Both the physician and patient should be on the alert whenever severe abdominal pain occurs during neutropenic episodes, in which case, hospitalization for IV antibiotics and fluids along with bowel rest, may prevent the development of bowel necrosis and spontaneous perforation. The disease course is usually benign, but fatalities from overwhelming infection, peritonitis, and complications of intestinal perforation have been reported. This includes several syndromes in which periodic weakness is the dominant feature. The primary clinical manifestation is episodic, flaccid weakness of voluntary muscles, ranging in duration from minutes to weeks and varying in severity from slight weakness of one or two muscles to quadriplegia. Permanent muscle weakness is present in all patients independent of paralytic attacks.
Therapy is either with acetazolamide or potassium salts.
This symptom complex differs from the other periodic syndromes in that it is not accompanied by fever and has no fixed rhythmicity. Described in 1933, since then the syndrome is frequently described in children. It has been defined as a constellation of symptoms characterized by at least one episode of abdominal pain per month for 3 consecutive months severe enough to interfere with routine functioning, separated by asymptomatic periods. The incidence of the syndrome is highest in children between 8 and 10 years of age. Most children who have recurrent abdominal pain have no underlying organic pathology.