Degenerative Diseases of the CNS Flashcards
What are common features amongst neurodegenerative diseases?
Gradual neuronal loss
Structural imaging is often normal
What does dementia consist of?
Disease progression results in loss of cognitive function leading to a loss of acquired skills and interference in occupational and social role.
What is the diagnosis of dementia?
Based on history and examination - has to be progressive (gets worse over time)
MULTIPLE areas of cognitive loss
What is the commonest reason to go into a nursing home
Dementia
What are the causes of dementia (late onset)?
Alzheimer’s (55%)
Vascular (20%)
Lewy body (20%)
Others (5%)
What are the causes of dementia (early onset)?
Alzheimer’s (33%)
Vascular (15%)
Frontotemporal (15%)
Other (33%)
- Toxic (alcohol)
- Genetic (Huntington’s)
- Infection (HIV, CJD)
- Inflammatory (MS)
What are the treatable causes and mimics of dementia?
Vitamin deficiency - B12
Endocrine - thyroid disease
Infective - HIV, syphilis
Mimics: Hydrocephalus
Tumour
Depression: “pseudodementia”
What is the examination and investigation for dementia?
Examination: Cognitive function, neurological, vascular
Investigations: Routine - bloods, CT / MRI
Others: CSF, EEG, functional imaging, genetics (biopsy)
What are the various domains of cognitive function?
Memory
Attention
Language
Visuospatial
Behaviour
Emotion
Executive function
Apraxias (can’t perform actions)
Agnosias (inability to interpret sensations and hence recognise things)
What are the screening tests for dementia?
Mini-mental (MMSE)
Montreal (MOCA)
What speed of progression does CJD and Vascular dementia have?
CJD - rapid progression - patients usually die within a year
Vascular - stepwise progression
What would abnormal movements (chorea) and myoclonic movements be suggestive of?
Other neurological signs
–abnormal movements (Huntington’s)
–parkinsonism (Lewy body)
–myoclonus (CJD)
What is the commonest neurodegenerative condition?
Alzheimers (temperoparietal dementia)
What are the different types of dementia?
Tempero-parietal
Frontotemporal
Vascular
What are the features of tempero-parietal dementia?
Early memory disturbance
Language and visuospatial problems
Personality is preserved until later
What are the features of frontotemporal dementia?
Early change in personality / behaviour
Often change in eating habits
Early dysphasia
Memory / visuospatial relatively preserved
What are the features of vascular dementia?
Mixed picture
Stepwise decline
What is the non pharmacological symptomatic treatment for dementia?
–Information & support, dementia services
–Occupational therapy
–Social work / support / respite / placement
–Voluntary organisations
What is the pharmacological intervention for symptomatic treatment of dementia?
Treatment for insomnia
Treatment for behaviour (care with antipsychotics)
Depression
What is the specific treatment for alzheimers?
Cholinesterase inhibitors (donepezil, rivastigmine)
Acetylcholine is lost in dementia, the effect of a cholinesterase inhibitor is to increase the amount of acetylcholine - although this doesn’t stop the degeneration of nerves, doesn’t delay progressive disability or entry into a care home
NMDA antagonist (memantine)
What is the treatment for frontotemporal dementia?
None
What is the specific treatment for vascular dementia?
No good evidence for reduced vascular risk factors
What is the clinical syndrome of parkinsonism?
2 or more of:
Bradykinesia (slowness of movement)
Rigidity (stiffness)
Tremor (shakiness)
Postural instability (unsteady, falls)
Where is the pathology of parkinsonism?
Basal ganglia
(REMEMBER - dementia - frontal, temporal and parietal lobes)
As a result of dopamine loss
What are the causes of parkinsons?
Idiopathic parkinsons
- Dementia with lewy bodies
Drug induced (dopamine antagonists)
Vascular parkinsonism
Parkinson’s plus syndromes (multiple system atrophy, progressive supranuclear palsy / corticobasal degeneration)
What is the diagnosis of parkinsons?
Must have bradykinesia (with on or more of tremor, rigidity and postural instability)
Slowly progressive
No other cause / atypical features
Asymetric rest tremor
Good response to dopamine repacement treatment
When is it less likely ot be parkinsons?
Rapid progression
Symmetrical
Lack of rest tremor
Poor response to treatment
Early falls
Early dementia
Other abnormal neurological signs
When does movement decrement in parkinsons?
Decrements after lots of repetitive movement on the affected side
What is the functional imaging scan for parkinsons?
DAT scan - dopamine transporter scan
What areas are seen to be reduced on a DAT scan?
Putamen
Caudate
What is the early medical treatment of PD?
Levadopa
COMT inhibitor (entacapone)
MAO - B inhibitor
- selegiline
- rasagiline
- safinamide
Dopamine Agonists
- ropinirole
- pramipexole
- rotigotine
What are drug induced complications associated with parkinsons?
Motor fluctuations - levadopa wears off
Dyskinesias - involuntary movements (levadopa)
Psychiatric - hallucinations, impulse control
What are the later complications associated with parkinsons (non-drug induced)?
Depression - more likely in patients with parkinsons
Dementia
Autonomic: BP, Bladder, Bowel
Speech
Swallow
Balance
What is late treatment for parkinsons?
DRUGS:
Aim is to increase levadopa half life:
MAO-B inhibitors
COMT inhibitors
Slow release levadopa
Or
Add oral dopamine agonist
Continuous infusion (apomorphine, duodopa - this is via peg)
FUNCTIONAL NEUROSURGERY: Deep brain stimulation
Allied health professionals
