4.1 Guillain Barré Syndrome Flashcards

1
Q

Summarise the case

A
  • Complex critically ill paediatric patient
  • Severe neurological pathology with threatened airway and failure of
    ventilation needing urgent multidisciplinary input from senior clinicians
  • Parents are Jehovah’s Witnesses
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2
Q

What abnormality do you note in the blood results?

A
  • Normocytic anaemia
  • Platelets are high, depicting inflammation
  • Low magnesium
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3
Q

What are the causes of normocytic anaemia?

A
  1. Decreased production of normal-sized red blood cells
    (e.g. anaemia of chronic disease, aplastic anaemia)
  2. Increased production of HbS as seen in sickle cell disease
  3. Increased destruction or loss of red blood cells
    (e.g. haemolysis, post-haemorrhagic anaemia )
  4. An uncompensated increase in plasma volume
    (e.g. pregnancy, fluid overload)
  5. B2 (riboflavin) and B6 (pyridoxine) deficiency
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4
Q

What are the causes of hypomagnesaemia?

A
    • Decreased magnesium intake
      ° Starvation
      ° Alcohol dependence
      ° Total parenteral nutrition
    • Redistribution of magnesium from extracellular to intracellular space
      ° Treatment of diabetic ketoacidosis
      ° Alcohol withdrawal syndrome
      ° Refeeding syndrome
      ° Acute pancreatitis
    • Gastrointestinal magnesium loss
      ° Diarrhoea, vomiting, and nasogastric suction
      ° Hypomagnesaemia with secondary hypocalcaemia (HSH)
    • Renal magnesium loss
      ° Renal tubular defects
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5
Q

interpret the arterial blood gas.

A
  • Compensated respiratory acidosis
  • Type 2 respiratory failure
  • Normal glucose and lactate
  • Anion gap 21
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6
Q

What is anion gap? How do you calculate it?

A
    • Anion gap is the difference
      in the measured cations
      and the anions in serum.

° Measured cations: Na+, K+, Ca2+, and Mg2+

° Unmeasured cations:
serum proteins (normal) and paraproteins (abnormal)

° Measured anions: Cl−, H2Po4 −, HCo3 −

° Unmeasured anions: sulphates and some serum proteins

    • Used to determine the cause of metabolic acidosis
    • Expressed as mEq/L

_________________

  • Anion gap = (Na + K) − (Cl + HCo3)
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7
Q

What are the causes for a raised AG

A
    • Lactic acidosis/ketoacidosis/alcohol abuse
    • Toxins: methanol/aspirin/cyanide
    • Renal failure causes
      high anion gap acidosis by
      decreased acid excretion and
      decreased HCO3 − reabsorption.

Accumulation of sulphates, phosphates, urate,
and hippurate accounts for the high anion gap.

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8
Q

What is the significance of a high anion gap?

A
    • The anion gap is affected by
      changes in unmeasured ions.

A high anion gap indicates acidosis
(e.g. in uncontrolled diabetes,
there is an increase in ketoacids
i.e. an increase in unmeasured anions)

and a resulting increase in the anion gap.

    • Bicarbonate concentrations decrease
      in response to the need to buffer
      the increased presence of acids
      (as a result of the underlying condition).

The bicarbonate is consumed by the
unmeasured anion
(via its action as a buffer),

resulting in a high anion gap.

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9
Q

comment on the CSF analysis.

A

Increase protein in the absence of organisms
is called albumino cytological dissociation

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10
Q

Why is there increase in CSF protein?

A

The increase in CSF protein is due to
widespread inflammation of the nerve roots.

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11
Q

What would you expect the CSF glucose to be?

A

Normal—i.e. approx. 2/3 of plasma glucose

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12
Q

What is the differential diagnosis?

A
  • Guillain Barre Syndrome (GBS)
  • Myasthenia Gravis
  • Multiple Sclerosis
  • Transverse myelitis
  • Encephalitis
  • Meningitis
  • Space-occupying lesion
  • Sepsis
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13
Q

What is the likely diagnosis?

A

My diagnosis is Guillain Barre Syndrome. The history of prodromal infection
and the course of presentation favour my diagnosis. There is often history
of campylobacter or cytomegalovirus infections or vaccinations (influenza,
polio, rabies, and rubella).

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14
Q

What is the pathogenesis GBS?

A

It is postulated that the immune responses

directed towards the infecting organisms

cross-react with neural tissues

resulting in widespread segmental
demyelination of peripheral nerves.

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15
Q

How will you differentiate this from myasthenia gravis?

A

Differences in Myasthenia (the following are features of myasthenia):

    • Early involvement of muscle groups
      including extra-ocular, levator, pharyngeal jaw,
      neck, and respiratory muscles.

Sometimes presents without limb weakness

    • Excessive fatigability and variation
      of symptoms and signs throughout the
      day are common
    • Reflexes are preserved and sensory features,
      dysautonomia, and bladder dysfunction are absent
    • Electrophysiological study shows
      normal nerve conduction and presence
      of decremental response to repetitive nerve stimulation
    • EMG shows abnormal jitter and blocking
    • Edrophonium test is normally positive
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16
Q

How will you manage this case?

A

General—ICU care

  • ABC approach: 30% cases require ventilation
  • Temperature control
  • VTE prophylaxis
  • Pain relief
  • Feeding

___________________________

Specific

  • Steroids
  • Immunoglobulin G (IgG)
  • Plasma exchange
  • CSF filtration
17
Q

Discuss autonomic dysfunction in these patients.

A
  • Autonomic dysfunction is a major cause of
    morbidity and mortality, particularly in ventilated patients
  • May cause refractory orthostatic hypotension,
    paroxysmal hypertension,
    bradycardia, ventricular tachyarrhythmias,
    ileus and urinary retention
  • Autonomic dysfunction is of particular importance
    at the induction of anaesthesia.

Careful consideration should be given to the
use of suxamethonium and inotropic and vasopressor agents
may produce markedly atypical responses in heart rate and
blood pressure. Even tracheal suction may lead to significant
cardiovascular instability

18
Q

How do you decide when to intubate this patient?

A
    • Intubation should be performed on patients
      who develop any degree of respiratory failure
    • Clinical indicators for intubation include
      hypoxia,
      rapidly declining respiratory function,
      poor or weak cough, and suspected aspiration
    • Typically, intubation is indicated when
      the FVC is less than 15 mL/kg
19
Q

How would you intubate this child?

A
  • Avoid suxamethonium
  • Size 7 cuffed tube (Age/2 +12 cm at lips)
20
Q

What are the specific treatment options?

A
    • Plasma exchange:
      removes auto antibodies,
      immune complexes, etc.,
      and has shown to halve the recovery time
    • Immunoglobulins:
      easier and safer than,
      and equally effective as
      plasma exchange.
      Useful in unstable patients
    • Steroids:
      ineffective as monotherapy
    • Complement inhibitors:
      e.g. Eculizumab has been trialed.
    • CSF filtration
21
Q

What is the problem with igG use in this patient?

A
  • Jehovah’s parents may refuse this
22
Q

How do you deal with this situation?

A
  • Assess the child’s capacity—Gillick competence.
  • Involve hospitals Jehovah liaison group/legal service/social
    services.
23
Q

What are the problems encountered during his stay?

A

This condition needs long-term care and ventilatory support. This makes
way for the usual and common ICU-related problems on top of the
pathology-related complications

Non-pathology-related
* Infections—lines, chest, UTI, septicaemia, etc.
* DVT, pressure sores, and contractures
* Nutritional deficits
* Psychological

24
Q

What are the problems encountered during his stay?

A

This condition needs long-term care and ventilatory support. This makes
way for the usual and common ICU-related problems on top of the
pathology-related complications

Non-pathology-related
* Infections—lines, chest, UTI, septicaemia, etc.
* DVT, pressure sores, and contractures
* Nutritional deficits
* Psychological

Pathology-related
* Autonomic neuropathy
* Pain

25
Q

The ICU team has tried to do an early percutaneous tracheostomy, which
was unsuccessful due to technical reasons. An X-ray is done after the
procedure. See Figure 4.1.

comment on the chest X-ray.

A

pneumomediastinum

26
Q

Cause of pneumomediastinum

A

Traumatic intubation
or tracheostomy/NG insertion
as children have fragile soft tissue in trachea and oesophagus

27
Q

Should a percutaneous tracheostomy procedure be followed by X-ray to look for complications?

A

Immediate CXR after uncomplicated percutaneous tracheostomy performed
under bronchoscopic guidance rarely reveals unexpected radiological
abnormalities.

The role of CXR appears to be restricted to those patients
undergoing technically difficult and complicated procedures.