shuffling, Freezing, Turns)
- Balance
- Functional Mobility
- Dexterity
- Brady Kinesia
- Tremors
- Fatigue
- Difficulty Dual tasking
- Pain
This can be known as Parkinson Plus Syndrome
Atypical Parkinsonian Syndromes
What are the 3 major syndromes for Atypical Parkinsonian Syndromes/
- Progressive Supranuclear Palsy (PSP)
- Multiple System Atrophy (MSA)
- Corticobasal Degeneration (CBD)
What is the Prevelence of Progressive Supranuclear Palsy (PSP)?
More frequent in men
Mean age onset 65 years of age
What is the most frequent Atypical Parkinsonian Syndrome?
Progressive Supranuclear Palsy
This form of Parkinson’s is marked by neuronal degeneration and neurofibrillary tangle and Tau-positive astrocytes in the basal ganglia and brainstem structures
Progressive Suprannuclear Palsy
This form of Parkinson’s has a poor response to levodopa and dopamine agonists
Progressive supranuclear palsy
What happens in Progressive Supranuclear palsy to their striatal neurons and their post synaptic dopamine receptors?
Loss of Striatal Neurons
Loss of Post Synaptic Dopamine Receptors
What is the disease course for Progressive Supranuclear Palsy?
Insideous
Progressive
More rapid than PD
What is the lifespan associated with progressive supranuclear palsy?
Death in 5-10 years often due to aspiration
Describe the disease course for Progressive Supranuclear palsy
- Parkinsonian Symptoms, cognitive involvement, early falls, Axial rigidity, and absence of tremors
- Freezing, Supranuclear Gaze Palsy, Wide based Gait, Affected Speech, Facial Dystonia
- Progressive dementia, Dysphagia, Dysarthria, Emotional lability, volatile mood
- Progressive motor and cognitive decline until death
What are the key clinical findings for Progressive Supranuclear Palsy (PSP)? (6)
- Progressive Parkinsonism
- Vertical Supranuclear ocular palsy or slow vertical Saccades
- Early onset of Falling
- Axial Rigidity
- Usually no Tremors
Thesepatients eventually develop symptoms of both Parkinson’s disease, Autonomic Dysfunction, and Cerebellar Signs
Multiple Systems Atrophy (MSA)
What is Multiple systems Atrophy
- Parkinsons disease
- Autonomic Dysfunction
- Cerebellar Signs
With Multiple system atrophy, there is marked Neuronal degeneration of what? (3)
- Striatonigral pathways
- Olivopontocerebellar pathways
- Corticospinal pathways
Multiple system atrophy, unlike Parkinson’s is more ________
Progressive
At what stage are patients with Multiple Systems Atrophy (MSA) wheelchair bound?
5 years
What is the mean survival rate of patients with Multiple systems Atrophy (MSA)
8-9 years
Describe the disease course for Multiple System Atrophy
- Parkinsonian symptoms, Cognitive involvement, Autonomic dysfunction, cerebellar Ataxia
- Postural Instability, UMN signs, Bowel and Bladder
- Progressive Dementia, Dysphagia, Dysarthria
- Progressive Motor and Cognitive Decline until death
What are the key feature for Multipe System Atrophy? (4)
- Parkinsonism
- Symptomatic Orthostatic Hypotension
- cerebellar Ataxia
- Poor Therapeutic Response to Levadopa
What are the additional Symptoms associated with Multiple System Atrophy? (5)
- Increased DTR
- Bowel/Bladder Dysfunction
- Dysphagia and Dysarthria
- Early Postural Instability
- May not have tremors
This is diagnosed as Nerve cell loss and atrophy of multiple areas of the brain including cortex and basal ganglia.
Corticobasal Degeneration
Corticobasal Degeneration leads to significant parietal atrophy and is
A. Symmetric
B. Asymmetric
B. Asymmetric
When does Corticobulbar Degeneration typically occur?
60
Corticobasal degeneration usually starts to affect how much of the body?
1 side, but eventually becomes symmetric
Patient’s with Corticobulbar Degeneration progress over the course of 6 to 8 years, but death is generally caused by …
Pneumonia
What are the key features for Corticobulbar Degeneration? (3)
- Parkinsonism
- Unilateral arm rigidity and Dystonia
- Cortical Sensory Deficits
What are the other possible symptoms of Corticobasal Degeneration aside from the key features?
- UMN Features (Hyperreflexia and + Babinski)
- Apraxia
- Dementia
- Rigidity
- Dysphagia
- Myoclonus
- Alien Limb Phenomenon
With Atypical Parkinsonian Syndromes, patients are often initially diagnosed with Parkinson’s Disease, but clinical findings and the fast rate of progression are indicative of Atypical progression. And MRI and PET can be performed, but are not diagnostic. PT’s should look for neuropsychiatric cognitive involvement.
With Atypical Parkinsonian Syndromes, patients are often initially diagnosed with Parkinson’s Disease, but clinical findings and the fast rate of progression are indicative of Atypical progression. And MRI and PET can be performed, but are not diagnostic. PT’s should look for neuropsychiatric cognitive involvement.
This is a rhythmical sinusoidal movement of body parts
Tremor Syndromes
With tremors, what part of the body may be involved?
All parts.
In order to diagnose tremor syndrome, what must be checked?
Distinction that is made between rest and action tremor
What is the definition of the resting tremor?
Tremor in body part not activated or supported against gravity
What is a postural tremor?
Tremor in body part being supported AGAINST GRAVITY
This is a tremor during any voluntary contraction of muscle
Action Tremor
This is a tremor in a body part during any voluntary movement
Kinetic Tremor
This is a tremor in a body part during non goal-directed movement
Simple Kinetic Tremor
This is a tremor where the tremor in the body part increases during pursuit of a goal
Intention Tremor
What is the different between simple kinetic tremor and intention tremor
Simple kinetic tremor is non goal directed
Intention tremor is goal directed
What is the most common adult-onset movement disorder?
Essential Tremor
When can essential tremors occur?
Most common on early 20’s or later in adulthood.
Who is more likely to have an essential tremor, males or females?
Both are likely
What is the Pathogeneis of Essential tremors?
Neuro Degeneration of the Cerebellum
Abnormal GABA function
What is the etiology of Essential Tremors?
Familial in 50-70% of patients with Autosomal dominant transmission
Essential tremors are not necessarily a progressive neurodegenerative disease, but tremor may do what?
Spread to different parts of the body
Essential tremors are what kind of tremors?
Action Tremors
What are the 4 clinical findings for Essential tremors?
- Action tremor of arms, head, voice
- Family history of tremors
- Absence of Parkinsonism
- Transient improvement of tremor with alcohol ingestion
How does one determine if a patient has essential tremors?
- Absence of rest tremor
- Symmetric onset of action tremor
- Absence of other neurological signs (PD and Cerebellum)
- 50% are alcohol responsive
What are the 3 differential diagnosis of Essential Tremors?
- Parkinson Disease
- Medication related tremor
- Enhanced Physiological Tremor
How are essential tremors medically managed?
- Primidone: Anticonvulsant
- Propranolol: non-selective beta blocker used in treatment of hypertension
- Botox
- Surgical treatment: DBS in the thalamus
What clinical management can be given for patients with essential tremors?
Adaptive equipment
-Tremor canceling spoons, pens weighted or larger utensils
PD is an extensive disease with both significant ________ and __________ involvement
Motor and Nonmotor
For the dx of PD, a person must have at least?
Tremors and/or Bradykinesia, and 1 other factor related to PD.
This is the most common adult-onset movement disorder
Essential Tremors
Essential tremors are what kind of tremors?
Action tremors
What is the key difference between Essential tremors and PD tremors?
Essential tremors do not occur at rest like PD tremors
What is the etiology of PD?
Unknown
-Genetic and Environmental factors
What is the etiology of Atypical Parkinsonian Syndromes (PSP, MSA, and CBD)
- PSP: Progressive Supranuclear Palsy
- MSA: Multiple System atrophy
- CBD: Corticobasal Ganglionic Degeneration
Unknown
What is the etiology of Essential Tremors
50-70% inherited
-Sporadic
What are the signs and symptoms associated with PD? (4 key)
- Bradykinesia
- Rigidity
- Resting Tremor
- Postural Instability
What are the signs and symptoms associated with Atypical Parkinsonian Syndrome?
- PSP: Progressive Supranuclear Palsy
- MSA: Multiple Systems Atrophy
- CBD: Corticobasal Ganglionic Degeneration
(4)
- Early Falls
- Gaze Abnormalities
- Autonomic Dysfunction
- Early Dementia
What are the signs and symptoms associated with Essential Tremors? (4)
- Action Tremors
- UE> Head
- Voice > LE
- Absence of other neurological signs related to PD
How is PD Diagnosed?
Clinically
- Motor Signs/Symptoms
- Asymmetric Onset
- Gradual
- Responsive to Levadopa
What is the Diagnosis for Atypical PArkinsinan Syndromes?
PSP: Progressive Supranuclear Palsy
MSA: Multiple Systems Atrophy
CBD: Cotricobasilar Ganglionic Degeneration
(2)
Clinical
- Non-Responsiveness to Levadopa
- Possible findings in MRI Imaging
What are the diagnoses for Essential Tremors?
Clinical
- > 50% alcoholic Responsiveness
- Large Tremulous writing
- Bilateral Symmetric Onset
What is the typical progression of PD?
Gradual Progression 20+ years
What is the typical progression of Atypical Parkinsonian Syndromes
PSP: Progression Supranuclear Palsy
MSA: Multiple Systems Atrophy
CBD: Corticobasaliar Ganglionic Degeneration
More Rapid Progression
What is the typical progression of Essential Tremors?
Stable or mildly progressive to other body areas
How is PD Treated? (3)
- Medication (Levadopa)
- DBS (Deep Brain Stimulation
- Exercise!
How is Atypical Parkinsoniann Syndrome typically treated?
PSP: Progresive Supranuclear Palsy
MSA: Multiple Systems atrophy
CBD: Corticobasilar Ganglionic Degeneration
Medication less effective
Symptomatic
Exercise
How are Esential Tremors Treated?
- Medication (Proponolol, Primidone)
2. DBS (Deep Brain Stimulation)