Degenerative Diseases of the CNS Flashcards
What are common features of neurodegenerative 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
Why is dementia important?
Common and increasing:
- incidence 200 per 100,000
- prevalence 1,500 per 100,000
- >850,000 patients in UK ( 1.6M by 2040)
Devastating impact on patient + family
Costly: ~£35 billion/yr (15% NHS, 40% social, 45% unpaid)
Projected £94 billion by 2040
66% of those in care homes
What are the causes on late onset dementia? (more common)
Late onset (65+ yrs):
Alzheimer’s (55%)
Vascular (20%)
Lewy body (20%)
Others (5%)
What are the causes on younge onset dementia? (rarer)
Young onset (<65 yrs):
Alzheimer’s (33%)
Vascular (15%)
Frontotemporal (15%)
Other (33%):
Toxic (alcohol)
Genetic (Huntington’s)
Infection (HIV, CJD)
Inflammatory (MS)
What are the treatable causes of dementia?
Vitamin deficiency - B12
Endocrine - thyroid disease
Infective - HIV, syphilis
What are mimics of dementia?
Hydrocephalus
Tumour
Depression: “pseudodementia”
How do you diagnose dementia?
(clinical diagnosis)
History (independent witness) - type of deficit, progression, risk factors, FH
Examination: cognitive function, neurological, vascular
Investigations:
routine - bloods, CT / MRI
others - CSF, EEG, functional imaging, genetics (biopsy)
WHat is the most important part of diagnosing dementia?
history!
How is the examination of cognitive function carried out?
Various domains: Memory, attention, language, visuospatial,
Behaviour, emotion, executive function, Apraxias, agnosias
Screening tests - Mini-mental (MMSE), Montreal (MOCA) - more common one
Neuropsychological assessment
What are some clues for diagnosis?
Type of cognitive deficit
Speed of progression
- rapid progression (CJD)
- stepwise progression (vascular)
Other neurological signs:
- abnormal movements (Huntington’s)
- parkinsonism (Lewy body)
- myoclonus (CJD)
If the dementia is a step wise progression what is the more likley cause?
Vascular
What is the mommonest neurodegenerative condition?
Alzheimer’s disease
What is the mean age onset of alzheimers disease?
mean age onset 70 yr (25% <65yrs)
What are the risk factors for alzheimer’s disease?
genetic
lifestyle (smoking, exercise, diet, alcohol)
What type of dementia can alzheimer’s disease cause and what is it?
Temporo-parietal dementia
– Early memory disturbance
– Language and visuospatial problems
– Personality preserved until later
What does Frontotemporal dementia cause?
Early change in personality/behaviour
Often change in eating habits
Early dysphasia
Memory/visuospatial relatively preserved
What does vascular dementia cause?
Mixed picture
Stepwise decline
What are the 2 types of symptomatic treatment for dementia?
Non pharmacological
Pharmacological
What are the non pharmacological treatments for symptomatic dementia?
Information & support, dementia services
Occupational therapy
Social work/support/respite/placement
Voluntary organisations
What is the pharmacological treatment of symptomatic dementia?
Insomnia
Behaviour (care with antipsychotics)
Depression
What is specific treatment for alzheimers disease (+/- Lewy body dementia)?
Cholinesterase inhibitors (cholinergic deficit)
• Donepezil, rivastigmine, galantamine
• Small symptomatic improvement in cognition (wash-out)
• No delay in institutionalisation
NMDA antagonist (memantine)
What is the specific treatment for frontotemporal dementia?
none
What is the specific treatment for vascular dementia?
No robust evidence for ↓ vascular risk factors
Does any treatment for dementia chance the progression of the disease?
no
What is Parkinsonism?
A clinical syndrome with 2 or more of the following:
- Bradykinesia (slowness of movement)
- Rigidity (stiffness)
- Tremor (shakiness)
- Postural instability (unsteadiness/falls)
Parkinsonism is a pathology where?
Pathology in basal ganglia - predominantly dopamine loss
What are 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
- Multiple system atrophy
- Progressive supranuclear palsy/corticobasal degen
What is the epidemiology of PD?
2nd commonest neurodegenerative disease
Incidence: 15-20 per 100,000 per year - age dependent, 1.5 M : F
Crude prevalence: 150-300 per 100,000: ~150,000 in UK
Cost in UK: ~£2 billion/year (most indirect)
60-90% dead or disabled at 10 yrs
What is the 2nd commonest neurodegenerative disease?
Parkinsons disease
What is the Incidence of PD by age?
How do you diagnose parkinsons disease?
Bradykinesia + ≥1 tremor, rigidity, postural instability
No other cause/atypical features
Slowly progressive (> 5-10 yrs)
Supported by asymmetric rest tremor, good response to dopamine replacement treatment
Less likely if rapid progression, symmetrical, lack of rest tremor, poor response to treatment, early falls, early dementia, other abnormal neurological signs
The dignosis of parkinsons is based on what kind of features?
clinical features
What is the early medical treatment of PD?
dopamine effecting the neurone synapses
What are some later drug induced complications of parkinsons disease?
Motor fluctuations - levodopa wears off
Dyskinesias - involuntary movements (levodopa)
Psychiatric - hallucinations, impulse control
What are some later non-drug induced complications of parkinsons disease?
Depression (20%)
Dementia (~50% after 10 yrs)
Autonomic: BP, bladder, bowel
Speech, swallow
Balance
What is the late treatment of parkinsons disease?
Drugs:
- Prolong levodopa half life
- Add oral dopamine agonist
- Continuous infusion (apomorphine, Duodopa)
NB PD medications are time critical (hospital admissions)
Functional neurosurgery (deep brain stimulation)
Allied health professionals +/- care package
Neurodegenerative conditions are common and ________
Main chronic health burden with ______ society and improvements in treating vascular disease & cancer
Need effective ________/_______ strategies
Neurodegenerative conditions are common and increasing
Main chronic health burden with ageing society and improvements in treating vascular disease & cancer
Need effective neuroprotective/restorative strategies
