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PATH FINAL > Neuro PATH > Flashcards

Neuro PATH Flashcards

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1
Q

Chronic menigitis causes ?

A
  • TB
  • Cryptococcus
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2
Q

Pyogenic meningitis causes by age
Neonate-
Young adult-
Older pts-

A

Neonate
- E-coli
- Group B strep

Young adult
- Neisseria Meningitidis

Older patients
- Strep pneumonia, listeria, H-influenza

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3
Q

Aseptic meningitis causes

A
  • Enterovirus
  • Self limiting
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4
Q

CADASIL
- inheritance ?

A

AD

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5
Q

CADASIL
- affects small, medium or large vessels ?

A
  • Small vessel vasculopathy without stroke risk factors*
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6
Q

CADASIL
- mutation ?

A

NOTCH 3 gene on Chromosome 19

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7
Q

CADASIL small vessel morphology ?

A
  • Fibrotic thickening of basement membrane
  • No atherosclerosis or amyloid deposition
  • Diagnosed with gene testing*
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8
Q

CADASIL affects which part of brain ?

A
  • Basal ganglia (most common) >
  • Paramedian superior frontal lobe**
  • Parietal lobe
  • External capsule*
  • Anterior temporal lobe** (classic)
  • Cerebral cortex and U-fibers spared**
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9
Q

What’s unique about CADASIL infarcts ?

A
  • Infarcts cross arterial territory
  • Less frequently hemorrhage
  • Diagnosed with skin/muscle biopsy**
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10
Q

4 types of vascular malformations ?

A
  • AVM
  • Cavernous malformations
  • Capillary telangiectasia
  • Venous angiomas
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11
Q

of the 4 types of vascular malformations, which type are associated with hemorrhage and development of neurologic symptoms ?

A
  • AVM
  • Cavernous malformations
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12
Q

AVM associated with what mutation ?

A

KRAS oncogene mutation

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13
Q

AVM vs Cavernous malformation

A

AVM
- intervening gliotic tissue
- no intervening capillary
- often have prior hemorrhage
- most common MCA territory posterior* branch
- AD familial form are common (will have multiple)
- High flow (dAVF, pial AVF, then everything else low flow)

Cavernous malformation
- No intervening brain
- Most common in cerebellum > pons > subcortical
- Low flow
- Old hemorrhage as well

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14
Q

Capillary telangiectasia (hemangioma) RF ?

A
  • Radiation*
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15
Q

Sinus Pericranii

A
  • Anomalous communication between intracranial dural venous sinus, extracranial venous
    circulation.
  • Majority congenital.
  • Probable anomalous venous development during late embryogenesis.
  • Incomplete sutural fusion over prominent/abundant diploic or emissary veins.
  • In utero DVS thrombosis*.
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16
Q

Capillary telangectasia

A
  • Cluster of dilated, but histologically normal capillaries. - Thin-walled, endothelial-lined vascular channels, largest channels may represent draining veins.
  • Normal brain interspersed between dilated
    capillaries.
  • No surrounding gliosis, hemorrhage or calcification.
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17
Q

Developmental venous anomaly

A
  • Radially oriented dilated medullary veins.
  • Separated by normal brain.
  • Normal white matter (no gliosis).
  • 20% have mixed histology (CM most common), may hemorrhage.
  • Variant: “Angiographically occult” DVA
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18
Q

Summary of tumor grades

A

Grade 1
- well circumscribed

Grade 2
- Poorly circumscribed

Grade 3
- Mitotic cells and atypia

Grade 4
- Necrosis and angiogenesis

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19
Q

Diffuse Astrocytic and Oligodendroglial Tumours ?

A

IDH Mutant (1p/19q codeletion present)
- Oligodendroglioma (grade 2)

IDH Mutant (1p/19q codeletion absent)
- Diffuse astrocytoma (grade 2)
- Anaplastic astrocytoma (grade 3)

IDH Wild type
- GBM (grade 4)

H3K27M
- Diffuse midline glioma (grade 4)

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20
Q

Ependymal tumors ?

A
  • Myxopapillary ependymoma : grade 1
  • Ependymoma : grade 2
  • Anaplastic ependymoma : grade 3
  • Subependymoma : grade 1
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21
Q

Choroid Plexus tumours (all enhance a lot)

A
  • Choroid plexus papilloma (grade 1) - kids
  • Atypical choroid plexus papilloma (grade 2)
  • Choroid plexus carcinoma (grade 3) - adults
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22
Q

Neuronal and Mixed Neuronal-Glial Tumours

A

Grade I
- Dysembryoplastic neuroepithelial tumour (DNET)
- Ganglioglioma and gangliocytoma
- Desmoplastic infantile astrocytoma and ganglioglioma
- Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos)

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23
Q

Embryonal Tumours

A

All are WHO 4
- Medulloblastoma
- Atypical teratoid/rhabdoid tumour
- CNS neuroblastoma
- CNS ganglioneuroblastoma
- Medulloepithelioma

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24
Q

Arachnoidal Tumours

A

All Meningiomas basically

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25
Craniopharyngioma arises from?
- Rathke pouch remnant - Epithelial tumor* - Benign grade 1 tumor
26
Craniopharyngioma clinical presentation
- Growth retardation due to pituitary dysfunction (Growth hormone deficiency) - Headache and visual disturbance
27
Ependymoma epidemiology
- Bi-Modal - first peak 1-5 year old (A subtype) - second peak mid 30s (B subtype)
28
Ependymoma arises from ?
- Glial cells of ependymal lining of ventricles / ependymal rests
29
Ependymoma subtype and prognosis and location ?
60% infratentorial - 2 subtypes in the posterior fossa - Subtype A: Most common, predominately infants, poor prognosis - Subtype B: Older children and adults, better prognosis 10% spinal
30
Supratentorial Ependymoma location ?
- Extra-ventricular (most)* - if intra-ventricular will be in 3rd ventricle* supratentorial ependymoma, ZFTA (RELA) fusion-positive (POOR PROGNOSIS) supratentorial ependymoma, YAP1-MAMLD1 fusion
31
Ependymoma microscopic ?
- Perivascular Pseduorosettes**
32
Myxopapillary ependymoma seen, next step ?
- MRI whole spine - Benign but up to 50% do have leptomeningeal disease at time of diagnosis so must image neural axis - Despite this, excellent prognosis*
33
GBM associations ?
- NF-1 - Li-Fraumeni - Turcot - Ollier - Maffuci
34
Uncal herniation causes:
- Duret Hemorrhages from basilar perforating arteries - CN3 compressed between PCA and SCA causing ipsilateral pupil dilatation and ptosis - "Kernohan's Notch": Ipsilateral hemiparesis. Mid brain compresses tentorium, cerebral peduncle on contralateral.
35
HTN causes what vessel changes
Accelerated atherosclerosis - larger arteries Hyaline arteriosclerosis + Hyperplastic - small arteries - vulnerable to rupture Lipohyalinosis --> Charcot buchard microaneurysm - basal ganglia - site of rupture Fibrinoid necrosis - small vessels In amyloid angiopathy there is deposition of AB in the walls of medium to small meningeal and cortical vessels - PERIPHERAL rather than central*
36
Global hypoxic ischemia - most common site
- Diffuse cortical Grey Matter perirolandic cortex (affected first) - Watershed infarcts after hypotensive episodes* - Cerebellum (more common in older patients) - Diffuse white matter
37
Meningioma morphology types
Rounded "globose" - well defined dural base - CSF cleft between tumour and cortex En plaque - sheet like over dura - Hyperostosis* --> Psamomma bodies
38
Meningioma genetic mutations
- loss of chromosome 22** (most common) - NF2 mutation seen in sporadic (60%) and almost all high grade meningiomas
39
Define grade II and III Meningioma features
Grade II = < 20 mitoses / brain invasion / 3 atypical features* Grade III = overt malignancy cytology or  20 mitoses / 10 HPF*
40
Multiple Sclerosis epidemiology
- Female - Peak at 35
41
MS pathophysiology
- Autoimmune attack against myelin sheath - EBV associated
42
MS active vs chronic plaque
Active - Periventricular - Myelin breakdown with abundant macrophages - Lyphocytes as perivascular cuffs Chronic - Marked loss of Myelin and Oligodendrocytes - Astrogliosis** - No perivascular inflammation
43
MS Macroscopy
- Firmer than surrounding white matter due to sclerosis
44
Clinical presentation, Inclusions, Genetic for: Prion disease Alzheimer FTLD Parkinson PSP Corticobasal degeneration (CBD) MSA Huntington ALS SBMA
Prion - Rapid progerss dementia Alzheimer - AB (plaques) - tau (tangles) - Trisome 21, PS-1, PS-2, APP FTLD - Behavioural, Language change - Tau* - TDP-43* Parkinson - a-synuclein - tau - Lewy bodies PSP - Parkinson + abnormal eye movement - tau Corticobasal degeneration (CBD) - Parkinson + asymmetric movement disorder - tau MSA - Parkinson + cerebellar ataxia + autoimmune failure** - a-synuclein Huntington - AD - Hyperkinetic movement (Chorea) - HTT gene** ALS - weakness with upper and lower motor neuron signs - SOD1 - TDP43 - FUS SBMA - Lower motor neuron weakness and low androgen - Androgen receptor
45
Huntington - Inheritance - Gene - Image
- AD - HTT gene with tri-nucleotide repeat - loss of GABAergic neurones at BG - Atrophy caudate head and putamen + box-like frontal horn
46
MSA - inheritance - gene
- Sporadic - Parkinson + cerebellar ataxia + autoimmune failure** - a-synuclein
47
Oligodendroglioma macroscopic appearance
- Cystic degeneration, Calcification, Hemorrhage common - Remodelling of adjacent bone - Supratentorial (adult) - Posterior fossa and spinal cord (children) - Fried egg appearance of tumor cells - Chicken wire pattern of microvasculature
48
Pituitary adenoma epidemiology
- peak 35-60 - 15% found on autopsy* - most are non-functional microadenomas* --> Pituitary carcinoma is rare, <1% of all pituitary tumours. Mostly functional. Differ from adenomas due to the presence of distant metastases.
49
Least common pituitary hormonal abnormality
- TSH (1%)
50
Pituitary adenoma RF
- MEN-1
51
Pituitary adenoma aetiology
- due to Mutation of G-protein - Sporadic (most)
52
Pituitary adenoma macroscopic appearance
- Cyst, necrosis and hemorrhage in large adenomas - Calcifications in Prolactinomas*
53
HSV-1 affects who ?
- adults and young children - Accounts for 90% of Herpes simplex encephalitis
54
HSV-2 affects who ?
- neonates and immunocompromised
55
CMV, HIV, JCV afects who ?
- Immunocompromised
56
HSV encephalitis brain imaging feature ?
- Medial temporal lobe* - Spares basal ganglia - restricted diffusion (earliest sign, most sensitive) Limbic encephalitis looks similar**
57
Limbic encephalitis imaging feature ?
(if they say HSV titer is negative..then its this) - Paraneoplastic syndrome from small cell lung ca* - Looks like HSV encephalitis on imaging: - Medial temporal lobe* (same as HSV encephalitis) - Spares basal ganglia
58
Microscopic finding of HSV encephalitis
- Cowdry Bodies** - Perivascular inflammation
59
HIV encephalitis is a chronic or acute inflammation ?
- Chronic inflammation - Associated with microglial nodules** - Aseptic meningitis occur after seroconversion
60
PML preferentially infects what type of brain cells ?
- Oligodendrocytes* - leads to demyelination*
61
What are causes of Basal meningitis ?
- Cryptococcus - TB - Sarcoidosis
62
SAE association
- HTN (strong association)**
63
SAE imaging ?
(looks like CADASIL) - ONLY involves white matter - Centrum semiovale white matter* - Fronto-temporal horn (classic) - Spares U-fibers - Seen in >55yo
64
Alzheimer vs Vascular dementia vs Lewy body dementia - imaging features
Alzheimer - hippocampal, temporal lobe atrophy - FDG posterior temporoparietal uptake (ear muff) Vascular Dementia - Cortical infarcts - Lacunar infarcts - Generalized brain atrophy Lewy body dementia - Generalized atrophy - a-synuclein* - clinically like Parkinsons, but dementia comes before parkinsonism - Visual hallucinations**
65
MELAS features
(MELAS) - Mitochondria disorder - Lactic Acidosis - Seizures - Strokes (in non-vascular distribution) - normal WM
66
Adrenal Leukodystrophy (ALD) - Imaging ? - F or M more common
- Parietal occipital predominance - Crosses splenium of Corpus Callosum - MALE predominance - can Enhance, restrict
67
Metachromatic - Imaging ?
- Frontal lobe - Periventricular Tigroid - Spares U-fibers - Most common leukodystrophy*
68
Multifocal brain tumors from seeding ?
- Medulloblastoma - GBM - Oligodendroglioma - Ependymoma
69
Germinoma of pineal gland* - epidemiology
- Exclusively in BOYS - the MOST COMMON pineal gland tumor
70
Germinoma pineal gland labs ?
- high bHCG
71
Pineoblastoma resembles what other tumors ?
Small round blue cell tumor, pNET of the pineal gland. WHO grade-4 highly aggressive tumor. Resembles: - Medulloblastoma (pineoblastoma also highly aggressive and often seeds) - Retinoblastoma
72
Pineoblastoma epidemiology
- girls (c.f. germinoma is boys)
73
Pineoblastoma association ?
- Retinoblastoma (5% with retinoblastoma gets pineoblastoma)
74
Pineoblastoma labs
- Homer Wright rosettes and Flexner-Wintersteiner rosettes - synaptophysin: positive - chromogranin-A - neurone-specific enolase: positive
75
Pineocytoma epidemiology ?
- Adult - RARE in childhood (c.f. pineoblastoma and germinoma) - non-invasive, well circumscribed (c.f. pineoblastoma)
76
Most common neonatal brain infection ?
- CMV (3x more common than toxo) - Polymicrogyria (highest association amongst TORCH)
77
What trimesters does TORCH infection matter most ?
- only matter in the first two trimesters (doesn't cause much harm in third)
77
Calcification pattern of CMV vs Toxo
- CMV (periventricular) - Toxo (Basal ganglia with hydrocephalus)
78
Most common opportunistic infection in AIDS ?
Toxoplasmosis
79
CJD 3 types ?
- Sporadic (90%) - Variant "mad cow" (rare) - Familial (10%)
80
CJD imaging
Restricted diffusion most sensitive sign - Cortex (most common early site) - Basal ganglia - Progressive brain atrophy** Hockey stick (variant) - FLAIR dorsal medial thalamus Pulvinar sign (variant) - FLAIR pulvinar thalamic
81
Things that involve corpus callosum
* Tumor: Lymphoma (most classic), GBM * Post Treatment: Radiation Necrosis * Demyelination; Big Tumefactive MS Plaque, Marchiafava-Bignami * Infection: Progressive Multifocal Leukoencephalopathy (PML) * Trauma: Diffuse Axonal Injury * Weird Shit: transient lesions of the splenium of the corpus callosum - cytotoxic stuff often from seizure medications (oval spot in the splenium)
82
Bilateral thalamic stroke differentials
- Artery of Percheron (arise from PCA) - Tip of basilar occlusion - Bilateral internal cerebral vein thrombosis
83
Caudate infarct which artery affected
- Recurrent artery of Heubner stroke (from ACA, can be due to clipping of ACOM aneurysm)
84
Infarct in Anterior and Posterior circulation of the same hemisphere
- Fetal PCOM stroke
85
Fusiform aneurysm association and site
Association: - PAN - Syphilis - Marfans, Ehlers Danlos Location: - Posterior circulation
86
2 types of paraganglioma and locations
Parasympathetic paragangliomas - Carotid body paraganglioma (most common - 40-60%) - Jugular Paraganglioma (invades occipital and petrous apex) - Jugulotympanic paraganglioma (erodes middle ear) - Tympanic paraganglioma (doesnt erode middle ear) Sympathetic paraganglioma - Intra-adrenal: Pheochromocytoma (most common) - extra-adrenal: organ of Zuckerkandl, bladder, mediastinum
87
Jugular Paraganglioma - Epidemiology - Clinical presentation - Imaging
Epi - 40% hereditary* Presentation - Hoarseness from vagal nerve compression (most common) Imaging - Can erode middle ear, petrous apex, occipital bone - Salt and pepper - FDG-avid
88
Cholesterol Granuloma - imaging ?
- most common petroud apex lesion with smooth bony expansile lesion - T1 and T2 bright - T2 dark hemosiderin rim and faint peripheral enhancement - slow growing one can watch and wait, fast growing needs surgery
89
Cholesteatoma in petrous apex - congenital or acquired ? - how to tell from cholesterol granuloma
- these are Congenital (c.f. middle ear cholesteatoma is acquired) - slow growing with similar bony changes to Cholesterol Granuloma - T1 dark, T2 bright with restricted diffusion* (c.f. Cholesterol granuloma is T1, T2 bright, No restricted diffusion)
90
Malignant "Necrotizing" Otitis externa - Causing agent ? - Risk factor ?
Causative agent - Pseudomonas (always) Risk factor - Diabetics* (always) Swollen EAC soft tissue with small abscesses and bony erosion
91
phthisis bulbi causes
IMage: Shrunken, crenated appearance of the left globe with associated calcification Cause - chronic end-stage condition due to severe ocular insults - Traumatic injury - Infection, or radiation.
92
What is LEAST LIKELY to be associated with enlarged nerves of the cauda equina? Dejerine-Sottas Hereditary sensory motor neuropathy Guillain-Barre syndrome Metastatic adenocarcinoma
Guillain-Barre typically shows diffuse cauda equina enhancement, but does not enlarge the nerves. - Chronic demyelinating polyneuropathies such as - Dejerine-Sottas and HSMN will enlarge the nerves of the cauda equina. - Leptomeningeal metastases will also enlarge the roots in a nodular pattern.
93
Saccular aneurysm in how many % population ? how many % multiple
2% of populatoin - 90% are found near major branch points in anterior circulation. - 10% posterior Up to 30% multiple
94
Features of intracranial hypertension
- papilloedema: flattening of the posterior sclera (~80%) - Prominent subarachnoid space around the optic nerves - Tortuous optic nerves - Empty sella - Enlarged Meckel cave - Slit like ventricle
95
Optic neuritis bilateral
- NMO (devics)
96
ischemic optic neuropathy in Vasculitis can look like ?
Optic neuritis
97
Rectus pseudotumor or thyroid disease show tendons sparing
Pseudotumor - Involves unilateral rectus muscle most common - involves tendons - will see peri-orbital oedema (if dont seen, then dealing with real tumor)
98
JNA most common arterial supply
Internal maxillary artery of the external carotid artery
99
Craniocynosotosis associations
- Apert syndrome - Crouzon syndrome - Carpenter syndrome - Choanal atresia
100
What are some conditions that are associated with BBB disruption besides Infarct, Tumor, Infection ?
- Temporal lobe epilepsy (TLE) - Posterior reversible encephalopathy syndrome (PRES) - Hypoxia, ischaemia and infarction, tumour - Inflammatory conditions, e.g. meningitis, trauma, intracranial irradiation, multiple sclerosis, progressive multifocal leukoencephalopathy: the JC virus can cross the BBB.
101
Examples of Communicative hydrocephalus ?
- meningitis, haermorrhage, overproduction of CSF
102
Which vascular malformation has NO normal intervening brain tissue ?
Cavernous haemoangioma
103
Which vascular malformation angiographically occult ?
DVA

PATH FINAL (12 decks)

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