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Flashcards in 9.7 Autoimmune of NS Deck (30)
1

What is MS?

EPisodes of demyelination affecting areas of the CNS separated by space and time

2

What is an environment factor of MS?

Vitamind D levels
- Further away from the equator has higher levels of MS

3

What occurs in MS?

Unregulated imune system dysfunction causes inflammatory lesions in the CNS. These lesions cause demyleination, followed by a period of remyelination . With time this will lead to a permanent loss of myelin with secondary axonal loss

4

What is the immune activation in MS?

1. Autoreactive myelin specific T helper cells (Th1 and 17)
2. Failure of regulation when stimulated by antigens
3. Th1 secretes IFN gamma and Th17 secretes IL-17 which can adhere and cross the BBB
4. These activated T cells encounter myelin and activate migroglia
5. Microglia express class II molecules which further promotes T cells, microglia and PMN

5

What is affected in MS?

White matter tracts

6

What are the presenting complaints of MS?

Optic neuritis
Sensory symptoms in unusual pattern
Motor deficit (10%)
Cerebellar: ataxia, dysarthria
Dipolpoia
Transverse myelitis
Fatigue

7

What is the clinical course of MS?

Attacks and recovery with an increasing likelihood after each attack that they are left with some kind of impairment

Secondary progressive: no attacks but symptoms are getting worse

8

What are the unfavourable prognosis factors of MS?

Male
Older age at onset
Motor/cerebellar signs at onset
Short interval between initial and second attack
High relapse rate in early years
Incomplete remission after first relapse
Early disability
High lesion load early by MRI

9

What is the diagnosis of MS?

One clinical episode
Clinical episodes separated by time and space
MRI to look for plaques
CSF analysis

10

What are the MS treatment aims?

Reduce relapses
Prevent permanent disability
Reduce disability from acute attacks
Symptom treatment

11

What is acute disseminated encephalitis?

T cell attacking the myelin after a viral illness or vaccination and is most common in children
Lesions will appear like MS but all of the same age

12

What are the investigations and treatment in ADEM?

CSF: raised WCC and protein
Evidence of infection
Exclude other causes
Follow risk MS

Treat: high dose methylprednisolone

13

What is Neuromyelitis optica?

inflammation and demyelination of the optic nerve and the spinal cord
Caused by antibodies against aquaporin-4

14

What is the diagnosis of Neuromyelitis optica

Optic neuritis
Acue myelitis
2 of: brain MRI at onset not meeting MS criteria, Continuous lesion over >3 veretebral segments, NMO-IgG seropositive

15

What will you see in Neuromyelitis optica?

Long cervical lesions (>3 segments)
Symmetrical cerebral lesions
Brainstem with extension to thalami
Acute attacks that respond to plasmapharesis but not steroids
Long term Rx with immunosuppression (prednisolone, azathioprine and rituximab)

16

What is primary cerebral vasculitis and who dos it affect and what is the presentation?

inflammation of the blood vessels of the brain and sometimes spinal cord
Affects middle aged males
Present with headache, stroke events, cognitive impairment and seizures

17

What is Guillian Barre?

Acute post infectious peripheral nauropathy which progresses for no more than 4 weeks - ascending paralysis the most common

18

What is the pathogenesis of Guillan Barre?

Antigen presentation, inflammatory actiation targeting myelin or axonal components, inflammatory response causing secondary axonal loss in primary demyelinating disease followed by remyelination and axonal regeneration

PERIPHERAL NERVES

19

What are the triggers for Guillan Barre?

Viral infection: campylobacter jejuni, cytomegalovirus, EBV, mycoplasma pneum
Vaccinations
Surgery
Unknown

20

Presenting symptoms of Guillan Barre?

Pain, numbness, parasthesia
Weakness LL > UL > face > respiratory
Areflexia (may have reflexes early)

21

Investigations for Guillan-Barre

CSF: cytoalbuminaemic dissociation, raised WCC, oligoclonal bands, protein
Nerve conduction studies
Urinary porphyins
ESR, CRP, ANA, HIV, drugs and toxins, MRI spinal cord, CXR, pulmonary function testing

22

What do you need to be wary of in Guillan-Barre?

Lung function - can lose respiratory muscles so ventilate at 15ml/kg of FVC

DVT management, cardaic monitoring, postural hypo/hypertension, pressure sore management

23

What is the treatment for Guillan-Barre?

IVIG
Treat complications: DVT prophylaxia, respiratory management, ICU support, Presure care, pain management

24

What is chronic inflammatory demyelinating polyradiculoneuropathy?

Infalmmatory neuropathy for at least 2 months. Sensory signs present in most cases and associated with paraproteins

25

When will you have Anti-Hu?

Encephalitis and neuropathy

26

When will you have affected voltage gated K channels?

limbic encephalitis

27

When will you have Anti-Yo (purkinje cells)

Cerebellar syndrome

28

When will you have Anti-Ri

Opsoclonus and myoclonus

29

When will you have Anti-GAD?

Stiff man syndrome

30

When will you have anti-NMDA?

Anti-NMDA syndrome