PATHOMA nervous system Flashcards
(164 cards)
1
Q
what is the most common cause of hydrocephalus in newborns
A
cerebral aqueduct stenosis
2
Q
where does CSF flow from the 4th ventricle
A
into the subarachdonic space via the forami=en of Lushka and Megendie
3
Q
what is dandy walker syndrome
A
it is the absence of formation of the cerebral vermis. It presents as an enlarged (huge) 4th ventrice with the absence of the cerebellum
4
Q
what is arnold chiari syndrome
A
it is the downward displacement if cerebellar vermis and tonsils into the foramen magnum
5
Q
what is arnold chiari syndrome associated with
A
- Meningomyelocele
2. Syringomyelia
6
Q
what is syringomyelia associated with
A
type I arnold chiari syndrome
7
Q
what is a syrinx
A
it is the cyst in syringomyelia (damage to anterior white commissure from C8 - T1)
8
Q
what happens if the syrinx expands (what tracts will be damaged)
A
- damage to anterior horn - lateral corticisoinal tract - LMN damage - negative babinski sign
- Lateral horn damage - hypothaloamosoinal tract - horners syndrome
9
Q
what is damaged in poliomyelitis
A
anterior horn - flaccid paralysis, fasciculations, negative babinski sign, decreased reflexes, muscular weakness and muscular atrophy
10
Q
what is Werdnig Hoffman disease
A
autosomal recessive damage to the anterior horn in a baby - floppy baby
11
Q
what mutation is seen in familial ALS conditions
A
zinc-copper superoxide dismutase (SOD-1) mutation
12
Q
what accumilates in fredrick ataxia
A
it tis the damage to spinal tarcts and to the cerebellum
13
Q
what are the symptoms of fredrick ataxia
A
proprioception problems, muscular weakness in lower extremities, vibration damage and damage todeep tendon reflexed (DTR)
14
Q
wht is frataxin useful for
A
it is used for mitochondrial iron regulation. It it is damaged, it’ll cause free radicle damage to hte nerves via phenytoin reaction
15
Q
what are the associations of fredrick ataxia
A
hypertrophic cardiomyoathy
16
Q
causes of meningitis in neonates
A
GBS, E.coli (both via birth canala) and Listeria
17
Q
causes of meningitis in teenagers
A
N. meningitidis
18
Q
causes of meningitis in unvaccinated people
A
H. influenza
19
Q
causes of meningitis in adults and elderly people
A
Strep pneumoniae
20
Q
causes of meningitis in immunocompromised people
A
fungal infections
21
Q
characteristics of CSF in bacterial meningitis
A
high neutrophils with low glucose
22
Q
characteristics of CSF in viral meningtis
A
high lymphocytes with high glucose
23
Q
characteristics of CSF in fungal meningitis
A
high lymphocytes with low glucose
24
Q
what is te classic triad of meningitis
A
headache, fever and nuchal rigidity…along with photophobia, vomiting and altered mental status
25
what is the sequale related to fibrosis after meningitis (bacterial)
hydrocephalus, hearing loss and seizures
26
what nucleotide is repeated in fredrick aatxia
nucleotide GAA is repeated in the frataxin gene
27
what are the causes for cerebrovascular diseases
ischemia (85%)and hemorrhage (15%)
28
how long does it take the neruron to undergo necrossis
3-5 minutes
29
etiology of global cerebral ischemia
low perfusion (atherosclerotic plaque), chrnoic hypoxia (ischemis), acute decrease in blood flow (cardiogenic shock) and repeated low glucose levels (insulinoma)
30
what does moderate global ischemia lead to
1. Pyramidal cells infarction in 3rd, 5th and 6th layers if cortex( corticolaminar necrosis)
2. Pyramidal cells in the hypothalamus in the temporal lobe (long term memory damage)
2. Purkinjie cells in the cerebellum (integration between sensory and motor control is lost)
31
what are watershed areas
the areas of low perfusion between the anterior and middle cerebral arteries. They are the main areas of moderate global ischemia
32
what are the subtypes of ischemic stroke
thrombotic, ebolic and lacunar
33
where does the thrombotic infarct usually occur
bifurcation of internal carotid and middle cerebral artery in the anterior circle of willis
34
where does embolic stroke ususally occur
middle cerebral artery
35
In which conditions does hyaline arteriosclerosis occur
HTN and DM
36
where does lacunar stroke occur
1. In the lenticulostriatal vessels from the middle cerebral artery in the deep poritons of the brain
2. Internal capsule - motor lock
3. Thalamus - sensory lock
37
what type of necrosis does ischemic stroke cause
liquefactive infarction
38
hematoma vs hemorrhage
hematoma is a solid collection that is clotted or in the process of clotting
hemorrhage is ongoing and active bleeding
39
where does intracerebral hemorrhage usualy occur
in the basal ganglia
40
causes for intracerebral hemorrhage
charcott bouchard microaneurysma - they occur due to HTN in lenticulostriatal vessels
41
presentation of intracerebral hemorrhage
headche, nausea, vomiting and eventually coma
42
where does a subarachnoid bleeding occur
at the base of the brain
43
causes of a subarachnoid aneurysm
berry aneurysms, anticoagulable state and AV malformations
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why do berry aneurysms rupture easily
because they dont have medial (middle) layer in the blood vessel
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where do berrry aneurysms uaually occur
in the anterior circle of willis in the branching point of anterior communicating artery
46
presentation of a subarachnoid hemorrhage
sudden headache (worst headache of life) with nuchal rigidity.LP has xanthochromia due to bilirubin breakdown
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association of berry aneurysms
marfan syndrome and ADPKD
48
what do cerebral traumas cause
hematomas
49
Fracture of the skull usually causes rupture of which vessel
middle meningeal artery
50
how does an epidural hematoma presented on a CT
lens shaped
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CT vs MRI
CT is used for hard tisue findings while MRI is used for soft tissue
52
what is the complication of an epidural hematoma
hermiation f thr brain due to increased intracranial pressure
53
talk and die syndrome
occurs in epidural hematoma due to sudden rupture of the hematoma nad sudden herniation of the brain
54
how does a subdural hematoma occur
due to breaking of the bridginf veins in the arachnoid space and also due to brain atrophy (as it pulls the brain vessels away frm the skull)
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presentation ofsubdural hematoma on CT
cresent shaped
56
what is herniation of the brain
it is the diaplacement of the brain tissue due to mass effect or due to increased intracranial pressure in the brain (protruion of one part of thr brain into other part)
57
what is the lethal complication of subdural hemorrhage
brain herniation
58
what are the types of herniation
1. subfalcime
2. uncal
3. tonsillar
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what is subfalcine herniation and its result
it is the folding of the cingulate gyrus under the flax cerebri.It causes compression of the anteriot cerebral artery and leads to infarction
60
what is uncal herniation and its result
it is the downward displacement of the uncal part of th temporl love into the firamen magnum and it causes :
1. compression of CN 3 - down and out eye
2. duret (brainstem) hemorrhage due to compression of paramedian arteries
3. posterior cerebral artery compression - contralateral homonymous hemianopsia
61
what is tonsillar herniation
downward displacement of the cerebral tonsils into the foramen magnum. It causes thecompression of brinstem and causes cardiopulmonary arrest
62
phases of ischemic stroke
1. 12 hrs to 14 hrs - esinophilia with red nerons (dead neurons)
2. 1-3 days :neutrophilic infiltration
3. 4-7 days : nacrophage infiltration
2-3 weeks : water filled cyst of necrotic area covered with gliosis
63
name the demyelinating disorders
1. leukoencephalopathy
2. Progressive multifocal leukodystrophy
3. MS
4. Subacute Sclerosing Panencephalitis.
5. Central Pontine Myelinolysis
64
what is central pontine myelinolysis
focal degeneration of anterior braonstem (pons). It results in licken in syndrome
65
what is locked in syndrome
It is acute bilateral paralysis- paralysis of all the voluntary motor movements except eyes muscles as all the other motor nerves go down through the pons
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wht does acute bilateral paraysis occur
It is due to rapid correction of hyponatremia in malnourished , alcoholics and in patietns with liver disease
67
what is preogressive multifocal leukoencephalopathy and wht causes it
it is caused by JC virus.
Here, the oligodendrocytes are destroyed or damaged. The patient has fast neurological problems like dementia, vision loss and weakness. It can only cause this in immunocompromised state
68
what is subacute sclerosing panencephalitis
it is caused by measels virus in childhood.It may lead to slow progresive neurological deficits and leads to death
Infection - infancy
panencephalitis - childhood (years later)
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wat is affected in subacute sclerosing panencephalitis
both grey mater (neurons) and white mater (myelin and axons)
70
what are leukodystrophies
it is the dengeneration of white mater (defective enzymes that are necessary for myelin production)
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what are the types of leukodystrophies
1. Metachromatic - damaged arylsulfatase :causes accumilation of sulfatides in lysosomes of oligodendrocytes...lysosomal storage disorders (autosomal recessive)
2. Krabbes disease - galactocerebrocidase deficinecy...galactocerebrocides accumilate iin macrophages (autosomal recessive)
3. Adrenoleukodystrophy - impaired coenzyme A attachment to long chani fatty acids.. fatty acids accumilation in the white mater and in the adrenal gland....X linked
72
What is MS
it is degeneration of myelin and oligodendrocytes
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who does MS generally affect
younger people (20-30 yrs) , mostly women and people away from the equater
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which HLA is MS associated with
HLA-DR2
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How is MS presented
1. optic nerve - blurred vision
2. Spinal cord - lower extremity weakness or sesory loss
3. Brainstem - vertigo and scannig speech similar to alcohol intoxication
4. Internuclear opthalmoplegia - damaged MLF
5. Cerebral white mater, usually periventricular - hemiparesis or inulateral sensation loss
6. Autonomic system - sexual,bowel and bladder functions are affected
76
Clinical featuress of MS
presents as recurrent neurological defects with rperiods of remission ( multple lesions in time and space)
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diagnosis of MS
MRI and LP
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features of MRI in MS
white plaques in white mater
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features of LP in MS
Oligoclonal IgG bands, increased lymphocytes, myelin basic protein and increased immunoglobulins
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Treatment of MS acute attacks
high dose steroids
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long term treatent ofMS
interferon beta slows the progression of the disease
82
gross features of MS
grey plaques in white matter
83
sources for brain metatastatic tumors
lung, breast and kidney
84
name all the glial cells
1. astrocytes (BBB)
2. oligodendrocytes (myelination- schwann cells in PNS)
3. ependymal cells (surround the ventricles)
4. mesothelial cells (surround the ventricles)
85
Adult brain tumor location
supratentorially
86
Child brain tumors location
infratentorially
87
most common primary brain tumors in adults
glioblastoma multiforme
schwannoma
meningioma
88
most common primary brain tumors in children
pilocytic astrocytoma,
ependymoma
medulloblastoma
89
what is meningioma
it is benign tumor od arachnoid cells in adults
90
who is suseptible to meningioma
females, as meningioma has estrogen receptors
91
imaging of meningioma
round tumor attached to the dura
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histology of meningioma
it has whorled cells , also may have psammoma bodies
93
presentation of meningioma
it compresses the brain but doesnt invade (and so causes seizures)
94
wht is GFAP
Glial Fibrillary Acidic Protein - it is a types III intermediate filament in the brain cells usually in the astrocytes and in the ependymal cells during development
It is encided by GFAP gene
95
what is glioblastoma multiforme (GBM)
It is the malignant tumor of astrocytes in adults
96
histology of GBM (glioblastoma multiforme)
It has pseudopallisading around necrotic tissue, endothelial cells proliferation and is GFAP positive
97
characteristics of GBM brain tumor
It has poor prognosis and invades the corpus callosum and goes to the other hemisphere. And so it is called as a BUTTERFLY LESION
98
what is schwannoma
It is a benign tumor od schwann cells in adults
99
staning for schwannoma
s-100
100
what is affected in schwannoma
the cranial and spinal neurins inside the cranium, but largely CN VIII is affected in the cerebellopontine angle causing tinnitis and heairng loss
101
associations of schwannoma
bilateral schwannoma is associated with neurofibromatosis II
102
what is an oligodendroglioma
it is a malignant tumor of oligodendrocytes in aduts
103
oligodendroglima imaging
It looks as a calcifies tumor on the frontal lobe
104
histology of oligodendroglima
it has fried eggs imaging
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where does oligodendroglima usually occur and what are its presentations
it occurs in the frontal lobe and it causes seizures
106
what is pilocytic astrocytoma and where does it occur
it is a benign tumor in children and it occurs in the cerebellum
107
imaging of pilocytic astrocytoma
it looks like a cyctic lesion with a mural nodule
108
histology of pilocytic astrocytoma
it is GFAP positive, it has rosenthal cells and has edinophilic granular cells in histology
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wht is a medulloblastoma
It is a malignant tumor of granular cells of the cerebellum (neuroectoderm) in children
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how and where does a medulloblastoma spread
it is highly spreading and has drop metastasis to cauda equina via the CSF
111
histology of medulloblastoma
it has small blue round cells called as Horner Wright rosette
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what is an ependymoma
It is a malignant tumor of ependymal cells in children
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ependymoma histology
It has periventricular pseudorosette on histology
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location of ependymoma
it is located in the 4th venetricle and may cause a hydrocephalus
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whatis a craniopharyngeoma
It is a benign tumor derived from the epithelial cells of rathkes pouch in children
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how does a craniopharyngeoma present as
It compresses the optic nerve and causes blurred vision and it may also cause seizures
117
medulloblastoma imaging
It look like a calcified tooth on imaging
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which brain tumors are GFAP positive
Glioblastoma multiforme
| pilocytic astrocytoma
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what are the malignant brain tumors in children
ependymoma
| medulloblastoma
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what are the malignant brain tumors in adults
Oligodendroglioma
| Glioblastoma multiforme
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which brain tumors arise from the cerebellum
Pilocytic astrocytoma
| Medulloblastoma
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What leads to movement problems
degeneration of brainstem and basal ganglia
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what leads to dementia
degeneration of cortical grey mater
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what is associated with increased risk of sporadic alzheimers
Epsilon 4 allele of apolipoprotein E (APOE)
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what is associated with decreased risk of sporadic alzheimers
Epsilon 2 allele of apolipoprotein E (APOE)
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what is the most common cause of demetia
alzheimers
127
wht is the cause of death (COD) in alzheimers
infections in bed-ridden patients
128
what are the clinical features of alzheimers
1. behavioral changes and
2. loss of cholinergic stimulation from the basal nucleus of meynert
3. slow onset dementia and progressive disorientation
4. lossof learned motor skills and language
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when is early onset alzhemiers seen
1. Down syndrome patients
| 2. familial cases witl presinilin 1 ans presinilin 2 mutation (mostly presenilin 1)
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how is down syndrome associated with alzheimers
Epsilon 4 allele of APOE is on chromosom 21 and down syndrome is trisomy 21. Epsilon 4 allele causes increased production of AB amyloid and since down syndrome patietns hav trisomy, it increases the conversion and it leads to early onset alzheimers
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what are the morphological features of alzheimers
1. Cortical degeneration with narrowing of gyrus , widening of sulsi and hydrocephalus ex vacuo
2. Neurofibrillary tangles
3. Neuritic plaques
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how are AB amyloid formed and wht do they result in
Every neuron has amyloid precursor protein (APP) and the action of alpha-secretase causes degeration and recycling of the APPs. But when beta-secretase acts on APPs, it formes beta pleated configurated AB amyloids which cant degrade and hence deposit in the brain and cause alzheimers
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what are neurofibrillary tangles
They are abnormal elongated tau proteins (microtubular associated proteins) due to hyperphosphorylation of tau protein
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how is alzheimers diagosed
by clinical features
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how is alzheimers confirmed
post-mortem brain autopsy
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what does amyloid deposition around or in the brain vessels cause
it causes CEREBRAL AMYLOID ANGOPATHY which causes weakening of the blood vessels and leads to hemorrhage
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what is the 2nd most cause of dementia
vascular dementia
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what is vascular dementia
it is due to degeneration of pyramidalcells in the cortical 3rd, 5th and 6th layers and the pyramidal cells in the hippocampus
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Name the degenerative disorders of the brain
1. Alzheimers
2. Vascular dementia
3. Parkinsons disease
4. Norma pressure hydrocephalus
5. Spongiform encephalitis
6. Huntingtons disease
7. Picks disease
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what is picks disease
It is selective frontal and temporal cortical degeneration
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clnical features of picks disease
early onset behavioral and language symptoms and progresses to dementia
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what is seen on histology for picks
round tau proteins aggregates (pick bodies) in the cortex
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what is masked face
It is the less expressive fase in parkinsons patients due to facial muscle rigidity
144
what is parkinsons disease
it is the degeneration of substancia niagra pars compacts (SNPC) in the basal ganglia that leads to deteriorated nigostraiatal pathway
145
causes of parkinsons disease
unknown, byt rarepy, MPTP exposure can lead to parkinsons
146
what are the clinical features of parkinsons disease
TRAP
1. Tremor - pill rolling tremor of the fingers and hands that reduced with movement
2. Rigidity - cogwheel rigidity of the expremities
3. Akinesia/bradykinesia - masked face and slowing of voluntary movements
4. Postural instability and gait problems
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histology of parkinsons disease
lewy bodies in the basal ganglia and degraded substantia nigra in the basal ganglia
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what are lewy bodies
round esinophilic inclusions with aphla synergin
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what is huntungtons disease
It is the degeneration of GABA in the caudate nucleus
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what is the clinical finding in Huntingtons disease
repeated trinucleotide sequence accumilation of CAG in the caudate nucleus
151
how is huntingtons transmitted
the CAG is added to the spem in every spermatogenesis and is autosomal dominant in trnasmission
152
presentation of huntingtons disease
presents with chorea and progresses to dementia and depression
153
CODin huntingons disease
suicide from depression
154
what are the clinical features of normal pressure hydrocephalus
triad of urinary incontinence, dementia nd gait problems
155
Rx of normal pressure hydrocephalus
VP shunt for excess CSF or repeated lumbar punctures
156
what is spongiform encephalitis
It is a prion disease in humans. Every neuro had normal prio protein PrPc (alpha form).
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how is spongiform encephalitis transmitted
1. Sporadic form
2. Familial form
3. Trasmitted form
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what are the features of spongiform encephalitis
When alpha form of PrPc changes to PrPsc of beta pleated configuration, an abnormal prion protein is formed and it cant be degraded and it leads to accumilstion of this abnormal protein in the cytoplasm of the neuron which forms spongy spaces in the white mater of the brain
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how does CJD occur
it can occur with a retinal or human growth hormone tranplant from a pron infected donor
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what is the most common form of spongiform encephalitis
creutzfeldt-jakob disease
161
whar are the features of CJD
progressivedementia , rapid ataxia (due to cerebellum involvement) and startle myoclonus (exacerbated muscular movement with minimal stimulus)
162
what is variant CJD
It is a form of CJD that occurs with beef consumption of a cow with mad cow disease
163
what are the EEG features of CJD
periodic sharp waves
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What is familial fatal insomnia
It is a form of spongiform encehalitis characteried by severe insomnia and exacerbated startle response
