pathology Flashcards

1
Q

define dementia please

A

a decrease in cognitive ability, memory of function with intact consciousness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what part of brain is affected in Alzheimer disease

A

frontal, temporal, parietal

occipital usually spared

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what part of brain is affected in frontotemporal dementia

A

spares parietal lobe and posterior 2/3 of superior temporal gyrus
frontotemporal atrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q
name the dementia:
dementia
aphasia
parkinsonism
changes in personality
A

frontotemporal dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

name the dementia:
dementia
visual hallucinations
parkinsonian features afterwards

A

lewy body dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is most common form of Alzheimer disease

A

late onset

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

APoE2 has what link to AD?

A

decrease risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

APO E4 has what link to AD?

A

increased risk for familial/early onset form

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what genes are associated with familial/early onset AD and their products pelase

A

chromosome 1 - presenlin-1
chromosome 14 - presenlin 2
chromosome 21 - APP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

describe gross appearance of brain in Alzheimer disease

A

widespread cortical atrophy with narrowed gyri and widened nuclei

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what are the neurotransmitter changes in alzhemier disease

A

decreased ach

also happens with aging

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what are two distinguishing histopathos features found in Alzheimer disease

A

senile plaques and neurofibrillary tangles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

please describe senile plaques

A

found in gray matter

extracellular beta amyloid core

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what is the potentnail consequence of senile plagues

A

deposition ofextracellular beta amyloid may lead to amyloid angiopathy - increase risk for intracranial hemorrhage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

how is Abeta/amyloid-beta made?

A

synthesized by cleaving amyloid precursor protein APP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what pathway is involved int eh phosphorylation of both Abeta and tau proteins in AD?

A

no more wnt to inhibit GSK

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

please describe a neurofibrillary tangle

A

intracellular hyperphosphorylated tau protein - insoluble ytoskeletal elements

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what is a marker o the degree of dementia in Alzheimer disease?

A

number of intracellular neurofibrillary tangles form hyper-pid tau protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

where do you see PICK bodies

A

frontotemporal dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what is a pick body?

A

silver-staining spherical tau protein aggregate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what is a lewy body?

A

alpha-synculein defect - lewy bodies are primarily cortical

seen in lewy body dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

time course of creutzdelft jakob disease please

A

rapidly progressive; less than 1 year survival ‘[ weeks to months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what does brain look like in Cj disease please

A

spongiform - bubbles and holes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q
name the dementia:
rapid ie weeks to months
dementia
myoclonus
''startle''
A

Creutzfeld Jacob disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
what causes CJ disease
prions -- PrPc --> PrPsc that form a beta pleated sheet resistant to proteases
26
risk groups for Cj disease please
corneal transplant contact with human brain - neurosurgeons/pathologists improper sterilized cortical electrodes eating beef from cattle with mad cow disease
27
what is the second most common cause fo dementia in elderly
multi infarcts from vascuarl disease
28
what else can cause dementia
``` syphilis HIV vitamin B1, 3, 12 deficiency Wilson disease normal pressure hydrocephalus ```
29
``` syphilis HIV vitamin B1, B3, B12 deficiency Wilson disease vascular disease - multi infarct normal pressure hydrocephalus ```
can all cause dementia
30
what is subacute sclerosing panencephalitis associated wtih
measles/rubeola
31
describe the pathogenesis fo multiple sclerosis please
autoimmune inflammation and demyelination fo the CNS @ brain and sc
32
how does multiple sclerosis present
``` remitting and relapsing optic neuritis - sudden loss of vision resulting in marcus gunn pupils hemiparesis hemisensory symptoms bladder/bowel incontinence ```
33
who does ms usually present in
white people living further away from equator females 20-30 years old
34
what is charcots triad of MS
SIIIN ``` scanning speech incontinence intention tremor intraopthalmoplegia - bilateral nystagmus ```
35
lab findings in MS please
oligoconal bands in CSF | increased Ig in CSF
36
gold standard for ms diagnosis please
MRI | shows periventricular plaques caused by areas of oligodendrocyte loss and reactive gliosis with destruction of aonxs
37
whats going on to cause problems in ms
autoimmune - oligodencrocyte loss and reactive gliosis with destruction of axons leads to periventricular plaques on MRI
38
how do you slow the progression of ms
disease modifying therapies - IFNb and natalizumba
39
how do you treat acute flares of ms
IV steroids
40
how do you treat neurogenic bladder in ms
catheter and muscarinic antagonists
41
how do you treat spasticity in ms
baclofen and GABAb receptor agonists
42
how do you treat pain in ms
opioids
43
``` marcus gunn pupils with suddne loss of vision intranuclear opthalmoplegia hemiparesis hemisensory symptoms bladder and bowel incontiencne scanning speech intention tremor incontinence intranuclear opthalmoplegia nystagmus ```
MULTIPLE SCLEROSIS young adult female northern hemispheres
44
what is the most common cause of acute peripheral neuropathy
GUILLIAN BARRE/acute inflammatory demyelinating polyradiculopathy
45
what is guillian barre?
inflammatory infiltrate in the endoneurium autoimmune condition that destroys schwann cells - inflammation and demyelination of peripheral nerves and motoro fibres after infection with campylobacter jeuni, mycoplasma pneumonia of viral - attach of myelin due to molecular mimcry, inoclautionad and stress
46
presentation of guillina barre pelase
rapid symmetrical ascending motor weakness/paralsysi facial parpalyss in 50% may also see autonomic dysregualtion - cardiac irregulatiris, hypo or hypertension may also see sensory abnormalities
47
prognosis of gullian barre
most survive after complete recovery following weeks to months
48
lab findings in guillina barre please
increased CSF protein with normal cell count | albunimocytlogic dissociation
49
what can happen due to increased protein in the CSF in guillian barre
papilloedema (enlarged blind spot and elevated opti disc with blurred margins on fundoscopy due to optic disc swelling from increased IOP)
50
how to treat guillian barre
respiratory support until recovery plasmapheresis IVIg
51
bugs associated with guillian barre/acute inflammatory demyelinating polyradiculopathy
campylobacter jejuni viruses mycoplasma pneumoniae
52
what is acute disseminated/post infectious encephalomyelitis
mutifocal periventricuarl infmmation and demyelination after infection or vaccinations
53
what can cause multifocal periventricular inflammation and demyelingation?
infectiosn of MEASLES or VZV | vacinnations of rabies or small pox
54
what is inheritance pattern of charcot-marie-tooth disease
autosomal ominant
55
what is charcot marie tooth disease
most common cause of herediatary neuropathy group fo progressive hereditary nerve disorders related to the defective production of proteisn involved in the structure and function of peripheral nerves or the myelin sheath
56
what is charco marie tooth usually associated with
scoliosis | foot deformites
57
scoliosis foot deformities peripheral neuropathis
charcot marie tooth
58
what is the inheritance pattern of krabbe disease
AR
59
what is the inheritance pattern of metachromatic luekodystrophy
AR
60
peripheral neuropathy developmental delay optic atrophy
krabb disease lysosomal storage disease definciency in galactocerebrosidase - increased galatocerebroside and psychosine - destroys myelin sheath
61
what enzyme is deficient in krabbe disease
galatocerebrosidase - leads to accumulation of galatocerebrosides and psychosine that destroy myelin sheath
62
central demyelination peripheral demyelination dementia ataxia
metachromatic luekodystrophy lysosomal storage disease arylsultafase A deficienty - sulfatides build up and imapri production and destroy myelin sheat
63
what is enzyme deficiency in metachromatic leukodystrophy
arylsulfatase A definciency - ulfatides build up that inhibit production and destroy myelin sheath
64
what is progressive multifocal leukoencephaopathy
demyelination of CNS due to destruction of oligodendrocytes
65
what demyelinating disease is JC virus associated with
progressive multifocal leukoencephaoloatphy (destruction of oligodendrocytes)
66
what increases risk of PML
natulizimab and rituximab use | HIV > 50 CD4/mm^3 due to reactivation of JC virus
67
what is clinical course of PML
rapidly progressive and usually fatal
68
what is a seizure
high frequency coordinated neuronal firing
69
inheritance pattery of arenoleukodystrophy
xlinked recessive
70
what is the pathophys of adrenoleukodystrophy
enzyme deficiency ro beta oxidation fo very/long chain fatty acids in perioxisomes - instead build up
71
where do VLCFA build up in adrenoluekodystrophy
adrenal cortex testes nervous system generalized loss of meylin
72
what are consequences fo adrenoleukodstryophy
can lead to long term coma/death and adrenal gland insufficiency
73
describe a partial seizure
affects single area of brain; often preceeded with an aura; can be followed by a gernalzied
74
which typically has an aura, generalized or partial zeisure
partial
75
where is origin most often in partial seizures
medial temporal lobe
76
what is a simple partial seizure
consciousness intact | motor, sensory, autominc, psychic symptoms
77
what is a complex partial seizure
no consciousness intact
78
what is epilepsy
disorder of recurrent seizures
79
are febrile seizures epilepsy
noppers
80
what is status epilepticus
continuous or recurring seizures that mayr esult in brain injury >10-30 mins but variable
81
what can cause seizures in children
genetics, infection, trauma, congenital and metabolic
82
what can cause seizures in adults
tumor, trauma, stroke infection
83
what can aause stroke in elderly
stroke, tumor trauma, etabolic or infection
84
what can cause seizures in children, adults and elderly
infection, trauma
85
what can cause seizures in children and adults
metabolic, trauma
86
what can cause seizures in adults and elderly
trauma, infection, stroke
87
what causesa headaches
pain due to irritation fo structures such as dura, cranial nerves or exgtracrinal structires
88
what type of headaches are unilateral
cluster and migraine
89
what type of headache are bilateral
tension
90
how long is a cluster headache
15 mins to 3 hours and repetititve
91
how long is a tension headache
> 30 mins (typically 4-6 hours) constant
92
how long is a migraine headache
4-72 hours
93
headache with excricuating perioribtal pain
cluster
94
headache with lacrimation and rhinorrhea
cluster
95
headache more common in males
cluster
96
headache with possibly horner syndrome
cluster
97
treatment of cluster headache
100% O2 | sumitriptan
98
bilateral headache with no photophobia or phonophobia or aura
tension headache
99
how to treat tension hedache
analgecis NSAIDs acetaminophem amitriptyline for chronic
100
pulsating pain, which head ache?
migraine
101
photophbia, nausea or phonophobia headache?
migraine
102
what causes pain in a migraine?
irritation of CN V, meningnes or blood vessels - dubtance P, calcitonin gene related petide and vaosciative petides are reelase
103
which headache may have aura
migraines
104
treatment of migraines
abortive therapies like triptan and NSAIDs | prophylaxis - propranolol, topiramate, CCB, amitriptyline
105
POUNDS for migrans please
``` Pulsatile One day duration Unilateral Nausea disabling ```
106
what else can cause headaches
subarachnoid hemorrhage, meningitis, hydrocephalus, neoplasia, arteritis
107
describe pain with trigeminal neuralgia
repetitive shooting pain in the CN V distribution that typically lasts for < 1 minute
108
define vertigo
sensation of spinning while actuallys tationaty
109
what type of vertigo is most common
peripheral
110
what causes peripheral vertigo
inner ear stuffs - semicircular canal debris, vetibular nerve infection, Meniere disease
111
what will show up in positional testing with peripheral vertigo
delayed horizontal nystagums
112
what causes cenral vertigo
brain stem or cerebellar lesion: stroke affectin estibular nuclie or posterior fossa tumor
113
``` directional change of nystagmus skew deviation diplopia dysmetria focal neurological findings ```
central vertigo
114
cxpx of central vertigo please
``` directional change of nystagmus skew deviation diplopia dysmetria focal neurological findings ```
115
what happens in positional testing for central vertigo
immediate nystagmus seen in any direction that mae change firections and focal nerological findings
116
genetics of struge weber syndrome
somatic mosaicism | GNAQ gene activating mutation that causes developmental anomaly of neural crest deriviatves in mesoderma nd ectoderm
117
pathophys of sturg weber syndrome please
GNAQ gene activiating mutation that causes developmental anomalies of neural crest cell derivatives in mesoderm and ectoderm affects small blood vessels
118
cxpx of sturg weber pelase
port wine stain/ nevus flammeus @ CN V1 and V2 unilateral leptomeningeal angiomas - seizure and epilepsy intellectual disability episcleral hemangiomas - increased IOP - early onset glaucoma
119
intellectual disability port winde staine seizures and epilepsy early onset glaucoma
surg weber syndrome
120
what si a nevus falmmeus
non neoplastic ''birthmark;; in CN V1 and V2
121
inheritance pattern of tuberous sclerosis please
autosomal dominant
122
describe cxpx of tuberous sclerosis please
``` angiofibromas in skin ash leef/sheegreen hypopigmented patches rhabdomyoma at heart mitral regurgitation angiomyolipoma at kidney mental retardation increased risk of subependymomal astrocytoma and ungal fibromas ```
123
main characteristic of tuberous sclerosis
hamartomas in the CNS and skin
124
``` angiofibromas at skin rhabdomyomas at heart hitral regurgitation angiomyolipoma t kidney mental retardation ash leaf shagreen ```
tuberous sclerosis
125
inheritance pattern of tuberous sclerosis
autosomal dominant
126
genetics of neurofibromatosis I pelase
chromome 17, NF1 gene, tumor supporessor of RAS, neurofibrominin
127
pathophys of nf-1
skin tumors are derivatives of abberant neural crest cells
128
cxpx of neurofibromatosis I
neurofibromas in skin optic gliomas lisch nodules - pigmented nodules in iris pheochromocytoma increased risk of wilms tumor, pheochromoctyoma and CML café au lait spots
129
what are of increased risk with NF1
CML wilms tumor pheochromocytomas
130
what are of increased risk in tuberous sclerosis
subependymal astrocytomas | uncal fibromas
131
describe genetics of von hippel lindau
chromosome 3 HVL - tumor supporessor of HIF-1
132
describe pathos of von hippel lindaue
hemangioblastmas - high vascularity with hyperchromatic nuclei
133
cxpx of von hippel lindau
hemangioblastomas in retina, cerebellum, spinal cord and brain stem angiomatosis in skin, mucosa, organs bilateral renal cell carcinoma pheocrhrmocytoma
134
hemangiobalstoma in retina, cerebellum, spc, brain stem angiomatosis in skin, m;ucos, organs bilateral renal cell carcinoma pheochromocytomas
von hippel lindau