Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: An analysis of antigenic targets in neural tissues

Objective: Opsoclonus-myoclonus (OM) is a rare neurologic syndrome affecting children and adults. In children it occurs as a parainfectious process or a paraneoplastic syndrome in association with neuroblastoma. Evidence for an immune mechanism includes the presence of serum autoantibodies to several neural antigens and improvement of symptoms with immunosuppressive therapy. We studied the neural antigenic targets of serum IgM and IgG autoantibodies from nine children with OM.Design: We studied sera from nine children with OM, three with associated neuroblastoma and six with a prodromal viral illness. Control subjects (n = 77) included four children with neuroblastoma but not OM, 32 children with other neurologic disorders, and 41 with nonneurologic illnesses. We studied the neural antigenic targets of serum IgM and IgG autoantibodies by the following methods: (1) immunostaining of human cerebellar sections and peripheral nerve, and (2) Western blot analysis with human brain fractions including white matter, gray matter, and cerebellar Purkinje cells and nuclei.Results: Sera from all nine children with OM had IgM and IgG binding to the cytoplasm of cerebellar Purkinje cells and to some axons in white matter. In peripheral nerve, IgM and IgG from all nine OM sera bound to large and small axons. Western blot analysis showed a distinctive pattern of binding to several neural proteins, including a 210 kd antigen identified as the high molecular weight subunit of neurofilament. No control serum showed a similar pattern of reactivity.Conclusion: Opsoclonus-myoclonus syndrome in childhood is associated with a distinctive pattern of serum IgM and IgG binding to neural tissues and antigens.     

Homer's syndrome: An unusual presentation of Hodgkin's disease

Horner's Syndrome (ptosis, anisocoria, and anhydrosis) developed in a 22-year-old woman. A mediastinal mass was noted on chest x-ray. Further evaluation led to a diagnosis of Hodgkin's disease, nodular sclerosis type. Horner's syndrome is an unusual initial manifestation of Hodgkin's disease, and, in this case, it was due to oculosympathetic damage from mediastinal compression. Because the potential for cure is high in Hodgkin's disease, this diagnosis should be considered in patients presenting with Horner's syndrome.     

Acute severe hepatitis with coagulopathy: An unusual presentation of Kawasaki syndrome in association with Epstein-Barr virus

The first case of Kawasaki syndrome presenting as acute severe hepatitis with coagulopathy, in association with an acute Epstein-Barr virus infection is reported. This case supports the hypothesis that Epstein-Barr virus may play a role in the etiology of Kawasaki syndrome and serves as a reminder that a diagnosis of Kawasaki syndrome should be considered for a persistently febrile child, even in the face of an atypical presentation.     

Sleep disturbance and rage attacks in opsoclonus-myoclonus syndrome: response to trazodone

OBJECTIVES: Parents of children with opsoclonus-myoclonus syndrome (OMS) frequently describe poor sleep and rage attacks. We hypothesized that these manifestations are related and could result from underlying monoaminergic dysfunction. STUDY DESIGN: We clinically characterized the sleep and behavioral characteristics of 51 young children with OMS; 19 of those with the most disruptive sleep patterns were treated with trazodone, a soporific serotonergic agent. RESULTS: Sleep disturbances, including prolonged sleep latency, fragmented sleep, reduced quantity of sleep, snoring, and non-restorative sleep, were reported in 32 children, and frequent rage attacks were reported in 25. In 59% of the poor sleepers, parents felt that the problem was severe enough to warrant treatment. Children sleeping <10 hours/night had a higher rage frequency than those who slept more. Of the children who required trazodone, 84% were receiving corticosteroids or adrenocorticotropic hormone (corticotrophin), compared with 37% in the subgroup with normal sleep. Trazodone (3.0 +/- 0.4 mg/kg/day) improved sleep and behavior in 95% of the children, significantly increasing total sleep time by 72%, decreasing the number of awakenings by 76%, and reducing rage attacks by 33%. CONCLUSIONS: Children with OMS exhibited multiple types of sleep disturbances, which contributed to rage attacks. Trazodone was effective in improving sleep and decreasing rage attacks and was well tolerated, even in toddlers.     

Acute appendicitis in a patient with hemolytic uremic syndrome: an unusual clinical scenario

Gastroenteritis due to Escherichia coli O157:H7 occurs in young children and is associated with consumption of under cooked beef. Approximately 5–10% of patients will develop hemolytic uremic syndrome (HUS): renal failure, microangiopathic hemolytic anemia, and thrombocytopenia. A 6-year-old boy was admitted with abdominal pain, guaiac positive stool, decreased urine output and elevated creatinine levels. Hemodialysis was initiated upon rapid progression to anuria. On hospital day # 5 he developed acute abdominal pain, which was different from his initial assessment. Exam revealed focal tenderness in the right lower quadrant with localized guarding and rebound. Ultrasound demonstrated a dilated, fluid filled tubular structure in the RLQ concerning for appendicitis. Based on these findings the patient was taken to the operating room for a laparoscopic appendectomy. The patient had undergone dialysis the previous day and was preoperatively treated with DDAVP to minimize the risk of bleeding. The procedure occurred without complication and final pathology confirmed acute appendicitis. This case highlights the unique clinical scenario in which patients with HUS require operative intervention. Surgical procedures can be performed on these patients, however, all precautions should be taken to minimize the risk of bleeding, including the use of preoperative DDAVP.