Readings Week Four Flashcards
What was Parkinson’s disease first called?
Paralysis agitans
In 1957, Dopamine was discovered as a _______ neurotransmitter
Dopamine = Putative neurotransmitter
Patients with PD lose cells in the _______ ______.
Substantia Nigra
Concentrations of the neurotransmitter ______ are _______ in the ____ of pratients with PD
Concentrations of the neurotransmitter DOPAMINE are DECREASED in the STRIATUM of patients with PD
In 1960, the first medication trialled for PD was _____.
Levodopa
What is akinesia?
Loss of ability to move muscles voluntarily
Which came first, the injectable or oral form of levodopa?
Injectable came first
What are the 5 biological processes/phenomenon that have been identified with cell death in PD?
- Genetic mutations
- Abnormal handling of misfolded proteins by the ubiquitin-proteasome & autophagy-lysosomal systems
- Increased oxidative stress
- Mitochondrial dysfunction
- Inflammation
What are the 4 cardinal features of PD?
TRAP
- Tremor at rest
- Rigidity
- Akinesia [loss of voluntary muscle control] (or bradykinesia [slowness of movements])
- Postural instability
What are the 2 additional features/symptoms of parkinsonism?
- Flexed posture
- Freezing (motor blocks)
What does the Hoehn and Yahr scale do?
Compares groups of patients and provides a gross assessment of disease progression, ranging from 0 (no signs of disease) to stage 5 (wheelchair-bound or bedridden unless assisted)
What is the most common and well-established scale for assessing PD?
The Unified Parkinson’s Disease Rating Scale (UPDRS)
When is deterioration most rapid in PD?
In the early stages of PD and for those who have the postural instability gait difficulty (PIGD) of PD. Also may progress more rapidly the older the onset begins.
In a longitudinal study of PD patients, what was one motor activity that showed no decline?
Handwriting
What age group is most likely to develop levodopa-induced dyskinesias?
younger PD patients
The UPDRS is undergoing revisions for what two reasons?
- Needs to be more sensitive in order to detect small changes in PD patients
- Needs to integrate non-motor elements of PD
What are two things that the UPDRS does not assess that other rating scales of PD do?
- Psychiatric manifestations (e.g. depression)
- Quality of life
Bradykinesia is…..
The slowness of movements
The most characteristic clinical feature of PD is?
Bradykinesia
Why should Bradykinesia be interpreted with caution?
Can also be caused by other disorders, such as depression
Bradykinesia is known as the hallmark of ______________ disorders
Basal Ganglia Disorders
Individuals with Bradykinesia may also experience difficulties with ….. (4)
- Planning
- Initiating/executing movement
- Performing sequential tasks
- Performing simultaneous tasks
How does Bradykinesia first manifest? (4 symptoms)
Initial manifestation includes:
- Slowness in performing daily activities
- Slow movement
- Slow reaction times
- Difficulties with fine motor tasks (e.g. buttoning)
What are some less common consequences/symptoms of Bradykinesia? (6)
- Loss of spontaneous movements & gestures
- Drooling (from impaired swallowing)
- Monotonic & Hypophonic Dysarthria
- Loss of facial expression (Hypomimia)
- Decreased blinking
- Reduced arm swing while walking
How might PD be suspected before any analysis is performed?
The presence of Bradykinesia
Assessment of bradykinesia usually includes having patients perform…..
perform:
- rapid,
- repetitive,
- alternating movements of the hand (finger taps, hand grips, hand pronation supination)
- heel taps and observing not only slowness but also decrementing amplitude.
Bradykinesia, like many other PD symptoms, is dependent on a persons _______ _______.
Dependent on a person’s emotional state
What is kinesia paradoxica?
The phenomenon that environmental cues (like an emotional trigger, loud noise, marching music, a visual cue of an obstacle to step over) allow an individual with Parkinson’s disease to have little or less difficulty in performing a motor action that would otherwise prove very difficult. For example, suddenly being able to run when someone screams “fire”.
The severity of the PD symptom _____ correlates with the person’s degree of ______ deficiency.
The severity of the PD symptom BRADYKINESIA correlates with the person’s degree of DOPAMINE deficiency.
Finish the sentence:
It is hypothesised that bradykinesia is the result of…………… mediated by ……….
It is hypothesised that bradykinesia is the result of a disruption in normal motor cortex activity mediated by reduced dopaminergic function.
Bradykinesia studies suggest that there is an:
- Impairment in the recruitment of …(2)….. that regulate …..(1)…….
- Increase in the recruitment of ….(1)…., such as those responsible for …(1)……
- Impairment in the recruitment of cortical and subcortical systems that regulate kinematic parameters of movement (eg, velocity)
- Increase in the recruitment of various premotor areas, such as those responsible for visuomotor control
Individuals with Bradykinesia have the most difficulty with:
a) Small and slow movements
b) Small and fast movements
c) Large and slow movements
d) Large and fast movements
D. They struggle to initiate and maintain large fast movements.
Individuals with Bradykinesia show anatomical deficits localised in the _____ and _______
Individuals with Bradykinesia show anatomical deficits localised in the PUTAMEN and GLOBUS PALLIDUS
What is the most common and easily recognized symptom of PD?
Rest tremor
What are 3 key features of rest tremors?
- Tremors are unilateral (one side of the body)
- Occur at a frequency between 4-6 Hz
- Most prominent in the distal part of the extremity
How is a rest tremor different to an essential tremor?
Rest tremor can happen in the hands, lips, chin, jaw, and legs. Common in PD. Disappear with action or during sleep.
Essential tremor can happen in the neck, head, and voice. Not common in PD. Becomes more frequent when performing an action.
How does an Essential Tremor link to PD?
Having an Essential Tremor is a risk factor for later developing PD
Other than a rest tremor, what is another tremor that can manifest when PD first develops?
Postural tremor (aka re-emergent tremor). It is more prominent and disabling than a rest tremor
How is re-emergent tremor different or similar to Essential and Rest tremors?
Essential Tremor: Different in that the appearance of tremor is often delayed after the patient assumes an out-stretched horizontal position
Rest Tremor: Similar in that it occurs at the same frequency and is responsive to dopaminergic therapy (classed as a variant of rest tremors).
What are some signs that a person might have an Essential tremor, not a PD re-emergent tremor?
- longstanding history of action tremor,
- family history of tremor,
- head and voice tremor,
- no tremor occurs when arms are outstretched in a horizontal position in front of the body
What type of tremor can be present at the start of PD but disappears later into the disease? (only happens to 9% of people)
Rest tremor
Tremor in PD patients is associated with the degeneration of neurons in the…..
Midbrain
What is the cogwheel phenomenon, or cogwheel rigidity?
muscular rigidity in which passive movement of the limbs (e.g. during a physical examination) elicits ratchet-like start-and-stop movements through the range of motion of a joint (e.g. bending the arm at the elbow). Can occur during limb flexion, extension, or rotation at a joint).
Is cogwheel rigidity proximal, distal, or both?
Can be both proximal (neck, shoulders, hip), and distal (wrist, ankle)
What is Froment’s Manoeuvre?
Voluntary movements of the contralateral (opposite to the side effected) limb that show an increase in rigidity experienced
What is Froment’s manoeuvre useful for?
Useful for detecting mild cases of rigidity
Pain in what part of the body is a common symptom at the beginning of PD? What is it often misdiagnosed as?
Pain in the shoulder. Often misdiagnosed as arthritis, bursitis, or rotator cuff injury.
Presence of what three symptoms in the elderly is associated with an increased risk for PD?
- Stiffness
- Tremor
- Imbalance
Rigidity of the ____ and ____ (also known as ____ rigidity) may result in abnormal _______.
Rigidity of the NECK and TRUNK (also known as AXIAL rigidity) may result in abnormal POSTURE.
What is striatal limb deformity?
Occurs to hands and feet, where fingers and toes extend and flex outwards.
Striatal deformities are most common in individuals with PD who:
- are younger and had an earlier onset of PD symptoms
- are older and had an earlier onset of PD symptoms
- are younger and had a later onset of PD symptoms
- are older and had a later onset of PD symptoms
- younger and had an earlier onset of PD symptoms
What are the 3 common skeletal abnormalities present in PD patients?
- Extreme neck flexion
- Truncal flexion
- Scoliosis
What is Camptocormia?
Extreme flexion of the thoracolumbar (junction between thoracic and lumbar spine) spine
What makes Camptocormia better and worse?
Camptocormia is:
Exacerbated by - Walking
Relieved by - sitting, lying in supine position, and extending trunk by leaning against a wall/table.
What is Pisa syndrome?
Tilting of the trunk when sitting or standing (like the leaning tower of Pisa)
For patients with PD, postural instability can be caused due to…..
loss of postural reflexes
Postural instability manifests in the early/middle/late stages of PD
Late stages
What is the pull test?
Used to test postural instability. Involves quickly pulling a patient backwards or forwards by the shoulders, and assessing the degree of retropulsion or propulsion.
What is considered an abnormal postural response in the pull test? (2)
- taking more than 2 steps backwards
- absence of any postural response
The L_____ L_____ for onset of ____ in PD patients distinguishes PD from other neurodegenerative disorders
Long Latency for onset of Falls
What are 5 other factors that contribute to postural instability in PD patients? (other than PD related loss of reflexes)
- parkinsonian symptoms
- orthostatic hypotension
- age-related sensory change
- ability to integrate visual, vestibular, and proprioceptive sensory input (kinesthesia)
- fear of falling
What form of akinesia (loss of movement) is the most disabling symptom of PD?
Freezing, also known as motor blocks. Does not occur in all PD patients.
What parts of the body can be affected by freezing (aka. motor blocks)?
Legs, arms, and eye lids
How long does feezing/motor blocks usually last?
<10 seconds
What are the 5 subtypes of freezing?
- Start hesitation
- Turn hesitation
- Hesitation in tight quarters
- Destination hesitation
- Open space hesitation
Risk factors for the development of freezing include (4)
Risk factors for the development of freezing include:
- presence of rigidity,
- bradykinesia,
- postural instability,
- longer disease duration
Tremor at PD onset is associated with an increased or decreased risk of freezing?
tremor = decreased risk of freezing
Does freezing respond to dopaminergic therapy during the ON or OFF period?
During OFF periods, dopaminergic therapy, like levodopa, do not work efficiently and freezing is not stopped. So, freezing will only respond during an ON period.
What are two primitive reflexes that tend to re-emerge in PD, due to a breakdown of the frontal lobe inhibitory mechanisms?
- Glabellar reflex
2. Palmomental reflex
What are mirror movements and what could they indicate?
Mirror movements are the presence of unintended movements that accompany the voluntary activity in homologous muscles on the opposite side of the body. They can be an indicator of early asymmetric PD.
What is bulbar dysfunction?
difficulty swallowing, weak jaw and facial muscles, progressive loss of speech, and weakening of the tongue
What is bulbar dysfunction?
Can include: difficulty swallowing, weak jaw and facial muscles, progressive loss of speech, and weakening of the tongue
Bulbar dysfunction symptoms are related to __________________ and ______
- orofacial-laryngeal bradykinesia
- rigidity
How are speech disorders in PD patients characterised?
Speech disorders in patients with PD are characterised by monotonous, soft and
breathy speech with variable rate and frequent word-finding difficulties( ‘tip-of-the-tongue phenomenon)
What neuro-opthalmological abnormalities can be presented in PD patients? (6)
- decreased blink rate,
- ocular surface irritation,
- altered tear film,
- visual hallucinations,
- blepharospasm,
- decreased convergence
Are respiratory disturbances a consequence of PD?
Yes, they can be
What are 4 non-motor symptoms of PD?
- autonomic dysfunction
- cognitive/neurobehavioural disorders
- sensory abnormalities
- sleep abnormalities
Is autonomic failure a sign of PD, or just somatic failure?
Failure can occur in both the autonomic nervous system (heart, kidney, stomach…) and somatic nervous system (arms, legs…)
How much higher is the risk of developing dementia in patients who are diagnosed with PD?
6 times higher risk for dementia
What are the 4 common neuropsychiatric comorbidities?
- Depression
- Apathy
- Anxiety
- Hallucinations
What is hedonistic homeostatic dysregulation, and what is it caused by?
A set of behavioural symptoms caused by dopamine dysregulation syndrome (from taking dopaminergic drugs). Behaviours include obsessive-compulsive behaviours, and impulsive behaviours. (e.g. hypersexuality, binge eating, compulsive arranging… etc)
In terms of sleep, what disorder is common among PD patients and is also a major risk factor for PD?
Rapid eye movement sleep behaviour disorder. Is characterised by an increase in violent dream content, accompanied by violent/dramatic motor activity (e.g. screaming, kicking, punching…etc).
Note: Insomnia (sleep fragmentation) is also frequent in PD patients
Sleep abnormalities in PD patients might be caused by….
the 50% loss of hypocretin (orexin) neurons that can occur in PD
Which sensory dysfunction has been found to be a marker for increased PD risk and early PD stage?
Olfactory dysfunction (hyposmia)
What might cause olfactory dysfunction? (2)
- Neuronal loss in corticomedial amygdala
- Decreased dopaminergic neurons in the olfactory bulb
Is there any definitive test that can be performed to ensure an individual has PD?
Not really, the only way to know from biological tests is an autopsy to find Lewy Bodys
What is tricky about early-stage PD symptoms and diagnosis?
Early-stage symptoms of PD majorly overlap with symptoms of other parkinsonism forms (other disorders that affect motor movements). Cases can be unclear if they are truly PD until the disease progresses.
What are 5 main causes of misdiagnosed PD?
- Essential tremor
- Alzheimer’s disease
- Vascular parkinsonism
- Normal aging (e.g. age-related rigidity, gait disturbance…)
- Multifactorial conditions (e.g. diabetes, cancer…)
What are the four classes of Parkinsonian Disorders?
- Primary (idiopathic) parkinsonism
- Secondary (acquired, symptomatic) parkinsonism
- Heredodegenerative parkinsonism
- Multiple system degeneration (parkinsonism plus syndromes)
What are the key traits of Parkinson’s Disease (PD) that distinguish it from Parkinsonian disorders? (5)
- tremor
- early gait abnormality (eg, freezing)
- postural instability
- pyramidal tract findings
- response to levodopa (MSA also show initial response to levodopa, but often develop dyskinesias and no longer respond to drug)
Neuroimaging of ______ ____ receptors, and ________ _________ _______ sites can help to diagnose and differentiate PD from other parkinsonian disorders.
Neuroimaging of DOPAMINE D2 receptors, and STRIATAL DOPAMINE REUPTAKE sites can help to diagnose and differentiate PD from other parkinsonian disorders.