1. Explanation of diagnosis, nurse specialist, patient support groups Physiotherapy, occupational therapy, SALT (speech and language therapy) ``` Drug treatments: L-dopa Dopamine agonist Amantadine COMT inhibitor MAO-B inhibitor ```

Movement disorders Flashcards by Maria Sasakis

Where in the brain is the substantia nigra?

Midbrain

How well did you know this?

Not at all

Perfectly

What are the 2 parts of the striatum?

Caudate and Putamen

How well did you know this?

Not at all

Perfectly

What is the globus pallidus also known as?

Pallidum

How well did you know this?

Not at all

Perfectly

Which parts of the brain are heavily involved in movement disorders and why?

The SN, striatum, pallidum, and subthalamic nuclei

Control movement via thalamo-cortical projections

Affected = movement disorders

How well did you know this?

Not at all

Perfectly

Which disease becomes very similar to Lewy Body Dementia later in the disease?

Parkinson’s (eventually it starts to affect the parietal lobe)

How well did you know this?

Not at all

Perfectly

What degenerates in PD?

Dopaminergic neurons in the SN degenerate

How well did you know this?

Not at all

Perfectly

What does too little dopamine cause?

Hypokinesia (Bradykinetic, akinetic)

Too little movement

How well did you know this?

Not at all

Perfectly

What does too much dopamine cause?

Hyperkinetsia (Dyskinetic)

Too much movement

How well did you know this?

Not at all

Perfectly

What effect does dopamine replacement have on patient life expectancies?

Dramatically increases the life expectancy

How well did you know this?

Not at all

Perfectly

What diseases are treated with dopamine blockers?

Schizophrenia

How well did you know this?

Not at all

Perfectly

What are the side effects of dopamine blockers?

Parkinsonian-like symptoms

How well did you know this?

Not at all

Perfectly

People used to believe PD wasn’t genetic.

What happened to change that?

1997

An autosomal dominant family with alpha synuclein mutation developed Parkinson

Alpha synuclein was discovered to be a hallmark of PD

How well did you know this?

Not at all

Perfectly

Describe the pattern of dopaminergic neuron loss in PD

Progressive and asymmetric

How well did you know this?

Not at all

Perfectly

What are the symptoms of PD?

Stiffness of arm/shoulder
Tremor
Handwriting/micrographia
Repetitive tasks gets slower on repeating
Generalised slowing down

How well did you know this?

Not at all

Perfectly

What are signs of PD?

Bradykinesia
Cogwheel rigidity
Gait disorder
Rest tremor

How well did you know this?

Not at all

Perfectly

What percentage of people with PD have a tremor?

50%

How well did you know this?

Not at all

Perfectly

How do you treat PD?

Explanation of diagnosis, nurse specialist, patient support groups Physiotherapy, occupational therapy, SALT (speech and language therapy)

Drug treatments:
L-dopa
Dopamine agonist
Amantadine
COMT inhibitor
MAO-B inhibitor

How well did you know this?

Not at all

Perfectly

What is the mechanism of action of amantadine?

Not well understood

An increase in synthesis and release of dopamine, with perhaps some inhibition of dopamine uptake

How well did you know this?

Not at all

Perfectly

Name 2 L-DOPA drugs

Co-Careldopa and Co-Beneldopa

both have L-DOPA and Carbidopa

Name 3 dopamine agonists

Ropinirolee
Pramipexole
Pergolide

Name a COMT inhibitor

Entacapone

Name 2 MAO-B inhibitors

Rasagaline and Selegeline

What are the non-motor complications of treatment for PD?

Nausea
Postural hypotension
Fatigue

What are the motor complications of PD treament?

Dyskinesias due to supersensitivity
On/off fluctuations
Wearing off

How does Parkinson's progress?

Starts with motor symptoms, can go to dementia and then cause hallucinations partially due to the treatment

What is Braak's hypothesis?

Sporadic PD is caused by a pathogen that enters the body via the nasal cavity This is swallowed and reaches the gut, initiating Lewy pathology (LP) in the nose and the digestive tract It is suggested that Parkinson's spreads from the periphery upwards (ascending theory)

Map out ascending progression of PD

SN --> temporal cortex --> frontal and parietal cortices

What are the prodromal symptoms of PD?

Cognitive: Frontal-executive impairment Affective: Depression, anxiety, decreased novelty seeking Sleep: REM behaviour disturbance Autonomic: constipation Special senses: loss of sense of smell

What causes the prodromal constipation in PD?

Affects neurons in gut and in brainstem that controls the autonomic NS

What are the NINDS criteria for possible symptoms of progressive supranuclear palsy?

Vertical supranuclear gaze palsy (loss of ability to look up and down) Falls or the tendency to fall in the first year of symptoms

What are useful clues to suggest someone has supranuclear palsy?

Frontalis over-activity Impaired verbal fluency without bvFTD Decreased blink rate Erect posture with ambling gait Falls with retained postural reflexes

What is seen in the histology of someone with supranuclear palsy?

Tau tangles and tufted astrocytes

What is supranuclear palsy usually misdiagnosed as?

Parkinson's (it's actually a parkinson's plus condition)

What are the diagnostic criteria for multiple system atrophy?

Autonomic failure --> urinary incontinence (ED for men) Postural hypertension Poorly L-DOPA responsive parkinsonism or cerebellar syndrome

What is cerebellar syndrome?

Gait ataxia with cerebral dysarthria (difficulty speaking) Limb ataxia Cerebellar oculomotor dysfunction

What are clinical features of multiple system atrophy?

Poly-mini-myoclonus Action tremor Incontinence "Strangulated" dysarthria -problem with speech and swallowing REM sleep behavioural disorders Postural hypotension - black out when standing up Cerebellar eye signs Cranial-cervical dyskinesia/dystonia

Which disorder is identical to multiple system atrophy in the early stages?

What are cerebellar eye sings?

Flutter, lack of coordinationn, instability of gaze, "dancing eyes" etc.

What part of the nervous system does multiple system ataxia affect?

The autonomic nervous system

What is muscle atonia?

It is how your muscles (except for respiratory muscles and eyes) get paralyzed in REM sleep.

What is a possible prodromal symptom of multiple system ataxia?

REM sleep and behavioural disturbances

What can you see in MSA histology?

Synuclein deposits Especially in glial cells

What are the symptoms of cortico-basal degeneration?

Limb apraxia Asymmetric stiffness Dystonia Action myoclonus Cortical sensory loss Eye movement apraxia Oro-buccal apraxia (alien limb)

What is cortico-basal degeneration often misdiagnosed as?

PD or stroke

How is cortico-basal degeneration different from PD?

Doesn't affect the substantia nigra, it affects the cortex Causes people to have a useless limb Doesn't involve memory problems early on

What is the age of onset for corticobasal degeneration?

50 or over

Do MSA, CBD and PSP respond well to L-DOPA?

No, because the down stream receptors/neurons are not there