Pathology - Neurodegenerative disorders Flashcards by Jenna Dymond

acquired global impairment of intellect , reason and personality without impairment of consciousness

a.alzheimers
b.dementia
c.parkinsons
d.huntigntons

dementia

emotional liability , memory dysfunction are prominent manifestations implying involvement of the cerebral cortex and limbic system

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how is alzheimers diagnosed

ask the person and a family member questions about overall health , mediation, changes in behaviour and daily activity

conduct memory, problem solving and language tests

standard medical tests eg. bloods etc

brain scans - CT, MRI, PET

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which of these is a secondary cause of dementia

a.alzheimers
b.diffuse lewy body disease
c.frontotemporal lobar disease
d. cerebrovascular disease
e.huntigntons disease

d. cerebrovascular disease
eg- multi infarct dementia

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which of these is NOT. a primary neurodegenerative disorder

a. alzheimers disease
b.wernicke korsakoff syndrome
c.frontotemporal lobar degeneration
d.huntingtons disease
e.diffuse lewy body disease

b.wernicke korsakoff syndrome

secondary cause

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secondary causes of dementia

cerebrovascular disease - multi infarct dementia

infection - CJD HIV

drugs

metabolic disorders

vitamin deficiency - wernicke korsakoff syndrome

paraneoplastic syndromes - limbic encephalitis

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infection with which of these can be a secondary cause of dementia

a.HSV
b.CJD
c.legionella
d.strep pneum
e. varicella zoster

b.CJD

Creutzfeldt-Jakob disease

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infection with which of these can be a secondary cause of dementia

a.HSV
b.HIV
c.legionella
d.strep pneum
e. varicella zoster

b.HIV

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what is the most common primary cause of dementia

a.diffuse lewy body disease
b.huntingtons
c.parkinsons
d.frontotemporal degeneration
e.alzheimers

e.alzheimers

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what is alzheimers disease associated with

a.gender
b.age
c.prior disease
d. substance abuse

b.age

genetic predisposition important and early onset heritable

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what type of alzheimers is heritable

a.late onset
b.early onset

b.early onset

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the amyloid precursor associated with alzheimers is found on which chromosome

a.21
b.14
c.1
d.5
e.6

a.21

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the ps1 gene associated with alzheimers is found on which chromosome

a.21
b.14
c.1
d.5
e.6

b.14

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the ps2 gene associated with alzheimers is found on which chromosome

a.21
b.14
c.1
d.5
e.6

c.1

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there is predisposition to early onset alzheimers in those with the e4e4 in the apo e gene on which chromosome

a.21
b.14
c.1
d.5
e.19

e.19

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which protein has a primary role in amyloid accumulation

a. AB
b.AC
d.AD
e.AE

a. AB

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AB protein is derived from the abnormal cleavage of the normal protein ….

a.APB
b.APA
c.APO-E
d.APP

d.APP

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which chromosome is the APP gene found on

a.21
b.14
c.1
d.5
e.19

a.21

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there is a predisposition for early alzheimers in those with e4e4 in which gene on chromosome 19

a.APO- B
b.APO-C
c.APO-D
d.APO-E
e.APO- A

d.APO-E

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individuals with which syndrome develop early onset alzheimer like changes in the brain as a result of a gene dosage effect

a.huntigtons
b. kleinfelter
c.brown sequard
d,cushings
e.downs

e.downs

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pathogenesis of alzheimers

Amyloid cascade

APP
abnormal cleavage (genetic influences)
AB made
microglial reaction and secretion
AB plaque
makes neurofibrillary tangles and dystrophic neurites
interact with apo e
leads to neuronal death and dementia

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the amyloid plaques in alzheimers disease are composed of the AB protein and form …………. rounded masses in the ECM of the cerebral cortex

a.irregular
b.regular

a.irregular

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the amyloid plaques in alzheimers are composed of AB protein and form brown irregular rounded masses in the ecm of what

a.skull
b.cerebellum
c.cerebral cortex
d.limbic system
e.ventricular system

c.cerebral cortex

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what protein is abnormally phosphorylated and accumulates intracellularly in neurofibrillary tangles (arrows) and dystrophic neurites (arrowheads)

a.apo - e
b.AB
c. amyloid
d.tau

d.tau

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abnormally phosphorylated tau protein accumulates intracellularly in neurofibrillary tangles in alzheimers how do these appear histologically

a.brown irregular rounded masses
b. brown arrows
c.brown arrowheads

b. brown arrows

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abnormally phosphorylated tau protein accumulates intracellularly in dystrophic neurites in alzheimers how do these appear histologically a.brown irregular rounded masses b. brown arrows c.brown arrowheads

c.brown arrowheads

what happens to the brain in alzheimers a.cortical atrophy, gyri narrowing and sulci widening b.cortical atrophy , gyri widening, sulci narrowing

a.cortical atrophy, gyri narrowing and sulci widening

white matter loss in alzheimers is accompanied by .................. of the ventricular system a. dilation b.constriction

a. dilation compensatory hydrocephalus

a group of proteins that may be deposited in tissues , in extracellular locations often basement membranes a.collloid b.tau c.amyloid d.AB

c.amyloid associated with beta pleated sheet conformation classified by fibril type / chemical composition

how does amyloid stain with congo red a.negative b.positive

b.positive

which form of amyloid is associated with significant medical complications a.localised b. systemic

b. systemic localised forms may be incidental or cause significant problems ef accumulation of amyloid in the myocardium and heart failure

AL amyloid is present in which condition a. myeloma associated b.reactive c.alzheimers disease d.hereditary and familial e.medullary carcinoma of the thyroid

a. myeloma associated

AA amyloid is seen in what conditions a. myeloma associated b.reactive c.alzheimers disease d.hereditary and familial e.medullary carcinoma of the thyroid

b.reactive

AB amyloid is present in which conditions a. myeloma associated b.reactive c.alzheimers disease d.hereditary and familial e.medullary carcinoma of the thyroid

c.alzheimers disease

AB 2M amyloid is present in which conditions a. myeloma associated b.reactive c.alzheimers disease d.hereditary and familial e.medullary carcinoma of the thyroid f. haemodyalisis associated

f. haemodyalisis associated

ATTR, AA, AGel amyloid is present in which conditions a. myeloma associated b.reactive c.alzheimers disease d.hereditary and familial e.medullary carcinoma of the thyroid f. haemodyalisis associated

d.hereditary and familial familial neuropathic - ATTR famiial mediterranean fever - AA AGel - finnish amyloidosis

AMCT amyloid is present in which conditions a. myeloma associated b.reactive c.alzheimers disease d.hereditary and familial e.medullary carcinoma of the thyroid f. haemodyalisis associated

e.medullary carcinoma of the thyroid calcitonin - amct

A 70 year old man with a long history of poorly controlled diabetes and plasma cell myeloma develops progressive proteinuria and nephrotic syndrome. Renal biopsy shows congo red positive material diffusely deposited within the glomeruli. Which of the following diagnoses is most likely? * AA amyloidosis * AL amyloidosis * Diffuse glomerulosclerosis * Kimmelstiel- Wilson lesion * Membranous glomerulonephritis

* AL amyloidosis AL deposits caused by myeloma

amyloidosis leads to what ...

heart failure organ enlargement nephrotic syndrome

AB amyloid accumulation in the walls of small arterioles and capillaries in the brain

cerebral amyloid angiopathy

what colour does amyloid stain on congo red stain a.dark pink b.salmon pink c.blue d.apple green

b.salmon pink

what colour does amyloid show under polarised light a.salmon pink b.blue c.apple green d.dark brown

c.apple green

cerebral amyloid angiopathy in isolation predisposes what a.spontaneous intracerebral haemorrhage b.ischaemic stroke d. dementia c.medullary carcinoma of the thyroid

a.spontaneous intracerebral haemorrhage

how can alzheimers be inherited a.autosomal recessive b.autosomal dominant c.x linked

b.autosomal dominant

a clinical syndrome characterised by tremor, rigidity , bradykinesia and postural instability

parkinsonism

impaired function of which neurons projecting from the substancia nigra to the corpus striatum leads to parkinsonism a.gaba b.dopamine c.serotonin d.glutamate e.acetycholine

b.dopamine

causes of parkinsonism

drugs - neuroepileptics cerebrovascular disease other progressive degenerative neurologic disease eg progressive supranuclear palsy PSP, MSA and and corticobasal degeneration post encephalitic CO poisoning

poisoning by what can lead to parkinsonism a. lead b. asbestos c.opiod overdose d.carbon monoxide e.nitrous oxide

d.carbon monoxide

most cases of parkinsons disease are... a.idiopathic b.idiosyncratic c. inherited

a.idiopathic autosomal dominant and recessive forms also exist

point mutations and duplications of which gene cause autosomal dominant parkinsons a.parkin gene b.a synuclein c.UCHL1 d.LLRK2 kinase e.PARK 7

b.a synuclein

which gene is involved in the recycling of ubiquitin and defects can cause parkinsons disease a.parkin gene b.a synuclein c.UCHL1 d.LLRK2 kinase e.PARK 7

c.UCHL1

genetic changes in which of these genes are associated with mitochondrial dysfunction and can cause parkinsons disease a.parkin gene b.a synuclein c.UCHL1 d.LLRK2 kinase e.PARK 7

d.LLRK2 kinase e.PARK 7 and PINK 1

parkinsons disease has inherited predisposition accompanied with what

enviromental triggers

patient with tremor, stiffness, postural imbalance and bradykinesian , diagnosed with parkinsons disease what will show on the histology a.rounded cytoplasmic inclusions with a dense core and pale halo b.irregular rounded brown masses c.brown arrowheads d.salmon pink

a.rounded cytoplasmic inclusions with a dense core and pale halo lewy bodies and aggregates of a synuclein with neurofilaments and ubiquitin

where are lewy bodies found in parkinsons disease a.red nucleus b. substancia nigra c,ventricular system d.sella turcica

b. substancia nigra

in a person with parkinsons the DAT scan shows asymetrically reduced activity in which area with preserved but lower than normal dopamine uptake in the caudate nucleus a.putamen b.globus pallidus c. 4th ventricle d.substancia nigra

a.putamen posterior section of comma shapes on either side (striatum - caudate nucleus and putamen) is gone showing reduced putament activity usually asymmetrical

on a normal DAT scan what shape does the striatum appear showing dopamine uptake of normal levels a.circle b.comma c.bracket d.star

b.comma round part - caudate nucleus posterior tail - putamen

non motor symptoms of parkinsons

hyperhidrosis - excessive sweating increased salivation and liquid anxiety , depression, dementia, hallucinations and delusions

excessive sweating, increased salivation and anxiety, depression, dementia, hallucination and delusions are non motor symptoms of which condition a.dementia b.alzheimers c.parkinsons d.huntingtons e.cerebral amyloid angiopathy

c.parkinsons

patient has tremor, rigidity, bradykinesia, impaired balance muscle cramps and smaller handwriting which diagnosis most likely a.dementia b.alzheimers c.parkinsons d.huntingtons e.cerebral amyloid angiopathy

c.parkinsons

in which condition are lewy bodies seen more widespread distribution including in the cerebral cortex and limbic system a.lewy body dementia b.alzheimers c.parkinsons d.huntingtons e.cerebral amyloid angiopathy

a.lewy body dementia

in which condition are lewy bodies seen in the lower brain stem and substancia nigra a.lewy body dementia b.alzheimers c.parkinsons d.huntingtons e.cerebral amyloid angiopathy

c.parkinsons

dementia within a year of onset of motor symptoms associated with visual hallucinations , confusion, sleep disturbances, fainting and falls . histology shows lewy bodies in cerebral cortex and limbic system which most likely diagnosis a.lewy body dementia b.alzheimers c.parkinsons d.huntingtons e.cerebral amyloid angiopathy

a.lewy body dementia

huntigtons is inherited in which pattern a.autosomal recessive b.autosomal dominant c. x linked

b.autosomal dominant

autosomal dominant condition associated with degenration of the striatum , associated with movement disorder ad changes progressing to dementia and associated with increased risk of suicide in early stages a.lewy body dementia b.alzheimers c.parkinsons d.huntingtons e.cerebral amyloid angiopathy

d.huntingtons

Huntigtons is caused by CAG trinucleotide .................. of a gene located on 4p16.3 that encodes the huntingtin protein a,point mutation b.deletion c.substiturion d.repeat expansion

d.repeat expansion normal alleles have 11-34 repeats , disease causing can have 100s larger numbers associated with earlier onset (genotype phenotype correlation)

huntingtons is caused by a ... trinucleotide repeat expansion of a gene located on 4p16.3 that encodes the protein huntingtin a. A A C b. C A G c.G A C d. T A C

b. C A G

which type of transmission of the repeat expansion in huntingtons is associated with an earlier onset of the disease a.maternal b.paternal

b.paternal

expanded .......... tracts in the huntigtin protein are associated with toxicity of the protein a. polyglutamine b.polyglutamate c.polydopamine d.polyacetylcholine

a. polyglutamine

CAG trinucleotide repeat expansion of a gene in 4p16.3 that encodes huntingtin protein leading to a mutant protein that is subject to proteolysis and ubiquitinations making polypetide fragments that are toxic and accumulate forming intranucleat inclusions in neurons leading to cell death atrophying the caudate nucleus and putamen in particular a.lewy body dementia b.alzheimers c.parkinsons d.huntingtons e.cerebral amyloid angiopathy

d.huntingtons

degenerative brain disease associated with a history of repetitive head impacts , eg in contact sport , classically associated with cognitive impairment / specific neurologival syndromes eg. parkinsonism, neurobehavioural dysregulation may also be associated a.lewy body dementia b.alzheimers c.parkinsons d.huntingtons e.chronic traumatic encephalopathy

e.chronic traumatic encephalopathy

patient with cognitive impairment, parkinsonism and neurobehavioural dysregulation , findings in the brain show structural abnormalities of the septum pellucidum , thinning of the corpus callosum , degeneration of the substancia nigra and cerebral cortical neurofibrillary triangles a.lewy body dementia b.alzheimers c.parkinsons d.huntingtons e.chronic traumatic encephalopathy

e.chronic traumatic encephalopathy

abrupt onset confusion abnormalities of eye movement and ataxia a.wernicke korsakoff syndrome b.korsakoff syndrome c.wernicke encephalopathy

c.wernicke encephalopathy secondary to thiamine defficiency caused by gastrectomy or chronic alcohoism

gastrectomy and chronic alcoholism can cause thiamine defficiency leading to what a.wernicke korsakoff syndrome b.korsakoff syndrome c.wernicke encephalopathy

.wernicke encephalopathy

irreversible memory disturbances due to damage to mamillary bodies is known as what a.wernicke korsakoff syndrome b.korsakoff syndrome c.wernicke encephalopathy

b.korsakoff syndrome

wernicke korsakoff syndrome

damage to mamillary bodies wernicke encephalopathy (abrupt onset confusion and eye moevemnt abnormalities ) korsakoff syndrome ( irreversible memory disturbance) stages of wernicke korsakoff syndrome

wernicke korsakoff syndrome is a defficiency of which vitamin a.b1 b.b2 c.b12 d.b4

a.b1 thiamine

which operation can cause wernicke korsakoff syndrome a.gastectomy b.open heart surgery c. coiling d. cervical disc surgery

a.gastectomy

abnormal protein that induce abnormal folding of other proteins

prion

which of these is an abnormal protein form that is resistant to proteolysis and can induce conformational change in the native protein in process of propagation and aggregation a.PrPc b. PrP sc

b. PrP sc

new variant of CJD in young adults associated with behavioural changes and slower onset of neurological signs and symptoms was associated with exposure to which disease a.bovine spongiform encephalopathy b. foot and mouth c.

a.bovine spongiform encephalopathy

bovine spongiform encephalopathy is associated with what a.lewy body dementia b.alzheimers c.cjd d.wernicke korsakoff syndrome e.chronic traumatic encephalopathy

c.cjd

cjd is associated with spongiform change in the cerebral cortex and deep ...... matter caused by accumulation of abnormal proteinase resitant prpsc a.gray b.whote

a.gray

cjd is associated with a spongiform change in the deep gray matter and where else a.cerebellum b.substancia nigra c.striatum d.cerebral cortex

d.cerebral cortexd

vcjd is characterised histologically by amyloid plaques in the regions of ............. spongiform change a.most b.least

a.most