dementia (excluding alzheimers) Flashcards

-frontotemporal -vascular -demetia with lewey bodies -parkinsons -huntingtons (37 cards)

1
Q

what is frontotemporal dementia

A

specific degeneration/atrophy of the frontal and temporal lobes of the brain

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2
Q

cause of frontotemporal

A

-genetic
-may be part of MND-FTD spectrum

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3
Q

presentation of frontotemporal dementia

A

-disinhibition
-apathy
-loss of empathy
-stereotypes or compulsive behaviours
-hyperorality
-early loss of insight
-difficulty in planning, executive function and impulse control

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4
Q

what is the behavioural variant

A

-behavioural changes
-executive dyfunction
-disinhibition
-impulsivity
-loss of social skills
-apathy
-obsessions
-change in diet

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5
Q

what is primary progressive aphasia

A

-effortless non-fluent speech
-speech errors
-lack of grammar
-lack of words

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6
Q

what is semantic dementia

A

-impaired understanding of meaning of words
-fluent but empty speech
-difficulty retrieving names

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7
Q

what is seen on MRI of frontotemporal dementia

A

atrophy of frontotemporal lobes

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8
Q

what is seen on SPECT in frontotemporal dementia

A

reduced metabolism in frontotemporal

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9
Q

what is seen in the CSF in frontotemporal

A

increased tau and normal amyloid

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10
Q

what is the most useful for frontotemporal

A

SPECT

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11
Q

management of frontotemporal

A

-trial of trazadone/antipsychotics to help behavioural features
-safety management
-power of attorney

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12
Q

onset of vascular dementia

A

late but abrupt

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13
Q

what causes vascular dementia

A

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

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14
Q

who gets vascular dementia

A

men over 60 with hypertension

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15
Q

presentation of vascular dementia

A

• 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

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16
Q

investigation for vascular dementia

A

evidence of stroke or small vessel disease may be seen on CT or MRI

17
Q

treatment of vascular dementia

A

-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

18
Q

what is dementia with lewey bodies

A

Deposition of abnormal protein (⍺-synuclein) within neurons in the brain stem and neocortex

19
Q

presentation of dementia with lewey bodies

A

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

20
Q

investigation for dementia with lewey bodies

A

DaT scan (dopamine active transporter imaging)
-CT
-MRI

21
Q

treatment for dementia with lewey bodies

A

-small dose levodopa for movement problems
-trial cholinesterase inhibitors - donepezil or rivastigmine
-if they don’t work then galantamine which is an acetylcholinesterase inhibitor

22
Q

what needs to be done before prescribing/increasing dose of cholinesterase inhibitors

23
Q

what is parkinson’s dementia

A

Late onset dementia
-progressive reduction of dopamine in the basal ganglia of the brain, leading to disorders of movement

24
Q

presentation of parkinson’s

A

-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

25
what is the parkinson's tremor like
-asymmetrical -4-6 Hz -worse at rest -improves with intentional movement -no change with alcohol
26
treatment options for parkinson's
-levodopa (usually combined) -COMT inhibitors (entacapone) -dopamine agonists -monoamine oxidase-B inhibitors
27
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
28
what are some parkinson's plus syndromes
-multiple system atrophy -dementia with lewey bodies -progressive supranuclear palsy -corticobasal degeneration
29
what is huntingon's disease
early onset dementia -progressive deterioration in the nervous system
30
what is the huntington's gene
HTT gene on chromosome 4 -CAG trinucleotide repeat autosomal dominant
31
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
32
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
33
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
34
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
35
investigations for huntington's
-genetic tests -CT scan
36
treatment for huntingtons
there isnt really any -support
37
life expectancy of Huntington's
15-20 yrs