A23. Multiple sclerosis

Question 1

Multiple sclerosis Definition

Answer 1

Chronic, progressive, multifocal, autoimmune inflammatory disease of the CNS.

Question 2

Multiple sclerosis is more common in which gender

Answer 2

Women:men = 2:1

Question 3

age onset of Multiple sclerosis

Answer 3

Disease of young adult, starts in 30-40s

Question 4

in n which region is Multiple sclerosis more frequent

Answer 4

More frequent in the northern hemisphere

Question 5

etiology of Multiple sclerosis

Answer 5

A. Genetic susceptibility: HLA, some increased risk in relatives
B. Environmental factors: Viruses, geographic latitude (more towards the poles), vitamin D

Question 6

Environmental factors that could cause Multiple sclerosis

Answer 6

• Viruses,
• geographic latitude (more towards the poles),
• vitamin D

Question 7

pathology Multiple sclerosis

Answer 7

• T-cells are activated in the periphery, then cross-react with parts of myelin in the CNS →
  Demyelination and secondary axon degeneration.
  The demyelinated regions are called plaques.
  ● Active plaques are characterized by blood-brain barrier dysfunction, perivascular infiltration of
  lymphocytes and plasma cells and segmental demyelination.
  ● In chronic plaques, scar tissue is formed (sclerosis) due to astrocyte proliferation

Question 8

Active plaques in multiple sclerosis are characterized by

Answer 8

• blood-brain barrier dysfunction,
• perivascular infiltration of lymphocytes and plasma cells
• and segmental demyelination.

Question 9

what happens In chronic plaques that occur in multiple sclerosis

Answer 9

scar tissue is formed (sclerosis) due to astrocyte proliferation

Question 10

what is meant by plaques in multiple sclerosis

Answer 10

The demyelinated regions are called plaques
(T-cells are activated in the periphery, then cross-react with parts of myelin in the CNS →
Demyelination and secondary axon degeneration)

Question 11

Pathological types of multiple sclerosis

Answer 11

a. T-cell and macrophage mediated inflammation
b. B-cell and complement mediated inflammation
c. Oligodendrocyte destruction, axonal lesion
d. Primary oligodendrocyte dystrophy

Question 12

Main points about Multiple sclerosis

Answer 12

• Demyelination
• Inflammation
• Secondary axonal damage
• Inflammatory processes → Relapses
• Secondary axonal damage → Brain atrophy → Deficits (delayed manifestation)

Question 13

Secondary axonal damage in multiple sclerosis leads to

Answer 13

→ Brain atrophy → Deficits (delayed manifestation)

Question 14

Possible symptoms of Multiple sclerosis

Answer 14

• Optic neuritis - Blurry vision, pain on movement, often first sign
• Sensory - Paresthesias, numbness, tingling, always distribution specific for central cause
• Motor symptoms - Paraparesis, hemiparesis, monoparesis, tetraparesis
• Brainstem symptoms - Gaze palsy, internuclear ophthalmoplegia (INO), trigeminal pain
• Cerebellar symptoms - Limb/trunk ataxia, dysarthria, nystagmus, tremor, coordination/balance
  problem
• Autonomic symptoms - Urge incontinence (detrusor hyperactivity), detrusor-sphincter dyssynergia
• Cognitive symptoms, subcortical dementia
• Paroxysmal symptoms (may occur several hundred times a day, short duration) - Lhermitte’s
  sign, trigeminal neuralgia, dystonia, hemiataxia
• Aspecific symptoms - Fatigue, depression

Question 15

which tracts are involved in multiple sclerosis?
which are more affected?

Answer 15

May involve all tracts of CNS, but longer tracts are more affected.

Question 16

which symptom often manifests first in multiple sclerosis

Answer 16

Optic neuritis -
* Blurry vision
* pain on movement
often first sign

Question 17

Sensory symptoms in multiple sclerosis

Answer 17

• Paresthesias,
• numbness,
• tingling,
• always distribution specific for central cause

Question 18

Motor symptoms in multiple sclerosis

Answer 18

• Paraparesis,
• hemiparesis,
• monoparesis,
• tetraparesis

Question 19

Brainstem symptoms -in multiple sclerosis

Answer 19

• Gaze palsy,
• internuclear ophthalmoplegia (INO),
• trigeminal pain

Question 20

Cerebellar symptoms -in multiple sclerosis

Answer 20

• Limb/trunk ataxia,
• dysarthria,
• nystagmus,
• tremor,
• coordination/balance problem

Question 21

Autonomic symptoms - in multiple sclerosis

Answer 21

• Urge incontinence (detrusor hyperactivity),
• detrusor-sphincter dyssynergia

Question 22

Paroxysmal symptoms in multiple sclerosis

Answer 22

(may occur several hundred times a day, short duration) -
* Lhermitte’s sign,
* trigeminal neuralgia,
* dystonia,
* hemiataxia

Question 23

Lhermitte’s sign

Answer 23

(Barber chair phenomenon)
* is a transient sensation of an electric shock that extends down the spine and extremities
* upon flexion and/or movement of the neck.

Question 24

Diagnostic criteria of multiple sclerosis

Answer 24

McDonald criteria (2017) →
* Prove that inflammation is chronic (dissemination in time) and
* widespread (dissemination in space)

Question 25

McDonald criteria (2017)

Answer 25

* for multiple sclerosis

Question 26

In case of zero attacks and lesions what additional criteria is needed for diagnosis of MS based on McDonald criteria

Answer 26

* One year of disease progression (retrospective/prospective) * and at least 2 out of 3 criteria: *dissemination in space in the brain *dissemination in space in the spinal cord based on 2 or more T2 lesions *Positive CSF

Question 27

In case of 1 attack, 1 lesion what additional criteria is need for diagnosis of MS

Answer 27

disseminatiton in space and time or await for further clinical attack implicating a different CNS site

Question 28

In case of 1 attack, 2 lesions what additional criteria is needed for diagnosis of MS

Answer 28

Dissemination in time on MRI or await for further clinical attack implicating a different CNS site

Question 29

In case of 2 or more attacks, and 1 lesion what additional criteria is needed for diagnosis of MS

Answer 29

Dissemination in space on MRI OR await for further clinical attack implicating a different CNS site

Question 30

In case of 2 or more attacks, and 2 or more lesions what additional criteria is needed for diagnosis of MS

Answer 30

None Clinical evidence alone will suffice

Question 31

First choice of diagnostic tool in Multiple sclerosis

Answer 31

MRI

Question 32

Diagnostic tools in Multiple sclerosis

Answer 32

* MRI (first choice) * CSF examination * Evoked potentials * Serology (differential)

Question 33

what is seen on MRI in multiple sclerosis

Answer 33

* typical distribution of demyelinated plaques around: *ventricles *juxtacortically *corpus callosum *brain, *spinal cord

Question 34

what is MRI used for in multiple sclerosis

Answer 34

* diagnosis * follow up

Question 35

CSF examinations in multiple sclerosis

Answer 35

* normal routine values, * OCBs (oligoclonal bands) * Not needed for diagnosis, but may support it. Characteristic to see **elevated IgG index or presence of oligoclonal gammopathy (OGP).**

Question 36

Evoked potentials use in diagnosis of MS

Answer 36

* VEP(Visual Evoked Potential) to investigate visual tract, * MEP(Motor evoked potentials ) * SEP( somatosensory evoked potention test)

Question 37

SEP Somatosensory evoked potential

Answer 37

* The **electrical response of the brain**, as measured by **electroencephalography**, in response to **electrical stimulation of superficial nerves** (e.g., tibial nerve). * studies the relay of body sensations to your brain and how the brain receives those sensations.

Question 38

Motor evoked potentials (MEPs)

Answer 38

* are electrical signals recorded from muscle tissue in response to stimulation of the motor cortex. * The stimulation may be magnetic or electrical * applied directly to the motor cortex or * applied transcranially through the skull

Question 39

Stimulus of Visual evoked potential (VEP) and in which disorders is it indicated

Answer 39

Light impulse Checkered patterns * Demyelinating disorders (e.g., optic neuritis in the case of multiple sclerosis)

Question 40

Somatosensory evoked potential (SEP) is indicated in

Answer 40

Demyelinating disorders (e.g., multiple sclerosis) and axonal damage

Question 41

Motor evoked potential (MEP) is indicated in

Answer 41

* Damage to the motor cortex and other motor neurons (e.g., amyotrophic lateral sclerosis) * Demyelinating disorders (e.g., multiple sclerosis) and axonal damage

Question 42

Serology in MS

Answer 42

(differential) - * Borreliosis, * Treponema, * anti-AQP4 (very specific for Devic’s disease)

Question 43

Devic disease or neuromyelitis optica) is also knows as

Answer 43

* **Neuromyelitis optica spectrum disorders** (NMOSD) * previously known as Devic disease or neuromyelitis optica) is also knows as

Question 44

definition of Devic disease

Answer 44

immune-mediated, chronic inflammatory disorders of the CNS that primarily affect the optic nerve and spinal cord Closely resembles the clinical appearance of MS