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Annual conversion rate from MCI to dementia?
10-15%
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What radiological feature can be found in TGA?
- Punctate ischaemia of the hippocampus
- 84% of cases
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What types of words are people with svPPA most likely to mispronounce?
- Exception words - Pint, soot, dough, etc.
- They have surface dyslexia and can’t read into the way to say the word because they have lost the word or the meaning of the word.
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Non-Dom lesions cause?
- Anosognosia,
- Dressing apraxia,
- Topographical agnosia,
- Constructional apraxia and
- Hemispatial sensory neglect.
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Lesions of the dominant hemisphere cause?
- Anomia,
- Finger agnosia,
- Acalculia,
- Agraphia,
- Alexia,
- Aphasia (primarily conduction aphasia and/or transcortical Sensory aphasia),
- Right-left disorientation
- Conduction apraxia.
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What is Cerebellar Cognitive affective syndrome?
- The cerebellum does more than just motor co-ordination
- Kids with cerebellar hypoplasia develop issues with the following domains:
- a) executive dysfunction including deficient planning, set-shifting, abstract reasoning and working memory
- b) impaired spatial cognition
- c) personality change including blunting or flattening of affect and disinhibition
- d) linguistic difficulties
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If you’re starting some one on an antipsychotic for their behaviour/risk in dementias - how often should you review them?
Every 6 weeks
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Which features make DLB most likely in the early stages of a dementing process?
- Visuospatial dysfunction
- Visual hallucinations
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When trying to manage behaviour in Alzheimer’s what meds are best?
- Memantine, SSRIs and SNRIs
- Antipsychotics carry a higher morbidity and mortality
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In what do you find the Posterior cingulate island sign on FDG PET?
- DLB
- Helps differentiate it from AD and FTLD
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How does FDG PET look in the dementias?
- AD - hypometabolism in the precuneus and bilateral temporal lobes
- DLB - hypometabolism in the primary visual cortex with posterior cingulate island
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Why is metachromatic leukodystrophy so named?
- Because the sulfatide granules that accumulate appear different colours down the microscope
- BUT!!
- Much more fun to think that its because of the MRI appearances of tigroid pattern white matter change.
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What is the molecular problem in Metachromatic Leukodystrophy?
Arylsulfatase A deficiency in leukocytes.
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What does Metachromatic leukodystrophy present with?
- Dementia, behavioural changes or, less frequently, psychosis.
- A demyelinating sensorimotor neuropathy is the give away.
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If you’re not an arc-welder - how else are you going to get too much manganese into your system?
Liver failure and portosystemic shunts will deposit it in your globus pallidus.
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Why is metronidazole bad from a neurological perspective?
- Metronidazole encephalopathy is a thing.
- Involves the cerebellar dentate nuclei, midbrain,
-
Niemann-Pick type C what visceral changes?
Splenomegaly in 90%
-
Neurological features of Niemann-Pick type C?
- Deafness
- Psychiatric symptoms
- Cognitive dysfunction
- Cerebellar ataxia
- Vertical supranuclear ophthalmoplegia,
- dysphagia,
- dystonia,
- parkinsonism.
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Most common cause of Familial AD?
PS1 - accounts for 80%
-
Where would you have to infarct your thalamus to cause confusion?
Anterior thalamus
-
Alpha-synulein intra or extra?
Intracellular. Amyloid is the only extracellular bastard.
-
Whats the best test for Narcolepsy-cataplexy syndrome?
MSLT
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Treatment for pseudobulbar affect?
Dextromethorphan-quinidine
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Ideomotor apraxia - clinical finding and localisation?
- Using body parts for actions instead of miming the use of the object.
- Localises to the dominant parietal lobe
-
Ideational apraxia - clinical finding and localisation?
- Difficulty in organising the sequence of movements - 'pour water out of a jug and drink from the cup'.
- Localises to bifrontal regions
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Conduction apraxia - clinical finding?
Inability to imitate movements
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Social disinhibition, inappropriate jocularity (witzelsucht) and utilisation behaviour localise to?
Orbitofrontal region
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Apathy, amotivation, abulia, reduction or abolition of volitional movement localise to?
DorsoMedial Prefrontal Cortex
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Anhedonia, Poor judgement, Inability to plan, multitask and problem solve localise to?
DorsoLateral Prefrontal Cortex
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Gaze deviation localises to 2 structures...?
- Frontal eye fields - govern contralateral eye movement so a lesion on the right will cause unopposed activity of the left leading to right eye deviation.
-
- Parapontine reticular formation - governs ipsilateral conjugate eye movements so a lesion in the right PPRF will lead to left hemiparesis and left eye deviation due to unopposed action of the left PPRF.
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What are the 3 repeat tau disorders?
Pick disease
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What are the 4 repeat tau disorders?
- PSP
- CBD
- Agyrophylic grain disease
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Where do you measure for evans index?
Widest part of the frontal horns and widest internal diameter of the skull on the same section.
-
What are the best predictors of poor outcome following cardiac arrest?
- Within 48hrs - status myoclonus
- After 72 hours - Absent pupillary and corneal reflexes
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