Pathology - Neurodegenerative disorders Flashcards
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
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
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
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
secondary causes of dementia
cerebrovascular disease - multi infarct dementia
infection - CJD HIV
drugs
metabolic disorders
vitamin deficiency - wernicke korsakoff syndrome
paraneoplastic syndromes - limbic encephalitis
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
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
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
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
what type of alzheimers is heritable
a.late onset
b.early onset
b.early onset
the amyloid precursor associated with alzheimers is found on which chromosome
a.21
b.14
c.1
d.5
e.6
a.21
the ps1 gene associated with alzheimers is found on which chromosome
a.21
b.14
c.1
d.5
e.6
b.14
the ps2 gene associated with alzheimers is found on which chromosome
a.21
b.14
c.1
d.5
e.6
c.1
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
which protein has a primary role in amyloid accumulation
a. AB
b.AC
d.AD
e.AE
a. AB
AB protein is derived from the abnormal cleavage of the normal protein ….
a.APB
b.APA
c.APO-E
d.APP
d.APP
which chromosome is the APP gene found on
a.21
b.14
c.1
d.5
e.19
a.21
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
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
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
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
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
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
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
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