case presentation ( dr soufi & dr hekmatnia ) 3.pdf

Rad cases for radiology residents

1. INTERACTIVE CASE
PRESENTATION
MAY 2022
DR. HEKMATNIA
DR. JMALIPOURSOUFI
RESIDENT : SHAMIMSHAFIEYOON

2. CASE 1 :
5 YEARS OLD CHILD WITH HEADACHE

3. FINDINGS AND DDX :
•Large intraaxialheterogenousmass lesion in right frontoparietotemporallobe
with pressure effect on right lateral ventricle
•Hemorrhagic component
•Heterogenousenhancement
•Ddx:
•1-PNET
•2-SUPRATENTORIAL EPENDYMOMA
•3-GBM
•4-MET

4. SUPRATENTORIAL EPENDYMOMA :
•Supratentorialependymomas are an uncommon type of ependymoma ,
found within the cerebral hemispheres, either remote to or abutting the
ventricle
•Overall, supratentorialependymomas affect primarily children and
adolescents but also, to a lesser degree, adults
•Although they can be located anywhere in the hemispheres, they seem to
have a predilection for the frontal and parietal lobe
•CT
•coarse calcification is common (50%)
•cystic areas (50%)
•solid component isoto hypodense
•heterogeneous enhancement
•a small proportion can have hemorrhage

5. •non-contrast CT appearances of an intraparenchymalependymoma is said to
resemble a periwinkle flower, thusperiwinkle sign(The central solid component with
centripetal calcification surrounding central necrotic area resembles a flower. More
fanciful still, the often present surrounding peripheral cyst has been likened to a leaf)
•The imaging findings can be remembered by the “1234 rule”:
•1: a single large tumor common in the first decade
•2P:Perilesional edema andPeriwinkle sign
•3L:LargeLobulatedLobar mass
•4C:CentralChunkyCalcification and large peripheralCyst

7. CASE 2 :
11 Y / SEIZURE

8. •MULTIPLE VIRCHOW ROBIN SPACE ARE NOTED IN BOTH CEREBRAL HEMISPHERE
•Enlarged perivascular spaces have also been reported with greater
frequency in a variety of settings, albeit generally in smaller size cohorts:
•autism spectrum disorder
•CADASIL
•cerebral amyloid angiopathy
•hypertensive encephalopathy
•mucopolysaccharidoses, e.g.Hunter disease,Hurler disease
•muscular dystrophies
•Parkinson disease
•Senersyndromesystemic lupus erythematosus (SLE)
•traumatic brain injury

9. •PATIENT SHOWS DIFFUSE HYPOPIGMENTATION SPOT IN HIS TRUNK.

10. HYPOMELANOSISOF ITO:
•hypomelanosisof Ito is a rare condition characterized by distinctive skin
changes, in which areas of the body lack skin color (hypopigmentation).
These skin changes may present as patches, streaks or spiral-shaped
(whorled) areas. In many affected individuals, additional symptoms affecting
areas outside of the skin also occur. There are a wide variety of symptoms
potentially associated with hypomelanosisof Ito. Neurological findings such
as seizures and developmental delays and musculoskeletal symptoms such
as abnormal curvature of the spine (scoliosis) are commonly associated with
this condition.

11. CASE 3 :
65Y/F/ PARESIS

12. FINDINGS AND DDX:
•There are high T2 whiter matter foci in both cerebral hemispheres. There are also
some tiny hypointensefoci scattered throughout the cerebral hemispheres and infra-
tentotrialbrain tend to be corticosubcortical(grey-white matter junction) in
distribution, suggestive of micro haemorrhages.
•Ddx:
•hypertensive microangiopathy
•hemorrhages, including microhemorrhages, are typically located in basal ganglia, pons and cerebellum
•not associated with subarachnoid hemorrhage or superficial siderosis
•multiple cavernomasyndrome
•lesions have a random distribution
•random size, althoughZabramskiclassificationtype IV cavernous malformations are indistinguishable from cerebral microhemorrhagesrelated to CAA
•hemorrhagic metastases(e.g. melanoma)
•lesions have a variable size and can often be larger than microhemorrhages
•enhancing
•diffuse axonal injury
•lesions are typically located at the grey-white matter junction, in thecorpus callosumand in more severe cases, in the brainstem
•neurocysticercosis
•nodular calcified stage visible on CT or phase-filtered SWI
•random distribution
•fat embolism syndrome
•'starfield' pattern of distribution
•lesions also show restricted diffusion on DWI and are likely visible on other sequences

13. CEREBRAL AMYLOID ANGIOPATHY(CAA)
•Cerebral amyloid angiopathy(CAA)is a cerebrovascular disorder caused
by the accumulation of cerebral amyloid-β (Aβ) in the tunica media and
adventitia of leptomeningeal and cortical vessels of the brain. The resultant
vascular fragility tends to manifest in normotensive elderly patients aslobar
intracerebral hemorrhage. It is, along withAlzheimer disease, a
commoncerebral amyloid deposition disease.

14. •Hemorrhage :intracerebral hemorrhage
•usually cortico-subcortical,in a so-calledlobarlocation
22
, but can also be seen in
thecerebellum(especially in the cerebellar cortex or vermis)
24
, may have finger-like
projections
26
•tend to spare thebasal gangliaandpons(cf.hypertensive 'deep' intracerebral
hemorrhage)
•cerebralmicrohemorrhage
•defined as 2-10 millimeter, round or ovoid areas of hemorrhage, and tend to be
corticosubcortical(grey-white matter junction) in distribution
25
, but can also be in
the superficial cerebellum
29
•tend to spare thebasal gangliaandpons(cf.hypertensive microhemorrhages)
•corticalsuperficial siderosis
•Ischemia :ischemic leukoencephalopathy
•chronic lesions, indistinguishable fromleukoaraiosisdue to other etiologies, but tends
to have a periventricular and posterior predominance
25
•CT:diffuse hypodensityof the white matter
25
•MR:T2 hyperintensityof the white matter without involvement ofsubcortical U-
fibers(cf.cerebral amyloid angiopathyrelated inflammation)

15. CASE 4 :30 Y /M/ ATAXIA

16. FINDINGS AND DDX:
•Selected FLAIR MRI images of the brain demonstrates hyperintensitiesin the
periaqueductal region and the medial thalami.
DDX:
•Leigh disease: mammillary bodies not involved
•metronidazole-induced encephalopathy:dentate nuclei,cranial nerve
nucleiand splenium also involved
•Wernike'sEncephalopathy
•bilateral medial thalami abnormalities:
•artery of Percheroninfarct
•central venous thrombosis

17. WERNIKE'SENCEPHALOPATHY
•Wernicke encephalopathy,also known asWernicke-Korsakoffsyndrome, is a
form ofthiamine (vitamin B
1) deficiencyand is typically seen in alcoholics.
•On imaging, it is commonly seen on MRI as areas of symmetrical increased
T2/FLAIR signal involving themammillary bodies,dorsomedial thalami,tectal
plate,periaqueductal areaand/or around thethird ventricle.
•T2/FLAIR:symmetrically increased signal intensity in the
•mammillary bodies
•dorsomedial thalami
•tectalplate
•periaqueductal grey matter
•around thethird ventricle
•T1 C+ (Gd):contrast enhancement can also be seen in the same regions,
most commonly of themammillary bodies
4
•DWI/ADC:restricted diffusion can also be seen in the same regions
4
•MR spectroscopy:may show decreased or normal NAA with the notable
presence of lactate

18. CASE 5 : 20 Y /F
RTLOWER LIMB PARASTESIS

20. FINDINGS AND DDX:
•This MRI study reveals T2 hyper intense multi lobulated / conglomerate
masses along right sided exiting sacral nerve roots in pre sacral region,
extending along sacral nerve at greater sciatic notch deep to Gluteal
muscles.
•Differential diagnosis:
•sarcoma
•plexiform schwannoma
•lymphatic malformation
•venous malformation

21. PLEXIFORM NEUROFIBROMA
•Plexiform neurofibromais an uncommon variant ofneurofibroma, a benign
tumor of peripheral nerves (WHO grade I), arising from a proliferation of all
neural elements. Plexiform neurofibromasare essentially pathognomonic
ofneurofibromatosis type 1 (NF1). Unlike small sporadiclocalized
neurofibromasanddiffuse cutaneous neurofibromas(both discussed
separately), these tumors are at significant risk of eventual malignant
transformation.
•Plexiform neurofibromasare usually diagnosed in early childhood. They are
found in approximately 30% of patients with NF1.

22. CASE 6 :
65 Y /F / RTSIDE PARESIS

24. FINDINGS AND DDX:
•MRI Brain Axial FLAIR and coronal T2w images show an extra axial nodular T2
flow voids, flow related signals on 3D TOF NoncontrastMR Angiography
suggestive of a high flow vascular malformation.
•Ddx:
•Ich
•AVM
•Dural AVF

25. DURAL ARTERIOVENOUS FISTULA
•Dural arteriovenous fistulas (dAVF)are a heterogeneous collection of conditions that
share arteriovenous shunts from duralvessels. They present variably with hemorrhage
or venous hypertension and can be challenging to treat.
•Complications
•The likelihood depends on venous drainage (reflected in both
theCognardandBordenclassification systems), and not arterial supply.
•hemorrhage
•subdural hemorrhage
•intracerebral hemorrhage
•subarachnoid hemorrhage
•venous congestion/hypertension and edema
•intracranial hypertension
•spinal myelomalacia

26. مکاخ رد هشیر اجنیا نم.
مکاپ یگدولآ زا ِکاخ نیا قشاع اجنیا نم.
منام یم تس یقاب سفن ات اجنیا نم.
مناد یمن ،مهاوخ یم هچ اجنیا زا نم!
،تسین اه یگ هریت نیا رد هچرگ ییانشور دیما
منار یم هنشت ِکشخ ِتشد نیا رد زاب اجنیا نم.
یهت ِتسد اب ،کاخ نیا لد زا رخآ یزور اجنیا نم
مناشفا یم رب لُگ.
دیشروخ نوچ ،هوک غیتس زا رخآ یزور اجنیا نم.
،مناوخ یم حتف دورس
مناد یم و
تشگ یهاوخ زاب یزور وت!

27. CASE 7:
2YO MALE WITH DELAYED MILESTONE. BIRTH HISTORY UNREMARKABLE.

28. IMAGING FINDINGS AND DDX :
.
CT brain salient features:
Hyperdensethalami.
Cortical atrophy marked in frontal regions.
•Differential diagnosis
•The differential diagnosis is largely that of otherleukodystrophies,
mainlymetachromatic leukodystrophy
12
.Neonatal hypoxic-ischemic
encephalopathycan also occasionally present with similar imaging findings.
Possible DDs:Krabbesdisease.
Profundperinatal ischemia unlikely as there is no supporting history and
periventricular leukomalacia.

29. KRABBEDISEASE
•Krabbedisease,also known asgloboid cell leukodystrophy,is an autosomal
recessivelysosomal storage disorderresulting in damage to cells involved in
myelin turnover. It thus affects both the peripheral nervous system and the
central nervous system (manifesting as aleukodystrophy).
•The majority of individuals with Krabbedisease present in early childhood
although adult presentations as late as the 5
th
decade are encountered
9
.
•Krabbedisease can be divided according to the age of onset into two or
three forms depending on the author
2,3,5
:
•infantile form (85-90%):rapidly progressive usual onset <2 years of age
•late-onset/adult form (10-15%):more slowly progressive

30. •T2:may show high signal involving periventricular white matter
,
centrum
semiovaleand deep grey matter;subcortical U-fibermay be spared until
late in the course of the disease
5
. Atigroidpatternof white matter
involvement can be seen.
•T1 C+ (Gd):no contrast enhancement in these areas
2
•MRS:adult form may show abnormal choline elevation in centrum
semiovale
7
•Enhancement and enlargement of the optic nervesand occasionally
peripheral nerves (e.g. lumbosacral plexus) can also be seen

31. CASE 8 :
A 40 Y O FEMALE WITH PROGRESSIVE LOWER LIMB

33. DESCRIPTION :
•This MRI sagittal T2w images and Post contrast fat sat T1w images shows an extra axial
solid signal intensity lesion, isointense on T2w images at foramen magnum causing
foramen magnum stenosis and significant cord compression. Intense homogeneous
enhancement of lesion on post contrast with duraltailing.
•Differential diagnosis
•The main duralmasses to consider include:
•solitary fibrous tumors of the dura
•more aggressive often destroying bone
•extensive peripheral vascularity
•more microlobulation
•duralmetastases(e.g. breast cancer)
•SCHEWNNOMA
•MENINGIOMA

34. CRANIOCERVICAL JUNCTION /
FORAMEN MAGNUM MENINGIOMA
•Meningiomasare a common primary intracranial neoplasm but location at the foramen magnum is
uncommon -accounting for ~2.5% (range 1.8-3.2%) of meningiomas
•Meningiomasareextra-axial tumorsand represent the most commontumor of the meninges. They
are a non-glial neoplasm that originates from the meningocytesor arachnoid cap cells of
themeningesand are located anywhere that meninges are found, and in some places where only
rest cells are presumed to be located.
•A number of helpful imaging signs have been described,including:
•CSF cleft sign, which is not specific for meningioma, but helps establish the mass to be extra-axial; loss
of this can be seen in grade II and grade III which may suggest brain parenchyma invasion
•duraltailis seen in 60-72%
2
(note that a duraltail is also seen in other processes)
•sunburstorspoke-wheelappearance of the vessels
•white matter buckling sign
•arterial narrowing
•typically seen in meningiomaswhich encase arteries
•useful sign in parasellartumors, in distinguishing a meningioma from apituitary macroadenoma ; the
latter typically does not narrow vessel

35. CSF CLEFT SIGN
Nice example of thespoke
wheelappearance andwhite matter
bucklingsigns in a case of a large
meningioma.

36. CASE 9 :
A 30 YOMALE, ADDICT, PRESENT WITH RIGHT SIDE
WEAKNESS.

38. PROGRESSIVE MULTIFOCAL
LEUKOENCEPHALOPATHY (PML)
•Findings:
Multiple patchy T2 white matter hyperintensitiesincerebral white matter in
frontoparietal region, basal ganglia, thalami as well as corpus callosum,
Bilateral asymmetrical involvement,
Non enhancing on post contrast T1.
Faint bright signal on diffusion attributed to prolonged T2 effect.
No mass effect.
Mild diffuse cerebral cortical atrophy.

39. •DDX:
HIV Encephalitis / Leukoencephalopathy : Usually characterisedby bilateral
symmetrical Periventricular T2 white matter hyperintensity, an associated
diffuse cortical atrophy and ventricular dilatation which are not predominant
findings of PML. Clinically PML is associated progressive focal motor and
sensory deficits, whereas HIV leukoencephalopathy ptpresent with altered
cognition.
•Lesions tend to have a confluent, bilateral butasymmetricalsupratentorial
white matter and thalamic involvement. However, basal ganglia, brainstem
and cerebellum also can be involved. Subcortical frontal and parieto-
occipital regions are common locations. However, isolated posterior fossa
involvement has also been reported.While the condition invariably involves
white matter,subcortical U-fibersand in late stages of disease grey matter
involvement is also seen

40. •multiple punctate high T2 signal lesions surrounding the main area (milky way
sign)
•barbell sign: parieto-occipital signal abnormality crossing the splenium

41. CASE 10:
A 19 YOMALE, HISTORY OF HOSPITAL ADMISSION SINCE
2WEEKS FOR HEADACHE ANDGENERALIZEDWEAKNESS,

43. FINDINGS AND DDX:
•
In Brain, bilateral asymmetric multifocal T2 hyperintensitiesin cerebral as well
as cerebellar white matter.
In spine, spinal cord show multisegementalcontiguousT2 hyper intensity
involving upper dorsal cord extending from C6-7 to D3-4 with focal cord
swelling.
•Ddx:
•Multiple sclerosis
•Vasculitis
Acute Disseminated Encephalomyelitis (ADEM)

44. Multiple sclerosis:Lesions are relatively smaller, involves sub cortical white
matter, commonly involves posterior fossa.Lesions are more
symmetric.Relapsing and remettingcourse.
Auto immune mediated vasculitis.
ADEM is a auto immune mediated demyelination.
Age group affected is children more than adults. More common in males than
female.
Variablesymptomsranging from headache fever drowsiness, CN palsy, hemi
paresis, loss ofconsciousnessandbehaviorchanges.

45. ADEM
•Acute disseminated encephalomyelitis (ADEM),as the name would suggest, is
featured by a monophasic acute inflammation anddemyelinationof white matter
typically following a recent (1-2 weeks prior) viral infection or vaccination
4,6
. Grey
matter, especially that of thebasal ganglia, is also often involved, albeit to a lesser
extent, as is thespinal cord
•Unlike many otherdemyelinating diseases(e.g.multiple sclerosisorneuromyelitis
optica), acute disseminated encephalomyelitis has no female predilection; if
anything, there is slight male predominanc
•Unlike multiple sclerosis, symptoms are more systemic rather than focal and include
fever, headache,decreased level of consciousness
•Compared tomultiple sclerosis, involvement of thecallososeptalinterfaceis unusual.
Lesions are usually bilateral but asymmetrical. Involvement of cerebral cortex,
subcortical grey matter -especially thethalami-and thebrainstemis not very
common, but if present are helpful in distinguishing frommultiple sclerosis

46. •In a small proportion (reported figures range from 10 to 20%
12
) the course is
more fulminant, frequently resulting in death. In such cases, the lesion may
demonstrate hemorrhage and the condition is then known asacute
hemorrhagic leukoencephalitis(Hurst disease)

47. CASE 11:
5Y / F/ BLURRED VISION

48. FINDINGS AND DDX:
•MRI of the brain demonstrates enlargement and enhancement of the optic
apparatus, including the posterior optic nerves, optic chiasm, optic tracts
and into the midbrain and basal ganglia.
•The pituitary gland can be seen as separate to the mass which pushes down
upon it.
•Ddx:
•Chraniopharyngioma
•Dysgerminoma
•Optic pathway glioma

49. •Optic pathway gliomasare relatively uncommon tumors, with a variable
clinical course and usually seen in the setting ofneurofibromatosis type I
(NF1). Histologically the majority arepilocyticastrocytomas.
•They are characterized by imaging by an enlarged optic nerve seen either
on CT or MRI.Usually showing low T1 and a high central T2 signal on MRI
images,enhancement is variable.
•stage 1:optic nerves only
•stage 2: chiasm involved (with or without optic nerve involvement)
•stage 3: hypothalamic involvement and/or other adjacent structures
•Males and females are approximately equally affected.

50. CASE 12:
25 Y / M / VOMITING

52. FINDINGS AND DDX:
•Mostly cystic partly soft tissue mass within the right cerebellar hemisphere.
•Significant mass effect on the brainstem and the fourth ventricle which
appears of abnormal shape.
•Intra-axial cystic right cerebellar hemispheric mass lesion containing intensely
enhancing peripheral mural nodule. The cystic part is isointense T1,
hyperintenseT2/FLAIR and the soft tissue nodule is isointense on T1 and T2.
•The mass effect is better appreciated by MRI; compression effect on the
brainstem and the fourth ventricle, crowded foramen magnum.
•The differential diagnosisfor a posterior fossa cystic mass with a mural nodule
in an adult includes hemangioblastoma, pilocyticastrocytoma and
metastasis, among others.

53. HEMANGIOBLASTOMAS
•Hemangioblastomasare tumors of vascular origin and occur both
sporadically and in patients withvon Hippel Lindau disease. They areWHO
grade 1tumors, which can occur in the central nervous system or elsewhere
in the body, including kidneys, liver, and pancreas.
•These tumors generally present on imaging as sharply demarcated
homogeneous masses composed of a cyst with non -enhancing walls, a
mural nodule which vividly enhances, often with prominent serpentine flow
voids.
•typically occur in young to middle-aged adults

54. LOCATION
•intracranial: 87-97%
•95% in the posterior fossa
•85% in cerebellar hemisphere
•10% in the cerebellar vermis
•5% medulla
•only rarely do they extend beyond the cerebellum into the cerebellopontine angle
•5% supratentorial(typically in the optic radiations)
•cerebral hemangioblastomasare only really seen in patients withvHL
•spinal: 3-13%

55. ASSOCIATIONS
•pheochromocytoma
•multipleRCCs
•von Hippel Lindau (vHL) disease
•~45% of those with vHLdevelop hemangioblastomas
•~20% of those with hemangioblastomahave vHL
•polycythemia: due to secretion of erythropoietin from lesions

56. CASE 13 :
56 YEARS OLD MAN / BACK PAIN

57. FINDINGS :
•Narrowing of the duralsac due to excessive fat (T1 & T2 high signal) within the
extradural space from L4-S1 levels.

58. EPIDURAL LIPOMATOSIS
•Epidural lipomatosisrefers to an excessive accumulation of fat within
thespinal epidural spaceresulting in compression of the thecal sac. In
severe cases, compression may be symptomatic. Thelumbar regionis most
frequently affected
•The demographics of affected individuals reflect the underlying causes:
•glucocorticoid excess
•long term steroid administration (e.g. for asthma): 55% -most common
•endogenousCushing syndrome: 3%
•obesity: 25%
•idiopathic: 17%
•Scheuermanndiseasefollowing spinal surgery

59. •Symptoms are often non-specific and may be similar to other degenerative
spinal conditions resulting in stenosis. Patients may present with a combination
of pain, radicular symptoms, weakness, and paresthesia.
•There is an often generalized excess of fat seen in the extradural space. As a
result, the duralsac can appear narrowed or even resemble a "Y" shaped
configuration.

60. TAKE HOME MESSEGES:
•1-Supratentorialependymoma : periwinkle signin non contrast ctscan
•2-MULTIPLE VIRCHOW ROBIN SPACE : hypomelanosisof itoand MPSand Parkinson
•3-Amyloid angiopathyusually dose not involve basal ganglia
•4-Wernike's Encephalopathy:commonlyseen on MRI as areas of symmetrical
increased T2/FLAIR signal involving themammillary bodies,dorsomedial thalami,tectal
plate
•5-plexiform neurofibroma: association with NF -1
•6-Krabbdisease : bilateral hyperdensethalami

61. •7-SPOKE WHEEL APPEARANCE : IN MENINGIOMA
•8-EXTRA AXIAL LESIONS : CSF CLEFT SIGN , WHITE MATTER BUCKLING , DURAL TAIL
•9-PML : barbell sign, CONFLUENT BILATERAL WHITE MATTER LESIONS, SUBCORTICAL
WHITE MATTER INVOLVEMENT
•10-Adem: systemic symptoms , callososeptalmargin usually spare , younger than
MS
•11-Optic pathway glioma HISTOLOGIC TYPE USUALY IS PILOCYTIC ASROCYTOMA
•12-SOLID CYSTIC MASS IN POSTERIOR FOSSA HEMANGIOBLASTOMA, PILOCYTIC
ASROCYTOMA , METASTASIS