NEURO/SPINE Flashcards

(388 cards)

1
Q

Acute spinal cord ischaemia clinical

A

Anterior
- bilateral
- paralysis below level
- pain and temperature loss
- sparing of proprioception and vibration
- anterior horn; sensation preserved
- cervical/man in barrel; bilateral arms, normal face and legs

Posterior
- unilateral
- complete sensory loss at level
- proprioception and vibration loss below
- minimal motor sx

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2
Q

Acute spinal cord ischaemia imaging

A

MRI
- T2 in cord, pattern based on artery
- restricted diffusion

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3
Q

Spinal subarachnoid haemorrhage (usually due to avm) also known as

A

Coup de poignard of Michon (poignon = french for dagger)

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4
Q

Spinal epidural haemorrhage aetiology

A

spontaneous (most common)
trauma
iatrogenic
AVM
tumours
pregnancy

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5
Q

Spinal AVM clinical

A

25% spinal vascular malformations
Clinically variable;
- foix alajouanine syndrome (progressive myelopathy)
- coup de poignard of Michon (stab in back)

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6
Q

Spinal AVM imaging

A

T1
- signal voids
- dilated perimedullary vessels, indent/scallop cord
T2
- signal voids
- increased cord signal due to oedema or myelomalacia

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7
Q

Spinal AVM classification

A

Intramedullary or extramedullary

four angio subtypes
1: single coiled vessel
2: intramedullary glmus AVM
3: juvenile
4: intradural perimedullary

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8
Q

Spinal AVF clinical

A

70% of all spinal vascular malformations
Cause venous congestion, vague sx
- motor; gait disturb and reduced power
- paraesthesia
- radicular pain
- later; incontinence, ED

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9
Q

Spinal AVF imaging

A

MRI
- tortuous enlarged vessel flow voids
- spinal cord oedema; usually centromedullary and multisegmental
- low T2 rim and peripheral of oedema, deoxy blood product

DSA

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10
Q

Spinal AVF classification

A

1: dural AVF
2: intramedullary glomus AVM
3: intramedullary juvenile AVM
4; perimedullary AVF
5: extradural AVF

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11
Q

Spinal cord cavernoma clinical

A

peak during fourth decade
blood filled endothelial lined spaces lined by thickened hyalinsed walls that lack elastic fibres and smooth muscle

typically thoracic

variable clinical course

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12
Q

Spinal cord cavernoma imaging

A

CT occult
DSA occult

MRI
minimal cord expansion unless recent hamorrhage
heterogenous, popcorn
bloom on GE
minimal enhancement

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13
Q

Cerebrovascular malformations classification

A

High flow
- AVM
- DAVF
- proliferative angiopathy
- pial AVF

Low flow
- Cavernoma
- Capillary telengiectasia
- DVA
- venous varix
- sinus pericranii
- mixed

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14
Q

Cerebral cavernoma/cavernous venous malformation clinical/path

A

40-60
can be single or multiple (?familial ?radiation)

incidental or presenting with haemorrhage

path; mulberry like cluster of hyalinsed dilated thin walled capilllaries with surrounding haemosiderin. No normal intersecting brain. Occ assoc with a DVA

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15
Q

Cerebral cavernoma/cavernous venous malformation imaging

A

difficult on ct, do not enhance
can be hyperdense if large or speckle calc

MRI
popcorn, rim of signal loss
T1; variable, fluid fluid levels
T2; hypointense rim, FF lvels, variable signal
SWI; blooming
C+ no ehnacment

ddx
amyloid
hypertensice
dai
vasculitis
hamorrhagic mets
parry romberg
mets/primary
calcified lesions (neurocyterocosis)

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16
Q

Cerebral cavernoma/cavernous venous malformation classification

A

Zabramski
1; subacute harmorrhage
2; classic popcorn
3; chronic haemorrhage
4; multiple punctate microhaemorrhage

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17
Q

Parry Romberg syndrome

A

Progressive facial hemiatrophy. rare phakamatosis.

IPSILATERAL: leptomeningeal enhancement, paracnchymal atrophy, microhaemorrhage, clacifications, aneurysms

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18
Q

Perimesencephalic haemorrhage is

A

SAH around midbrain cisterns. 95% cause not found ?venous SAH. Better outcomes than if aneurysm, AVM AVF or trauma

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19
Q

Hypertensive microangiopathy is

A

sustained elevated blood pressure leading to lipohyalinosis and charcot boiuchard aneurysms prone to rupture

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20
Q

Hypertensive microangiopathy imaging

A

microhaemorrhages affecting the basal ganglia, pons and cerebellar hemispheres

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21
Q

Hypertensive microangiopathy ddx

A

cerebral amyloid (more peripheral)
multiple familial cavernous malformation syndrome
neurocystericosis
calcified treated mets

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22
Q

Cerebral AVM is

A

intracranial high flow vascular malformation composed of enlarged feeding arteries, nidus closely associated with parenchyma and draining veins

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23
Q

Cerebral AVM syndromes

A

typically single but when multiple ?syndromes;
Osler weber rendu HHT
Wyburn mason syndrome CAMS

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24
Q

Cerebral AVM imaging

A

CTA bag of worms
DSA
MRI

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25
Cerebral AVM grading
Perioperative morbidity. Spetzler Martin Size: <3, 3-6, >6 Eloquence of brain Veins: superficial or deep
26
Cerebrofacial arteriovenous metameric syndrome encompasses
Sturge Weber and Wyburn Mason syndrome. Intracranial and maxillofacial components required. Three types based on location 1: medial prosencephalic - nose and hypothalamus 2: lateral prosencephalic - occipital, chiasm, optic tract, thalamus, retina, maxiall 3: rhombencephalic = cerebrallum, pons, mandible
27
Wyburn Mason syndrome is
rare, non hereditary neurocutaneous disorder. Unilateral vascular malformations incolving the brain, orbits and facial structures. Features: facial vascular naevus visual pathway/orbital ACM intracranial AVM, commonly midbrain
28
Dural AVFs are
high flow vascular malformations that share AV shunting from dural vessels.
29
Dural AVF imaging
CTA: abnormal enlarged cortical veins, enlarged transosseous vessels, abnormal dural venous sinuses MRI: same. watch out forsuperiorly flowing venous blood as arterialised in the left jugular. DSA
30
Dural AVF classification
Borden or Cognard. Single most important feature is presence of retrograde leptomeningeal venous drainage. Borden 1: drain into meningeal veins, spinal epidural veins or dural venous sinus. normal antegrade flow. - cognard 1 and 2a 2: drain into meningeal veins, epidural veins or dural venous sinus. Retrograde flow into subarachnoid veins. - cognard 2b and 2a+b 3: direct drainage to subarachnoid veins or isolated segment of venous sinus - cognard 3, 4, 5
31
Capillary telangiectasia are
small low flow vascular lesions of the brain. dilated capillaries interspersed with normal brain parenchyma.
32
Capillary telangiectasia imaging
commonly in hte brainstem, especially the pons. usually solitary. can be assoc with OWR syndrome. usually only seen on MR T2 subtle high signal FLAIR subtle high signal SWI low signal C+ faint stippled enhancement
33
Developmental venous anomalies are
congenital malformations of veins which drian normal brain. Characterised by a caput medusae sign of veins draining into a single larger collecting vein (palm tree).
34
DVA imaging
commonly frontoparietal, cerebellar usually solitary except in blue rubber bleb naevus syndrome assoc with cavernomas CT; curvilinear enhancing structure. can have dystropic calcification. DSA caput medusae appearance MRI; postcon t1 and SWI
35
Cerebral varix is
an uncommon vascular malfomation, usually found in combination with another vascular malformation. Focal dilation of a single vein with no neural tissue or vessel anomalies
36
Sinus pericranii is
a cranial venous anomaly where there is abnormal communication between intracranial dural sinuses and extracranial venous structures usually via an emissary transosseous vein
37
Caroticocavernous fistulas are
abnormal communications between the carotid circulation and cavernous sinus. Can be direct or indirect.
38
Caroticocavernous fistula classification
Barrow classification A: direct B: indirect with ICA branches C: indirect with ECA branches D: B+C
39
Caroticocavernous fistula causes
Direct - trauma - aneurysm - CTD Indirect - probably secondary to cavernous thrombosis with revascularisation. - maybe pregnancy, surgery, sinusitis
40
Caroticocavernous fistula imaging
orbital congestion - proptosis and exopthalmos - retrobulbar fat stranding - enlarged EO muscles venous engorgement - enlarged SOV - bulging cavernous sinus - asymmetric enhancement Dehiscent ICA - snowman haemorrhage
41
Reversible cerebral vasoconstriction syndrome is
a group of conditions characterised by thunderclap headache and reversible vasoconstriction of the cerebral arteries
42
Reversible cerebral vasoconstriction syndrome associated conditions/risk factors
Pregnancy Drugs (lots) migraines hypercalc phaeos carcinoid bronchial idiopathic
43
Reversible cerebral vasoconstriction syndrome imaging
vascular narrowings and/or - convexity SAH - lobar hamorrhage - watershed infarct - vasogenic oedema smooth tapered narrowings invovling large to medium sized arteries followed by abnormally dilated segments. beaded or sausage shaped.
44
Reversible cerebral vasoconstriction syndrome differentials
SAH with vasospasm Vasculitis Arterial dissection untreated fungal/bacterial meningitis
45
Central nervous system vasculitis are
a heterogenous group of inflammatory diseases affecting the walls of blood vessels in the brain, cord and meninges. Can be primary or secondary Primary - confined to the CNS, primary angiitis Secondary - in the context of a systemic process
46
Central nervous system vasculitis imaging
variable and non specific. infarctions are most common, 53%. focal or multifocal segmental narrowing T2 FLAIR high intensity white matter (non spec). meningeal enhancement and ICH can be seen.
47
Cerebral amyloid angiopathy is
a disorder caused by the accumulation of cerebral amyloid in the tunica media and adventitia of leptomeningeal and cortical vessels causing fragility.
48
Cerebral amyloid angiopathy forms
Sproadic - incidental, prominent in autopsy of elderly and alzheimers patients - not assoc with systemic amyloidoses Familial - rare - usually autosomal dominant - lots of types includ; AB peptide with precursor protein APP, ACys peptide with precursor protein cystatin c Assoc; alzheimers, downs, chronic traumatic encephalopathy
49
Cerebral amyloid angiopathy presentation
Cortical vessels - lobar/cerebellar haemorrhage - cognitive impairment Leptomeningeal vessel involvement - convexity SAH Other - inflammatory type; rapid cognitive decline - amyloidoma
50
Cerebral amyloid angiopathy imaging
Haemorrhage - ICH; corticosubcortical, lobar. tends to spare BG and pons - microhaemorrhage; tends to spare BG and pons - convexity SAH - superficial siderosis Ischaemia - ischaemic leukoencephalopathy - microinfarcts and lobar lacunes Others - dilated perivascular spaces of the centrum semiovale - cortical atrophy
51
Cerebral amyloid angiopathy ddx
Hypertensive microangiopathy Multiple cavernoma Haemorrhage mets DAI Neurocystericosis Fat embolism syndrome Radiation induced vasculopathy
52
Cerebral amyloidoma is
a rare manifestation of cerebral amyloid deposition, appearing as a solid enhancing mass.
53
Cerebral amyloidoma imaging
Nodular solid masses with vivid contrast enhancement centred within the white matter and often abutting the ventricles. Mantle of vasogenic oedema. CT: hyperattenuating typically. enhancing. MR T1 and T2 variable Vivid enhnacement, can have peripheral radial enhancement microhaemorrhages
54
Sturge Weber syndrome is
a phakomatosis characterised by facial port wine stains and pial angiomas
55
Sturge Weber syndrome path
sporadic, no hereditary component. associated gene GNAQ 9q21. leptomeningeal haemangioma results in a vascular steal affecting the cortex and white matter producing ischaemia
56
Sturge Weber syndrome imaging
XR - gyral calcification CT - subcortical and cortical calc (tram track). calvarial and regional sinus enlargement. dyke davidoff masson appearance. orbital choroidal haemangiomas. ipsilateral cavernous and choroidal enlargement. MR T1: volume loss C+: leptomeningeal enhancement T2: low signal white matter subjacent SWI: calcification Spectro: decreased NAA
57
Sturge Weber syndrome ddx
AVM Torch infections neurocystericosis PHACE syndrome health cortical infarct radiotherapy gobbi syndrome
58
PHACE syndrome
aka cutaneous hamangioma vascular complex syndrome phakamatosis comprised of: P: posterior fossa malformation H: haemangiomas A: arterial anomalies C: coaractation of the aorta/cardiac anomalies E: eye anomalies
59
Gobbi syndrome aka CEC syndrome
coliac, epilepsy, cerebral calcifications bilateral occipital calc. no atrophy.
60
Superficial siderosis is
a rare condition which results from deposition of haemosiderin along the leptomeninges with eventual neurological dysfunction. classically presents with gradual bilateral sensorineural hearing loss, cerebellar dysfx and pyraimydal signs
61
Superficial siderosis imaging
MR pial and ependymal surfaces coated with low signal haemosiderin, particularly brainstem and cerebllum should image whole axis to look for causative lesion, if presnet
62
CADASIL is
cerebral autosomal dominant arteriopathy with subcortical infarcts and lekuencephalopathy AD microvasculopathy characterised by recurrent lacunar and subcortical white matter ischaemic strokes and vascular dementia in young and middle aged patients without known vascular risk factors.
63
CADASIL path
chromosome 19p13.12 NOTCH 3 gene small vessel and arteriole stenosis secondary to fibrotic thickneing of the basement membrane.
64
CADASIL imaging
CT non spec wm hypoattenuation MR confluent white matter hyperintensities lesions can be seen in pons, BG, thalami initial course anterior temporal lobe and external capsule. can be diffuse subcortical sparing occipital, orbitofrontal white matter, cortex and subcortical U fibres
65
CADASIL ddx
hypercoaguable state MELAS subcortical arteriosclerotic encephalopathy SAE Susac syndrome CNS vasculitis CARASIL
66
Susac syndrome quick hitter
retinocochleocerebral vasculopathy. middle aged women. triad of subacute encephalopathy, bilateral sensorineural hearing loss, branch retinal arterial occlusion. imaging - small rounded T2 hyperintense snowball lesion, with one in the CC. - CC predilection; central fibres of the body and splenium without abutting the callosal undersurface - leptomeningeal enhancement - punched out T1 holes when chronic retinal/vestibulocochlear involvement clinically
67
Remote cerebellar haemorrhage is
benign complication of craniotomy, spinal surgery, LP and shunt insertion. may be secondary to post surgical CSF hypovolaemia, tends to be self limiting. layering of blood over the superior folia - Zebra sign
68
Brain abscess imaging features
CT - outer hypo inner hyperdense rime (double rim) - uniform hyperdense ring - central low attenuation - vasogenic oedema - ventriculitis - obstructive hydro MRI T1 - low intensity centrally and peripherally - ring enhancement T2/FLAIR - central high intensity - peripheral high intensity vasogenic - capsule may have intermediate to low thin irm DWI - high DWI centrally SWI - low intensity rim; typically smooth and complete - dual rim sign Perfusion - CBV reduced in surrounding oedema Spectro - elevated peaks corresponding to lipids/lactate, succinate, acetate, amino acids
69
Brain abscess differentials
Mets or GBM - abscesses have smooth wall, satellite lesions,low intensity capsule, reduced perfusion, restriction Subacute iunfarct, haemorrhage, contusion Demyelinating lesion Radiation necrosis
70
Ventriculitis is
inflammation/infection of the ependymal lining of the ventricles. most often due to meningitis
71
Ventriculitis imaging
US - periventricular echogenicity and irregularity of the surface - choroid plexus irregularity - intraventricular debris CT - hyperdense layering material - hydrocephalus - periventricular low density - thin uniform ependymal enhancement post con MR - debris layering - intensely restricuted diffusion of the debris - periventricular oedema - ependymal enhancement
72
Subdural empyema is
intracranial infection with a suppurative collection b/w the dura and the arachnoid. Commonly seen as a complication of sinusitis, otitis, mastoiditis or surgical intervention.
73
Subdural empyema imaging
CT - resemble subdural haematomas - crescentic, although can seem biconvex - enhancing surrounding membrane MR - same as CT but better - better for complications like cerebritis, abscess and venous thrombosis.
74
Intracranial epidural abscess is
a pyogenic collection within the epidural space. sinusitis again most common. strep, h influ, staph.
75
Intracranial epidural abscess imaging
CT - less good than MR - similar to EDH - peripheral enhancement post con MR - T1 hyper - C+ peripheral enhancement - T2/FLAIR iso to hyper - PD same - DWI restricted
76
Child/Adult HSV encephalitis is
most common cause of fatal sporadic fulminant necrotising viral encephalitis. Typically HSV1.
77
Child/Adult HSV encephalitis imaging
General - typically bilateral, asymmetric - limbic, medial temporal, indular cortices and inferolateral frontal - BG spared CT - low density in thje anterior and medial temporal lobe and island of reil (insular cortex) - enhancement rare in first week, then patchy MR T1: usually low, unless haemorrhage C+: enhancement absent early, variable late (gyral, lepto, ring, diffuse) T2: hyperintensity of white matter and cortex DWI: restricted due to cytotoxic oedema, less than infarct SWI: blooming in haemorrhagic
78
Child/Adult HSV encephalitis differentials
Limbic encephalitis Gliomatosis Status epilepticus MCA infarct Trauma Ohter viral encephalitis. - EBV - HHV 6 - VZV - Flu a - Rabies Neurosyphlis
79
Neurocysticercosis is
a CNS infection caused by the pork tapeworm taenia solium which is endemic in low income countries
80
Neurocysticercosis stages
Escobars pathological stages 1. Vesicular - viable, no host reaction 2. Colloidal vesicular - cyst fluid becomes turbid. l;eaky oedema. 3. granular nodular - oedema decreases as cyst retracts, enhancement persists 4. nodular calcified - calcified cyst
81
Neurocysticercosis locations
SAS - lack a scolex Parenchyma, most common, GW junction Basal cisterns "grape like" lack scolex Ventricles, most common 4th Spinal
82
Neurocysticercosis imaging
Vesicular - cyst with a dot - CSF density/intensity - eccentric hyperintense scolex T1 - minimal enhancement/oedema Colloidal vesicular - cyst fluid turbid, T1 hyper, CT attenuating - oedema - peripheral enhancement - scolex initially, shrinks down Granular nodular - oedema decreases - retracts to small enhancing nodule - less enhancement, but persits Nodular calcified - end stage quiescent calcified nodule - no oedema or enhancement - T2 and * signal dropout
83
Neurotoxoplasmosis is
an opportunistic infection caused by toxoplasma gondii. Typically in patients with HIVAIDS and most common cause of abscess in these
84
Neurotoxoplasmosis imaging
Multiple lesions with a predilection for the BG, thalami and CM junction. CT - multiple hypodense regions - BG and CM junction - variable size - thin nodular or ring enhancement - dot like calcification in treated MR T1: iso to hypo T2: - variable intensity. hyperintense necrotising, iso organising abscess - concentric alternating zone of hypo hyper iso signal - perilesional oedema C+: ring or nodular enhancement Spectro: incr lactate and lipids. Red cho/cr/naa PET cold
85
Neurotoxoplasmosis vs CNS lymphoma
Lymphoma - subependymal - solitary - solid enhancement usually - haemorrhage uncommon - more diffusion retriction - increased choline - increased rCBV - high signal thallium Toxoplasmosis - BG and CMJ - multiple - ring or nodular enhancement - haemorrhage may be seen - more facilitated diffusion - decreased choline - decreased rCBV - low signal thallium
86
CMV encephalitis is
CNS infection, almost always profoundly immunocompromised. Affects entire neural axis.
87
CMV encephalitis imaging
meningoencephalitis and ventriculitis/ependymitis MRI - non spec increased WM T2 signal, periventricular - no enhancement, unless ventriculitis - no mass affect
88
CMV encephalitis ddx
HIV encephalitis PML CNS lymphoma
89
Neurotoxoplasmosis ddx
CNS lymphoma Cerebral mets Other infection- TB, crypto, bacterial, neurocysticercosis
90
CNS cryptococcosis is
most common fungal infection and second most common opportunistic infection of the CNS. Predominantly AIDS, but if competent than history of birds. Can be meningeal, parenchymal or perivascular space predominant
91
CNS cryptococcosis imaging
range of appearance influenced by degree of immunocompromise and therapy. more effective therapy has enhancement. range includes - hydrocephalus - dilated perivascular spaces coalescing - leptomeningeal/pachymeningeal enhancement - cryptococcomas - miliary nodules - choroid plexus plexitis perivascular/BG pattern is common, but also white matter, brainstem, cerebellum MRI Meningeal - leptomeningeal/pachymeningeal enhancement - FLAIR C+ enhancement Cryptococcomas - T1 low - T2/FLAIR high - C+ variable - DWI variable Perivascular - T1 low to intermediate - T2 high - FLAIR variable - DWI variable
92
CNS aspergillosis is
one of the most common fungal CNS infections, resulting from angioinvasive infection from aspergillus spp. Typically AIDS, steroid use, neutropaenia or GVHD
93
CNS aspergillosis imaging
Variable but three main patterns; - brain abscess; often multiple, rando distribution - cerebral infarctions, more likely perforators - invasive paranasal rhinosinusitis MR Abscess - often multiple, rando distribution - ring enhancing with high DWI - DWI can be variable - may also have peripheral low T2 signal, low GRE (perilesional hamorrhage or iron in fungi) without inner high signal rim (absent dual rim) Infarction - multiple, random - haemorrhage/mycotic aneurysms can be present Rhinosinusitis - paranasal disease, invasive features - OM - dural enhancement and subdural empyema Other - can have a granulomatous mass lesion - hypo to iso T1 - hypo T2
94
Progressive multifocal leukoencephalopathy is
a demyelinating disease resulting from reactivation fo the JC virus infecting oligodendrocytes in patients with compromised immune systems. Typically AIDS CD4 50-100. Non HIV - post transplant - leukaemia - solid malig - isolated CD4 lymphocytopaenia - MABs Can also be encountered in immune reconstitution inflammatory syndrome.
95
Progressive multifocal leukoencephalopathy imaging
CT - asymm focal zones of low attenuation involving periventricular and subcortical white matter. HIV more symmetrical MR - Typically multifocal, asymmetric, periventricular, subcortical - little to no mass effect or enhancement - subcortical U fibres - parieto occipital predilection T1 - hypo T2 - hyper - multiple punctate T2 lesions (milky way sign) - barbell sign; PO region crossing splenium C+ - typically no enhancement - can be seen in IRIS, natalizumab DWI - patchy leading edge restriction Spectro - reduced NAA, lactate presence, incr choline/lipids MRP - increased leading edge
96
Progressive multifocal leukoencephalopathy ddx
MS (more well defined, periventricular) HIV encephalopathy (more diffuse, atrophic, symmetric, no U fibres) PRES (different hx, grey and white matter) ADEM (hx, grey and white matter, enhances)
97
Progressive multifocal leukoencephalopathy signs
milky way - punctate T2 foci around primary lesion barbell - extending across splenium
98
Creutzfeldt Jakob disease is
a transmissable spongiform encephalopathy resulting in rapidly progressive dementia and death. Sporadic, but familial and acquired occasionally. Typically hyperintense DWI in cerebral grey matter - cortex, striatum, thalamus.
99
Creutzfeldt Jakob disease types
Sporadic Variant Familial Iatrogenic
100
Creutzfeldt Jakob disease variants
Sporadic - rapidly progressive dementia and other features of neuropsychiatic decline Heidenhain - isolated visual Brownell oppenheimer - cerebellar ataxia Vairant - psychiatric symptoms
101
Creutzfeldt Jakob disease markers
EEG findings S100 CSF 14 3 3 protein CSF RT QulC seeding assay
102
Creutzfeldt Jakob disease affected regions (good luck idiot)
Sporadic distribution: cortex and deep grey matter. from most to least; - insula, cingulate, superior frontal - striatum (caudate and putamen) - precuneus, cuneus, paracentral lobule, medial frontal, occipital, angular/supramarginal, superior parietal, inferior frontal - thalamus - post central, pre centra, medial and superior temporal Usually bilateral. Heidenhain - parietooccipital Bronwell Oppenheimer - cerebellum, BG Variant - hockey stuck/pulvinar (although more commonly seen in sporadic due to overall greater prevalence)
103
Creutzfeldt Jakob disease imaging and ddx
MRI DWI: hyperintensity ADC: variable, depends on timging FLAIR/T2: hyperintense, more subtle T1: high signal globus pallidus C+ no enhancement DDX - autoimmune encephalitis - hypoxic injury - osmotic demyelination - hepatic encephalopathy - hypoglycaemic encephalopathy - mitochondrial disease
104
Autoimmune encephalitis is
antibody mediate brain inflammatory process, typically involving the limbic system. Broadly divided into - paraneoplastic - non neoplastic
105
Autoimmune encephalitis causes
tumouts - small cell - testicular - thymic - breast - ovarian - haemotological - GI - neuroblastoma non paraneoplastic - VGKC antibody - anti NMDA receptor - systemic autoimmune conditions
106
Autoimmune encephalitis imaging
most common location limbic system cortical thickening and increased T2/FLAIR bilateral is most common basal ganglia are frequently involved patchy enhancement can be seen restriction and haemorrhage uncommon
107
Primary ameorbic meningoencephalitis is
rare, usually fatal CNS infection of naegleria fowleria. non specific imaging features in keeping with a haemorrhagic meningoencephalitis
108
Rocky mountain spotted fever is
an infectious disease caused by Rickettsia rickettsii transmitted by tick bites
109
Lyme disease is
caused by borrelia burgdorferi. non specific features including periventricular subcortical T2 hyperintensity, nerve root enhancement (esp facial), meningeal enhancement
110
Immune reconstitution inflammatory syndrome is
paradoxical deterioration following abrupt improvement in immune function. Classiccally seen in HIV patients on ART, but also mabs in MS
111
Immune reconstitution inflammatory syndrome imaging
underlying pathogen or may mimic woresening of underlying condition or be atrypical. typically enhancing masses gain mass effect rapidly. enhancement is variable and may be bizarre mycobacterial TB - new lns may be necrotic, pulmonary nodules or new abscesses PML - new enhancement and mass effect in exisiting white matter lesions pulmonary kaposi sarcoma - similar but increasing in extent
112
HHV 6 encephalitis is
rare CNS infection in immunosuppressed patients, esp following haematopoietic cell trnasplant who develop limbic encephalitis syndrome with MRI signal intensity abnormalities of the medial temporal lobe
113
Subacute sclerosing panencephalitis is
also known as Dawsons disease. rare, chronic progressive fatal encephalitis. persistent measles infection immune resistant.
114
Subacute sclerosing panencephalitis imaging
T2/FLAIR: high in parietal and temporal lobes C+ enhancement early lateral more extensive, atrophic
115
Rasmussen encephalitis is
a chronic inflammatory disease of unknown origin affecting one hemisphere
116
Rasmussen encephalitis imaging and ddx
MRI unilateral cortical atrophy with exvacuo dilatation starting in caudate T2 hyperintense DWI restricted can be seen no enhancement\ DDX Dyke Davidoff Massson Sturge Weber unilateral megaencephaly hemiconvulsion hemiplegia epilepsy syndrome
117
CLIPPERS is
chronic lymphocytic inflammation with pontine perivascular enhancement response to steroids predilection for the pons. characteristic curvilinear regions of enhancement.
118
Rasmussen encephalitis imaging
multiple punctate patchy and linear regions of contrast enhancement confined to the pons. can also be in the cerebellar peduncles, cerebellar hemispheres and cervical cord/ reatively little oedema. SWI signal loss.
119
Multiple sclerosis classification
Variants: Classic (Charcot) Tumefactive Marburg (acute malignant) Schilder type Balo concentric sclerosis Patterns: Relapsing remirrting Secondary progressive Primary progressive Progressive with relapses Benign
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Multiple sclerosis imaging
Plaques in the CNS system, typically ovoid and perivenular MRI T1 - iso to hypointense - callososeptal interface many small lesions (venus necklace) T2 - hyper - acute have oedema SWI - central vein FLAIR - hyperintense - epndymal dot dash sign - perpindicular to lat ventricles (dawsons fingers) C+ - active enhance - usually incomplete, open ring of enhancement at the periphery DWI - active may be high or low ADC - typically open ring
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Multiple sclerosis complications
PML PML IRIS CNS lymphoma
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Multiple sclerosis ddx
Intracranial - CNS fungal infection - mucopolysaccharidosis - Marchiafava Bignami disease - Susac syndrome - antiphospholipid syndrome - CLIPPERS Spine - Transverse myelitis - infection - tumours
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Tumefactive MS is
an MS variant where patients develop large aggressive demyelinating lesions
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Marburg variant MS is
Extensive and fulminant acute demyelination. Typically youynger patients, die within a year. Extensive confluent areas of tumefactive demyelination are seen with mass effect, defined rings and incomplete rim enhancement.
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Schilder type MS is
an extremely rare, progressive demyelinating process that begins in childhood.
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Balo concentric sclerosis is
a rare and severe monophasic demyelinating disease. Considered an MS variant. Rounded lesion with alternating layers of high and low signal intensity "bullseye" "onion bulb".
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Balo concentric sclerosis imaging
Alternating bands of demyelinated and myelinated white matter, seen as concentric rings. T1: irregular, concentric rings of iso to low signal T2: irregular concentric rings of iso to hyper C+ usually periphal enhnacement in the area of active demyelination DWI: some restriction in outer ring
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Neuromyelitis optica (NMO) and NMO spectrum disorder (NMOSD) is
severe demyelinating disease caused by autoantibody to aquaporin 4 water channel. classic triad of optic neuritis, longitudinally extensive myelitis and postive anti AQP4. Previously known as Devic disease.
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Neuromyelitis optica imaging
Orbits - swollen/hyperintense/enhancing CN2 - bilateral involvement, to optic chiasm - atrophy in chronic Brain - periventricular (no dawsons fingers) - periaqueductal GM - hypothalamus/medial thalamus - dorsal pons/medulla - CC - deep punctate white matter lesions - corticospinal trtact invovlement - larger >3cm hemispheric lesions (radially orientated, little mass effect) - fever juxtacortical lesions - larger, more confluent, more CC Spine - at least three levels (long extensive) - ring enhancing, or patchy enhancement - prefers central cord
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Neuromyelitis optica ddx
cerebral - MS - Susac - Neuro Behcet - Primary angiitis of the CNS - ADEM - ALS Optic neuritis Spinal - NMO - MS (confluent lesions) - anti MOG encepha - Systemic (neurosarcoid, sjogrens, SLE, behcet) - other causes transverse myelitis - vascular (AVF, infarct)
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Acute disseminated encephalomyelitis ADEM is
a monophasic acute inflammation and demyelination of white matter following viral infection or vaccination,
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ADEM imaging
Vary from small punctate to tumefactive Callososeptal interface involvment is less typical Bilateral but asymmetrical Can sometimes involve subcortical grey matter and brainstem unlike MS
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Acute haemorrhagic leukoencephalitis is
also known as Hurst or Weston Hurst syndrome. Very rare form of demyelinating disease. Acute rapidly progressive fulminant inflammation of the white matter. Typically young adults (ADEM kids). Large tumefactive lesions, sparing the cortex. Assoc punctate haemorrhage. POssible BG/thalami involvement.
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Acute necrotising encephalopathy is
a rare encephalopathy with bilateral brain lesions, involving the thalami, putamina, internal/external capsule, cerebellar white matter and tegmentum. Typicall bilateral and symmetrical thalamic involvement. Canhave restricted diff, haemorrhage, cavitation and enhancement.
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Vascular dementia is
second most common after alzheimers. seen in atherosclerosis and htn. MRI more sensitive to white matter change, changes of amyloid and chronic htn encephalopathy. patterns of vascular damage - binswanger (small vessel) - cerebral infarct - lacunar infarctr - hjaemorrhage
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Binswanger disease is
a slowly progressive non hereidtary white matter vascular dementia. subcortical and periventricular hjypertinscve T2/FLAIR usually small lesions moderate diffuse atrophy ddx CADASIL (hereditary, genetic, anterior temporal and superior frontal) CNS vasculitis
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Alzheimers imaging
most common degenerative disease. most common cerebral amyloid deposition disease. characteristic volume loss 1. mesial temporal lobe - particularly hippocampus, entorhinal cortex and perirhinal cortex 2. temporoparietal cortical atrophy NM SPECT/PET to see hypoperfusion/metabolism om a biparietal bitemporal distribution with amyloid/tau agents in the grey matter and medial temporal lobes respectively
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PRES three main patterns
Holohemispheric at watershed zones Superior frontal sulcus Parieto occipital dominance
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PRES is
an acute hypertensive encephalopathy secondary to inability of the posterior circulation to autoregulate in response to bp changes. Hyperperfusion with disruption in the BBB resulting in vasogenic oedema.
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PRES aetiology
Severe HTN Haemolytic uraemic syndrome TTP SLE Drugs BM t/p
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PRES imaging
Bilateral vasogenic oedema, typically parietooccipital Can also be non posterior; frontal, inferior temporal, cerebellar, central. Can be unilateral. MRI T1 hypo C+ patchy, variable T2 hyper DWI vaRIABLE SWI may have haemorrhage
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PRES differential
inflammatory cerebral amyloid angiopathy PML hypoglycaemia posterior infarct hypertensive brainstem encephalopathy gliomatosis
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Hypertensive brainstem encephalopathy is
severe htn, brainstem vasogenic oedema and various neurology. Can be isolated or with PRES.
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Hypertensive brainstem encephalopathy imaging and differentials
CT: diffuse hypoattenuation, mostly at the pons MRI: increased FLAIR/T2 brainstem, lack of diffusion restriction ddx - PRES - osmotic demyelination - infarct - neoplasia
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Hypoglycaemic encephalopathy is
brain injury from severe/prolonged hypoglycaemia. Typically affects posterior internal capsule, cerebral cortex, hippocampus and basal ganglia.
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Hypoglycaemic encephalopathy imaging
Typically bilateral characteristically - posterior limb internal capsule - cerebral cortex (PO and insula) - hippocampus and basal ganglia - cerebellum, brainstem, thalami (spared in adults, present in neonates) - splenium of CC (boomerang)
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Osmotic demyelination syndrome is
seen in the setting of osmotic changes typically with rapid correction hyponatremia
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Osmotic demyelination syndrome imaging
T2 bright DWI bright T1 hypo piglet sign trident sign
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Status epilepticus imaging
Increased T2/FLAIR, increased DWI. Variable distribution - cerebral cortex and subcorticla WM - hippocampi and mesial temporal lobes - thalamus, pulvinar region - cerebellum
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Carbon monoxide poisoning imaging
Bilateral with globus pallidus most commonly affected CT GP low attenuation Can have diffuse white matter hypoattenuation MRI T1 low, can have areas of haemorrhage T2/FLAIR high C+ can have peripheral enhancement DWI: increased restriction
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Carbon monoxide differentials
Mitochondrial encephalopathies - leigh - kearns sayre Other toxic encephalopathies - cyanide - methanol - organophsophate Other causes of anoxia Metabolic - Wilsons Prion - CJD
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Hepatic encephalopathy imaging
T2/FLAIR - mild; symmetric high in insula, thalamus, posterior internal capsule, cingulate - severe; diffuse cortical. perirolandic/occpital spared. DWI - similar to t2 SWI - 50% have microhaemorrhage
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Fahr syndrome is
abnormal vascular calcium deposition in the basal ganglia, cerebellar dentate nuclei, white matter, with subsequent atrophgy. Can be primary or secondary.
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Fahr syndrome secondary causes
Endocrinopathies - hypoparathyroidism/hyper Vasculitis Mitochondrial disorders Infection - brucellosis - EBV -HIV Inherited - neuroferritonpathy - other conditions Radiation Chemo CO poisoning
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Fahr disease imaging
Symmetrical calcification of the causate, lentiform, thalamus and dentate nuclei Globus first Subcortical white matter later
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Non ketotic hyperglycaemic hemichorea is
A rare complication of non ketotic hyperglycaemia, causing hemichorea hemiballismus syndrome
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Non ketotic hyperglycaemic hemichorea imaging
typically unilateral, contralateral to symptoms. causes abnormality in the striatum (caudateand putamen) T1 hyper T2 variable, generally low SWi increased DWI high
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Non ketotic hyperglycaemic hemichorea differential
Other BG T1 high - calcium - wilson and non wilson hepatic - NHH - haemorrhage - japanese encephalitis
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Wernickes encephalopathy is
a form of thiamine deficiency typically seen in alcoholics. Can also be seen in starvation, TPN, post bariatic surg, hyperemesis
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Wernickes encephalopathy imaging
T2/FLAIR: symetrically increased in - mam bodies - dorsomedial thalami - tectal plate - periaqueductal grey matter - peri third ventricle C+: enhancey same, particularly mam bodies DWI: same but retricted
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Wernickes encephalopathy ddx
Leighs disease (no mamm bodies) Metronidazole induced encephalopathy (dentate, splenium, cranial nerve nuclei) Artery of percheron/central venous infarcts
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Vitamin B12 deficiency imaging
subacute combined degeneration of the cord - bilateral symmetrical high signal in the dorsal columns - iinverted V sign - start upper thoracic, ascend or descend - can get lateral tract involvement
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Uraemic encephalopathy is
an acquired toxic syndrome in patients with chronic kidney disease.
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Uraemic encephalopathy imaging
Cytotoxic oedema in the subcortical grey and white matter, midbrain and mesial temporal lobes. minimal nehnacement. variable restriction. lentiform fork sign; white matter surrounding the basal ganglia hypoertintense
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Wilson disease CNS imaging
Frequently affects BG (especially putamen), midbrain, pons, thalamus. Bilateral and symmetric. CT: atrophy MRI T2 high signal; putamen, tegmentum midbrain (giant panda), pons (panda cub/double panda). T1: hypo intense, contrast to acquired non wilson hepatocerebral degeneration atrtreibuted to manganese deposition.
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Storage disorders classification
Share the accumulation of a metbolite within various cells due to dysfunction of specific enzymes or transport proteins, resulting in cellular or organ dysfunction. Majority are autosomal recessive. Broadly divided to the type of of metabolic defect. Carbohydrate metabolism - carbo intolerance - glycogen storage disorders Lysosomal storage disorders - leukodystrokphies - mucopolysaccharidoses - glycogenoses, mucoliposes, sphingolipodoses - gaucher most common Mitochondrial Peroxisomal Protein Purine/pyrimidine
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Glycogen storage disease are
characterised by a defect in synthesis, metabolism or storage of glycogen many types, 1-12 1: von gierke 2: pompe Can be grouped into myopathic, hepatic and miscellaneous forms depending on predominant organ. Hepatomegaly most common.
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Mucopolysaccharidoses are
a group of hereditary disorders, type of lysosomal storage disorder. Excessive accumulation of mucopolysaccharides secondary to deficiencies in specific enzymes responsible for degradation of mucopolysaccharides (or glycosaminoglycans). Types 1-9 1H Hurler syndrome 2: Hunter syndrome 4: Morquio
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Whatre the common leukodystrophies
Alexander disease Canavan disease Krabbe disease Metachromatic leukodystrophy X linked adrenoleukodystrophy Megalencephalic leukoencephalopathy with subcortical cysts Pelizaeus Merzbacher Vanishing white matter disease
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Alexander disease is
a rare, fatal leukodystrophy. Usually infantile but can be juvenile/adult.
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Alexander disease imaging
Frontal region, extends posteriorly Subcortical U spared intially, involved late End stage cystic leukomalacia Caudate - globus - thalamus - brainstem Periventricular rim
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Canavan disease is
a leukodystrophy characterised by megaencephaly, severe deficits and blindness. Deficiency in enzyme for NAA, leading to accumulation in the brain, CSF, plasma and urine.
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Canavan imaging
Megaencephaly Diffuse, bilateral Involves subcortical U fibres mainly subcortical white matter, extending periventricular Involves the globi pallidi and tyhalami Generally spares the CC, caudate, putamen and internal capsule Markedly elevated NAA on spectro
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Krabbe disease is
an autosomal recessive lysosomal storage disorder resulting in damage to cells involved in myelin turnover. Typically infantile and rapidly progressive but can be late onset.
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Krabbe disease imaging
Changes involve the corticospinal tracts. CT Hyperdense areas involving the thalami, cerebellum, caudate, posterior internal capsule and brainstem Lateral hypoattenuating centrum semiovale. Evetually, atrophy MRI T2: high signal periventricular white matter and deep grey matter. Subcortical U fibre may be spared until late. Tigroid pattern can be seen. C+: no enhancement Enchnacing/enlarging peripheral nerves can be seen
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Metachromatic leukodystrophy is
the most common hereditary leukodystrophy and a lysosomal storage disorder. Characteristic imaging features invluding periatrial and frontal horn wm and periventricular perivenular sparing "tigroid"
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Metachromatic leukodystrophy imaging
Bilateral symmetrical confluent areas of periventricular deep white matter signal change. Typically around the atria and frontal horns, sparing the sub cortical u fibres. Butterfly pattern. Tigroid pattern on axial or leopard on saggital representing sparing along the venules.
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Tigroid pattern
Krabbe Metachromatic Pelizaeus Merzbacher
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X linked adrenoleukodystrophy is
an inherited metabolic peroxisomal disorder characterised by oxidation of very long fatty acids resulting in severe inflammatory demyelination of the periventricular deep white matter. Posterior predominant change and early involvement of the splenium.
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X linked adrenoleukodystrophy imaging
Location - five distinct patterns 1. PO deep white matter and splenium 2. frontal lobe or genu CC 3. frontopontine or corticospinal projection fibres 4. cerebellar white matter 5. combined PO and frontal Tends to be subcortical u fibre sparing Three zones: central gliosis, intermediate inflammation, peripher active demyelination T1: central hypointense C+ peripherally enhancing T2: centra hyper, intermediate iso, peripheral hyper
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Megalencephalic leukoencephalopathy with subcortical cysts is
a rare inherited autosomal recessive disease characterised by diffuse subcortical leukoencephalopathy with cystic white matter degeneration.
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Megalencephalic leukoencephalopathy with subcortical cysts imaging
Megalencephaly Diffuse bilateral and symmetric T2 hyper and T1 hypo of the cerebral white matter Have have restriction Subcortical U fibres invovled Sparing of deep and cerebellar Bilateral subcortical cysts anterior temporal and frontoparietal Eventually atrophyu
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Pelizaeus Merzbacher disease is
an x linked leukodystrophy with arrest of myeline development
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Pelizaeus Merzbacher imaging
T1 lack of myelination, low signal regions involving internal capsule, corona radiate and optic radiation T2 diffuse or patchy, tigroid, atrophy
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Vanishing white matter disease is
a rare genetic leukoencephalopathy, extensive white matter involvement with cavitatory changes.
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Vanishing white matter disease imaging
Diffuse white matter T2 high signal, eventually replaced by CSF intensity fluid (suppresses on FLAIR).
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Primary mitochondrial disorders are
a clinically heterogenous group of conditions caused by vairants in mitochondrial DNA or nuclear DNA Include: Leigh syndrome Kearns Sayre syndrome MELAS MERFF Gnerally have bilateral deep grey matter involvement and peripheral white matter delayed myelination in children. Elevated lactate on MRS.
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Leigh disease imaging
T2 high signal in - brainstem - periaqueductal gm - putamen - remainder of the corpus striatum T1 low DWI can restric acutely ddx Wernicke (involves mamillary, enhances, hamoerrhagic change) Other mitochondrial disorders Acute necrotising encephalitis of childhood
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Kearns Sayre syndrome imaging
Basal ganglia siderocalcific deposits and subcortical calcifications
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MELAS is
mitochondrial encephalomyopathy with lactic acidosis and stroke like episodes. Mitochondrial d/o. Manifests as multifocal stroke like cortical lesions in different stages "shifting spread", crossing vascular territories. Predilection for posterior parietal and occipital.
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MELAS imaging
CT multiple infarcts, multiple territories BG calc MR Acute and chronic infarcts elevated lactate on spectro parieto occipital and parieto temporal most common
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Marchiafava Bignami disease is
a rare CNS disorder seen in the context of alcoholism and malnutrition. Classically involved necrosis and demyelination of the corpus callosum.
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Marchiafava Bignami disease imaging and ddx
Typically begins in the corpus callosum and later involves the genu and splenium. Classically involves the central layers with sparing of the dorsal/ventral extremes "sandwich sign" on saggital CT hypoattenuating MR T1 hypointense acute T2 hyperintense acute, hypointense subacute (haemosidering), ears of a lynx sign (frontal horns) ddx - MS - transient lesions of the splenium of the CC - DAI
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Meningiomas WHO 2021 subtypes (there are 15 lol)
angiomatous atypical anaplastic chordoid clear cell fibrous lymphoplasmacytic rich meinigothelial metaplastic microcystic papillary psammomatous rhabdoid secretory transitional
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Meningioma imaging
CT can be slightly hyperattenuating to brain can have calc majority have homogenous enhancement can be heterogenous hyperostosis, typically BOS reactive vs direct invasion vs primary intraosseous MRI variable intensity, typically iso T1 with homogenous enhancement post con iso to gm t2 can restrict signs - csf cleft - dural tail -suinburt or spoke wheel vessels - white matter buckling - arterial narrowing variable vasogenic oedema
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Meningioma differentials
Dural masses - solitary fibrous tumours of dura - dural mets location - CP angle; schwannoma - pituitary; macroadenoma, cranipharyngioma - bos; pachymeningitis, EMH, chondrosarc, chordoma
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Burnt out meningiomas are
completely calcified.ossified. typically indolent. likely to be psammomatous meningioma.
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Cystic meningioma is
applied to both meningiomas with intratumoural degenerative cyst formation as well as those with peritumoural arachnoid cysts or reactive intraparenchymal cysts. Five subtypes - intratumoural, central - intratumoural, peripheral - cyst wall nest of tumour cells - cysts in adjacent brain - adjacent arachnoid cysts
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Intraosseous meningioma imaging
2/3 blastic 1/3 lytic CT - commoner sclerotic type shows diffuse sclerosis with bony expansion MR - T1 iso, expanded bony component hypo - T2 iso to grey matter - uniform enhancement
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Intraosseous meningioma ddx
BLASTIC Pagets FD Osteoma Osteosarcoma Mets LYTIC plasmacytoma lytic
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En plaque meningioma
diffuse and extensive dural invovlement usually with extracranial extension
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Intraventricular meningioma imaging and ddx
Location - mostly trigone - 15% third ventricle - 5% fourth ventricle Iso to grey matter pre and homogenous contrast enhancement post. ddx - glial tumour; ependymoma, astrocytoma - choroid plexus mets; RCC, melanoma - choroid plexus papilloma - CNS lymphoma - central neurocytoma
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Optic nerve sheath meningioma imaging
Morphology - tubular - exophytic - fusiform CT - iso to nerve - enhancing relative to nerve "TRAM TRACK" sign - coronal "DOUGHNUT" MR - T1 hypo to nerve - T2 hyper to nerve - homogenous enhancement ddx - optic nerve flioma - orbital pseudotumour - orbital lymphoma - orbital mets - sarcoid
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Primary CNS lymphoma subtypes
immunocompetent immunodeficient assocaited - aids related - ebv positive - lymphmatoid granulamatosis - primary CNS post transplant lymphoproliferative intravascular MALT of dura
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Primary CNS lymphoma imaging
typically ct hyper, avidly enhancing, t1 hypo, t2 iso to hypo with restricted diffusion diffusion. subependymal and crossing the CC - predominantly seen in untreated, non immunocompromised immunocompromised; more heterogeous, central non enhancement and haemorrhage. may have peripheral rim enhancement. typically supratentorial, solitary or multiple usually contact subarachnoid/ependymal surfaces can cross the cc pronounced enhancement limited mass effet and vasogenic oedema NOTCH sign deep depression at the margin
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Primary CNS lymphoma ddx
Secondary CNS lymphoma Cerebral toxoplasmosis - toxo no subependymal spread - more likely BG, CMJ - toxo not pet/thallium avid butterfly glioma - more haemorrhage tumefactive MS/ADEM cerebral abscess - central restriction, thinner peripheral enhancement neurosarcoid
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Intravascular lymphoma is
a rare variant of extranodal dlbcl that affects small and medium sized vessels
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Intravascular lymphoma imaging
T2/FLAIR high signal in a dynamic pattern DWI retriction in a dynamic pattern C+ mass like enhancement in proximity to t2 or DWI changes
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MALT lymphoma dura imaging
share imaging features with other small round blue cell tumours and other dural masses. extra axial lobulated mass, either solitary or multiple ct slightly hyperdense enhance mri T1 iso to grey C+ homogeneous enhancement T2 iso to gry DWI retricts
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MALT lymphoma dura ddx
meningioma mets Erdheim chest Rosai dorfman
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Adamantinomatous craniopharyngioma imaging
Primarily suprasellar Intrasellar component in 25% Lobulated contour Calcification is common 90% CT large dominant cysts solid component enhances calcification stypically stippled and peripheral MRI Cysts - T1 iso to hyper - T2 variable Solid - enhances - variable T2 Calcification
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Adamantinomatous craniopharyngioma ddx
Rathke cleft cyst - no solid/enhancing - calc rare - unilocular - mostly intrasellar Pit macroadenoma - usually intrasellar epicentre - calcification often absent Apoplexy - clinical pres more acute Teratoma - presence of fat
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Papillary craniopharyngioma imaging
primarily suprasellar with a small intrasellar component in the minority of cases CT cysts not a significant feature solid part enhances calcification uncommon MRI cysts: when present, variable but typically low T1 Solid; T1 iso, T2 variable, enhances
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Papillary craniopharyngioma ddx
pituitary macroadenoma pituicytoma chordoid glioma meningioma
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Pilocytic astrocytoma are
who grade 1 astrocytic gliomas in young people majority cerebellum, can be optic pathway or spinal strong assoc with NF1
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Pilocytic astrocytoma location
cerebellum 60% optic pathway 30% brainstem/hemispheres/ventricles/spinal
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Pilocytic astrocytoma imaging
General - large cystic with enhancing mural nodule - heterogenous with mixed solid/cystic/necrotic - completely solid - usually enhancement present - can have calcs - haemorrhage is an uncommon complication MRI T1 - iso to hypo solid part - cystic fluid signal C+ - vivd - wall enhances half the time T2 - solid hyper to brain - cystic high SWI - calc or haemorrhage
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Pilocytic astrocytoma ddx
High grade astrocytoma with piloid features - NF1 Haemangioblastoma - usually adults - children VHL - no calc - smaller mural nodule with angiographic blush Medulloblastoma - typically midline - usually younger 2-6yo ATRT - larger, heterogenous Ependymoma - fourth ventricle - large cyst less common Ganglioglioma PXA haemorrhage
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Optic nerve gliomas are
relatively uncommon tumours usually seen in NF1 and predominantly pilocytic astrocytomas. Typically enlarged, low T1 high central T2, variable enhancement.
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Spinal pilocytic astrocytoma imaging
Thoracic > cervical > lumbar Eccentric location in the cord, typically dorsal Fusiform enlargement Well defined and displace cord Assoc cysts and syringomyelia Hamorrhage uncommon Vasogenic oedema rarely present T1 iso to hypo T2 hyper C+ mild variable enhancement
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Spinal pilocytic astrocytoma ddx
Astrocytoma - similar - also nf1 ependymoma - enhance strongly - central position - hamoerrhage common ganglioglioma - calcification - mixed t1 paraganglioma - inferior to conus - flow voids along surface and within tumour - cap sign from haemorrhage mets - more oedema
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Medulloblastoma subtypes (fuck you)
Medulloblastoma WNT activated Medulloblastoma SHH activated - TP 53 wildtype - TP 53 mutant - subgroups 1-4 Non WHT/non SHH - group 3 - group 4 Epidemiology - WNT; children and adults - SHH wild; infants and adults - SHH activated; children - gr 3; infants and children - gr 4; typically children Location location location Cerebellar peduncle/foramen of luschka - WNT Cerebellar hemisphere - SHH Midline - 3, 4 or SHH - 3 infants, prominent enhancement, ill defined margins - 4 children, well defined, minimal enhancement - SHH adults, variable defined/enhancing Spectro Gr3/4 - taurine peak - high cr SHH - little or no taurine - low creatine Proggy - WNT v good - SHH good - gr3 poor - gr 4 intermediate
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Medulloblastoma imaging
Strongly influenced by subtpye But small round blue cell tumour Typically cerebellum, majority vermis. protrude into fourth from roof. cerebellar peduncle WNT. CT - obstructive hydrocephalus - dense, cysts/necrosis, calcs - prominent enhancement MRI T1 hypo C+ typically enhance heterogenously T2/FLAIR iso to hyper, heteorgenous, oedema DWI; restrict
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Medulloblastoma ddx
Ependymoma - floor 4th, toothpaste ATRT - very young children, aggressive Pilocytic astro - cystic brainstem glioma Choroid plexus papilloma adult - mets - haemangioblastoma -cpp - epndymoma
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Atypical teratoid/rhabdoid tumour imaging
Location - cerebellum/brainstem - cerebral hemi - pineal - septum pellucidum - hypothalamus CTs Iso to GM deterogenous enhancement calc is common obstructive hydro MRI necrosis, cysts, haemorrhage T1: iso to hyper T2: generally hyper C+ heterogenous DWI restrict
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ATRT ddx
Embryonal tumour with multilayered rosettes Medulloblastoma Intracranial teratoma
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Embryonal tumour with multilayered rosettes (ETMR) are
rare small round blue cell tumours one of the most aggressive tumours in children
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Embryonal tumour with multilayered rosettes imaging
large, demaracted, solid, patchy enhancement, oedema, signifincant mass effect minority have cystic comonents and microcalc t1 hypo t2 hyper c+ patchy or no
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Astrocytoma IDH mutant tumours are
WHO grade 2, 3 and 4. diffuse infiltrating astrocytic tumours. IDH mutation and absence of 1p19q deletion.
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Astrocytoma IDH mutant tumours imaging
Low grade infiltrating astrocytomas, iso or hypodense without any enhancement. Can have cystic components. T1; iso to hypo T2/FLAIR - mass like hyperintense that suppresses on FLAIR - white matter distribution - can have microcystic changes at periphery DWI: facilitated diffusion. lower adc values suggest higher grade. C+: no enhancement in lower grades, solid enhancement/necrosis suggests higher Perfusion; elevated in higher grade
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Astrocytoma IDH mutant tumours ddx
GBM infarct cerebritis, encephalitis cortical based tumour
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Oligodendrogliomas are
intracranial tumours characterised by IDH mutation and 1p19q codeletion. present as masses involving the cortex or subcortical grey matter with low attenuation on CT, hypointensity on T1, and hyperintensity on T2. Can be heterogenous due to calc, cysts and haemorrhage
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Oligodendrogliomas imaging
CT 70-90% calc can have central, peripheral or ribbon calc variable enhacement MR T1 hypo T2 hyper, but for calc SWI; calc seen as blooming DWI; variable enhancement, not indicative of grade DWI no restriction perfusion; chicken wire increased vasc
234
Oligodendroglioma ddx
astrocytoma - t2/flair mismatch - more well defined margins - no calc ganglioglioma PXA CAPNON (entirely calc)
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Glioblastoma IDH wild type tumours are
diffuse astrocytic tumours that are IDH wild type. most common adult primary brain tumour. typically heterogenous, irregular peripheral enhancement, central necrosis, vasogenic oedema. three variants - giant cell glioblastoma - gliosarcoma - epithelioid glioblastoma
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Glioblastoma imaging
large at diagnosis thick irregular enhancing margins central necrotic core may have haemorrhage vasogenic type oedema, actually infiltration multifocal 20% with connections multicentric; no connection CT irregular thick margins slightly hyperattenuating marked mass effect vasogenic oedema haemorrhage/calc occasionally intense irregular heterogenous marginal enhancement MRI T1 - hypo to iso - central heterogenous C+ - peripheral and irregular T2/FLAIR - hyperintense - vasogenic oedema - flow voids SWI - low intensity rim, incomplete and irregular - absent dual rim sign DWI - solid usually restricts - necrotic/cyst facilitated Perfusion - rCBV elevated
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Glioblastoma ddx
astrocytoma IDH mutant 4 - generally younger mets - usually grey white matter junction - rCBV in oedema reduced CNS lymphoma - central necrosis in aids abscess - should have dual rim sign - central restricted diff tumefactive demyelination - open ring pattern enhancement - younger subacute infarct - hx - low rcbv toxo
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Pleomorphic xanthoastrocytomas are
astrocytic tumour in young patients who gr 2 or 3. most often cortical, cystic component with vivid enhancement. temporal lobe common. minimal oedema. can have overlying scalloping and dural tail. calcs rare.
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Pleomorphic xanthoastrocytoma imaging
solid enhancing nodule with peripheral cystic component may have a dural tail (reactive) MR T1 - cystic low, solid hypo to gm - leptomeningeal involvement in 70% C+ - solid enahnces vividly T2 - solid hyper - cystic high signal - not quite as suppressed on FLAIR as CSF - little vasogenic oedema
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Pleomorphic xanthoastrocytoma ddx
Ganglioganglioma - more calc - no dural tail DNET - enhancement uncommon - bubbly Oligodendroglioma - calc
241
Diffuse midline glioma H3 K27 altered is
a specific entity that represents the majority of diffuse intrrinsic pontine gliomas. Aggressive tumours with poor prognosis, who grade4. Typically young children and in the pons.
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Diffuse midline glioma H3 K27 imaging
located in the pons pons enlarged with basilar displaced anteriorly floor of the fourth ventricle flat can be exophytic CT hypodense, minimal enhancement MRI T1 low T2 heterogenously incr C+ usually minimal DWI mild restriction, usually normal
243
Focal brainstem glioma is
a relatively uncommon brainstem glioma, carries a more favourable prognosis than diffuse brainstem glioma. Majority low grade, pilocytic astro, can be ganggliogliomas or oligodendrogliomas. higher grade can be GBM
244
Tectal gliomas are
brainstem gliomas, typically low grade astros. Assoc with NF1. Obstructive hydro or parinaud syndrome
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Tectal gliomas imaging
CT homogeneous expansion of the tectal plate minimal enhancement can have central calc MRI T1 iso to hypo GM T2 hyper to grey C+ no enhancement
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Tectal gliomas ddx
aquaduct stenosis pineal parenchymal tumours germ cell tumours meningioma mets malformation
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Subependymomas are
slow growing and non invasive WHO grade 1 lesions
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Subependymomas imaging
Most commonly seen in the 4th ventricle Followed by lateral ventricles Third ventricle and spinal cord rare Typically small, 1-2cm CT hypodense/isodense intravenciular non enhancing may have cystic or calcific components MRI T1 iso to hypo, generally homogeneous when small T2 hyper, heterogenous when large, no oedema C+ no enhancement generally
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Subependymoma ddx
Ependymoma - children, younger adults - heterogenous enhancement Choroid plexus papilloma - vividly enhancing - children, young adults - in adults, fourth ventricle Central neurocytoma - typically younger patients SEGA - TS
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Calcified glial tumours
Old elephants age gracefully (and Eat PNETS) Oligodendoglioma Ependymoma Astrocytoma Glioblastoma EMTR/PNET
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Supratentorial ependymomas are
an uncommon type, with distinct molecular features to posterior fossa and spinal. Molecular subtypes = ZFTA fusion positive = YAP1 fusion positive
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Supratentorial ependymoma imaging
heterogenous parenchymal masses calcification, cystioc components, solid enhancing components surrounding oedema predilection for frontoparietal, but can be anywhere CT coarse calcs are common cystic areas are comon solid part iso to hypo can have haemorrhage periwinkle sign MRI T1; iso to hypo T2; hyper SWI: foci of blooming from haemorrhage/calc C+ heterogenous enhancement DWI can restrict
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Supratentorial ependymoma ddx
Parenchymal - diffuse astro, oligo, gbm - ETMR - pilocytic astrocytoma - met Intraventricular - central neurocytoma - choroid plexus papilloma - SEGA - subependymoma
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Posterior fossa ependymomas are
the most common type, typically occuyring in children. divided on the basis of DNA methylation profiling into A and B groups. A infants and young children B adolescents and adults
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Posterior fossa ependymoma imaging
PFA; lateral recess of fourth ventricle PFB midline floor of fourth ventricle near the obex Extend through foramina, plastic appearance Hterogenous, necrosis/calc/cystic/haemorrhage Can have intraparenhcymal lesiosn in the cerebellum CT coarse calc cystic areas iso to hypo heterogenous enhancement variable haemorrhage MR T1; iso to hypo T2; hyper SWI; foci of blooming C+ heterogenous enhancement DWI can have restriction
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Posterior fossa ependymoma ddx
Medulloblastoma - less calc - more homogeneous - vermis - less plastic Suybependymoma - usually non enhancing Choroid plexus papilloma - children trigone usually - adults fourth ventricle - homogeneous enhance - lacks adjacent oedema - carcinoma can be heterogenous andinvasive Choroid plexus met
257
Dysembryoplastic neuroepithelial tumours (DNET) are
benign who 1 slow growing glioneuronal tumours arising from cortical or deep grey matter. Arise from secondary germinal layers, typically cortical. Frequently assoc with cortical dysplasia. Cause intractable focal seizures. Specific glioneuronal element SGNE is characteristic. Simple, complex or non spec microscopically
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Dysembryoplastic neuroepithelial tumours (DNET) imaging
Location - temporal lobe 2/3 - frontal 1/5 - caudate - cerebellum - pons predominantly cortical, well circumscribed CT low density no or minimal enhancement may scallop/remodal skull calc in 1/3 MR T1 hypo C+ variable enhancement in 20% T2 high signal, bubbly. minimal oedema FLAIR mixed intensity, bright rim sign, partial bubble suppression SWI: calc DWI no restriction
259
Dysembryoplastic neuroepithelial tumours (DNET) DDX
Ganglioglioma - non bubble - more enhancement PXA - more enhancement - dural tail Low grade astro - IDH Oligo - IDH and 1q19p codeletion Desmoplastic infantile astro/gang - young - dural involvement prominent - large/multiple lesions Multinodular and vacuolating neuronal tumours (juxtacortical)
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Temporal lobe jenny diffs
Tumours - ganglioglioma - DNET - Pilocytic astro - diffuse astro oligodendroglioma - Pleomorphic xanthoastrocytoma Cysts - neuroepithelial - choroid fissure Other - herpes simplex - limbic encephalitis - mesial temporal scleoriss
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Gangliogliomas are
uncommon usually low grade tumours. can have epilepsy. commonly in the temporal lobes. variable appearance; cytic wih a nodule, solid mass expanding the gyris. variable enhancement
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Ganglioglioma imaging
Variable. Can be solid or cystic with a nodule. Infiltrating is uncommon and suggests higher grade CT - iso or hypo - calc 35% - bony remodelling - enchnaing 50% MRI T1; solid iso to hypo C+ solid variable enhancement T2; hyper, variable cystic signal. Peritumoural oedema is uncommon SWI; blooming with calc
263
Ganglioglioma ddx
DNET - bubbly PXA - dural tail - more enhancy oligo - calcs more common DIG - young children - prominent dural invovelemtn
264
Choroid plexus papillomas are
benign who 1 neuroepithelial intraventricular tumours, more common in paedis. Usually lateral ventricle in kids and fourth ventricle in adults. Solid vascular tumour with vivid frond like enhancement.
265
Choroid plexus papilloma imaging
CT Well defined lobulated mass mildly hyperdense cauliflower appearance homogenerously enhance fine speckled calc 25% MRI frond like morphology assoc hydrocephalus T1; iso to hypo T2 iso to hyper, flow voids can be seen C+ marked
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Choroid plexus papilloma ddx
Atypical choroid plexus papilloma Choroid plexus carcinoma - usually young children - heterogenous enhancement - parenchymal invasion Choroid mets Posterio fossa - medulloblastoma - AT RT - ependymoma Adults Ependymoma intraventricular meningioma subependymoma central neurocytoma
267
Choroid plexus carcinomas are
malignant neoplasms, who 3. Predominantly in children 0-5. assoc with liframeni and aicardia
268
Choroid plexus carcinoma imaging
Markedly enhancing intraventricular tumours, typically in the trigone and invading brain parenchyma. CT heterogenous iso to hyper calc in 25% prominent enhancement but heterogenous MRI T1 iso to hypo T2iso to hypo with hyper necrotic areas GRE blooming foci C+ marked heterogenous enhancement
269
Choroid plexus carcinoma ddx
Papilloma and atypical p[apiloma - homogenous - lack of necrosis - lack of invasion - younger Central neurocytoma - older - usually body lat ventricle abutting the septum pellucidum Intraventricular meningioma - more homogeneous Choroid plexus mets
270
Pineocytomas are
relatively benign who 1 pineal parenchymal tumours. mostly in adults 20-60. can get hydro or parinaud syndrome
271
Pineocytoma imaging
slow growing well circumscribed solid, sometimes focal cystic change CT intermediate density calcifications dispersed peripherally [ MR T1 hypo to iso T2 solid iso, areas of cysts C+ solid enhances vividly
272
Pineocytoma ddx
Pineal cyst Other pineal parenchymal tumours - pineal parecnhymal with indermediate differentiation - pineoblastoma - papillary pineal tumour Germ cell tumour - germinoma - embryonal - choriocarcinoma - teratoma Atrocytoma Mets
273
Pineoblastomas are
like small round blue cell tumours located in the pineal region. Highest grade amongst pineal parenchymal tumorus who 4. Typically found in young children. Associated with retinoblastomas. Associated with DICER1.
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Pineoblastomas imaging
Large, poorly defined masses, with frequent CSF seeding atpresentation. Directly involve brain structures. CT slightly hyperdense peripherally dispersed or exploded calcification, similar to pineocytoma MRI Sizeable, typically >4cm Irredular invasion into brain T1 iso to hypoi T2 iso, cystic, necrotic C+ heterogenous DWI restricted whole neural axis imaging is needed for CSF seeding
275
Pineoblastoma ddx
Other pineal parecnhymasl - pineocytoma - intermediate diff pineal parenchymal -0 papillary Germ cell tumours - germinoma - embryonal - chorio - teratoma Pineal cyst - thin wall Astrocytoma Mets Medulloblastoma
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Intracranial germ cell tumours are divided into
Germinomas Embryonal carcinomas Yolk sac tumours Teratomas - immature - mature - malig transformation Mixed
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Intracranial germ cell tumour locations
Tend to cluster in the midline Predilection for pineal and suprasellar regions. Also; - floor of the third - basal ganglia - thalamus - fourth
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Germinoma imaging
Engulf normal pineal tissue with assoc central calcification. Cystic components in 45% CT high cellularity, hyperdense bright enhancement presence of calc in young child <6.5 MR ovoid/lobulated, enfulfing the pineal T1 iso to hyper T2 - iso to hyper - cyst formation - areas of haemorrhage - predilection for invading parenchyma - central calcification C+ vivid and homogenous
279
Intracranial teratoma imaging
Intra or extra axial - intra; antenatal. typically larger - extra; adulthood, pineal/suprasellar CT majority have some fat and calc, usually solid/clumpy cyystic and solid components usually solid bits variably enhance MR T1 - hyper fat/proteinaceous - intermediate soft tissue - hypo calc and blood C+ solid parts enhance T2 mixed/heterogenous
280
Intracranial germ cell tumours markers
YS; AFP chorio; HCG teratoma and embryonal; variable germinoma; not AFP/HCG
281
Haemangioblastomas are
tumours of vascular origin which occur sporadically and in VHL. typically sharply demarcated, homogenous, cyst with an enhancing mural nodule. flow voids. assoc phaeos rcc's VHL polycythemia
282
Haemangioblastoma imaging
intracranial - 95% posterio fossa - 5% supratent, usually optic radiation spinal 3-13% homogenous well defined masses. cyst with mural nodules. non enhcaing walls, nodule vivid enhancement. prominent serpentine flow voids. can be totes solid. CT nodule iso to brain homogenous enhancement cyst walls dont enhance no calc MR T1; hypo to iso C+ nodule enhances T2 bright, flow voids
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Haemangioblastoma ddx
Brain mets AStrocytoma - PA in kids - GBM in adults Ependymoma Vascular lesions - AVM - cavernoma with subacute bleed - subacute infarct Medulloblastoma
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Pituitary microadenoma imaging
Bulkiness of the gland ipsilateral Subtle remodelling of the floor Deviation of the infundibulum T1 iso C+ delayed nehancement relative to gland on dynamic can be hypoenhancing to hypernehcning on delayed T2 variable Inferior petrosal sinus sampling; normal mr - confrim presence in setting of cushings - lateralise to aid surgical exploration
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Pituitary macroadenoma imaging
>10mm usually extend superiorly, can compress chiasm snowman appearance by indentation of diaphragm sellae enlarged pituitary fossa, thinning and remodelling CT - can be heterogenous due to haemorrhage, cystic, necrosis MR T1: typically iso, can be heterogenous C+ solid components enhance T2 typically iso, can be heterogenous SWI can have harmorrhage <90 deg encasement ICA unlikely involvement >270 deg encasement very likely involvement knosp classification
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Knosp classification:
three lines - medial tangent - intercarotid line - lateral tangent four grades 0: medial to medial line 1: bw medial and inter 2: between inter and lateral 3: lateral to lateral - 3a: superior cavernous sinus compartment - 3b: inferior cavernous sinus compartment 4: complete encasement
287
Pituitary macroadenoma ddx
mets - known ca - less well defined, bony destc pituitary carcinoma - rare, but indistinguishable meningioma - dural tail, more enhancing craniopharyngioma - more likely to be cystic/calc lymphocytic hypophysitis - post partum
288
Hyperprolactinaemia ddx
Stalk effect - interruption of dopamine from hypothalamus to ant pit, reduces inhibition - impingement/interruption - increased intrasellar pressure due to a mass - congenital ectpic posteiror pit/pituitary stalk interruption Medications - dopamine antagnoists; haloperidol, chlorpromazine Prolactin secretion - sectroy pit macroadenomas
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Ectopic posterior pituitary is
disruption of normal embryogenesis of the psoterior pituitary, common cause of pit dwarfism. also hyperprolactinaemia. when assoc with a thin or absent infundibulum and hypoplastic anterior pituitary then pituitary stalk interruption syndrome
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Ectopic posterior pituitary imaging
Absent posterior bright spot High T1 signal at the median eminence (floor of 3rd) ddx - fat (lipoma, dermoid, teratoma), chemical shift/FS - craniopharyng (adamantinomatous), larger, calc - thrombosed aneurysm
291
Pituitary stalk interruption/transection is
syndrome characterised by an absent or hypoplastic anterior pit, thin or absent infundibulum and ectopic posterior pituitary
292
Acromegaly is
excessive GH in skeletally mature patients, usually from an adenoma. In skeletally immature, its giganticism.
293
Acromegaly imaging
Skull - calvarial thickening - frontal bossing - enlarged paransal sinuses - enlarged sella - prognathism/protruding mandible - gaps in teeth Spine - vert fractures - can also have dish appearance, scalloping, increased vert hegiht joints - OA Hands - spade terminal tufts
294
Pituitary apoplexy is
an acute clinical condition caused by haemorrhagic or non haemorrhagic necrosis of the pituitary gland. Variable presentation, typically headaches, visual distuirbances, ophthalmoplegia and AMS. Existing pit macroadenoma commonly present.
295
Pituitary apoplexy RFs
medical treatment of a macroadenoma prior radiation pregnancy (sheehan) cerebral angiography trauma/surgery anticoags changes in icp
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Pituitary apoplexys imaging
Enlarged pit gland with or without bleeding Haemorrhage in 85% CT may show frank haemorrhage may have fluid debris level can be insensitive MR mass T1 variable, hyper if haemorrhagic T2 variable C+ variable, usually peripheral and may be hard to see DWI restricted in solid infarcted components
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Pituitary apoplexy ddx
pit masses with high t1 necrotic/haemorrhagic macroadenoma - not acute adamantinomatous cranio - calc 90% - usually children - not acute rathke cleft cyst - asx, spherical dermoid/teratoma - fat component - if ruptures, locules of fat elsewhere
298
Sheehan syndrome is
a rare cause of apoplexy and hypopituitism. Occurs in post partum females who experience large volume harmorrhage and hypovolaemic shock
299
Diabetes insipidus - whatisdus? and causes?
deficiency or resistance to vasopressin resulting in polyuria and polydipsia central causes - trauma - nsx - malignancy (craniopharyn, germinoma, mets) - autoimmune (lymphotic) - inflamm (sarcoid, LCH, IgG4) - infection/tb - preggo peripheral - congenital renal insens - lithium - metabolic hypok hypercalc - CKD
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SIADH what is it and causes
excessive ADH resulting in dilutional hyponatremia etiology - malignant tumours - lung diseases - cns diseases - drugs
301
IgG4 hypophysitis is
a rare cause of pituitary inflammation by a rare manifestation of systemic igg4 related disease. Clinical ft relate to part involved - can be anterior, posterior or pan. lymphoplasmacytic infiltrate rich in igg4 postivie plasma cells.
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IgG4 hypophysitis imaging
non spec enlargement of the pit gland with or without infundibulum involvement. May enhance post con. Other H/N manifestations of igg4 may be seen including igg4 related hypertrophic pachymeningitis.
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Lymphocytic hypophysitis is
an uncommon non neoplastic inflammatory condition that affects the pituitary gland. Closelt realted to orbital pseudotumour and tolosa hunt syndrome. frequently in pregnancy/post partum women.
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Lymphocytic hypophysitis imaging
CT enhancing soft tissue mass extending to suprasella MR appears as a pituitary region mass T1: iso, with slight heterogeneity. may have an absent bright spot. C+ variable homogeneous enhancement. infundibulum may be thickened. can have a dural tail. T2: parasellar region hypointensity
305
Rathke cleft cysts are
non neoplastic sellar/suprasellar cysts arising from the embryologic remnants of rathke pouch in the pituitary gland. common and usually incidental.
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Rathke cleft cyst imaging
well defined non enhancing midline cyst within the sella arising between the anterior and intermediate lobes of the pituitary. 60% suprasellar extension. XR can cause sellar enlargement CT non clacified, uncommon cuvilinear clac in wall homogeneous low attenuation non enhancing MR T1 50/50 hypo/hypo intense T2 70/30 hyper/hypo intense C+ no enhancement, may have a rim of enhancing compressed pit Small non enhancing intracystic nodule, pathognomic. hyperintense to fluid on t1 and hypo on t2.
307
Hypothalamic hamartomas are
also known as tuber cinereum hamartomas. they are benign non neoplastic heterotopias that typically occur in the region of the hypothalamus arising from the tuber cinereum, between teh mamm bodies and the optic chiasm. gelastic seizures classic history but also precocious puberty.
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Hypothalamic hamartoma imaging
can be sessile or pedunculated iso attenuating/intense to cortex without enhancement
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Pituicytomas are
rare indolent tumours only found in the neurohypophysis and infundibulum of the pituitary gland.
310
Pituicytomas imaging
CT homogensouly enhancing pit fossa/suprasella MR T1 iso, absent bright spot C+ bright enhancement T2 heterogenous, hypo to iso
311
SATCHMOE
Sarcoid Aneurysm Teratoma/TB Craniopharyngioma, cleft cyst, chordoma Hypothalamic hamartoma, hamartoma of tuber cinereum, histiocytosis (LCH, IGG, Lympho) Meningioma, mets Optic nerve glioma Eosinophilic granuloma, epidermoid
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Neurosarcoid imaging
five compartments - skull vault - pachymeningeal - pituitary/hypothalamic - cranial nerve - parenchymal CT can appear hyperdense enhance, less dramatically MR T1 iso to hypo T2 variable, usually hyper C+ homogensou enhancement
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Craniosynostosis - types
brachycephaly - bicoronal and or bilambdoid scaphocephaly/doliocephaly - sagittal plagiocephaly - unilateral coronal and lambdoid - frontal or occipital trigonocephaly - metopic Oxycephaly/turricephaly - sagittal, coronal, lambdoid cloverleaf - intrauterine sag, coronal, lambdoid harlequin eye - ipsilateral coronal
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Skull vault haemangioma imaging
expansile bone lesion thin borders sunburst pattern or trabecular thickening
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Skull pagets imaging
Osteoporosis circumscripta Cotton wool appearance Diploic widening Tam o shanter - platybasia and basilar invagination
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Chordomas are
uncommon malignant tumours originating from embryonic remnants of the primitive notochord. can be conventional, chondroid or dededifferentiated most commonly sacrococcygeal, then sphenooccipital then vertebral.
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Chordoma imaging
CT central well circumscribed destrtive lytic lesion exp[ansile soft tissue mass irregular intratumoural calc enhancing MRI T1 intermediate to low T2 high C+ heterogenous, with honeycomb appearance SWI variable blooming "Thumbing the pons" sign
318
chondrosarcoma base of skull imaging
CT tumour, rings and arcs calc MR T1 low T2 high SWI lwo for clacs C+ heterogenous
319
Ecchordosis physaliphora is
a congenital benign hamartomatous lesion derived from notochord remnants, usually in the retroclival prepontine region can can be anywhere to sacrum
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Ecchordosis physaliphora imaging
CT - bony clival defect, benign looking. near csf density. can see an osseous stalk at the base MR T1 hypo T2 hyper C+ varibable, typically non
321
Dysgenesis corpus callosum associations
aneuploidy non aneuploidy syndromes other cns - hydrocephalus - lipoma - chiari 2 - DWS - holoprosencephaly inborn errors of metabolism
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Dysgenesis corpus callosum imaging
antenatal - dilated/displaced third ventricle - colpocephaly - racing car sign - absent septum pellucidum MR - racing care - colpocephaly - dilated high riding third ventricle - bundles of probst - radial gyri, absent cingulate - hypoplastic fornices/hippocampi
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Chiari malformation quick breakdown
Chiari 1 - peg like cerebellar tonsils Chiari 1.5 - tonsils and brainstem Chiari 2 - medulla, fourth ventricle, cerebellar vermis - assoc myelomeningocoele Chiari 3 - similar to 2 but high cervical or occipital encephalocoele Chiari 4 - severe cerebellar hypoplasia without displacement Chiari 5 - absent cerebellum - occipital herniation
324
Lissencephaly pachygyria spectrum is
a basket term for congenital cortical malformations characterised by absent or minimal sulcation. - agyria no gyri - pachygyra broad gyri - lissencephaly smooth brain surface Can be further divided to type 1 (classic) and type 2 (cobblestone)
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Type 1 Lissencephaly (classic) imaging
grossly abnormal outline of the brain, hourglass or figure 8 cortex is thickened subcortical band heterotopia is strongly associated can have - enlarged ventricles - flattening anterior corpus callosum - cavum septum pellucidum et vergae
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Type 2 Lissencephaly (cobblestone) is
reduction in normal sulcation, associated with bumpy or pebbly cortical surface. While type 1 is neuronal undermigration, type 2 is due to overmigration. Heterogenous group of disorders, with similar morphology changes and congenital muscular dystrophy. Three most common; - Walker Warburg - Fukuyama congenital muscular dystrophy - muscle eye brain disease
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Type 2 Lissencephaly imaging
Lack or normal sulcation - small sylvian fissure - hour glass or figure 8 Multinodular corttical surface, particularly anteriorly Additional - hypomyelination - hydrocephalus - posterior cephalocoele - abnormal brainstem - abnormal cerebellum - abnormal globes
328
Grey matter heterotopia is
a group of conditions characterised by interruption of normal neuronal migration from near the ventricle to the cortex
329
Grey matter heterotopia types
Nodular - subependymal - subcortical Diffuse - band - lissencephaly - laminar
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Grey matter heterotopia associ
agenesis CC pachygyria schizencepahly polymicrogyria chiari 2
331
polymicrogyria is
a malformation of cortical development, cahracteised by abnormalities of both migration and organisation. strong assoc with schizencepahly.
332
Polymicrogyria imaging
Predilection for perisylvian region. bilateral 60%. MR abnormal morphology subjacent abnormal white matter with T2 hyperintensity
333
Schizencephaly is (incl types and assoc)
a cortical malformation that manifests as a grey matter lined cleft extending from the ependyma to the pia. can be open or closed assoc - septo optic dysplasia - GM heterotopia - absent septum pellucidum - dysgenesis of the CC
334
Holoprosencephaly is
a congenital brain malformation resulting from incomplete separation of the two hemispheres. three subtypes - alobar - semilobar - lobar other entities in the spectrum - syntelencephaly - septo optic dysplasia - central incisor syndrome
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Alobar holo imaging
single monoventricle absent midline structures - septum pellucidum - cc - interhemispheric dissure and falx - olfactory tract dorsal cyst absent or fused optic nerves abnormal arrangement of artereies craniofacial ft - proboscis - cyclopia - mononostil - hypotelorism - cebocepahly dx - semilobar - hydranencephaly - severe hydro
336
Semilobar holo imaging
absence of the septum pellucidum monoventrcile, partially developed occipitala nd temporal horns rudimentary falx incomplete interhemispheric fissure partial or complete thalamic fusion hypoplasia cc
337
Lobar holo imaging
fusion of the frontal horns wide communication to third ventricle partial fusion of the fornices and anterior frontal lobes absence of the septum pellucidum hypoplastic cc anteriroly displaced ACA, may be azygous
338
Septo optic dysplasia is
a condition characterised by hpoplasia of the optic nerves and absence of the septum pellucidum. 2/3 also have hypothalamic/pit dysfunction. part of the holoprosencepahly spectrum. assoc - schizencephaly - rhomboencephalosynapsis - chiari 2 - aqueductal stenosis
339
Septo optic dysplasia imaging
absent septum hypoplastic pit stalk hypoplastic optic nerves and globes point down appearance of the lateral ventricualr frontal horns on coronal
340
Syntelencephaly is
also known as middle interhemispheric variant, a mild subtype of holoprosencephaly characterised by an abnnormal midline connection of the cerebral hemispheres between the posterior frontal and parietal regions.
341
Syntelencepahly imaging
vertically orientated sylvian fissures, connected across midline cortical dysplasia subcortical heterotpoic grey matter dorsal cyst hypoplasia/aplasia of the CC body interhemispheric fissure present absent septum pellucidum separate frontal and occpital lobes
342
Focal cortical dysplasia imaging
MR cortical thickening blurring of the GWMJ T2/FLAIR hyperintensity in the grey/white matter transmantle sign abnormal sulcual or gyral pattern segemntal and or lobar hypoplasia
343
Focal cortical dysplasia classification
BLUMCKE 1. focal cortical dysplasia with abnormal lamination a; radial lamination b tangential 6 layer lamination c; radial and tangential lamination Type 1 - usually temporal, blurring of GWMJ, prominent atrophy, increased t2/flair 2 FCD with dysmorphic neurones a; without balloon cells b; with balloon cells type 2 - commonly frontal, abnormal gyri and sulci, marked blurring, cortical thickening, moderately increased signal, transmantle sign 3. architectural distortion of cortical layer a; temporal lobe with hippocampal atropgy b; abjacent to tumour c; adjacent ot vascular malformation d; abjacent to other lesions of early childhood
344
Classic dandy walker malformation is
triad of - hypoplastic vermis and cephalad rotation of the vermian remnant - cystic dilatation of the fourth ventricle - enlarged posterior fossa with torcula lambdoid inversion
345
Dandy walker variant is
a less severe posterior fossa anomaly than the classic, charactersed as partial vermian hypoplasia with partial obstruction of the fourth ventricle. usually no enlargement of the posterior fossa.
346
Blakes pouch cyst is
a cystic appearing structure that represents posterior ballooning of the inferior medullary velum into the cisterna magna below and psoterior to the vermis that communicates with an open fourth ventricle. It is caused by failure of regression of the blakes pouch secondary to non perforation of the foramen of magendie
347
Blake pouch cyst imaging
infravermian cyst that communicates with the fourth ventricle no vermian hypoplasia or rotation usually hydrocephalus elevation of the tentorium but normally positioned torcula
348
mega cisterna magna is
normal variant charactereised by focal enlaregement of the SAS in the inferior and posteiror portions of the posterior cranial fossa
349
Megalencephaly is
a disorder characterised by an abnormally large brain, primarily a prolferative disorder of embryonic origin. can be all or part, bilateral or unilateral. often assoc with polymicrogyria or agyria. assoc - achondroplasia - beckwith Wiedemann syndrome - NF1 - TS - Klipper trenaunay syndrome - epidermal naevus syndrome imaging - thick cortex - ipsilateral ventricular dilatation
350
Hemimegaencephaly is (and assoc)
rare congenital disorder of cortical formation with hamartomatous overgrowth of all or part of a cebral hemisphere. Assoc: isolated syndrome - epidermal naevus - klippel trenanay - mccune albright - proteus - nf1 - ts - cloves total (involving brainstema and cerebellym
351
Hemiemegalencephaly imaging
increased lat ventricle size shallow sulci enlarged gyri enlarged/thickened calvarial vault contralateral displacement of the falx white matter calc DVAs can have: polymicrogyria/lissencephaly GMH DDX - enlarged hemisphere (gliomatosis) - small hemisphere [rasmussen (no clavarial cahnges), DDM (calvarial changes), Sturge weber] - other neuronal migration anomalies with overgrowth
352
Brain cysts breakdown
Parenchymal - porencephalic (surrounding gliosis, usually comminicates with ventricle) - perivascular - neuroglial - neurocystericosis Intraventricular - ependymal - intraventricular simple/arachnoid - colloid (formane of monroe) - choroid plexus/xantholmatous Subarachnoid - arachnoid cyst - epidermoid (restricting) - choroid fissure
353
Intracranial epidermoid cysts are
uncommon congenital lesions resulting from inclusion of ectodermal elements during neural tube closure
354
Intracranial epidermoid cysts imaging
location - intradural 90%; CP angle, supra sellar, fourth ventricle - extradural 10% CT - similar to CSF - calcification can be seen - can be hyperdense due to haemorrhage, saponification or high protein (white epidermoid) - non enhancing MR T1: usually iso to CS, can be high for white C+ can have peripheral enhancment T2: usually iso to CSF, can be hypo if white FLAIR: heterogenous/dirty DWI: restricting engulf arteries
355
Porencephalic cysts are
focal cystic areas of encephalomalacia that communicate with the ventricular system and or suybarachnoid space
356
Intracranial dermoid cysts are
uncommon lesions, thought of along a spectrum from epidermoid cysts (containing only desquamated squamous epithelium)_ and teratomas (containing any kind of tissue from all three layers). Thought to occur as a developmental anomaly in which embryonic ectoderm is trapped in the closing neural tube. Lined by stratified squamous epithelium like epidermoids, but contain epidermal appendages as well, such as hair, sweat glands and sebaceous glands. the latter secrete the sebum that gives their appearance.
357
Intracranial dermoid imaging
Typically midline. Locations - sellar/suprasella - parasella - frontonasal - posterior fossa/vermis CT - low attenuating well defined - may have calc at rim - enhancement uncommon, if present usually rim - can rupture with IV FF levels of fat in sulci MR - more variable than a lipoma T1: typically hyper due to cholesterol C+ typically not enhancing, although if ruptures that pial enhancement T2: variable
358
Scoliosis is (and causes)
lateral curvature with a cobb angle >10 can be levo (left) or dextro (right) Terminology - apex: furthest vertebral body or space from centre/greatest rotation - end: vertebra most tilted toward each other - neutral: no rotation, not closer to apex than end - stable: first below the lowest curve thats midline Etiology Neuromusular - CP - chiari - friedreich - syringomyelia/diastematomyelia - tethered cord, dysraphism - musuclar dystrophies - connective tissue Congenital - segmental/fusion - skeletal dysplasia Tumours - bone - soft tissues Infection
359
Kyphosis and causes
sagittal curvature of the spine increased in - scheuermann disease - spondyloarthropathies - OP - fractures Decreased in - straight back syndrome
360
Spinal dysraphism classification
Open: cord and covering communicate with the outside, nothing covers teh sac - myelomeningocoele (98%) - myelocoel - hemimyelomeningo - hemimyelo Closed: cords is covered by other normal mesenchymal elements. can be with or without a subcutaneous mass. Closed, with subcutaneous mass - Lipoma with dural defect (lipomyelomeningo, lipomeylo) - terminal myelocystocoele - meningocoele - limited dorsal myeloschisis Closed, without subcut mass - posterior spina bfida - intradural lipoma - filar lipoma - tight filum terminale - persistent terminal ventricle - disorders of midline notochordal integration (dorsal dermal sinus, neurenteric cyst, split cord malformations) - disorders of notochordal formation (spinal regression, segmental spinal dysgenesis)
361
Scheuermanns disease imaging
Sorensen criteria - thoracic spine kyphosis >40 or - thoracolumbar kyphosis >30 and - at least 3 vertebrae wedging >5 Assoc - schmorls nodes - limbus vertebrae - scoliosis - spondylolisthesis
362
Basilar invagination is (and causes)
congenital or acquired craniocervical junction abnormality wherte teh tip of the odontoid goes above the FM. often assoc with platybasia. Stenosis of the foramen magnum and compression of the medullar. Congenital - OI - Klippel feil - achondroplasia - chiari 1 and 2 - cleidocranial dysostosis Acquired - RA - Pagets - Hyperparathyroidism - osteomalacia/rickets Pagets Fibrous dysplasia Rheumatoid/rickets Osteogenesis imperfect, osteomalacia Achondroplasia Chiaris, cleidocranial Hyperparathyroidism
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Klippel feil is (and assoc)
complex heterogenous entity resulting in cervical vertebral fusion. original classification 1. fusion of cervical and upper thoracic vert 2. fusion of two or three with assoc hemivert, occipitoatlantal fusion or other abnormality 3. cervical fusion with lower thoacic or lumbar fusion assoc - sprengel deformity - wildervanck syndrome - duane syndrome - arach anomalies - scoliosis - renal anomalies
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Congenital lumbar spinal stenosis is
stenosis affecting younger patients, typically with short pedicles. assoc with achondroplasia.
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Achondroplasia is
a congenital genetic disorder resulting in rhizomelic dwarfism. most common skeletal dysplasia. sporadic or AD. mutation in FGFR3 causing abnormal cartilage formation in bones formed by endochondral ossification
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Achondroplasia imaging (US, cranial, spinal, chest, pelivs/hips, limbs)
US short femur length trident hand frontal bossing depressed nasal bridge Cranial - large vault, small base - frontal bossing - narrowed FM - hydrocephalus - elevated brainstem Spinal - scalloping - progessive pedicle shortening - gibbus - laminar thickening - widenening of discs Chest - anterior flaring of ribs - anteroposterior narrowing Pelivs/Hips - horizontal acetabulum - trigent acetabulum - champagne glass pelvis - tombstone iliac wings - short sacroiliac notches Limbs - metaphyseal flaring - rhizomelic hosrtening - bowing mesial segment - trident hand - chevron sign - short metacarpal/metatarsals
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Sacrococcygeal teratoma is
a teratoma in the SC region, common in fetus and neonate. Assoc with meylomeningocoele and vert anomalies. elvated afp and bhcg. classified into benign and malignant. complications - high output heart failure - GU/GI obstruction - nerve compression - anaemia - dystocia - rupture
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Sacrococcygeal teratoma imaging
XR large mass over lower pelivs may show calcs US can be cystic or solid. Marked vascularity. MR variable depending on consituetion of the lesion T1 fat high, calc low T2 fluid hihg, clac low GRE calc C+ enhancing solid bits ddx - sacral chordom - terminal myelocystocoele - sacral meningocoele - sacral haemangioma - low lying neuroblastoma - low lying rhabdomyosarcoma
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Caudal regression syndrome is
spectrum of structural defects of the caudal region. results from an insult in early pregnancy.
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Caudal regression imaging
XR l/s vertebral dysgenesis usually below L1, often just sacrum truncated blunt cord above expected level severe canal narrowing Ante US blunt sharp cord conus way above expected level absent hypoplastic sacrum shield sign opposed iliac bones crossed leg position MR similar to US canal stenosis wedge sahped cord terminus
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Pars defects are
defect in pars interarticularis. Can be developmental or acquired. Acquired can be from repeated microtrauma or high energy trauma. Usually L5 or sometimes L4. Can be unilateral or bilateral.
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Pars defects imaging
Assoc - spondylolisthesis - spina bfida - scoliosis XR - scotty dog sign - inverted napolean hat
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Hollenberg classification (MR pars defect)
0 normal 1 stress rxn 2 incomplete fracture 3 complete fracture with oedema 4 chronic fracture no oedema
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Synovial cysts are
cystic formations connected to the facet joint containing synovial fluid. can cause radiculopathy. predominantly lumbar, predilection L4/5
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Synovial cyst imaging
CT - calc cystic lesions adjacent to facet - adjacent facet arthropathy - presence of gas MR - difficult to distinguish from ganglion cyst without intraarticular injection - gas is pathognomonic - complex fluid - neural cysts will intimate with a nerve
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Disc herniation types
protrusion - base wider than herniation - confined to disc level - outer annular fibres intact Extrusion - base narrower than dome - may extend above or below - complete annular tear - disc can sequester
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PLL ossification assoc
asians dish ank spond ossification lig flavum
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Kummell disease i
osteonecrosis of the vertebral body. RF: osteoporosis, steroids, alcoholism, radiation
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Kummell disease imaging
XR - collapse - invertebral vacuum cleft and fluid MR - intervertebral vacuum cleft - intravertebral dluid high t2
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Bertolotti syndrome is
controversial but essentially a L/S transitional vertbra and back pain
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Baarstrup is
interpsinous bursitis and other degenerative changes between adjacent spinous processes
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Bone marrow infiltration causes
Diffuse - multiple myeloma - mastocytosis - myelofibrosis - leukaemia Focal - mets - lymphoma Mnemonic MMLMML - mets - myeloma - lymphoma - myelofibrosis - mastocytosis - leukaemia
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Mastocytosis is
excessive accumulation of mast cells in one or more organs.
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Mastocytosis imaging
Skeletal - lytic/sclerotic/mixed bone invovlmenet - typically diffuse Abdo - PUD - small bowel thickneing - omental and mesenteric thickening - hepatosplenomegaly - ascites - lymphadenopathy chest - pumonary nodules, rare
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Primary myelofibrosis is
replacement of bone marrow with collagenous connective tissue and progressive fibrosis characterised by - EMH - progressive splenomegaly - anaemia - variable no granulocytes and platelets
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Primary myelofibrosis imaging
lymph nodes MSK - osteoscelrosis, diffuse - can give superscan abdo - hepatosplenomegaly - portal hypertension cv - failure
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Temporal lobe lesions ddx
PGPDM PXA Ganglio Pilocytic astro DNET MVNT
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swallow tail sign
normal axial hyperintensity in substantia nigra on T2*/SWI. Loss of sign may inidicate parksinsons, or LBD