MS and demyelinating diseases Flashcards

1
Q

What is the typical age and gender when MS begins?

A

ages 20 - 40 , female:male 2:1

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2
Q

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

A

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

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3
Q

Where is the prevalence of MS the highest?

A

furtherst away from the equator (both hemispheres)

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4
Q

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

A

antibodies to KIR4.1 (potassium channel ab)

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5
Q

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

A

Demyelinating plaques

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

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6
Q

Is MS a purely inflammatory demyelinating disease?

A

No, axonal loss occurs even at early stages

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7
Q

What can be clinical signs of MS? (13)

A
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
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8
Q

What four clinical types of MS exists?

A

Remitting-relapsing
Sexondary progressive
Primary progressive
Progressive-relapsing

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9
Q

What can be triggers for MS relaps?(4)

A

viral infections
bacterial infections
3 months - post partum
stress?

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10
Q

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

A
optic neuritis
brainstem syndrome
spinal cord syndrome
transverse myelitis
multifocal lesions
cerebral lesions
lhermitte's sign
sensory sympt
bladder dysfynction
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11
Q

What is needed to diagnose MS?

A

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)

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12
Q

What can be seen in CSF in MS?

A

oligoclonal bands

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13
Q

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

A
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
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14
Q

What can indicate worse prognosis of MS?(2)

A
  • shorter first attack interval

- incomplete recovery from first attack

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15
Q

How is a MS relapse treated?

A

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

OBS avoid in first trimester and breastfeeding

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16
Q

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

A
  • Interferon Beta1 a/b
  • Glatimer acetate
  • Natalizumab (alpha4integrin ab) - relapsing/remitting
  • Mitoxantrone - secondary progressive
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17
Q

What is the mechanism of Mitoxantrone?(2)

A

Topoisomerase inhibitor

intercalating agent

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18
Q

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

A
Baclofen (GABAb agonist)
Tizanidine (alpha 2 agonist)
Tetrazepam
Diazepam
Botulinum toxin
physiotherapy
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19
Q

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

A

Amantadine(NMDA antag, dopa reuptake, dopa release)

Modafinil (dopamine reuptake inhibitor)

20
Q

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

A

TCAs (amitryptiline)

SSRIs (Fluoxetine)

21
Q

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

A
Acute
 - Carbamazepine
 - Benzodiazepines
 - Baclofen
Chronic
 - Amitryptiline
 - Carbamazepine
 - Baclofen
 - Physiotherapy
22
Q

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

A

oxybutinine (anticholinergic)

tolterodine (antimuscarinic)

23
Q

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

A

self-cathetherization

distigmine (blocks ACHE)

24
Q

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

A

Terazosine (alpha 1 block)
Doxazosine
Tamsulozine

25
Q

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

A
Carbamazepine
Clonazepam
Primidone (barbituate)
Propanolol
Surgery
(Thalamotomy, deep brain stimulation)
26
Q

What are used to manage chronic vertigo in MS?

A

betahistine (Histamine 3 antag, H1 agonist)

27
Q

What are used to manage sexual dysfunction in MS?

A

Sildenafil

28
Q

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

A

anticonvulsants

29
Q

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

A

viral infection

vaccination

30
Q

What is ADEM?

A

Acute/subacute disorder
multifocal symptomatology
immunological
response to immunosuppression

31
Q

What are the clinical features of ADEM?(7)

A
prodromal symptoms
altered consciousness/confusion
ataxia
bilateral optic nerve damage
aphasia
spinal cord-related signs
dysarthria, dysphagia
32
Q

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

A

MRI:
multiple hyperintense T2 leisons
edeme
affected gray matter(thalamus, basal ganglia)

CSF:
lymphocytes increased
protein increased
oligoclonal bands

33
Q

What is the treatment for ADEM?(4)

A

Methylprednisone
Plasmapheresis
IVIG
Mitoxantrone

50-70% recover

34
Q

What is transvere myelitis?

A

ACUTE focal inflammatory disorder of spinal cord
motor,sensory, autonomic disturbances

prognosis 1/3 rule

35
Q

What are the clinical features of transverse myelitis?

A

UMN syndrome, sensory level, autonomic disturbance

36
Q

What are the causes of transverse myelitis?(4)

A

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

37
Q

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

A

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

38
Q

What is the treatment of transverse myelitis?(4)

A

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

39
Q

What is neuromyelitis optica?(NMO)

A

Chronic inflammatory demyelinating disorder

with severe recurrent myelitis and optic neuritis

40
Q

What gender and race are more commonly affected by NMO?

A

Females (9x), Asians

41
Q

Why does NMO sometimes cause dysphagia and dysarthria?

A

cervical lesion extends to brainstem

42
Q

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

A

NMO-igG autoantibodies against aquaporin4

43
Q

What examinations should be performed in NMO?(3)

And what do they show?

A

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

44
Q

What is the treatment of NMO?

A

Relapses: IV methylprednison / plasmapheresis
Prevention: Azathioprine+prednisone, rituximab,IVIG

OBS: interferons/glatimers may be even harmful!!

45
Q

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

A

Demyelinating diseases!