This document discusses various demyelinating diseases that can be imaged in neurology. It provides images and descriptions of findings for multiple sclerosis, ADEM, NMO spectrum disorder, Susac syndrome, CLIPPERS, acute disseminated encephalomyelitis, acute hemorrhagic leukoencephalitis, acute necrotizing encephalopathy, and osmotic demyelination syndrome. It compares imaging features of MS and NMOSD that can help differentiate the two conditions. The document also discusses variants of MS like Marburg disease, Schilder disease, and Balo concentric sclerosis.

1. IMAGING IN NEUROLOGY
DEMYELINATING DISEASES
Dr. Rahi Kiran.B
DM Resident
Dept. of Neurology
GMC Kota

2. Normal myelination
• does not reach maturity until 2 years
• myelination progresses from central to peripheral
• caudal to rostral
• dorsal to ventral
• sensory then motor
• As myelination progresses myelin deposition causes an increase in T1 signal that
completes at 1 year of age - adult T1 myelination pattern
• Between 1-2 years of age the drawing out of water from myelinating areas results
in a decrease in T2 signal - adult myelination pattern achieved by 3 years

4. A
D
B
E F
C

5. 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.

6. Multiple Sclerosis

7. Multiple Sclerosis
• Over 95% CDMS have positive findings on MRI
• linear, round, or ovoid lesions surrounding the medullary
veins that radiate centripetally away from the lateral ventricles
• One of the earliest findings is alternating areas of linear
hyperintensity along the ependyma on sagittal FLAIR
- "ependymal 'dot-dash'" sign
• Spinal cord lesions can be found in 50% to 90% of CDMS
• The most common site - cervical cord.
• Typical MS lesions –
• do not extend beyond 2 vertebral segments,
• tend to involve the posterior and lateral regions,
• occupy less than half the area of the cord on axial images.
triangle-shaped hyperintensities . at the
callososeptal interface and a "dot-dash"
appearance . along the ventricle.

8. multiple foci of punctate and ring enhancement in the cerebral white matter.
Note "target" appearance
• Before steroids after steroids

9. "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
Hypointense rims of
the lesion show
striking but
Incomplete
ring enhancement
DWI shows that the
enhancing rims
restrict moderately
Rims demonstrate low
ADC values
The presence of T2 hypointense rim and open-ring enhancement are potentially suggestive of demyelination.

10. MS: DIFFERENTIAL DIAGNOSIS
• MULTIFOCAL T2/FLAIR HYPERINTENSITIES
• ADEM-history of viral prodrome or recent vaccination
• Hypoxic-ischemic lesions- small-vessel disorders- cortical infarcts, border zone, or watershed
lesions, lacunes
• Lyme disease-Cranial nerve enhancement is more common than in MS.
• Vascular-preferentially involves the basal ganglia and spares the callososeptal interface
• Susac syndrome-preferentially involve the middle of the corpus callosum, not the callososeptal
interface.
• MASS-LIKE ("TUMEFACTIVE") LESION(S)
• Neoplasm
• ○ Glioblastoma multiforme
• ○ Metastases
• • PML/PML-IRIS
• ○ HIV/AIDS
• ○ Natalizumab-treated MS
• • Medication-related-Enbrel

11. MS: DIFFERENTIAL DIAGNOSIS
Brainstem - symmetrical and central peripheral

12. Multiple Sclerosis Variants-Marburg disease
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.
T1 C+ -through ventricles
shows the necrotic,
cavitating, acutely
enhancing right
parietal "tumefactive"
mass
Coronal T1C+ -shows
extension around the left
ventricle

13. Multiple Sclerosis Variants-Marburg disease
• acute, severe fulminant MS
• Multifocal > solitary disease
• ○ Characterized by coalescent white matter plaques - Brain , spinal cord
• Lesions characterized by massive inflammation, necrosis
• • diffusely disseminated disease
• ○ Large cavitating lesions
• ○ Incomplete ("open") enhancing rim
• ○ Multiple other patchy enhancing foci

14. • monophasic with a low rate of recurrence
• Signs of increased ICP, aphasia, and behavioral symptoms are typical.
• CSF is usually normal
• no history to suggest acute disseminated encephalomyelitis (ADEM)
• Approximately 15% of cases progress to MS.
• Solitary unilateral masses are present in two-thirds
• Differential Diagnosis-Tumefactive" MS, Pyogenic abscess, neoplasm, metastasis
and glioblastoma multiforme.
Multiple Sclerosis Variants-Schilder Disease

15. Multiple Sclerosis 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

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

18. Chronic Relapsing Inflammatory Optic Neuropathy
(CRION)
• Young middle age female with recurrent sequential optic neuritis(Steroid responsive)
T2 - high-signal intensity foci -
left optic nerve
T1C+ enhancement DWI - expanded left optic nerve
with bright signal

19. Chronic Relapsing Inflammatory Optic Neuropathy
(CRION)
• Relapsing ON without known involvement in other areas of the CNS.
• unilateral relapsing optic neuritis, sequential relapses of both optic nerves, and
simultaneous, bilateral optic neuritis.
• Characteristic features –
• Middle age female
• history of optic neuritis with at least one relapse,
• objective loss of visual function
• NMO IgG negative
• contrast enhancement of the affected optic nerve on MRI
• Response to steroid treatment followed by relapse with dose reduction
• Compared to inflammatory ON – Severity less, response more

20. ADEM
FLAIR-bilateral white matter lesions with a "fluffy"
appearance and "fuzzy" margins
T1 C+ -enhance intensely
but heterogeneously
DWI shows acute diffusion
restriction in the lesions

21. ADEM numerous, large, same stage , include
white(asymmetric) and gray matter
(symmetric-thalamus and basal
ganglia ),do not always show
gadolinium enhancement, also show
good resolution compared with MS
lesions.
Spinal cord lesions 10-30% >50%

22. Acute Hemorrhagic Leukoencephalitis
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
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.

23. Mortality is 60-80%.

24. Petechial microhemorrhages similar to those seen in
AHLE
• Diffuse vascular injury,
• disseminated intravascular coagulopathy,
• Fat emboli,
• thrombotic thrombocytopenic purpura,
• sepsis,
• vasculitis,
• hemorrhagic viral fevers,
• malaria, and rickettsial diseases.

25. A-Axial T2 WI - showing swelling and bright T2
B- Axial CE MRI -no evident post-contrast enhancement.
C- Axial - DWI ADC -bilateral symmetrical parenchymal areas
of bright signal in DWI and low values in ADC

26. acute necrotizing encephalopathy (ANE)
• young children, is often associated with influenza,
• results from a para- or postinfectious cytokine storm.
• Strikingly symmetric thalamic necrosis with bilateral T2/FLAIR hyperintensities
and bithalamic hemorrhages is common

27. T2 FLAIR- medulla, hypothalamus,optic chiasma,
midbrain, internal capsule, periventricular white matter,
corpus callosum,
T2 hyperintense C1-5 with patchy
enhance ment
Neuromyelitis Optica Spectrum
Disorder

28. Neuromyelitis Optica Spectrum Disorder
• NMO-IgG, is 90% specific and 70-75% sensitive ..
• 10-25% of NMOSD patients are seronegative -The F:M ratio - 1:1
• one or both optic nerves are involved together with the spinal cord- most
commonly cervical
• Brain - cluster around the third and fourth ventricles and the dorsal
midbrain/aqueduct of Sylvius.
• 15-20% of patients with NMOSD are over age 60 at onset
• (85- 90%) are relapsing,
• 30% of NMOSD are initially misdiagnosed with MS.
• NMO-IgG seropositivity is detected in 3-5% of patients with CIS
• brain is more involved in MS, whereas multisegmental contiguous spinal cord
disease is typical of NMOSD

29. -U fibres

30. MS- multiple lesions
most often the cervical cord
usually less than 2 vertebral segments
relatively small and peripherally located
NMO- more than 3 vertebral segments
swelling of the cord.
often involve most of the cord.

31. Susac Syndrome
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

32. Susac Syndrome SICRET (Small Infarcts of Cochlear, Retinal, and Encephalic Tissue)

33. 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.

34. 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
• differential diagnosis - autoimmune encephalitis , Bickerstaff brainstem encephalitis,
vasculitis, intravascular lymphoma, lymphomatoid granulomatosis, neuro-Behçet,
neurosarcoidosis, CNS histiocytosis, multiple sclerosis, and NMOSD.
• Dramatic response to glucocorticosteroids (GCSs) supports
• relapses are common
• therapy failure is a strong indication for an alternative diagnosis.

35. Osmotic demyelination syndrome
T1 C+ (Gd): no enhancement trident shaped appearance FLAIR: hyperintense DWI: hyperintense

36. THANK YOU