Pediatric emergency medicine trisk 2642 2642
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areflexia, without motor weakness of the extremities. The most common cranial
nerve deficit is a seventh (facial) nerve palsy, followed in decreasing frequency
by impairment of cranial nerves IX, X, and XI and oculomotor abnormalities.
Autonomic dysfunction occurs commonly and results in blood pressure lability,
postural hypotension, and cardiac abnormalities; autonomic dysfunction is a
disproportionate cause of morbidity and mortality. Urinary retention, if it occurs,
is usually seen late in the illness. As the paralysis ascends, muscles of breathing
may become involved, leading to respiratory embarrassment.
The primary aid in diagnosis is LP, which demonstrates an elevated protein
level and fewer than 10 white blood cells per cubic millimeter—the so-called
albuminocytologic dissociation. CSF glucose is normal. The protein elevation
occurs in almost all cases but may be delayed for weeks, usually peaking in the
second or third week of illness. Emergency electromyography (EMG) and nerve
conduction velocity testing are not indicated. EMG may detect the presence of
nerve conduction velocity delay; however, it is usually not demonstrable until the
second or third week of illness. Contrast-enhanced MRI imaging has been shown
to be a sensitive and useful diagnostic adjunct, and imaging will typically
demonstrate enhancement of the spinal nerve roots.
Because of the potential for progression to life-threatening respiratory
compromise, the child with Guillain–Barré syndrome should be hospitalized and
observed closely. Impending respiratory distress must be anticipated, and routine
respiratory monitoring should be aided by specific measures of respiratory
function, particularly measurement of negative inspiratory force. Because
autonomic dysfunction is common, blood pressure must be monitored closely and
abnormalities treated vigorously.
Acute polyneuritis is generally self-limiting, with more than 90% of children in
most series having complete or near-complete recovery. In mild cases, in which
children retain the ability to ambulate, only supportive care is required. However,
immunomodulatory therapy may be of benefit in more severely affected children.
Plasmapheresis and IV immunoglobulin both have been used. Although wellcontrolled, blinded studies of these treatments in children are lacking, the
available data suggest that both are effective in reducing the duration and severity
of illness in those most severely affected. Corticosteroids have not been shown to
be beneficial in acute Guillain–Barré syndrome, and some evidence suggests they
may actually delay recovery.
Myasthenia Gravis
