9.7 Autoimmune of NS

Question 1

What is MS?

Answer 1

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

Question 2

What is an environment factor of MS?

Answer 2

Vitamind D levels

- Further away from the equator has higher levels of MS

Question 3

What occurs in MS?

Answer 3

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

Question 4

What is the immune activation in MS?

Answer 4

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

Question 5

What is affected in MS?

Answer 5

White matter tracts

Question 6

What are the presenting complaints of MS?

Answer 6

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

Question 7

What is the clinical course of MS?

Answer 7

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

Question 8

What are the unfavourable prognosis factors of MS?

Answer 8

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

Question 9

What is the diagnosis of MS?

Answer 9

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

Question 10

What are the MS treatment aims?

Answer 10

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

Question 11

What is acute disseminated encephalitis?

Answer 11

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

Question 12

What are the investigations and treatment in ADEM?

Answer 12

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

Treat: high dose methylprednisolone

Question 13

What is Neuromyelitis optica?

Answer 13

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

Question 14

What is the diagnosis of Neuromyelitis optica

Answer 14

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

Question 15

What will you see in Neuromyelitis optica?

Answer 15

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)

Question 16

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

Answer 16

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

Question 17

What is Guillian Barre?

Answer 17

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

Question 18

What is the pathogenesis of Guillan Barre?

Answer 18

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

Question 19

What are the triggers for Guillan Barre?

Answer 19

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

Question 20

Presenting symptoms of Guillan Barre?

Answer 20

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

Question 21

Investigations for Guillan-Barre

Answer 21

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

Question 22

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

Answer 22

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

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

Question 23

What is the treatment for Guillan-Barre?

Answer 23

IVIG

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

Question 24

What is chronic inflammatory demyelinating polyradiculoneuropathy?

Answer 24

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

Question 25

When will you have Anti-Hu?

Answer 25

Encephalitis and neuropathy

Question 26

When will you have affected voltage gated K channels?

Answer 26

limbic encephalitis

Question 27

When will you have Anti-Yo (purkinje cells)

Answer 27

Cerebellar syndrome

Question 28

When will you have Anti-Ri

Answer 28

Opsoclonus and myoclonus

Question 29

When will you have Anti-GAD?

Answer 29

Stiff man syndrome

Question 30

When will you have anti-NMDA?

Answer 30

Anti-NMDA syndrome