Flashcards in PARKINSON'S DISEASE AND PARKINSONIAN DISORDERS Deck (76):
What do the basal ganglia consist of?
What are the akinetic rigid syndromes?
Multiple System Atrophy
Progressive Supranuclear Palsy
Is Parkinson's more common in men or women?
What is the average age of onset for Parkinson's disease?
What part of the brain is affected in Parkinson's disease?
Mainly the dopaminergic neurones of the substantia nigra as well as other brain areas.
What is the first presenting complaint in 60% of people who subsequently are diagnosed with Parkinson's?
What part of the substantia nigra contains the dopaminergic neurones that are affected in Parkinson's disease?
What are Lewy bodies?
Eosinophilic intra neuronal inclusions which contain alpha-synuclein protein, aggregated with abnormally phosphorylated neurofilaments and ubiquitin.
Where do the pars compacta cells of the substantia nigra project within the basal ganglia?
Other than the substantia nigra what other parts of the brain stem may be affected in Parkinson's disease?
(There may be more)
What is the classical triad of clinical features of Parkinson's disease?
Does Parkinson's normally affect limbs symmetrically?
No. The asymmetry is actually a characteristic feature.
How is the resting tremor associated with Parkinson's disease often described?
How might you exacerbate a resting tremor during an examination which might indicate Parkinson's as a more likely diagnosis?
Distraction - get the patient to concentrate on using the other limb. This should increase the tremor.
How is the increase in tone found in Parkinson's disease described?
Cog-wheel rigidity (when combined with the tremor)
Lead pipe rigidity
What is the difference between spasticity and rigidity?
Spasticity is an increased resistance to the passive movement of a joint due to abnormally high muscle tone (hypertonus) which varies with the amplitude and speed of displacement of a joint. The increased tone is more marked in the flexors of the arms and the extensors of the legs (decorticate position).
Rigidity is an increased resistance to the passive movement of a joint which is constant throughout the range of joint displacement and not related to the speed of joint movement; resistance is present in both agonist and antagonist muscles.
Rigidity is associated with a lesion in which part of the nervous system?
Basal ganglia and connections
Spasticity is associated with a lesion in which part of the nervous system?
Upper motor neuron
What is bradykinesia?
Slowness of movement with additional fatiguing and decrement of repetitive alternating movement
What is hypokinesia?
Reduced amplitude of movement
What features might be seen in the face of someone with Parkinson's?
Mask like - hypomimia
How is speech altered in someone with Parkinson's?
Monotomous hypophonic disarthria due to a combination of bradykinesia, rigidity and tremor.
How is power affected in someone with Parkinson's disease?
It is usually preserved, however bradykinesia and rigidity make testing power difficult in advanced disease.
How is sensation affected in Parkinson's disease?
Sensation is usually normal but patients may report discomfort and sensory abnormalities in the leg.
How is the gait of a Parkinson's disease patient affected?
Festinant - steps that become increasingly fast
What are the non-motor features of Parkinson's disease?
Constipation and urinary difficulties
Dementia is a common complication later on in disease
What is the cumulative incidence of dementia in Parkinson's patients?
What is the usual cause of death in Parkinson's patients?
Name 6 classes of drug used in the management of Parkinson's disease.
Monoamine oxidase B inhibitors - rasagiline
How does L-dopa work in the treatment of Parkinson's disease?
L-dopa is the precursor to dopamine so dopamine is more readily available to the dopaminergic neurones.
What is L-dopa given in conjunction with, in the management of Parkinson's disease?
A peripheral decarboxylase inhibitor such as carbidopa or benserazide. This prevents the conversion of L-dopa into dopamine in the periphery and hence prevents some of the side effects such as nausea.
What are some of the central side effects of L-dopa?
Psychiatric complications - vivid dreams, nightmares, illusions, hallucinations and impulse control disorder.
Name some of the dopamine agonists used in the treatment of Parkinson's disease.
Which of the dopamine agonists should be used in early management of Parkinson's disease?
The non-ergot derived
What are some of the side effects of the dopamine agonists used in the management of Parkinson's disease?
Why are anti-cholinergic drugs used in the management of Parkinson's disease?
To control the resting tremor
Name some of the anti-cholinergic drugs used in the management of Parkinson's disease.
What are some of the side effects of the anti-cholinergic drugs used in the management of Parkinson's disease?
What is the mechanism of action of amantidine, used in the management of Parkinson's disease?
Amantidine is an anti-viral drug which has dopamine re-uptake blocking and anticholinergic properties.
What are the side effects of amantidine, used in the management of Parkinson's disease?
Name some of the MAO-B (monoamine oxidase B) inhibitors used in the treatment of Parkinson's disease?
What is the mechanism of action of the MAO-B inhibitors used in the management of Parkinson's disease?
They block monoamine oxidase B which is used to break down dopamine in the CNS. It therefore prolongs the duration of action of dopamine.
What are the side effects of the MAO-B inhibitors, used in the management of Parkinson's disease?
Name the catechol-O-methyltransferase inhibitors used in the management of Parkinson's disease.
What are the side effects of the COMT inhibitors, used in the management of Parkinson's disease?
What are the surgical options for the management of Parkinson's disease?
Deep brain stimulation of the subthalamic nucleus has a significant effect.
What are the differential diagnoses for someone who presents with the signs and symptoms of Parkinson's disease?
Medication - Dopamine antagonists
Trauma - subdural haematomas, repetitive head injury (boxing)
Cerebrovascular disease - lacunar infarcts of the basal ganglia
Hydrocephalus or tumour
Infections - encephalitis lethargica and Japanese B encephalitis
Atypical parkinsonian disorders - multisystem atrophy, progressive supranuclear palsy, corticobasal degeneration.
What is progressive supranuclear palsy (PSP)?
A rare atypical parkinsonian condition characterised by falls early on, symmetrical parkinsonism and a prominence of axial features with cognitive decline, dysarthria, dysphagia and a striking supranuclear gaze palsy.
What is a supranuclear gaze palsy?
This is when the patient appears to become transfixed on something so that they cannot move their eyes in a vertical plane, most often downwards, and then eventually horizontal plane. However, when their head is passively tilted the eyes do stay transfixed on the same object, which differentiates this from a problem with the ocular muscles (dolls-head manoeuvre) or cranial nerves.
What are the clinical features of progressive supranuclear palsy?
History of falls early on
Supranuclear gaze palsy (initially vertical plane)
Inability to control movements - eg when asked to clap three times they will clap but won't be able to stop even though they count to three
What are the pathological findings in someone with progressive supranuclear palsy?
Neuronal loss, gliosis (proliferation of glia) and aggregates of tau protein and neurofibrillary tangles in the following parts of the CNS:
What might be seen on the MRI of someone with progressive supranuclear palsy?
Atrophy of midbrain (Hummingbird sign) and superior cerebellar peduncles
How is progressive supranuclear palsy managed?
Amantadine can be useful.
Davunetide is a neuroprotective peptide which has preclinical evidence for neuroprotective, neurotrophic and cognitive protective properties.
What is multisystem atrophy (MSA)?
Neurodegenerative disorder characterised by either parkinsonism or cerebellar signs, as well as autonomic failure. Split into two types MSA-P and MSA-C.
What is MSA-P (multisystem atrophy P)?
Asymmetrical parkinsonism that is poorly responsive to L-dopa, as well as autonomic failure including drop in blood pressure, loss of sweating, urinary incontinence or retention and early erectile dysfunction.
What is MSA-C (multisystem atrophy C)?
MSA where the cerebellar ataxia predominates with autonomic failure.
What are the clinical features of multisystem atrophy (MSA)?
Autonomic dysfunction - drop in blood pressure, loss of sweating, urinary incontinence or retention and early erectile dysfunction
Abnormal respiratory patterns - stridor, gasps and sleep apnoea
Is cognitive decline a feature of multisystem atrophy (MSA)?
What is the underlying pathology in multisystem atrophy (MSA)?
Glial cytoplasmic inclusions that contain alpha-synuclein in the basal ganglia, cerebellum, pons/medulla and motor cortex.
How is multisystem atrophy managed?
Treatment is basically symptomatic, although amantadine and L-dopa should be trialled.
What will MRI of someone with multisystem atrophy (MSA) show?
Degeneration of the middle cerebellar peduncles and pons - Hot cross bun sign
What is corticobasal degeneration (CBD)?
A rare disorder that is characterised by strikingly unilateral involvement with rigidity and dystonia in an arm.
What are the clinical features of corticobasal degeneration (CBD)?
Patients often report alien limb phenomenon - where arm appears to have mind of its own.
Parkinsonian signs except for the tremor
Limb apraxia (inability to make purposeful movements)
Myoclonus of affected arm
What are some of the drugs that can cause parkinsonism?
Dopamine antagonist neuroleptics:
Phenothiazines - eg chlorpromazine
Butyrophenones - eg haloperidol
Thioxanthenes - eg flupentixol
Benzamides - eg sulpiride
What feature might help you differentiate between Parkinson's disease and cerebrovascular disease causing parkinsonism?
In cerebrovascular disease, patients typically have a small-stepped gait (marche a petit pas) with an upright stance as opposed to the stooped posture of Parkinson's.
What is Wilson's disease?
Inherited autosomal recessive disorder of copper metabolism, resulting in low levels of the copper binding protein caeruloplasmin. This causes elevated levels of free copper in the blood, which results in copper deposition in the brain, particularly in the basal ganglia.
What are the clinical features of Wilson's disease?
Prominent psychiatric symptoms
What is the peak age for patient with Wilson's disease to present?
Teens or early 20s
Not after 40
How is the diagnosis of Wilson's disease made?
Penicillamine challenge - leads to low levels of caeruloplasmin and high levels of free copper in urine and serum.
Liver biopsy can provide tissue diagnosis
What might the MRI of someone with Wilson's disease show?
Signal change in the putamen
How is Wilson's disease treated?
Copper binding agents such as penicillamine, trientine or tetrathiomolybdate
What are the non-motor features of Parkinson's disease?
Sleep related problems:
Excessive daytime sleep
REM sleep behaviour
(Look up in the movement disorder lecture)
What imaging can be used to differentiate between Parkinson's disease and essential tremor?
Other than drugs, what other causes of secondary Parkinson's disease can you think of?
Repeated head trauma - boxing
What additional part of the examination might you get a Parkinson's patient to do over the next few days?
A Parkinson's diary