Degenerative disease of the CNS Flashcards
What is common features of neruodegerative diseases
aetiology largely unknown
(mendelian genetic cases rare, often younger onset)
usually late onset
gradual progression
neuronal loss (specific neuropathology)
structural imaging often normal (atrophy)
What is dementia
A syndrome consisting of progressive impairment of multiple domains of cognitive function in alert patient leading to loss of acquired skills and interference in occupational and social role
What is three causes of late onset dementia (>65years)
Alzheimers
Vascular
Lewy body
What is the potentially causes of younger onset (<65 years) dementia
Alzheimer’s
Vascular
Frontotemporal
Toxic (alcohol)
Genetic (Huntington’s)
Infection (HIV, CJD)
Inflammatory (MS)
What are treatable causes of dementia
Vitamin deficiency - B12
Endocrine - thyroid disease
Infective - HIV, syphilis
What are treatable disorders that mimic dementia
Hydrocephalus
Tumour
Depression: pseudo-dementia
What information do you gather from the patient when diagnosing dementia
type of deficit
progression
risk factors
family history
What is examined when diagnosing a patient with dementia
Cogntive function
neurological examination
Vascular examination
What is the routine investigation for dementia
Bloods
CT
MRI
What are further investigation for dementia
CSF EEG Functional imaging Genetic Biopsy
What is examined in cognitive function
Memory,
Attention,
Language,
Visuospatial,
Behaviour,
Emotion,
Executive function
Apraxias - difficulty with motor planning
Agnosia - inability to interpretate sensations and recognise
What are the screening tests used in examining cognitive functions
Mini- mental (MMSE)
Montreal (MOCA)
What assessment take place when examining cognitive function
Neuropsychological assessment
Rapid progression dementia is usually a sign of what type of dementia
CJD dementia
A stepwise progression is usually as signs to what kind dementia
Vascular dementia
Demential with abnormal movement is usually a sign to what kind of dementia
Huntingtons dementia
Demential with parkinsonism is usually a sign to what kind of dementia
Lewy body dementia
Demential with myoclonus is usually a sign to what kind of dementia
CJD dementia
What is the commonest neurodegenerative condition
alzheimers disease
mean age onset >70years
What is the pathology of alzheimers disease
proteins build up in the brain to form structures called ‘plaques’ and ‘tangles’ leading to the loss of connections between nerve cells, and eventually to the death of nerve cells and loss of brain tissue
What is the affect of alzheimers
Tempo -parietal dementia
What temp-parietal dementia associated with
- early memory disturbance
- language and visuospatial problems
- personality preserved until later
What is frontotemporal dementia associated with
Early change in personality / behaviour
Often change in eating habits
Early dysphasia
Memory / visuospatial relatively preserved
What is vascular dementia associated with
A mixed picture of symptoms with a stepwise decline
What is the non pharmacological symptomatic treatment of dementia
Information & support, dementia services
Occupational therapy
Social work / support / respite / placement
Voluntary organisations
What symptoms can be pharmacological treatment for dementia
Insomnia
Behaviour (care with antipsychotics)
Depression
What is the specific treatment for Alzheimer’s (+/- Lewy body dementia)
Cholinesterase inhibitors
NMDA antagonist
how does Cholinesterase inhibitors work in treating Alzheimers
inhibits the acetylcholinesterase enzyme from breaking down acetylcholine, thereby increasing both the level and duration of action of the neurotransmitter acetylcholine
leading to small symptomatic improvement in cognition
What is three examples of Cholinesterase inhibitors
Donepezil, rivastigmine, galantamine
How does NMSA antagonist work in the treatment of alzheimers
regulating the activity of glutamate, an important neurotransmitter in the brain involved in learning and memory
What is an example of NMSA antagonist
memantine
What is the specific treatment for frontotemporal dementia
Nothing
Parkinsonism is a clinical syndrome with 2 or more of what four symptoms
Bradykinesia (slowness of movement)
Rigidity (stiffness)
Tremor (shakiness)
Postural instability (unsteadiness / falls)
what is the pathology of Parkinsonism
Occurs in the basal ganglia due to loss of pigmented dopaminergic neurons of the substantia nigra
What can also be present in parkinsonism
Lewy bodies
What is the causes of parkinsonism
Idiopathic Parkinson’s disease
-Dementia with Lewy bodies
Drug-induced
(e.g. dopamine antagonists)
Vascular parkinsonism (lower-half)
Parkinson’s plus syndromes
What parkinson plus syndromes
Group of neurodegenerative diseases featuring the classical features of Parkinson’s disease
Give examples of parkinson plus syndromes
Multiple system atrophy
Progressive supranuclear palsy
corticobasal degeneration
What is the cause of vascular parkinsonsim
“atypical parkinsonism” in which parkinsonian symptoms are produced by one or more small strokes
What is the clinical presentation that leads to the diagnosis is parkinson
Bradykinesia + ≥1 tremor, rigidity, postural instability
No other cause / atypical features
Slowly progressive (> 5-10 yrs)
What supports your diagnosis of parkinson disease
good response to dopamine replacement treatment
What signs make it less likely to be parkinson disease
rapid progression,
symmetrical,
lack of rest tremor,
poor response to treatment,
early falls,
early dementia,
other abnormal neurological signs
What specific symptoms indicates the diagnosis to be parkinson s
asymmetric rest tremor
What imaging technique is used for the diagnosis of parkinson
Domapine transporter SPECT
What does early medication of parkinsons disease involve
Increasing dopaminergeric neurone
What is examples of how dopaminergeric neurone is increased
and used in the treatment of Parkinson’s
Levodopa
COMT inhibitor
MAO-B inhibitor
Dopamine agonist
What is three examples of MAO-B inhibitors
selegiline
rasagiline
safinamide
What is three examples of dopamine agonists
ropinirole
pramipexole
rotigotine
What are drug induced later complications of parkinson s
Motor fluctuations - levodopa wears off
Dyskinesias - involuntary movements (due to levodopa)
Psychiatric - hallucinations, impulse control
What is non motor later complications of Parkinson’s
Depression
Dementia
Autonomic: BP, bladder, bowel
Speech, swallow
Balance
What is the is the late treatment for parkinsons
prolong levodopa half life
add oral dopamine agonist
Functional neurosurgery - deep brain stimulation
allied health professional +/- care package
