Rheumatic chorea: relationship to systemic manifestations and response to corticosteroids.

OBJECTIVE: To describe Sydenham chorea among children in a cohort of patients with rheumatic fever (RF). STUDY DESIGN: An existing database was used to identify demographic characteristics, clinical manifestations, and therapy in persons with RF identified in Salt Lake City, Utah, from 1985 through January 2002. RESULTS: Of 584 cases in the database, 537 (91%) were new-onset RF (median age of 10 years) and 177 (33%) had chorea. Patients with chorea were more often female (OR = 0.37, 95% CI = 0.25-0.55, P < .0001) and were less likely to have carditis or arthritis. Prednisone treatment may lead to a shortened course of chorea (4.0 weeks in prednisone-treated [n = 32] vs 9.0 weeks in untreated [n = 14]; P < .0001). Among 33 patients seen at a median of 10.3 years (range 6.3-14.9 years) after their initial bout of chorea, 20% reported residual tremor or mood swings. Ten of the 33 (30%) had one or more recurrences of chorea. CONCLUSIONS: Chorea affected one-third of the children with RF. Patients with chorea were less likely to have severe cardiac or rheumatologic complications of RF. Therapy with prednisone shortened the duration of rheumatic chorea; some reported recurrences of chorea and had minor neurologic sequelae.

Cerebral sinovenous thrombosis in children: another reason to treat iron deficiency anemia.

Iron deficiency anemia is a rare cause of cerebral sinovenous thrombosis in children. We report three cases of cerebral sinovenous thrombosis and iron deficiency anemia treated at Primary Children's Medical Center in Salt Lake City, Utah, between 1998 and 2001. The children were 9, 19, and 27 months old at the time of admission. Hemoglobin levels ranged from 6.6 to 7.0 g/dL, mean corpuscular volume levels from 45 to 56 fL, and platelet counts from 248,000 to 586,000/microL. Magnetic resonance imaging and magnetic resonance venography revealed thrombosis of the straight sinus and internal cerebral veins in all three children, with the addition of the vein of Galen, left transverse and sigmoid sinuses, and upper left internal jugular vein in one child. Recovery ranged from excellent to poor in 3 months to 3 years of follow-up. Four additional cases, ages 6 to 22 months, were found in the English-language literature. Evaluation for prothrombotic disorders was negative in all children, including the current cases. Treatments have included thrombectomy, corticosteroids, mannitol, heparin, low-molecular-weight heparin, warfarin, aspirin, blood transfusion, and iron supplementation, but there is no consensus regarding therapy, other than to correct the anemia and treat iron deficiency. Iron deficiency anemia, a preventable cause of cerebral sinovenous thrombosis, deserves consideration when cerebral sinovenous thrombosis is detected in young children.

Leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia (LACH) in two brothers: a novel syndrome?

We report a pair of brothers who have leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia. Both presented initially with seizures in the early postnatal period. They have significant developmental delay, and brain MRIs demonstrate leukoencephalopathy, characterized by profound hypomyelination. They have developed arthritis, which in one brother has required chronic treatment; and persistent intermittent diarrhea, necessitating treatment for inflammatory bowel disease. Finally, they have had multiple hospitalizations, including several for sepsis; an immunological analysis revealed that they have IgG1-subclass hypogammaglobulinemia and low B cell levels. There is no family history of similar problems, and these brothers have an unaffected brother. We believe this constellation of symptoms represents a novel syndrome: LACH syndrome (leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia). The etiology of this syndrome remains unknown despite extensive investigations.