DISEASE- Parkinson plus syndromes

Question 1

what neurodegenerative disease is lewy body dementia associated with?

Answer 1

it is a type of parkinson plus syndrome
-dementia associated with features of parkinsonism

Question 2

at what age do people tend to develop lewy body dementia?

Answer 2

> 65 years

Question 3

pathophysiology of Lewy body dementia?

Answer 3

alpha- synuclein aggregates -> cell dysfunction -> cell damage -> disruption of dopimenergic and cholinergic pathways

Question 4

criteria for dementia with lewy bodies diagnosis?

Answer 4

Core criteria:
1. Fluctuating cognition
PLUS
2. Recurrent well formed visual hallucinations
+/-
3. Presence of extra pyramidal features (75%)/ Parkinsonism

Question 5

Presentation of Lewy body dementia?

Answer 5

Core criteria:
1. Fluctuating cognition
PLUS
2. Recurrent well formed visual hallucinations
+/-
3. Presence of extra pyramidal features (75%)/ Parkinsonism

-Dementia typically occurs before the motor
-Hallucinations, REM sleep disorder, fluctuating cognition levels, Parkinsonsim

Question 6

investigations for dementia with Lewy bodies?

Answer 6

positive DAT scan (dopamine transporter imaging)

Question 7

treatment- dementia with lewy bodies?

Answer 7

Trial cholinesterase inhibitors - rivastigmine + donepezil

Question 8

what are the 4 parkinson plus syndromes?

Answer 8

-progressive supranuclear palsy
-multiple system atrophy
-corticobasal degeneration
-lewy body dementia

Question 9

what are parkinson plus syndromes?

Answer 9

-progressive supranuclear palsy
-multiple system atrophy
-corticobasal degeneration
-lewy body dementia

diseases that present as parkinsonism (resting tremor, hypertonia and bradykinesia) with additional clinical features

Question 10

what is multiple system atrophy?

Answer 10

a condition where the neurones of multiple systems in the brain degenerate, including the basal ganglia leading to Parkinson like presentation

Question 11

who is typically affected by multiple system atrophy?

Answer 11

> 30s (but peaks 50-60 years)

Question 12

presentation of multiple system atrophy?

Answer 12

Parkinsons tremor due to degeneration of basal ganglia
-More symmetric and tremor negative Parkinsonism

Autonomic dysfunction: due to generation of neurones in other systems
* Postural hypotension
* Constipation
* Abnormal sweating
* Sexual dysfunction

Cerebellar dysfunction:
-Ataxia

Question 13

what is progressive supranuclear palsy?

Answer 13

-Parkinson plus syndrome where there are problems with the muscles involved in looking upwards leading to vertical gaze palsy

Question 14

presentation of progressive supranusclear palsy?

Answer 14

-vertical gaze palsy
-Parkinsonism that is more symmetric and tremor negative

Question 15

cortico basal degeneration presentation?

Answer 15

Patients sometimes present with apraxia - the inability to conceptulise movement i.e. description of not being able to follow instructions, but being able to do them automatically

Involved spontaneous activity by an affected limb, or akinetic rigidity of that limb

‘alien hand syndrome’