Question 3

Compare and contrast Guillian-Barré syndrome (GBS), and acute transverse myelitis (ATM) in terms of the relevant history, the clinical features, and the relevant investigation findings. 

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College answer

Guillian-Barré

Acute Transverse Myelitis

Relevant History

Antecedent respiratory or diarrhoeal illness

Campylobacter Jejuni

Viral – EBV, HSV

Mycoplasma

Vaccination

Antecedent respiratory, gastrointestinal, or systemic illness in 30-60%, can occur as part of the spectrum of multiple sclerosis, may be seen in patients with acute disseminated encephalomyelitis, other CNS infections or associated with a systemic autoimmune disease

Motor

Weakness

Ascending, symmetrical motor weakness

Parasthesia/pain

Hypo/areflexia

Pyramidal weakness below level of spinal cord lesion,

bilateral signs

Cranial Nerve Palsy

Common

Uni-/bilateral facial

Bulbar

Rare, may be associated with optic neuritis if part of MS spectrum

Dysautonomia

Yes

May be present

Sensory Deficit

Absent or Mild, distal

Clearly defined sensory level all modalities (spinothalamic and posterior columns) on the trunk at level of involvement

CSF

Elevated protein.

No pleocytosis.

Abnormal in 50%, moderate lymphocytosis (typically <100/mm3) and an elevated protein. Glucose levels are normal. Oligoclonal bands are usually not present in isolated TM, and when present suggest a higher risk of subsequent MS

Neurophysiology

Abnormal spontaneous

activity

Decreased/ unrecordable motor evoke potentials to lower limbs especially on

Normal MUPs initially. Reduced recruitment.

lumbar stimulation and evidence of denervation in leg muscles

MRI 

Not diagnostic

Gadolinium-enhancing signal abnormality

(extending over one or more cord segments. Cord oedema at the level. 

Discussion

This is another question in a long series of neurology questions asking the trainees to compare Guillain-Barre with other spinal weakness syndromes.   The official college answer to this SAQ was actually quite extensive, and likely represents an earnest effort from the examiners. It would be difficult to improve on such an answer. One can merely rearrange the categories and add some irrelevant tidbits here and there. The product appears below.

Guillian-Barre versus Acute Transverse Myelitis
Guillian-Barré Acute Transverse Myelitis
Pathophysiology

Acute inflammatory demyelinating peripheral neuropathy associated with infection such as:

  • Antecedent viral illness; usually with diarrhoea
  • EBV
  • HSV
  • Campylobacter jejuni
  • HIV

Autoimmune inflammation of the spinal cord; may be idiopathic or associated with other illnesses:

  • Usually occurs as a postinfectious complication
  • Can fall within the spectrum of coexisting MS
  • Can coexist with acute disseminated encephalomyelitis
  • Autoimmune diseases are associated (eg. SLE, scleroderma, etc)
Typical features of history
  • Sub-acute onset
  • Ascending pattern of clinical signs
  • Often, very rapidly progressing
  • Weakness nadir is achieved within 4 hours in some cases (though some take as long as 21 days)
Power
  • Bilaterally decreased
  • Symmetrical
  • Weakness ascends over time
  • Bilaterally decreased
  • Symmetrical
  • Weakness remains at and below the level of the lesion
  • "Pyramidal" preference: flexors of the legs and the extensors of the arms 
Tone
  • Flaccid 
  • Later, remains flaccid
  • Initially flaccid
  • Later, hypertonic spasticity
Reflexes
  • Diminished or absent
  • Later, remain diminished
  • Depressed initially
  • Hyperreflexia subsequently
Cranial nerves
  • Usually, not involved
  • Miller Fischer variant involves (usually, medullary) cranial nerves
  • Usually, not involved
  • When it forms a part of the MS spectrum, there may be optic neuritis
Autonomic features
  • Usually present
  • Not usually involved, unless the level of the lesion is high
  • High lesions may present with spinal shock
Sensory findings
  • Sensation usually preserved or oly mildly affected
  • Sensation is usually absent 
  • There is usually a distinct symmetrical sensory level
CSF features
  • Raised protein
  • Usually no white cells
  • Antibodies (Anti-GM1) or GQ1b antibodies in the Miller Fischer variant
  • Raised protein
  • Lymphocytosis
Nerve conduction studies
  • Marked slowing, conduction block
  • Reduced amplitude sensory nerve action potential (SNAP)
  • Pathological F-wave responses
  • Decreased conduction velocity of motor and sensory nerves.
Electromyography
  • Abnormal spontaneous activity, reduced recruitment, normal MUPs (early in disease). Later, reduced
  • Reduced amplitude of motor (MUP) action potentials
MRI
  • Noncontrast MRI is essentially normal
  • Gadolinium reveals surface thickening and contrast enhancement on the conus medullaris and the nerve roots of the cauda equina
  • Noncontrast MRI reveals cord oedema at the level of the lesion (but in 40%, looks totally normal)
  • Gadolinium-enhancing signal abnormality extending over one or more cord segments.
  • Lesions ccupy most of the transverse diameter of the cord (2/3rds)
   

References

Oh's Intensive Care manual:

Chapter   57   (pp. 617)  Neuromuscular  diseases  in  intensive  care by George  Skowronski  and  Manoj  K  Saxena

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