Movement disorders Flashcards by Adele Harris

In movement disorders what are the three anatomical areas there could be problems with?

Corticospinal/pyramidal tract
Basal Ganglia
Cerebellum

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What are the three features of Parkinsonism?

Akinesia
Tremor
Rigidity

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What is akinesia?

Slowness of movement (bradykinesia) or difficulty initiating movement with small amplitude of movements (hypokinesia)

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What is the tremor in Parkinsonism?

4-6Hz tremor, described as “pill-rolling tremor”

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If Parkinsonism will have a positive DAT scan TRUE/FALSE

TRUE

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What is Parkinson’s disease?

Common and complex progressive neurodegenerative movement disorder

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I am Parkinsons disease

OLD >55 (more common with age)

Male ( Males 1.5x likely to develop)

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80% will have dementia after 15-20years of Parkinson’s disease - Must have Parkinsonism for how long prior?

1 year

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What is Parkinson’s disease characterised by?

Levodopa responsive parkinsonism

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Histopathologically what is Parkinson’s disease characterised by?

Dopaminergic neuron loss in the substantial nigra pars compacta
Lewy Bodies

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Postural instability is an early feature of Parkinson’s disease TRUE/FALSE

FALSE

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In parkinsons non-motor symptoms are disabling and often preceed motor symptoms. Name them

Depression
Sleep disturbances
Dementia
Hallucinations
Anosmia
Cognitive impairment
GI dysfunction

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TRUE/FALSE

Symptoms are asymmetrical in PD whereas often symmetry in drug induced or atypical PD

TRUE

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There is nothing to stop the neurodegeneration in Parkinson’s disease TRUE/FALSE

TRUE sadly

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How does the symptomatic drug treatment for Parkinson’s work?

Increase dopamine concentration
OR
Directly stimulate dopamine receptors

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What is the mode of action of levodopa?

Crosses BBB and converted to dopamine

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What are the SE of levodopa?

Hypotension
Nausea
Dyskinesia

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What do the benefits of levodopa look like?

Initially smooth throughout the day (honeymoon period)
Over time patients notice akinesia or wearing off their doses, and around the same time may develop an “overshoot” from akinesia to dyskinesia when the dose is working

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How do MOAB inhibitors work?

Inhibit monoamine oxidase B (an enzyme responsible for catabolising dopamine to homovanillic acid)

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How do dopamine agonists work?

Cross BBB

Act directly on dopamine (D2 type) receptors

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With what drug can you get a hypertensive crisis when consuming tyramine-containing foods?

MOAB

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Why might Dopamine agonists be considered for 1st line in younger patients with Parkinson’s?

Due to high risk of dyskinesia in younger patients using levodopa

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Why are dopamine agonists not prescribed to the elderly?

Associated with hallucinations

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With drug induced Parkinsonism what is the treatment?

Cessation of causative drug
If on typical antipsychotic change to atypical due to lower risk of extrapyramidal symptoms
Anticholinergics e.g. Benztropine & procyclidine (can be used for symptomatic relief)

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What is Lewy body dementia?

Dementia/cognitive symptoms occurring before/same time as parkinsonism

What are the three core features of Lewy body dementia?

Dementia Recurrent visual hallucinations Parkinsonism features

REM sleep behaviour disorder may also be present in Lewy body dementia. What is REM sleep behaviour disorder>

Acting out their dreams, purposeful movement

What are Lewy bodies?

Aggregates of a-synuclein and ubiquitin Single/multiple intracytoplasmic, eosinophilic, round to elongated bodies that have a dense core and a pale surrounding halo

What is the treatment for Lewy body dementia?

Cholinesterase inhibitors

Multiple system atrophy has a good response to levodopa TRUE/FALSE

This is a lie | Poor levodopa response

What does multiple system atrophy present as?

Autonomic dysfunction and/or cerebellar dysfunction

What is the MRI appearance of multiple system atrophy?

Putaminal atrophy | "Hot-cross bun" appearance of pons in the axial section

What sign is seen on MRI is the patient has progressive supra nuclear palsy?

"hummingbird" sign

What is progressive supra nuclear palsy?

Degenerative disease that causes axial akinesia and rigidity, loss of balance and unexplained falls, forgetfulness, dysarthria and loss of eye movements

TRUE/FALSE | Those with vascular Parkinsonism tend to have more problems with gait than tremor

TRUE

Vascular Parkinsonism progresses quickly in comparison to the other types of Parkinsonism

FALSE | It progresses very slowly

What can be used to differentiate vascular Parkinsonism from other Parkinsonism syndromes?

SPECT

What is a tremor?

Rhythmical sinusoidal oscillation of a body part

What are the causes of a resting tremor?

Parkinson's disease Drug-induced Parkinsonism Psychogenic tremor

Intention tremor is worse as it gets closer to the target TRUE/FALSE

TRUE

What is essential tremor?

Movement disorder characterised by a postural/action tremor rather than a resting tremor as seen in Parkinsons Progressive neurological disease AD Bilateral action tremor of the hands/forearms, absence of other neurological signs

What are triggers for essential tremor?

Alcohol, sleep deprivation. and carbamazepine

What is the treatment for essential tremor?

Propanolol Primidone It often actually improves after alcohol

What is an important DDx to consider withqdystonia/tremor/chorea especially if young?

Wilsons disease

What condition is intention tremor seen most commonly in?

Multiple sclerosis

What is a Holmes (rubral) tremor?

A tripartite tremor incorporating... - -> Tremor at rest - -> Postural tremor - -> Intention tremor

What is dystonia?

Abnormal posture of the affected body part

What causes dystonia?

co-contraction of agonist and antagonist muscles

What is the age that determines is the dystonia is classed as early-onset or late-onset?

26 years

What is the treatment for focal dystonia?

Botulinum toxin

If there is any treatment for dystonia then what is it?

Anticholinergics

What is the typical onset of young onset primary dystonia?

Late childhood/early teens | Limb onset is typical with spread of symptoms over months to 2 years often generalisation

With young onset primary dystonia 50-60% of patients will have a mutation in what gene?

DYT1 (on chromosome 9q)

What is the inheritance of young onset primary dystonia?

AD inheritance with reduced penetrance

What is chorea?

Continuous spontaneous jerky movements

How do you differentiate chorea from myoclonus and tics?

Myoclonus (short and not flying around) | Tics (suppressible)

What is the treatment for chorea?

Treat underlying cause | Symptomatic treatment with - terabenazine or dopamine receptor blocking drugs

What is Huntington's disease?

Slowly progressive AD neurodegenerative disorder (associated with CAG triplet repeat affecting Huntington gene on Chr4)

How many CAG repeats have to be present for Huntington's disease to occur?

>=40

What are the early signs of Huntington disease?

``` Personality changes Depression Apathy Clumsiness Dysarthria Dysphagia Abnormal eye movements ```

What are the late signs of Huntington's disease (as there is progressive degeneration of the basal ganglia and cerebral cortex)?

Chorea Rigidity Dementia

What are the neurotransmitter changes in Huntington's disease?

Decreased GABA Decreased Ach Increased dopamine

Name an anti-choric drug that can be used in Huntington's disease?

Tetrabenazine

What is Sydenham's chorea?

One of the major manifestations of rheumatic fever

How does Sydenham's chorea present?

Widespread chorea Behavioural disturbances Obsessive-compulsive symptoms

Who does sydenhams chorea affect and how long for?

In children , mainly girls 5-15 Rare in developed world Self-limiting, usually resolves in 6 months

What are tics?

Relatively brief rapid intermittent stereotyped movements/sounds

TRUE/FALSE | Adult onset tics are rare and are often due to a secondary cause

TRUE

What must be met for Gilles de la Tourette syndrome to be diagnosed?

Tourette syndrome inc multiple motor tics and 1+ phonic and/or vocal tics, which must last longer than a year (<3m tic free)

When is the onset of Gilles de la Tourette syndrome?

<18 years

Gives examples of simple tics?

Sniffling, throat clearing, gulping, snorting and coughign

Give examples of vocal tics

Barking, making animal noises, inappropriate voice intonations and uttering strings of words

TRUE/FALSE | 90% of those with Tourettes have psychiatric co-morbidity

TRUE(most common is ADHD followed by OCD)

What medication can be used for Tourettes?

Risperidone | If co-existing ADHD can use clonidine

What is myoclonus?

Sudden, brief shock-like involuntary movements

Name 3 reasons behind myoclonus?

Symptom of neurogenic diseases Severe hypoxia Secondary to drugs/toxic causes

What is hemiballism?

Wild flinging/throwing movements of one arm/leg | Usually as a result of a cerebrovascular lesion to sub thalamic nucleus

Restless leg syndrome is what?

Unpleasant sensation or urge to move in the legs, classically at night, relieved by getting up and walking about

What are some of the associations with restless leg syndrome?

Iron deficiency Uraemia Pregnancy possibly with Parkinson's

How is restless leg syndrome treated?

Dopamine agonists (pramipexole)