dementia (excluding alzheimers) Flashcards
-frontotemporal -vascular -demetia with lewey bodies -parkinsons -huntingtons
what is frontotemporal dementia
specific degeneration/atrophy of the frontal and temporal lobes of the brain
cause of frontotemporal
-genetic
-may be part of MND-FTD spectrum
presentation of frontotemporal dementia
-disinhibition
-apathy
-loss of empathy
-stereotypes or compulsive behaviours
-hyperorality
-early loss of insight
-difficulty in planning, executive function and impulse control
what is the behavioural variant
-behavioural changes
-executive dyfunction
-disinhibition
-impulsivity
-loss of social skills
-apathy
-obsessions
-change in diet
what is primary progressive aphasia
-effortless non-fluent speech
-speech errors
-lack of grammar
-lack of words
what is semantic dementia
-impaired understanding of meaning of words
-fluent but empty speech
-difficulty retrieving names
what is seen on MRI of frontotemporal dementia
atrophy of frontotemporal lobes
what is seen on SPECT in frontotemporal dementia
reduced metabolism in frontotemporal
what is seen in the CSF in frontotemporal
increased tau and normal amyloid
what is the most useful for frontotemporal
SPECT
management of frontotemporal
-trial of trazadone/antipsychotics to help behavioural features
-safety management
-power of attorney
onset of vascular dementia
late but abrupt
what causes vascular dementia
Brain damage due to cerebrovascular disease: either major stroke, multiple smaller unrecognised strokes (multi-infarct) or chronic changes in smaller vessels (subcortical dementia)
-causes areas of cell death
who gets vascular dementia
men over 60 with hypertension
presentation of vascular dementia
• Dysphasia, dyscalculia, frontal lobe symptoms and affective symptoms more common than in Alzheimers
-decreased attention, executive dysfunction, slowed processing
• May have focal neurological signs
• May have vascular risk factors
• May have step wise decline
• Cognitive functions not fully regained after each step
-Impairments seen in the areas most affected by vascular injury
investigation for vascular dementia
evidence of stroke or small vessel disease may be seen on CT or MRI
treatment of vascular dementia
-vascular risk factors
-secondary prevention to protect against further vascular injury
-only consider cholinesterase inhibitors if they have suspected comorbid Alzheimer’s, parkinson’s or lewy bodies
what is dementia with lewey bodies
Deposition of abnormal protein (⍺-synuclein) within neurons in the brain stem and neocortex
presentation of dementia with lewey bodies
dementia
two of:
○ Visual hallucinations
○ Fluctuating cognition (delirium-like)
○ REM sleep behaviour disorder
○ Extrapyramidal features (Parkinsonism)
- Not more than 1 year prior to onset of dementia
-Positive DAT scan
investigation for dementia with lewey bodies
DaT scan (dopamine active transporter imaging)
-CT
-MRI
treatment for dementia with lewey bodies
-small dose levodopa for movement problems
-trial cholinesterase inhibitors - donepezil or rivastigmine
-if they don’t work then galantamine which is an acetylcholinesterase inhibitor
what needs to be done before prescribing/increasing dose of cholinesterase inhibitors
check pulse
what is parkinson’s dementia
Late onset dementia
-progressive reduction of dopamine in the basal ganglia of the brain, leading to disorders of movement
presentation of parkinson’s
-bradykinesia (movements get smaller and slower, handwriting gets smaller)
-rigidity (cogwheel, movement with small jerks)
-resting tremor
-symptoms are classically asymmetrical
-dementia
-decrease attention
-slow processing
-loss of memory
-forward tilt
-reduced arm swing
-facial masking
-stooped posture
-shuffling gait
what is the parkinson’s tremor like
-asymmetrical
-4-6 Hz
-worse at rest
-improves with intentional movement
-no change with alcohol
treatment options for parkinson’s
-levodopa (usually combined)
-COMT inhibitors (entacapone)
-dopamine agonists
-monoamine oxidase-B inhibitors
pathophysiology of parkinsons
Part of the basal ganglia called the substantia nigra produces a neurotransmitter called dopamine. Dopamine is essential for the correct functioning of the basal ganglia. In Parkinson’s disease, there is a gradual but progressive fall in the production of dopamine
what are some parkinson’s plus syndromes
-multiple system atrophy
-dementia with lewey bodies
-progressive supranuclear palsy
-corticobasal degeneration
what is huntingon’s disease
early onset dementia
-progressive deterioration in the nervous system
what is the huntington’s gene
HTT gene on chromosome 4
-CAG trinucleotide repeat
autosomal dominant
pathophysiology of huntington’s
-cells are lost from the basal ganglia causing flattening of the normal convex curve of the lateral ventricles
-cells are lost from other brain areas including the cerebral cortex
what is genetic anticipation of huntington’s
this is where successive genertations have more repeats in the gene resulting in:
-earlier age on onset
-increased severity of disease
presentation of huntingtons
-dementia
-dysexecutive syndrome (frontal lobe damage leading to emotional, motivational and behavioural symptoms)
-involuntary movements
-slow processing
-changes in mood and personality
-anxiety, psychosis, compulsions, suicidality, aggression
-usually presents with the cognitive, psychiatric and mood problems first then movement disorders
what are the late signs of huntington’s
-rigidity
-bradykinesia
-severe chorea (involuntary abnormal movements)
-serious weight loss
-inability to walk
-inability to speak
-swallowing problems
investigations for huntington’s
-genetic tests
-CT scan
treatment for huntingtons
there isnt really any
-support
life expectancy of Huntington’s
15-20 yrs
