multiple sclerosis

1. IMAGING IN
DEMYELINATINGDISEASES
Dr. Shubham Rai

2. Topics covered
• MS and variants
• ADEM
• AHEM
• NMOSD
• SUSAC syndrome
• CLIPPERS

3. INTRODUCTION
White matter diseases affect the pattern of normal myelination and include a
large diversity of congenital and acquired processes. They can be divided into
demyelinating (secondary destruction of normal myelin) or dysmyelinating
(primary abnormality of myelin formation) processes. Demyelinating diseases
can be further divided into autoimmune, infectious, vascular, and toxic-
metabolic categories
Primary demyelinating disorders are of unknown etiology, and their
prototype is multiple sclerosi.

4. MULTIPLE SCLEROSIS
Multiple sclerosis - is the most common inflammatory
demyelinating disease of the central nervous system in young
and middle-age adults.
Most patients initially present in the third and fourth decade.
The incidence of MS is two to three times higher in females
than in males

5. The first clinical symptom is
• often impaired or double
vision;
• other common complaints
include weakness,
numbness, tingling, and
gait disturbances.
• As the disease progresses,
loss of sphincter control,
blindness, paralysis, and
dementia may develop.

6. • Radiologically Isolated syndrome:
Mildest subtype
RIS refers to MR findings of t2 flair regions (suggestive of MS in persons) with no history of
neurologic symptoms and with a normal neurologic examination.
• Clinically Isolated syndrome:
It refers to a first episode of neurologic symptoms that lasts atleast 24 hours and is caused by
inflammation or demyelination in the CNS.
• Relapsing –remitting MS :
Mc type
Patients experience relapses alternating with remission phases.
• Primary progressive MS: it is characterised by worsening neurologic function and
lacks period of remission.

7. IMAGING
CT : NCCT is often normal but
may show solitary or multiple ill
defined white matter
hypodensities.
Acute or subacute lesions may
show puntate, patchy or ring
enhancement on CECT.
Right side frontal hypodensity in
periventricular area

9. MR Findings
TlWI
⚫Most MS plaques are hypo or
isointense on T1WI . The
hypointensity (black holes )
correlate with axonal
destruction.
⚫A faint rim of hyperintensity
secondary to lipid peroxidation
and macrophage infiltration-
sorround blackholes-lesion
within lesion appearance

10. T2/FLAIR
Multiple hyperintense round or ovoid
lesions sorrounding the medullary
veins that radiate away from the
lateral ventricles.
MS plaques often assume a triangular
shape with base adjacent to the
ventricle .( dawson fingers)
Alternating areas of linear
hyperintensity along the ependyma
on saggital FLAIR ,known as
ependymal dot dash sign
triangle-shaped hyperintensities . at the
callososeptal interface and a "dot-dash"
appearance . along the ventricle

13. Tl C+
Transient enhancement can be seen during active demyelination
Nodular
Ring
Semilunar, incomplete, "horseshoe-shaped"
MRS
Decreased NAA
Increased Choline
Increased Myoinositol

14. MRS
Decreased NAA
Increased Choline
Increased Myoinositol

15. Spinal Cord
• Typical spinal cord lesion in MS are
relatively small,peripherally located and
multiple
• They are most often found in the
cervical cord and are usually less than 2
vertebral segments in length .
• Isolated spinal cord involvement is seen
in upto 20% cases of MS

16. Extensivecervical spinal cord lesions with multiple sclerosis.
A: Sagittal T2-weighted imageshows extensive irregular high signal within
thecord substance.
B: Axial T2-weighted imageshows the typical peripheral location of the
multiplesclerosis plaque.
most often the cervical cord
usually less than 2 vertebral segments
relatively small and peripherally located

17. C: A patientwith multiplesclerosis shows an ill-defined lesion on
T2-weighted imageextending from thedorsal medulla to C2. D: Postcontrast T1-
weighted imagedemonstrates minimal peripheral enhancement.

18. multiplefociof punctateand ring enhancementin the cerebral whitematter.
Note "target"appearance
• Before
steroids
after
steroids

19. Multiple Sclerosis

20. A
D
B
E F
C

21. Multiple Sclerosis
• A- FLAIR best for periventricular and juxtacortical
lesions.
• B- T2 images are often best for viewing infratentorial
lesions.
• C- Homogenous uptake of contrast.
• D- Black hole sign On MRI T1W
• E- dawsons fingers
• F- Open-ring pattern, specific for demyelinating lesions.

23. McDONALD CRITERIA FOR MS DIAGNOSIS

24. The assessment of
IgG oligoclonal bands (IgG-OCB)
is routinely done in clinical
practice in patients suspected of
having a demyelinating disease.
The presence of CSF OCB
provides evidence of an
abnormal intrathecal B-cell
response and is present in over
95% of MS patients.

25. TumefactiveMS
• Tumefactive MS is a variant of Multiple Sclerosis
• It presents as a large intra parenchymal lesion (>2cm)
usually without any significant mass effect
• After the administration of gadolinium,there may be
peripheral enhancement often with an incomplete ring
• D/D- gliomas or intraparenchymal abscesses which typically
have a closed ring enhancement

26. "Tumefactive"MS.
T1WI – large hypointense lesions in
both cerebral Hemispheres with
significant perilesional edema
T2WI-very hyperintense and
surrounded by a thin hypointense rim
and perilesional edema
.

27. Hypointense rims of the
lesion show striking but
Incomplete
ring enhancement
DWI shows that the
enhancing rims restrict
moderately
Rims demonstrate low ADC values

28. Multiple Sclerosis Variants-Marburgdisease
• acute, severe fulminant MS
• c/b Rapid neurological deterioration
• Monophasic,relentless progression
• Death usually within one year
• Affects young adults

29. Pathology and imaging
• Multifocal > solitary disease
• Characterized by coalescent white matter plaques - Brain , spinal cord
• Lesions characterized by massive inflammation, necrosis
• Imagingshowsdiffusely disseminated disease
• Large cavitating lesions
• Incomplete enhancing rim

30. Multiple Sclerosis Variants-Marburgdisease
Axial FLAIR- Large
heterogeneously hyperintense
lesion in the right parietal WM
with
a smaller lesion on the left
Axial T1 C+- multiple bilateral
incomplete ring- enhancing lesions
in the deep and periventricular
WM.

31. • Myelinoclastic diffuse sclerosis
• It is a rare acute/subacute demyelinating disorder
• Affects young adults (mean age 18yrs)
• Signs of increased ICP, aphasia, and behavioral symptoms are typical.
• CSF is usually normal, no h/o fever/vaccination
• Approximately 15% of cases progress to MS.
• Solitary>multifocal lesions
• Lesions look like tumefactive MS.
Multiple Sclerosis Variants-SchilderDisease

32. • Concentric rings ofdemyelination/myelin
preservation
• Resemble tree trunk or onion bulb
• Solitary > multifocal
• "Whirlpool"hyperintense concentric rings on T2WI
• Minimal mass effect, edema
• Actively demyelinating layers enhance
MultipleSclerosis Variants-Balo Concentric Sclerosis

33. MultipleSclerosis Variants-Balo Concentric Sclerosis
Acute lesions-hyperintense on FLAIR,
restrict on DWI, show concentric
"onion bulb" enhancement
Follow-up scans show alternating
rings
of iso- and hyperintensity on T1 and
T2WI,
no enhancement

34. Acute Disseminated Encephalomyelitis
• It is the 2nd most common acquired demyelinating disease
• It is a postinfection/postimmunization disorder
• It can involve both the brain and spinal cord . White matter lesions
usually predominate,but basal ganglia involvement is seen in nearly
half of all cases.
• No female preponderance
• Can occur at any age but maximum incidence at 5-8 years of age
• Symptoms typically occur a few days to a few weeks following
infection/vaccination

35. ADEM

36. IMAGING

37. ADEM
FLAIR-bilateral white matter lesions with a "fluffy" appearance and
"fuzzy" margins

38. T1 C+ -enhance intensely but heterogeneously
DWI shows acute diffusion
restriction in the lesions

40. Acute Hemorrhagic
Leukoencephalitis
AHLE predominantly affects the white
matter but may involve both brain and
spinal cord.
Both the cerebral hemispheres and
posterior fossa structures are affected.
Despite its name it may affect gray matter.
Most cases begin with a viral illness f/b
rapid neurologic deterioration

41. Acute HemorrhagicLeukoencephalitis
Axial FLAIR-splenium and genu of the corpus
Callosum, bifrontal focal
hemispheric white matter lesions , subtle
confluent hyperintensity in the occipital
subcortical white matter
DWI shows restricted diffusion in the corpus
callosum splenium

42. GRE - punctate hypointensities in the corpus
callosum with subtle "blooming" in the subcortical
WM
SWI-Innumerable bilaterally symmetric punctate and
linear "blooming" hypointensities are seen
throughout the WM with striking sparing of cortical
gray matter.

44. T2 FLAIR- medulla, hypothalamus,optic chiasma,
midbrain,internalcapsule,periventricularwhitematter
,
corpuscallosum,
T2 hyperintense C1-5with patchy
enhancement
Neuromyelitis OpticaSpectrum
Disorder

45. Neuromyelitis Optica
⚫Neuromyelitis optica (Devic type) is a syndrome of acute onset of optic
neuritis and transverse myelitis (LETM) thatdevelopatapproximately
the same time
⚫ Classically, NMO patients have relapsesconsisting of optic neuritisand
myelitis
⚫The targetof the NMO antibody is the Aquaporin-4 (AQP4).

47. Clinical Manifestations
• Optic nerve involvement typically manifests as bilateral optic
neuritis involving the optic chiasm with severe vision loss.
• Complete acute spinal cord syndrome is a classic clinical
manifestation of spinal cord lesions.
• Intractable nausea, hiccups, and vomiting are related to area
postrema syndrome.
• Patients with diencephalic involvement may have narcolepsy,
anorexia, inappropriate diuresis, hypothermia, and hypersomnia.
• In brainstem involvement, oculomotor dysfunctions, long tract signs,
and ataxia can be seen

48. Imaging Findings
Optic Nerve/Chiasm :
Optic neuritis is a core clinical syndrome related to NMOSD.
Bilateral and longitudinally extensive optic nerve involvement
Optic neuritis typically includes the intracranial segment of
the optic nerves, usually extending to the chiasm and optic
tract
• optic neuritis is usually identified as a thickened optic nerve
with hyperintensity on T2-weighted images and
enhancement on gadolinium-enhanced T1-weighted
images.

49. (a, b) Axial (a) and sagittal (b) gadolinium-
enhanced fat-saturated T1-weighted images
show enhancement of the bilateral posterior
optic nerves (arrows in a) and chiasmatic
region (dotted oval in b). There is also typical
enhancement of the periaqueductal region
and area postrema (arrowheads in b)

50. Spinal Cord
• longitudinal involvement of the spinal cord at three or more
contiguous vertebral segments
• The central gray matter along the central canal of the spinal cord is
the preferred area of involvement
• lesions are located centrally or both centrally and peripherally on axial
images and involve more than 50% of the cord area
• Cervical, thoracic, or cervicothoracic spinal segments are usually
compromised
• When the cervical spine is involved, extension into the brainstem,
typically to the area postrema, is commonly observed

51. (a) SagT2-w
image shows
LETM
extending
into the area
postrema
(arrowhead).
(b) Axial T2w
image shows
characteristic
involvement
of the central
gray matter
(arrow). (c)
Axial T2-w
image shows
a bright
spotty lesion
(arrow). (d)
Axial T1-
weighted
image shows
a

52. BRAIN
The prevalence of brain lesions is variable, occurring in 24%–89% of NMOSD
patients
MR Imaging Findings
To recognize typical brain lesions in NMOSD, one must primarily remember the
areas where AQP4 is consistently expressed— subpial regions, periependymal
regions, circumventricular organs, brainstem, chiasm/hypothalamus, and corpus
callosum.

54. MRS
⚫ NAA (NAA/Cr), Choline
(Cho/Cr
MS- multiple
lesions most often the cervical cord
usually less than 2 vertebral
segments
relatively small and peripherally
NMO
-
more than 3 vertebral
segments swelling of the
cord.
often involve most of the

55. Susac Syndrome SICRET(SmallInfarctsof Cochlear,Retinal,and Encephalic Tissue)

56. SusacSyndrome
T2WI - hyperintense
foci, the middle of
the corpus callosum
genu and thalamus
Axial T1 C+ shows
that the corpus
callosum genu lesion
enhances.
Enhancing lesions in the left temporal
lobe, left thalamus,pons

57. CLIPPERS
Chronic Lymphocytic Inflammation with Pontine Perivascular
Enhancement Responsive to Steroids.
• onset is is 40-50 years, minor male predominance
• subacute brainstem symptoms such as gait ataxia,
diplopia, facial paresthesias, and nystagmus.
• Dramatic response to glucocorticosteroids (GCSs)
supports
• relapses are common
• therapy failure is a strong indication for an alternative
diagnosis.

58. CLIPPERS
Sagittal FLAIR -
multiple punctate
hyperintensities
"peppering" the pons,
Medulla extending into
the upper cervical
spinal cord
T1 C+ - punctate and
curvilinear foci of
enhancement,
extension into the
cerebellum and
superior cerebellar
peduncle
DWI - scattered foci
of restricted
diffusion
Axial T2 SWI shows
multiple hemorrhagic
foci in the pons.

59. THANK YOU