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Critical
illness polyneuropathy and myopathy
Updated May 2007 by Charles Gomersall
Difficult to distinguish between critical illness polyneuropathy and critical
illness myopathy and it may be preferable to group the two together
Epidemiology
- occurs in 25-63% of patients who have been mechanically ventilated for
≥1 week
- 70-100% of those who are also septic
- rare in children
- twice as common in males
- associated with:
- sepsis, SIRS
- multiorgan dysfunction
- neuromuscular blocking agents
- corticosteroids
- cytotoxics
- status asthmaticus
Clinical features
- often presents with difficulty in weaning. (Note that time to successful
discontinuation of ventilation is not reduced by respiratory muscle
"training" and that it is determined by time to resolution of
neuropathy)
- mixed motor and sensory neuropathy but motor signs tend to predominate
- tends to cause distal muscle weakness (as do inflammatory, diabetic, porphyria
neuropathy), particularly affecting lower limbs
- cranial nerves unaffected (cf Guillain Barre) - in common with other axonal
neuropathies. Discrepancy between facial grimacing and decreased limb movement
on painful stimulus often striking
- reflexes usually disappear during the course of the illness but absent
reflexes are not a prerequisite for diagnosis
- subclinical in 50%
- NB features may be modified by presence of co-existing septic encephalopathy
Investigations
- EMG & nerve conduction
- relative preservation of conduction velocities
and latencies (primary axonal degeneration)
- reduction of amplitude and duration of muscle compound action potentials
- ± decreased amplitured of sensory nerve action potentials
- needle EMG show fibrillation potentials
and positive sharp waves, particularly in distal muscles. Comprehensive
needle EMG testing is necessary.
- can give information on severity of polyneuropathy
- muscle biopsy
- CPK: normal or near normal in most patients
- CSF: at most mildly raised protein
Differential diagnosis
Guillain-Barre syndrome
other causes of predominant motor neuropathy (eg lead poisoning)
diabetic neuropathy
porphyria
acute quadriplegic myopathy
- more common in patients with severe asthma treated with high dose
corticosteroids but may occur in the absence of steroid use
- sensation and reflexes relatively spared
- may be distinguished from critical illness polyneuropathy and myopathy by
EMG
- CPK elevated
Prevention
- subgroup analysis suggests that intensive insulin therapy/tight glucose
control may reduce the incidence
Treatment
- no specific treatment
- treat underlying condition
Prognosis
- associated with increased mortality, prolonged mechanical ventilation
and prolonged rehabilitation
- majority of survivors have persistent functional disability, reduced
quality of life and restriction of autonomy
Further reading
Maramattom BV, Wijdicks EFM. Acute neuromuscular weakness in the intensive
care unit. Crit Care Med, 2006; 34(11): 2835-2841
Visser LH. Critical illness polyneuropathy and myopathy: clinical features, risk
factors and prognosis. Eur J Neurology, 2006; 13(11):1203-12
© Charles Gomersall December 1999, May 2007
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