Neurodegenerative Diseases Flashcards
What are the common features of neurodegenerative diseases?
Aetiology largely unknown
(mendelian genetic causes rare)
Usually late onset
Gradual progression
Neuronal loss (specific neuropathology)
Structural imaging often normal (atrophy)
Dementia is 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 the epidaemiology of dementia?
Incidence = 200 per 100,000
Prevalence 1,500 per 100,000
>750,000 patients in UK
What is the cost of dementia?
Devastating impact on patient and family
£20 billion per year (£6 billion by carers)
-66% of those in care homes have dementia
What are the causes of late onset (65+ years) dementia?
Alzheimer’s (55%)
Vascular (20%)
Lewy body (20%)
Others (5%)
What are the causes of young onset (
Alzeimer's (33%) Vascular (15%) Frontotemporal (15%) Other (33%) -Huntington's, CJD -Infection (HIV) -Inflammatory (MS) -Toxic (Alcohol)
What are the treatable causes of dementia like conditions?
Vitamin deficiency (B12) Endocrine (Thyroid disease) Infective (HIV, Syphilis)
What are mimics of dementia?
Hydrocephalus
Tumour
Depression “pseudodementia”
How do you diagnose dementia?
History (independent witness)
- Type of deficit
- Progression
- Risk factors
- Family history
Examination:
- Cognitive function
- Neurological
- Vascular
Investigations:
- Routine (bloods, CT/ MRI)
- Others (CSF, EEG, Functional imaging, genetics)
-Investigations to exclude other causes
What are the various domains for examining someones cognitive function?
Memory Attention Language Visuospatial Behavior Emotion Executive function Apraxias Agnosias
What screening tests can you use for examination of cognitive function?
Screening tests:
- Mini mental (MMSE)
- Montreal (MOCA)
Mini mental misses alot so montreal may be better
NEUROPSYCHOLOGICAL ASSESSMENT
What is apraxia?
Cannot plan complex motor tasks.
Wants to put a t-shirt on but cannot plan out all the sequences of movement to do so
What is agnosia?
Inability to interparate sensations and therefore recognise things.
Can be handed a coin with their eyes closed. Will be able to feel it and describe it in detail but not actually recognise its a coin
What are the clues to diagnosis in neurodegenerative diseases?
Type of cognitive deficit
Speed of progression
- Rapid (CJD, tumour, infection)
- Stepwise progression (vascular)
Other neurological signs:
- Abnormal movements (Huntington’s)
- Parkinsonism (Lewy body)
- Myoclonus (CJD)
What are the signs of temporo-parietal dementia?
Early memory disturbance
Language and visuospatial problems
Personality preserved until later
What are the signs of frontotemporal dementia?
Early change in personality/ behaviour
Often change in eating habits
Early dysphagia
Memory/ visuospatial relatively preserved
What is the non-pharmacological symptomatic treatment for dementia?
Information and support, dementia services
Occupational therapy
Social work/ support/ respite/ placement
Voluntary organisations
What is the pharmacological treatment of dementia?
Insomnia
Behaviour (care with antipsychotics, consider behavioural therapy)
Depression
What is the specific treatment for Alzheimers?
(+/- Lewy body dementia)
Colinesterase inhibitors (cholinergic deficit)
- Donepezil, rivastigmine, galantamine
- Small symptomatic improvement in cognition (wash out)
- No delay in institutionalisation
NMDA antagonist (memantine) -Basically same as above
What is parkinsonism?
A clinical syndrome with 2 or more of:
- Bradykinesia (slowness of movement)
- Rigidity (stiffness)
- Tremor (shakiness)
- Postural instability (unsteadiness/ falls)
Pathology in basal ganglia:
-Predominantly dopamine loss
How do you diagnose Parkinson’s disease?
- Bradykinesia + >/= 1 tremor, rigidity, postural instability
- No other cause / atypical features
- Slowly progressive (>5-10 years)
Supported by asymmetrical onset, rest tumor, response to dopamine replacement treatment
Less likely if repid progression, symmetrical, lack of rest tremor, poor response to treatment, early falls, early dementia, other abnormal neurological signs
What are the drug induced complications in parkinsons?
Motor fluctuations: drugs wear off
Dyskinesias: involuntary movements
Psychiatric: hallucinations, impulse control
What are the later complications of parkinson’s that are non-drug induced?
Depression (20%) Dementia (about 50% after 10 years) Autonomic: BP, Bladder, bowel Sensory and sleep Poor swallowing Falls
What can be done in the late treatment of parkinson’s disease?
Prolong levodopa half life
- MAO-B inhibitors
- COMT inhibitor
- Slow release levodopa
Add oral dopamine agonist Continuous infusion (apomorphone, duodopa)
Functional neurosurgery = deep brain stimulation
Allied health professionals +/- care package
