pathology final Flashcards
true or false
All degenerative diseases show fast
deterioration
false , slow deterioration
how do the aggregated proteins cause the dysfunction
1) they have a direct toxic effect
2) the more they accumulate the less there are proteins which perform their function
true or false
protein aggregates behave like
prions (INFECTIOUS FROM PERSON TO
PERSON)
false , they are not infectious from person to person
if proteins accumulate in the motor neurons this will lead to?
motor weakness , the main disease is amyotrophic lateral sclerosis
causes of dementia?
1) Alzheimer disease
2) Lewy body disease
3) frontotemporal dementia
4) infections
5) metabolic and endocrine disorders
6) nutritional deficiencies
7) reactions to medications
8) subdural hematoma
9) poisoning
10) brain tumors
11) anoxia
complications of dementia
1) inadequate nutrition
2) pneumonia
3) inability to perform daily tasks
4) personal safety challenges
5) death
what is the most important risk factor for Alzheimer
Age
The most commonly recognized symptom of
Alzheimer is
inability to acquire new memories and not difficulty in recalling recently observed facts
what is the affect of the accumulation of both AB amyloid proteins and tou protein
they both cause neuronal death and dysfunction, they accumulate due to increased production and decreased removal
true or false
APP is a cell surface protein with multiple transmembrane domains
false, it only has a single transmembrane domain
first cleavage is in the extracellular domain , the second cleavage is in the intra-membranous domain
what is the function of APP ?
it’s primary function is unknown but it has been implicated as a regulator of synapse formation and iron export
which pathways cut in the middle of the APP and which pathway cuts at the N terminus
the non-amyloidogenic pathway cuts in the middle while
the amyloidogenic pathway cuts at the N terminus
true or false
aggregation of beta amyloid leads to alteration in neurotransmission, damage or even death of neuron, inflammation
true
true or false
aggregation of tau causes neuronal damage and it causes microtubules instability
true
true or false
Certain polymorphisms in this locus on chromosome 19
increases the risk of inherited AD
false, sporadic AD
note: Point mutations in APP are a cause of familial
AD
true or false
diagnosis of AD
needs both clinical and histological findings
true
the amyloid plaques are divided into ?
focal and diffuse plaques
how does the FTLD differ from AD
changes in personality and language precede
memory loss .
When Tau is hyperphosphorylated two changes
occur:
1) decrease binding ability to microtubules
2) it’s ability to aggregate increases
true or false
INHERITED forms have mutations in Tau protein
causing increased accumulation
true
FTLD is a neurodegenerative disease where the primary abnormality is in?
Tau protein
parkinsonism is characterized by:
bradykinesia, instability, rigidity, tremors
causes of parkinsonism :
1) dopamine antagonists ,
2) toxins
3) Parkinson disease
true or false
AD is the most common cause of dementia nd it is the most common cause of neurodegenerative disease
true
true or false
the incidence of Parkinson’s increases with age
true
the rigidity and the tremor in Parkinson’s are known as
cogwheel rigidity, resting tremor ( pin-rolling tremor, postural tremor , lower limb tremor(in supine position))
note: the tremors diminish completely during sleep
if dementia occurs within the FIRST year in Parkinson’s then it is known as
Lewy body dementia
what protein accumulates in Parkinson’s
alpha synuclein
Lewy bodies are?
alpha synuclein inclusions
true or false
alpha synuclein has prion like pattern , it starts in the brain stem then the basal ganglia, then the cerebral cortex where it causes cognitive impairment
true
note: the first alpha synuclein aggregates are found in the medulla
Characteristic features of this PD with dementia are
fluctuating course and hallucinations
true or false
Huntington’s disease had a sporadic form
false
what is the role of the huntingtin protein
it has a role in long term memory storage
VERY IMPORTANT
true or false
the course of Huntington’s disease is not affected by the number of repeats
true
huntingtin’s disease causes what histologic changes
intra-nuclear inclusions
the Huntington’s protein is cleaved by ?
caspases
true or false
Huntington disease can cause memory loss , severe dementia and behavioral changes such as suicide
true
all spinocerebellar ataxia are caused by
trinucleotide repeat expansion
true or false
ataxia plays a role in cognitive and mood problems
true, because the cerebellum plays a role in some forms of thinking
what are the symptoms of Fredrich ataxia ?
1)gait ataxia 2) hand clumsiness 3) dysarthria
it is associated with cardiac abnormalities and DM
what is the function of frataxin?
a protein that regulates cellular iron level especially in the mitochondria
very imp
true or false all spinocerebellar ataxias cause neuronal damage by protein accumulation
false, in Fredrich ataxia it is caused by protein loss
true or false
ALS is 100% lethal
true
true or false
in ALS : because there are several mutations, there are also several types of misfiled proteins and several types of inclusions
true
can vitamin B 12 cause brain atrophy
yes, which causes the dementia
the main cells affected by hypoglycemia in the brain
hippocampal neurons and Purkinje cells
what are the most common primary tumors
gliomas
note: 80% of gliomas are diffuse astrocytoma’s
true or false
grade 1 diffuse astrocytoma is known as pilocytic astrocytoma
false, there is no DIFFUSE astrocytoma
the characteristics of glioblastoma multiform
1) necrotic( pesudopalisading) OR 2)vascular proliferation
note: looks like an anaplastic pus
if the vascular proliferation is marked and clear it forms?
glumeruloid body
pilocytic astrocytoma’s are most commonly found in
cerebellum
pilocytic astrocytoma has the same mutation as diffuse astrocytoma ?
false, it doesn’t have the IDH mutation but it has the BRAF mutation
are the Rosenthal fibers specific for pilocytic astrocytoma
no, they can also be found in chronic gliosis
true or false
oligodendrogliomas are mainly found in the cerebral hemispheres in the gray matter.
false,. in the WHITE matter
which one has a better prognosis astrocytoma or Oligodendroglioma
oligodendroglioma
describe the appearance of cell in oligodendrogliomas
fried egg appearance
histology of ependymoma
Rosette around canals and pseudo-Rosette around blood vessels
what is the origin of Embryonal neoplasms
they have a neuroendocrine origin
the most common embryonal neoplasm is ?
medulloblastoma
medulloblastoma occurs Exclusively in
cerebellum
occurs predominantly in children
which medulloblastoma has a better prognosis, MYC amplification or WNT pathway
WNT pathway
true or false
NUCLEAR beta catenin is a 100% indicator for the presence of a mutation in the WNT pathway
true
true or false
WNT tumors arise exclusively in an older age group of children over the age of three years
true
meningiomas arise in
ADULTS
what are the 4 histologic types of meningiomas
1) syncytial
2) fibroblastic
3) transitional
4) Psammomatous
which type of brain tumors resemble sarcoma
anaplastic meningioma
what tumor is the most common CNS tumor in immunocompromised individual’s
primary CNS lymphoma
manifestations of paraneoplastic syndromes
dementia, ataxia, psychosis, sensory neuropathy
Von Hippel Lindau syndrome mutation?
mutation in VHL tumor suppressor gene
note: Increase risk of renal cell carcinoma
