Weakness

Question 1

isolated progressive weakness, involving a number of limbs progressively, with muscle wasting

Answer 1

ALS

Question 2

name 3 causes of subacute/acute onset of weakness

Answer 2

• Guillian Barre syndrome
• Cauda equina
• Transverse myelitis

Question 3

condition that can present with both peripheral weakness + loss of peripheral sensation

Answer 3

GBS

Question 4

investigations for a patient presenting with peripheral weakness + loss of sensation

Answer 4

FBC, U+E, Ca, LFTs, Phosphate, Magnesium, CRP, ESR, CXR

Question 5

How can GBS affect breathing?

Answer 5

by causing neuromuscular ventilatory failure by causing muscular weakness of diaphragm

Question 6

what position makes neuromuscular ventilatory failure worse?

Answer 6

lying down

Question 7

examination signs of worsening GBS

Answer 7

• worsening neck weakness
• SoB worse on lying flat
• reduced chest excursion on deep inspiration
• CO2 retention flap

Question 8

signs of GBS related autonomic instability

Answer 8

tachycardia

BP changes

Question 9

investigation for GBS

Answer 9

LP

Question 10

landmarks for LP

Answer 10

between lateral aspects of iliac crest at L4

Question 11

complications of LP

Answer 11

• cerebellar herniation
• pain
• headache
• bleeding
• infection
• CSF leakage
• subarachnoid epidermal cyst

Question 12

CSF finding in GBS

Answer 12

normal cell count + elevated protein level

= cytoalbuminologic dissociation

Question 13

Presentation of transverse myelitis

Answer 13

rapid onset of weakness, bladder/bowel and sensory alterations

Question 14

frequency of FVC measurements in GBS

Answer 14

4-hourly

Question 15

GBS is…

Answer 15

immune mediated demyelination of the peripheral nervous system

Question 16

antibody found in 25% patients with GBS

Answer 16

Anti-GM1 antibody

Question 17

autonomic involvement in GBS

Answer 17

urinary retention
\+ 
labile BP 
\+ 
constipation

Question 18

Poss CN involvement in GBS

Answer 18

• diplopia
• ophthalmoplegia
• facial weakness

Question 19

triggers of GBS

Answer 19

• EBV, HIV, CMV,
• Campylobacter jejuni
• E. Coli
• Hodgkin’s disease
• Pregnancy

Question 20

examination findings in GBS

Answer 20

• ascending weakness
• areflexia
• distal paraesthesia
• slurred speech (if oropharyngeal muscle weakness)

Question 21

Investigations for ascending weakness:

Answer 21

• Nerve conduction studies
• LP
• LFTs
• Spirometry
• Antibody

Question 22

Management of GBS

Answer 22

• Thromboprophylaxis
• IVIG or Plasma exchange
• FVC monitoring
• Neurorehabilitation

Question 23

Triad in Miller fisher syndrome

Answer 23

• ophthalmoplegia
• ataxia
• areflexia

Question 24

CIDP is …

Answer 24

chronic, progressive relapsing remitting course of GBS; if the symptoms progress after 6 weeks

Question 25

when do symptoms in GBS peak?

Answer 25

1-4 weeks after starting

Question 26

how does plasma exchange hep to treat GBS?

Answer 26

In GBS your plasma contains harmful proteins, this treatment removes your blood, separates out the plasma and combines your remaining blood with a donors plasma which is then put back into you

Question 27

majority fully recover by how long?

Answer 27

6-12 months

Question 28

Myasthenia Gravis is ...

Answer 28

chronic autoimmune disorder of the post-synaptic membrane at the NMJ in skeletal muscle

Question 29

MG antibodies ?

Answer 29

antibodies to Acetylcholine receptors

Question 30

Differentials of weakness?

Answer 30

- Lambert-Eaton syndrome - GBS - Transverse Myelitis

Question 31

often the first symptoms to appear in MG are?

Answer 31

ocular symptoms

Question 32

Muscle weakness in MG is dependant on what?

Answer 32

fatiguability

Question 33

what is muscle fatiguability?

Answer 33

muscles become progressively weaker through periods of activity + diurnal variation

Question 34

Levator palpebrae superioris weakness in MG causes

Answer 34

ptosis/dopping eyelids

Question 35

double vision in MG is caused by>

Answer 35

extraocular muscle weakness

Question 36

speech changes in MG and why?

Answer 36

nasal speech: dysarthria; due to bulbar muscle weakness

Question 37

transverse smile in MG caused by

Answer 37

facial muscle weakness

Question 38

difficulty getting out a chair in MG caused by

Answer 38

proximal muscle weakness

Question 39

specific examination tests in MG (2)

Answer 39

asking patient to count to 50 and listen to voice become quiter (positive finding) + ask patient to stare at your finger held above their forehead

Question 40

investigations for MG

Answer 40

- CT thorax (thyoma) - EMG - autoantibodies - Pulmonary function tests

Question 41

symptomatic treatment of MG

Answer 41

pyridostigmine

Question 42

1st line treatment of MG

Answer 42

- Pyridostigmine - Thymectomy +/- Immunosuppressants: corticosteroids, rituximab

Question 43

Pyridostigmine is a...

Answer 43

anticholinesterase

Question 44

Myasthenic crisis is...

Answer 44

complication of MG: characterised by worsening muscle weakness resulting in respiratory failure requiring intubation + mechanical ventilation

Question 45

management of Myasthenic Crisis

Answer 45

IVIG or plasmaphoresis

Question 46

indication for mechanical ventilation

Answer 46

FVC < 15 ml/kg

Question 47

complications of MG

Answer 47

- respiratory failure - impaired swallowing (following mechanical ventilation) - acute aspiration - secondary pneumonia - plasma exchange induced adverse reactions

Question 48

Cauda equina examination findings

Answer 48

- reduced perianal sensation - reduced anal tone - urinary retention - bilateral leg weakness - areflexia

Question 49

what FVC is an indication for intubation>

Answer 49

< 15 ml/kg