Complications
Patients with seizures during the acute illness are at risk of future episodes.
If considered at risk, anticonvulsants should be continued. Decision to cease anticonvulsants should be individualized.
Reported as a cause of mortality.
Treatment with fluid resuscitation, vasopressors, or inotropes is necessary.
Reported as a cause of mortality. Treatment of underlying coagulopathy and surgical intervention may be necessary.
Reported as a cause of mortality.
Treatment with antibiotics, fluid resuscitation, vasopressors, or inotropes is necessary.
Long-term sequelae are primarily neurologic.
Manifestations include reduced general intellectual function, verbal and visuospatial skills, and behavioral activity. Patients also have significantly lower self-esteem.[28]
Patients with a higher incidence of neurologic complications include: those <2 years old; those with ammonia levels >45 micrograms/dL; those who show rapid progression from stage 1 to stage 3; those who present with stage 4 or 5; and those with both liver and muscle involvement.[22]
The best predictor for neurologic sequelae is the ammonia level. Only 3% of patients with ammonia levels <45 micrograms/dL will exhibit permanent neurologic sequelae, whereas 11% have neurologic deficits if levels are >45 micrograms/dL.[22] Levels >350 micrograms/dL are associated with increased mortality.[20]
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