A 32-year-old woman presented with gradually worsening weakness on the left side of her body over the last 5 days along with new weakness in her right arm. On examination, she had decreased strength in her left arm and leg and right arm, as well as left facial nerve palsy and increased reflexes on the left side. These findings are consistent with multiple sclerosis, a chronic inflammatory demyelinating disease of the central nervous system characterized by relapses and remissions. MRI of the brain would be pursued to identify lesions in multiple areas and confirm the diagnosis.
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Multiple sclerosis
1.
2. Case history
• A 32yrs old lady is brought to ER with c/o
gradually developing weakness of left half
of body for last 5 days. Now there is
weakness of right arm as well. No h/o
fever, headache, vomiting or
unconsciousness.
3. O/E
• GPE
• CNS:
Power
normal
2/5 in left arm & leg
3/5 in right arm
left facial nerve palsy LMN type
left plantar upgoing
reflexes brisk on left side
6. Case history
• A 35 yr old gentleman presents with
sudden onset paraplegia. O/E there is
UMN paraplegia with a sensory level at
T8.
• h/o sudden transient unilateral visual loss
7 yrs back.
• Whats the pathology?
7. Case history
• A young girl of age 17 presents in opd with
c/o diplopia & unsteadiness developing
over 2months. O/E :
• Features of right cerebellar lesion
• Bilaterally upgoing plantars
• Left lateral rectus palsy
All routine labs are normal
9. Case history
• A diagnosed patient of multiple sclerosis
presents with complaints of severe
excruciating pain over left half of face.
There is electric spark like sensation. Pain
aggravates even on touching the face. Its
not relieved even by use of NSAIDs.
• What is the underlying problem?
12. • Also called as disseminated sclerosis or
encephalomyelitis disseminata
• French neurologist Jean Martin Charcot
was the first to recognize multiple
sclerosis as a distinct separate disease in
1868
13. Epidemiology
•
•
•
Male: Female= 1:2
Peak age of onset is in the fourth decade
Incidence varies with latitude, low in
equatorial areas and higher in temperate
zones
• More relapses occur during spring and
summer
14.
15. Aetiology
Genetic factors
• Environmental factors
• Autoimmune factors
The risk of familial recurrence is 15% with
highest being for first degree relatives.
Monozygotic twin concordance is 35%
16. Pathology
• The major target is the myelin- producing
OLIGODENDROCYTES of the central
nervous system
• An underlying autoimmune mechanism
may be involved as there are increased
number of activated T lymphocytes in the
CSF and increased immunoglobulin
synthesis in CNS
20. Pathogenesis
• CNS inflammation in MS starts with entry of
•
•
activated T lymphocytes through blood brain
barrier
The resulting inflammatory cascade releases
cytokines and initiates destruction of
oligodendrocyte-myelin unit by macrophages
Demyelination mostly occurs in periventricular
regions of brain, optic nerves and subpial
regions of spinal cord
24. Common presentations
• Optic neuritis
• Bilateral internuclear ophthalmoplegia is
•
•
•
•
•
characteristic of MS
Relapsing and remmitting sensory symptoms
Subacute painless spinal cord lesion
Acute brain stem syndrome
Subacute loss of function of upper limb (dorsal
column deficit)
6th cranial nerve palsy
25. Other symptoms and
syndromes
• Afferent pupillary deficit and optic
atrophy (previous optic neuritis)
• Lhermitte´s symptom
(tingling in spine or limbs on neck
flexion)
• Progressive non-compressive
paraperesis
(Devic´s variant)
26. Clinical features
• Uhthoff´s symptom; transient unilateral
•
•
•
•
visual loss or blurring after hot shower or
exercise
Partial Brown-Sequard syndrome
Postural (rubral , Holmes ) tremor
Trigeminal neuralgia
Recurrent facial palsy
27. Variants of MS
•
•
•
Relapsing remitting (80%)
Primary progressive (10-20%)
Fulminant (<10%)
Marburg´s variant
• Secondary progressive
28.
29. Diagnosis
A diagnosis of multiple sclerosis requires
the demonstration of lesions in more than
one anatomical site at more than one time
for which there is no other explanation
30. INVESTIGATIONS
• No specific diagnostic test for multiple
sclerosis.
• Diagnosis is mainly clinical & tests are
done to confirm the diagnosis and to
exclude other conditions.
• Investigations can also predict prognosis
after first episode.
31. INVESTIGATIONS IN A PT
SUSPECTED TO HAVE MS
• Exclude other structural disease & identify
plaques of demyelination:
MRI , Myelography
• Demonstrate other sites of involvement:
MRI ,evoked potentials
• Demonstrate inflammatory nature:
CSF examination (oligoclonal bands)
• Exclude other conditions:
CXR, ACE levels, serum B12, antinuclear &
antiphospholipid antibodies.
36. ACUTE RELAPSE
Pulses of high-dose methylprednisolone either
IV 1gm daily for 3 days or orally 500mg daily for
5 days, shorten the duration of relapse but do
not affect long-term outcome. So long use of
steroids should be avoided.
37. PREVENTING RELAPSES
• Immunosuppressive agents like
azathioprine reduces relapse rate.
• Subcutaneous or IM interferon beta-1a/b
reduces the number of relapses by about
30% with a small effect on longterm
disability
• Glatiramer acetate has similar effects.
39. NON SPECIFIC THERAPIES
• Special diets including gluten free or
linoleic acid supplements
• Hyperbaric oxygen
are being used but of no proven
benefit.
42. PROGNOSIS
• About 15% of those having an attack of
•
•
•
•
demyelination do not suffer any more events.
If optic neuritis is initial manifestation, there may
be no recurrence.
In relapsing remitting disease, there are on
average 1- 2 relapses every 2 years.
Approximately 5% of patients die within 5yrs of
onset.
33% patients are dependant by 10yrs rising to
50% after 15 yrs.