MS and demyelinating diseases

Question 1

What is the typical age and gender when MS begins?

Answer 1

ages 20 - 40 , female:male 2:1

Question 2

The excact trigger of MS is uknown, but what are some possible triggers?(4)

Answer 2

viruses /other infectious - EBV, HHV-6, HTLV-1
environmental toxins
lack of nutrients - inverse vit D/sunlight correlation
genetic - IL2/7 R

Question 3

Where is the prevalence of MS the highest?

Answer 3

furtherst away from the equator (both hemispheres)

Question 4

What antibody is found in 47% of people with MS?

Answer 4

antibodies to KIR4.1 (potassium channel ab)

Question 5

What pathology can be seen in CNS white matter in patients with MS?

Answer 5

Demyelinating plaques

- predilection for periventricular regions, brainsterm, optic nerves, spinal cord

Question 6

Is MS a purely inflammatory demyelinating disease?

Answer 6

No, axonal loss occurs even at early stages

Question 7

What can be clinical signs of MS? (13)

Answer 7

OPTIC NEURITIS (50% early, 75% late)
Limb weakness
Vision disturbances
Sensory loss
loss of balance/coordination
Sphincter disturbances
Cognitive decline
spasticity (70%)
tremor
fatigue
depression
sexual dysfunction
pain

Question 8

What four clinical types of MS exists?

Answer 8

Remitting-relapsing
Sexondary progressive
Primary progressive
Progressive-relapsing

Question 9

What can be triggers for MS relaps?(4)

Answer 9

viral infections
bacterial infections
3 months - post partum
stress?

Question 10

clinical isolated syndrome is usually the first clinical event in MS. What are the subtypes?(9)

Answer 10

optic neuritis
brainstem syndrome
spinal cord syndrome
transverse myelitis
multifocal lesions
cerebral lesions
lhermitte's sign
sensory sympt
bladder dysfynction

Question 11

What is needed to diagnose MS?

Answer 11

Damage to the CNS which is:

1. Disseminated in time (2 relapses)
   and
2. Disseminated in space (2 locations)

evidence in time/space can also be on MR (T2)
(lesions in brainstem/spinal cord doesn’t count)

Question 12

What can be seen in CSF in MS?

Answer 12

oligoclonal bands

Question 13

What other diseases can look like MS clinically and on MRI?(11)

Answer 13

Acute disseminated encephalomyelitis
Progressive multifocal leukoencephalopathy
Transvere myelitis
Neuromyelitis optica (Devic syndrome)
small vessel disease
brain vasculitis
metastasis (melanoma)
Neuroborreliosis
Neurosarcoidosis
Bechet disease
central pontine myelinolysis

Question 14

What can indicate worse prognosis of MS?(2)

Answer 14

• shorter first attack interval

- incomplete recovery from first attack

Question 15

How is a MS relapse treated?

Answer 15

corticosteriods
- methylprednisone 1g I.V for 3-5 days

OBS avoid in first trimester and breastfeeding

Question 16

What disease-modyfing drugs are used in MS?(4)

Answer 16

• Interferon Beta1 a/b
• Glatimer acetate
• Natalizumab (alpha4integrin ab) - relapsing/remitting
• Mitoxantrone - secondary progressive

Question 17

What is the mechanism of Mitoxantrone?(2)

Answer 17

Topoisomerase inhibitor

intercalating agent

Question 18

What are used to manage spasticity in MS?(6)

Answer 18

Baclofen (GABAb agonist)
Tizanidine (alpha 2 agonist)
Tetrazepam
Diazepam
Botulinum toxin
physiotherapy

Question 19

What are used to manage chronic fatigue in MS?(2)

Answer 19

Amantadine(NMDA antag, dopa reuptake, dopa release)

Modafinil (dopamine reuptake inhibitor)

Question 20

What are used to manage depression in MS?(2)

Answer 20

TCAs (amitryptiline)

SSRIs (Fluoxetine)

Question 21

What are used to manage pain in MS? (7)

Answer 21

Acute
 - Carbamazepine
 - Benzodiazepines
 - Baclofen
Chronic
 - Amitryptiline
 - Carbamazepine
 - Baclofen
 - Physiotherapy

Question 22

What are used to manage “autonomic bladder” in MS?(2)

Answer 22

oxybutinine (anticholinergic)

tolterodine (antimuscarinic)

Question 23

What are used to manage atonic bladder in MS?(2)

Answer 23

self-cathetherization

distigmine (blocks ACHE)

Question 24

What are used to manage atonic bladder and hyperactive sphincters in MS?(3)

Answer 24

Terazosine (alpha 1 block)
Doxazosine
Tamsulozine

Question 25

What are used to manage tremor in MS?(6)

Answer 25

``` Carbamazepine Clonazepam Primidone (barbituate) Propanolol Surgery (Thalamotomy, deep brain stimulation) ```

Question 26

What are used to manage chronic vertigo in MS?

Answer 26

betahistine (Histamine 3 antag, H1 agonist)

Question 27

What are used to manage sexual dysfunction in MS?

Answer 27

Sildenafil

Question 28

What are used to manage seizures and other paroxysmal symptoms in MS?

Answer 28

anticonvulsants

Question 29

What agents might be a trigger of Acute disseminated encephalomyelitis(ADEM)

Answer 29

viral infection | vaccination

Question 30

What is ADEM?

Answer 30

Acute/subacute disorder multifocal symptomatology immunological response to immunosuppression

Question 31

What are the clinical features of ADEM?(7)

Answer 31

``` prodromal symptoms altered consciousness/confusion ataxia bilateral optic nerve damage aphasia spinal cord-related signs dysarthria, dysphagia ```

Question 32

What examinations (2) should be perfomed in ADEM, and what will they show?

Answer 32

MRI: multiple hyperintense T2 leisons edeme affected gray matter(thalamus, basal ganglia) CSF: lymphocytes increased protein increased oligoclonal bands

Question 33

What is the treatment for ADEM?(4)

Answer 33

Methylprednisone Plasmapheresis IVIG Mitoxantrone 50-70% recover

Question 34

What is transvere myelitis?

Answer 34

ACUTE focal inflammatory disorder of spinal cord motor,sensory, autonomic disturbances prognosis 1/3 rule

Question 35

What are the clinical features of transverse myelitis?

Answer 35

UMN syndrome, sensory level, autonomic disturbance

Question 36

What are the causes of transverse myelitis?(4)

Answer 36

idiopathic 2/3 infection related collagenosis related secondary to MS, ADEM, NMO

Question 37

What needs to be excluded in the diagnosis of transverse myelitis?

Answer 37

exclude cord compression - MRI Exclude primary MS, ADEN, NMO also confirm inflammatory CSF markers

Question 38

What is the treatment of transverse myelitis?(4)

Answer 38

IV methylprednisone - then oral predn. Plasmapheresis cyclophosphamide sympt treatment + physiotherapy

Question 39

What is neuromyelitis optica?(NMO)

Answer 39

Chronic inflammatory demyelinating disorder | with severe recurrent myelitis and optic neuritis

Question 40

What gender and race are more commonly affected by NMO?

Answer 40

Females (9x), Asians

Question 41

Why does NMO sometimes cause dysphagia and dysarthria?

Answer 41

cervical lesion extends to brainstem

Question 42

What antibody are found in 85% of patients with NMO?

Answer 42

NMO-igG autoantibodies against aquaporin4

Question 43

What examinations should be performed in NMO?(3) | And what do they show?

Answer 43

MR: longitudinal T2 hyperintense lesions CSF: elevated neutrophils, sometimes oligoclonal bands Serum: NMO-IgG antibodies

Question 44

What is the treatment of NMO?

Answer 44

Relapses: IV methylprednison / plasmapheresis Prevention: Azathioprine+prednisone, rituximab,IVIG OBS: interferons/glatimers may be even harmful!!

Question 45

What are the major causes of neurological disabillity in young people?

Answer 45

Demyelinating diseases!