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Flashcards in OHCM - MS Deck (55):
1

Demyelination in MS, heals ...?

POORLY - Causing relapsing and remitting symptoms.

2

Prolonged demyelination causes ...?

Causes axonal loss and clinically progressive symptoms.

3

Prevalence of MS:

1. Common in temperate areas (England >42/100.000, Scotland 200/100.000).
2. Rarer in Black Africa/Asia).

4

Lifetime UK risk:

1/1.000

5

Mean age of onset:

30yrs.

6

Female:Male ratio:

3:1.

7

Early exposure to sunlight ...?

VitD is important, and vitD status relates to prevention of MS, and fewer symptoms and fewer new lesions on MRI in established MS.

8

Presentation:

Usually MONOsymptomatic.
1. Unilateral optic neuritis (pain on eye movement and rapid decr. central vision).
2. Numbness or tingling in the limbs.
3. Leg weakness.
4. Brainstem or cerebellar symptoms (diplopia, ataxia).

9

Presentation - Other signs:

Symptoms may worsen with heat (eg hot bath) or exercise.
--> Rarely polysymptomatic.

10

Progression:

1. Early on, relapses (which can be stress-induced) may be followed by remission and full recovery.
2. With time, remissions are incomplete, so disability accumulates.
3. Steady progression of disability from the outset also occurs, while some patients experience NO PROGRESSIVE DISABLEMENT at all.

11

Poor prognostic signs:

1. Older males.
2. Motor signs at onset.
3. Many relapses early on.
4. Many MRI lesions.
5. Axonal loss.

12

Diagnosis is ...?

CLINICAL - No test is pathognomonic.

13

Diagnosis requires lesions ...?

Disseminated in time and space, unattributable to other causes - Thus, after 1st episode further evidence is needed.

14

Early diagnosis and treatment ...?

Reduces relapse rates and disability.

15

Diagnosis - MRI is ... but not ...?

Sensitive but NOT SPECIFIC for plaque lesions (McDonald criteria).

16

...% presenting with an MS-like 1st episode and consistent MRI lesions go to develop MS.

90%.

17

MRI may also ...?

EXCLUDE other causes, eg cord compression.

18

Diagnosis - CSF:

Oligoclonal bands of IgG on electrophoresis that are NOT PRESENT in serum suggest CNS inflammation.

19

Diagnosis - Evoked potentials:

Delayed visual, auditory, and somatosensory evoked potentials.

20

Devic syndrome:

NMO-IgG antibodies are highly specific for Devic's syndrome.
(NMO = Neuromyelitis optica).

21

Diagnosis of MOG and MBP antibodies:

MOG and MBP antibodies in those with a single MS-like clinical lesion can predict time to conversion to definite MS.

22

Rx:

1. Encourage a happy, stress-free life if possible (decr. stress can reduce development of new lesions).
2. Minimize disability.
3. If poor diet or decr. sun exposure, give vitD to achieve serum 25(OH)D levels of >50mmol/L.

23

Rx: Steroids:

1. Methylprednisolone, eg 1/2-1g/24h IV/PO for

24

Rx - Interferons (IFN-1beta and IFN-1alpha):

1. Decreases relapses by 30% in active relapsing-remitting MS and decrease lesion accumulation on MRI.
2. Their power to delay disability is MODEST at best, as is their role in PROGRESSIVE MS.

25

Rx - Interferon side effects:

1. Flu symptoms.
2. Depression.
3. Abortion.

26

Rx - Interferon - New gadolinium-enhancing lesions on IFN correlate with ...?

Severe disability 15yrs later.

27

Rx - Monoclonal antibodies:

1. Alemtuzumab.
2. Natalizumab.

28

Rx - Alemtuzumab:

1. Acts against T cells in relapsing-remitting MS.
2. 2 trials show its better than IFN.

29

Rx - Alemtuzumab - Side effects:

1. Infections, while the immune system reconstitutes itself.
2. Autoimmune disease (thyroid, skin, kidney).

30

Rx - Natalizumab:

1. Acts against VLA-4 receptors that allow immune cells to cross the BBB.
2. Decreases relapses in relapsing-remitting MS by 68% and decrease MRI lesions by 92%.

31

Rx - Natalizumab - Side effects:

1. PML by JC.
2. Antibody-mediated resistance.

32

Rx - Non-immunosuppressives:

1. Glatiramer.
2. Mitoxantrone (doxorubicin analogue).

33

Rx - Mitoxantrone:

Helps in secondary progressive MS - Safety is an issue.

34

Rx - Other drugs:

Azathioprine may be as good as IFNs for relapsing-remitting MS and is 20x cheaper.

35

Rx - Primary progressive MS:

There are no good drugs for primary progressive MS.

36

Palliation - Spasticity:

1. Baclofen 5-25mg/8h PO.
2. Diazepam 5mg/8-24h PO (addictive).
3. Dantrolene 25mg/24h (max 100mg/6h).
4. Tizanidine 2mg/24h PO - Incr. every 4d in steps of 1mg/12h (max 9mg/6h).
5. Endocannabinoid system modulation (Sativex) has a role.

37

Palliation - Tremor:

Botulinum toxin type A injections improve arm tremor and functioning.

38

Palliation - Urgency/frequency:

1. If post-micturition residual urine >100mL --> Teach intermittent seld-catheterization.
2. If

39

Clinical features - Sensory:

1. Dysaesthesia.
2. Pins and needles.
3. Decr. vibration sense.
4. Trigeminal neuralgia.

40

Clinical features - Motor:

1. Spastic weakness.
2. Myelitis.

41

Clinical features - Sexual/GU:

1. Erectile dysfunction.
2. Anorgasmia.
3. Urine retention.
4. Incontinence.

42

Clinical features - GI:

1. Shallowing disorders.
2. Constipation.

43

Clinical features - Eye:

1. Diplopia.
2. Hemianopia.
3. Optic neuritis.
4. Visual phenomena (eg on exercise).
5. Bilateral internuclear ophthalmoplegia.
6. Pupil defects.

44

Clinical features - Cerebellum:

1. Trunk and limb ataxia.
2. Intention tremor.
3. Scanning (ie monotonous speech).
4. Falls.

45

Clinical features - Cognitive/visuospatial decline:

1. A big cause of unemployment.
2. Accidents/isolation.
3. Amnesia.
4. Up or down mood (avoid ECT).
5. Decr. executive functioning.

46

Clinical features that are RARE in MS:

1. Fever.
2. Malaise.
3. Nausea.
4. Vomiting.
5. Positional vertigo.
6. Seizures.
7. Aphasia.
8. Meningism.
9. Bilateral optic neuritis.
10. CSF leukocytosis and CSF incr. protein.
--> May suggest non-MS recurrent demyelinating disease, eg vasculitis or sarcoidosis.

47

Transverse myelitis is indicated by ...?

Loss of motor, sensory, autonomic, reflex, and sphincter function below the level of a lesion indicates TRANSVERSE MYELITIS.
(Longitudinal myelitis may also occurs).

48

Devic's syndrome:

Neuromyelitis optica - NMO: An MS-variant with:
1. Transverse myelitis.
2. Optic atrophy.
3. NMO-IgG antibodies.

49

Lhermitte's sign:

Neck flexion causes "electric shocks" in trunks/limbs. Also +ve in:
1. Cervical spondylosis.
2. Cord tumors.
3. Subacute combines degeneration of the cord (down B12).

50

Lhermitte's sign along with ...?

1. Dysesthetic pain.
2. Trigeminal neuralgia.
3. Painful tonic MS spasms.
comprises the MS-related central pain disorders.

51

Optic neuritis symptoms:

1. Decr. acuity.
2. Temporary blindness +/- complex hallucinations of faces (Charles Bonnet syndrome - rare).

52

Optic neuritis symptoms - Uhthoff's phenomenon:

Decr. vision on exercise, hot meals, hot baths.

53

Optic neuritis symptoms - Pulfrich effect:

1. Unequal eye latencies.
2. Causing disorientation in traffic as straight trajectories seem curved and distances are misjudged on looking sideways.

54

Argyle Robertson-type pupil is rare in MS - DDx:

1. Syphilis.
2. DM.
3. MS.
4. Sarco.
--> Lesion in or near the Edinger-Westphal nucleus.

55

Main problem with MS:

Discrete plaques of demyelination occur at multiple CNS sites, from T-cell mediated immune response.