Hind ALNajashi ,MD
R3 neurology resident
Neurology Teacher Assistent KAU
Demyelinating Diseases
Demyelinating
Diseases are
disabling disease
of the central
nervous system. It
result from injury
to the sheath that
covers the nerve
fibers( myelin) in
the brain, spinal
cord, and optic
nerves.
autoimmune
Herdiatry
Vascular
•ADEM
•MS
•Adrenolukodystrophy.
•Krabbe’s disease.
•PKU.
•Binswanger’s
disease.
Infectious
• PML
Toxic/metab
olic
•CO
•VitB12
deficency.
•Hypoxia.
•Alchol.
•Mercury.
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Attacks of demylination, disseminated in time
& space leaving gliotic scars (plaques) in the
white matter of the brain , spinal cord and optic
nerve.
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Autoimmune.
Infection ??.
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Age of Onset:
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29 to 32 yr.
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Sex:
female > male.
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Mortality:
Patients with MS tended to have normal life
expectancy, and other diseases were more likely to
be the cause of death.
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High-frequency areas of the world, with
current prevalence of 60 per 100,000 or more,
include all of Europe ,southern Canada, the
northern United States, New Zealand, and
Australia.
Low-risk areas include most of South America,
Mexico, most of Asia, and all of Africa.

if persons migrating from an area of high risk to an area of low
risk after the age of puberty carry their former high risk with
them. With migration during childhood, the risk seems to be
that of the new area to which the person has migrated.
Clinical feature
The symptoms and
signs of MS are the
manifestations of the
pathological process
seen in the CNS,
namely
demyelination .
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Symptoms
Numbness or tingling in the face or limbs
Impaired vision in one or both eyes, including:
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Blurred vision
Double vision
Loss of vision
Eye pain
Fatigue
Dizziness
Muscle weakness
Incoordination or falling
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Trouble walking or maintaining balance
Weakness in one or more limbs
Bladder problems including:
Urgency
 Incomplete emptying
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Bowel problems.
Sexual dysfunction
Difficulty swallowing
Forgetfulness, memory loss, and confusion
Depression.
C/P SUGGESTIVE OF
MS
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Heat sensitivity
(uthoff’s phenomena ).
Lehermitte’s sign .
Optic neuritis.
Involvement of multiple
area of CNS.
Age of onset between 15
and 50.
C/P NOT
SUGGESTIVE OF MS
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Involvement of PNS.
Deficit developing
within minutes.
Early Dementia.
Age of onset before 15
or after 50.
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There are several types of MS:
Relapsing-remitting MS —Symptoms suddenly
reappear every few years, last for a few weeks or
months, then go back into remission. Symptoms
sometimes worsen with each occurrence.
Primary progressive MS —Symptoms gradually
worsen after symptoms first appear. Relapses and
remissions usually do not occur.
Secondary progressive MS —After years of relapses
and remissions, symptoms suddenly begin to
progressively worsen.
Progressive relapsing MS —Symptoms gradually
worsen after symptoms first appear. One or more
relapses may also occur.
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Sex: MS appears to follow a more benign
course in women than in men.
Onset at an early age is seemingly a favorable
factor, whereas onset at a later age carries a less
favorable prognosis.
Initial complaints: impairment of sensory
pathways or cranial nerve dysfunction, has
been found in several studies to be a favorable
prognostic feature.
Although the diagnosis of MS remains clinical, a
number of ancillary laboratory tests can aid in
the diagnosis of MS. Neuroimaging,
particularly MRI, usually provides the most
critical information.
MRI Evidence Of Dissemination In Space is when
three out of four criteria are seen:
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1 Gd-enhancing or 9 T2 hyperintense lesions if no
Gd-enhancing lesion
1 or more infratentorial lesions
1 or more juxtacortical lesions
3 or more periventricular lesions (1 spinal cord
lesion can replace a missing infratentorial lesion
and contribute to the 9 T2-lesions).
MRI Evidence Of Dissemination In Time is
defined as:
 A Gd-enhancing lesion demonstrated in a scan
done at least 3 months following onset of
clinical attack at a site different from attack
 Any new T2 lesion detected in a scan done at
any time compared to a reference scan done at
least 30 days after initial clinical event.
Circled is an active lesion, showing hyperintensity on T2-weighted images
(A), hyperintensity on postgadolinium T1-weighted images (B), hypointensity
on T1-weighted images without gadolinium (C).
MRI IN MULTIPLE SCLEROSIS.
Bermel, Robert; Fox, Robert
© 2010 American Academy of Neurology. Published by Lippincott Williams & Wilkins, Inc.
2
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The appearance of a single lesion on MRI is
never pathognomonic of the disease, but some
distributions and types of lesions are highly
suggestive while others should raise
considerable suspesion of the clinical diagnosis.
Classic Characteristics of Typical Multiple
Sclerosis Lesions on T2-Weighted Brain Imaging
MRI IN MULTIPLE SCLEROSIS.
Bermel, Robert; Fox, Robert
CONTINUUM: Lifelong Learning in Neurology.
16(5) Multiple Sclerosis:37-57, October 2010.
DOI: 10.1212/01.CON.0000389933.77036.14
© 2010 American Academy of Neurology. Published by Lippincott Williams & Wilkins, Inc.
2
MAGNETIC RESONANCE IMAGING IN
MULTIPLE SCLEROSISWolinsky, Jerry
CONTINUUM: Lifelong Learning in
Neurology. Multiple Sclerosis.
10(6):74-101, December 2004.
This is a parasagittal fluidattenuating inversion
recovery image from a 23year-old woman with
relapsing-remitting multiple
sclerosis of 2 years'
duration. The image shows
two well-defined lesions
arranged perpendicular to
the lateral ventricle within
the corpus callosum, one
with a flamelike appearance.
This characteristic pattern
suggests the classic
neuropathologic description
of "Dawson's fingers" for
plaques centered on the
draining veins and is a
finding highly suggestive of
multiple sclerosis.
© 2004 American Academy of Neurology. Published by Lippincott Williams & Wilkins, Inc.
2
This transaxial T2weighted image
shows multiple
well-defined brain
stem lesions. Note
also the
juxtacortical lesion
in the left inferior
temporal white
matter.
MAGNETIC RESONANCE IMAGING IN MULTIPLE
SCLEROSIS.
Wolinsky,
Jerry Inc.
© 2004 American Academy of Neurology. Published by Lippincott Williams
& Wilkins,
2
Typical changes of
multiple sclerosis (MS) in
the brain on axial FLAIR
images (A). Note the
periventricular ovoid
lesions oriented
perpendicular to the
lateral ventricle, and
lesions in the corpus
callosum, with
predilection for the
frontal and posterior
horns. Sagittal FLAIR (B) is
a useful sequence for
identifying MS
lesions.FLAIR = fluidattenuated inversion
recovery.
MRI IN MULTIPLE SCLEROSIS.
Bermel, Robert; Fox, Robert
© 2010 American Academy of Neurology. Published by Lippincott Williams & Wilkins, Inc.
2
Typical for MS in this case
•Involvement
of the
temporal lobe (red
arrow)
•Juxtacortical lesions
(green arrow) touching the cortex
•Involvement of the
corpus callosum (blue
arrow)
•Periventricular
lesions - touching the
ventricles
White
matter
lesion is not
always
Multiple
sclerosis
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In aging WMLs are
normal findhng:

Widening of sulci,
periventricular caps
(arrow) and bands
and some punctate
WMLs in the deep
white matter seen.
multiple WMLs in a
hypertensive patient.
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MS is one of the most common demylinating
disease.
Characterized by attacks of demylination that
produce the symptoms and signs related to the
affected area.
Possible environmental factor play role in
increasing disease risk.
MRI play major role in diagnosis.
Be careful from MS mimickers.