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56 Cards in this Set
- Front
- Back
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Which CNS regions are responsible for procedural memory (skills)?
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Basal ganglia and cerebellum
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A lesion in which CNS region may cause neglect?
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Parietal association cortex (mainly non-dominant)
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Which region of the CNS is responsible for visual attention (i.e. "eye-catchers"?
Where do its efferents project? |
Superior colliculus -
efferents to pulvinar |
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Which regions of the brain are responsible for processing of declaritive memory?
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hippocampus and entorhinal cortex (medial temporal lobe)
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Which regions of the brain are responsible for long-term memory storage?
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No specific area. It is thought that LTM is spread over modality-specific cortex
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What is 'working memory'?
Which regions of the CNS are associated with 'working memory'? |
The memory required for reasoning, comprehension and learning.
Prefrontal cortex and diencephalon |
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A lesion to which region of the CNS is most likely to cause anterograde amnesia of declaritive memory?
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Medial temporal lobe
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Name two factors that may inhibit the conversion of sensory to short-term memory
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Inattention
Competition |
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Name two factors that may inhibit the conversion of short-term to long-term memory
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Alcohol
Loss of consciousness GABA agonist Inattention Low salience |
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What is the most common cause of motor neuron disease?
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Amyotrophic lateral sclerosis (~80%)
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In amyotrophic lateral sclerosis, are upper or lower motor neurons effected?
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Atrophy of both occurs
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Which aggregates are you most likely to see in motor neurons in amyotrophic lateral sclerosis:
TDP-43 Beta-pleated sheets Amyloid plaques A-Beta-42 |
TDP-43
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Name two possible causes of amyotrophic lateral sclerosis
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Enterovirus (e.g. polio, echo, coxsackie)
Heavy metals Mutation (gain of function SuperOxide Dismutase-1) |
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What therapeutic approaches are available for amyotrophic lateral sclerosis?
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Riluzole (reduced glutamate release)
Beta-lactam (increased glutamate uptake) Caspase or COX2 inhib (anti-apoptotic) Free radical agents Gene therapy |
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What is the prion/abnormal protein responsible for Creutzfeldt Jakob disease?
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PrPsc
It is the abnormal version (increased beta-pleated sheets) of the PrPs protein (which is predominantly alpha -sheets) |
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T/F Sporadic CJD is the most common form of Creutzfeldt Jakob disease and predominantly is found in the elderly
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True
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Which form of CJD would you suspect for the following case:
young person living in the UK, rapid loss of general condition, progressive ataxia, behavioural changes and a codon 129 polymorphism. |
Variant CJD
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T/F Creutzfeldt Jakob disease can be inherited
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True
50% of genetic CJD patients have a family history |
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Cadaveric growth hormone use from 1960-1985 was associated with which disease?
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Iatrogenic CJD
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What signs would you expect to see on an MRI of a patient wit sporadic CJD?
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High signal in basal ganglia and cerebellum (diffusion weighted MRI)
Progressive, asymmetrical atrophy |
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Define dementia
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Disturbance of multiple higher functions without clouding of consciousness (c.f. delirium)
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T/F Dementia is part of the aging process
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False
Only 24% of 85+ year olds show signs of dementia |
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What are the risk factors for Alzheimer's disease?
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Age
Family history Head trauma Down's syndrome Vascular risk factors |
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Name two mutations that may be found in familial early onset Alzheimer's disease
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Presenilin 1, Chr 14, commonest, 25-40yo
APP, Chr 21, 45-60 yo Presenilin 2, Chr 1 |
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Name two common pathological findings in Alzheimer's disease
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Tangle (tau) formations: intraneuronal,
Amyloid plaques: beta-pleated sheets, extraneuronal Diffuse synaptic loss |
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T/F Amyloid plaques are extraneuronal, tangles are intraneuronal
(Alzheimer's disease) |
True
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An MRI may show atrophy of which region of the brain in early Alzheimer's disease
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Hippocampus
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Where are tangles (tau) most likely to first be found in Alzheimer's disease?
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Entorhinal cortex
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Which investigations should be ordered for suspected Alzheimer's disease
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MRI : exclusional
FDG-PET: hippocampal atrophy and hypometabolism |
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Which type of memory is affected in Alheimer's disease? Which type is spared?
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Affected: anterograde (story recall)
Spared: working memory (reasoning, comprehension, learning) |
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What are the differential points for dementia with DLB from Alzheimer's disease?
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REM-SBD (violent enactment in sleep) - good differential
Better recall Worse praxis Fluctuations |
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What type of dementia does the following describe: Symetric ‘akinetic-rigid’ parkinsonism, early cognitive and psychiatric disorders (precedes or within 12 months parkinsonism), fluctuation of cognition
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Dementia with Diffuse Lewy Bodies (DLB)
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What percentage of people with Parkinson's disease for at least 20 years have dementia?
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~80%
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What symptoms occur with fronto-temporal dementia?
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Behavioural variance
non-fluent aphasia (problems naming objects, laboured speech, halting) semantic dementia (problems understaning meaning of words) |
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Name two risk factors and two protective factors for Alzheimer's disease
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RF:
Family history Age APO E4 allele PF: Education NSAIDs Low dose alcohol (J curve) |
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What are the most common mutations in familial Alzheimer's disease?
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Presenilin 1> APP > Presenilin 2
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Cleavage of the transmembrane APP protein by which enzymes forms the insoluble Abeta-42 amyloid protein ?
(found in neuritic plaques in Alzheimer's disease) |
beta and gamma secretase
normally alpha secretase cleaves forming soluble products |
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What percentage of people worldwide aged 85+ show signs of Alzheimer's dementia?
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~45%
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Describe the neuritic plaques found in Alzheimer's disease
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Extracellular
Central amyloid (Abeta-42) core with corona of distorted processes and glia Found in frontotemporal --> limbic --> primary motor, sensory and visual regions |
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Describe the neurofibrillary tangles associated with Alzheimer's disease
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Intracellular
Hyperphosphorylated tau accumulates and kills cell Frontal and hippocampus predominantly |
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T/F Both neuritic plaques and neurofibrillary tangles are found in aged subjects without Alzheimer's disease
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True
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Name two potential treatments for Alzheimer's disease
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beta-secretase inhibitors
statins clioquinol (metal chelator) NSAID ACh (symptomatic) |
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Which of the following is the most significant risk factor for Alzheimer's disease:
High cholesterol Smoking Hypertension Severe head trauma |
Severe head trauma
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Which means of prevention is currently considered the best for Alzheimer's disease? :
treat vascular risk factors lifestyle modification dietary supplements avoidable known causes medication |
avoidable known causes:
head trauma, illicit drugs, excess alcohol |
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What are acetylcholinesterase inhibitors used for in Alzheimer's disease and when is their use justified?
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Therapeutic effect on cognitive decline. Cholinergic cell loss in AD may be an important pathway for memory loss.
Used only in moderate to severe AD |
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T/F Patients with the ApoE4/4 allele respond best to anticholinesterase inhibitors in Alzheimer's disease
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False
They respond worst |
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T/F Behavioural changes associated with Alzheimer's disease are often the most traumatic for the carer
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True
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Which of the following is not indicated in the treatment of agitation associated with Alzheimer's disease:
Benzodiazepines SSRIs Antipsychotics Mood stabilisers |
Benzos
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Describe the difference between type 1 and type 2 muscle fibres
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Type 1 (one slow fat red ox):
Slow Lipid and myoglobin abundant Oxidative metabolism Type 2: Fast Glycogen abundant Glycolytic metabolism |
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Which cells are responsible for regeneration following damage to myocytes? How can you tell if a section of muscle has been recently repaired?
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Satellite cells
Regenerated cells have a characteristic central nuclei (c.f. peripheral nuclei in normal myocytes) |
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What are the central pathological features of myaesthenia gravis? How is it tested? How is it treated?
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Auto-immune loss of post-synaptic ACh receptors.
ACh-ase is used to test and treat the disease (increases the amount of ACh in the NMJ, good response is indicative of disease) |
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Which protein is deficient in Duchenne-Muscular Dystrophy? What type of mutation is generally responsible? What is the normal protein's function?
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Dystrophin
Generally a due to deletion mutation. It is a cytoplasmic protein that normally binds actin to the ECM |
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T/F Myotonic dystrophy is the second most common adult dystrophy and is due to a CTG repeat expansion
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False
It is the most common adult dystrophy |
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For which of these inflammatory myopathies is immunosuppression NOT an effective treatment:
Polymyositis Dermatomyositis Inclusion body myositis |
Inclusion body myositis
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Which of these inflammatory myopathies is often mistakenly diagnosed as vaccine-induced inflammation:
Polymyositis Dermatomyositis Inclusion body myositis |
Polymyositis
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Which myopathy gives the characteristic 'red ragged' muscle fibres on biopsy and commonly causes extraocular weakness?
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Mitochondrial myopathy
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