Degenerative disease of the CNS Flashcards
What causes neurodegenerative disease?
The aetiology of Neurodegenerative diseases such as dementia and parkinsonism is largely unknown
Rarely linked to mendelian genetic cases
Describe the general onset of neurodegenerative diseases as a whole
Onset is usually late - ie typically in older people
Onset and progression of Neurodegenerative diseases is gradual
What is dementia?
A syndrome consisting of progressive impairment of multiple domains of cognitive function
Leads to loss of acquired skills and interference in occupational and social aspects
What are the causes of dementia?
Note - causes are also used as ‘types’ of dementia
Alzheimer’s - most common
Vascular
Lewy body
Frontotemporal
Complete the diagram of causes of dementia in old and young people
Alzheimer’s is by far the most common cause
What ‘other’ causes of dementia are there?
Alcohol abuse
Huntington’s disease
Infections:
- HIV
- CJD
Multiple sclerosis (inflammation aspect)
These are more common causes of dementia in under 65’s (young people)
What are the risk factors for dementia
Vitamin B12 deficiency
HIV, syphilis
Thyroid disease (endocrine problems)
Huntington’s, MS
Alcohol abuse
Old age
What conditions can mimic dementia (differentials)?
Hydrocephalus
Tumours
Depression - ‘pseudodementia’
What is the approach to diagnosing dementia?
History (with independent witness):
- type of deficit, progression, risk factors, FH
Examination:
- cognitive function, neurological, vascular
Investigations:
- Bloods + CT / MRI
others - CSF, EEG, functional imaging, genetics (biopsy)
Describe how you would assess a patient (with potential dementia)’s cognitive function?
Assess the various domains of cognitive function:
- memory, attention, language, behaviour, visuospatial etc
Screening tests:
- Mini-mental test (MMSE)
- Montreal test (MOCA)
Neuropsychological assessment
What type of onset is specifically associated with Vascular dementia?
Stepwise progression - is indicative of Vascular dementia
A rapid progression of dementia symptoms is associated with what cause of dementia?
CJD (Creutzfeldt–Jakob disease)
Fuck ever spelling that
For each cause of dementia below, state any specific neurological signs that may indicate that cause…
A) Huntington’s
B) Lewy body
C) CJD
A) Huntington’s:
- Abnormal movements
B) Lewy body:
- Parkinsonism
C) CJD:
- Rapid onset/progression
- Myoclonus
What are the risk factors for Alzheimer’s disease?
Being old
Some Genetic risks
Lifestyle:
- Smoking
- Exercise
- Diet
- Alcohol
What neurological signs are indicative of Temperoparietal dementia (which btw is a type of Alzheimer’s)
Early memory disturbance
Visuospatial problems:
- falling over
- bruises from bumping into things etc
Personality preserved until later stages so this will likely be normal at time of presentation
What neurological signs are specific to frontotemporal dementia?
Early change in personality/behaviour
Change in eating habits
Early dysphasia - communication problems
However - memory & visuospatial function remains okay at early stages
Describe the symptomatic treatment for dementia
Non-pharma:
- Information & support, dementia services
- Occupational therapy
- Social work / respite / placement (care homes?)
- Voluntary organisations
Pharma:
- Insomnia medication
- Antipsychotics - for extreme behavioural problems
- Anti-depressants
What treatments are available for Alzheimer’s?
Cholinesterase inhibitors (cholinergic deficit)
- Donepezil, rivastigmine, galantamine
- Pretty shit though
NMDA antagonist (memantine)
What is the treatment route for Lewy body dementia?
Same as alzheimer’s
Cholinesterase inhibitors - Donepezil, rivastigmine, galantamine
NMDA antagonists - memantine
How is frontotemporal dementia specifically treated?
No available treatments exist for FT dementia
Treatment is purely symptomatic
What specific treatments exist for vascular dementia?
Nothing really exists
Decreasing vascular risk factors has no robust evidence to support it
What is Parkinsonism?
A clinical syndrome with 2 or more of:
- Bradykinesia - slow movement. Pretty much always seen.
- Rigidity - stiffness
- Tremor
- Postural instability - falls, unsteadiness
What causes parkinsonism?
Pathology in the basal ganglia - predominantly dopamine loss
What are the types of parkinson’s disease?
Idiopathic Parkinson’s disease:
- Dementia with Lewy bodies
Drug-induced:
- Dopamine antagonists
Vascular parkinsonism
Parkinson’s plus syndromes:
- Multiple system atrophy
- Progressive supranuclear palsy / corticobasal degeneration
- Group of conditions which feature classic parkinsonism but with extra distinct features
What is the prognosis for Parkinson’s disease?
60-90% dead or disabled at 10 yrs
Because of a lack of effective specific treatments
Describe the typical presentation for a patient with Parkinson’s disease
Bradykinesia + 1/more of:
- Tremor - affects one side (hand), at rest, ‘pill-rolling tremor’
- Rigidity
- Postural instability
Slow progression (>5-10 years)
± Dementia symptoms/signs, neurological features
Why is a trial of dopamine replacement treatment a good investigation for Parkinsonism?
Good response to treatment would support a diagnosis of Parkinsonism
