RESUMEN
A 3-year-old boy with Kawasaki disease developed a cerebral infarction after high-dose intravenous immunoglobulin. Aspirin did not prevent the stroke. Based on the numerous reports of thrombosis due to high-dose immunoglobulin in older individuals, we conclude that it is necessary to be aware of this complication when treating children with Kawasaki disease.
Asunto(s)
Infarto Cerebral/inducido químicamente , Inmunoglobulinas Intravenosas/efectos adversos , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Preescolar , Relación Dosis-Respuesta a Droga , Humanos , Inmunoglobulinas Intravenosas/administración & dosificación , MasculinoRESUMEN
We report here a 6-year-old boy with narcolepsy. The diagnostic criteria were met by the clinical symptoms including excessive daytime sleepiness and cataplexy, and by the results of overnight polysomnography (PSG), multiple sleep latency test (MSLT), and human leukocyte antigen (HLA). PSG showed increased ratio of sleep stages 1 and 2 due to frequent awakening. All the five test session of MSLT showed a sleep onset REM period. HLA typing was positive for DRB1* 1501 and DQB1* 0602. Though the present case had very early onset, all the clinical symptoms and results of sleep studies met the criteria of narcolepsy. The CSF orexin level was far below the lower limit of the control values. It is very useful to measure CSF orexin for the diagnosis of early onset narcolepsy.
Asunto(s)
Péptidos y Proteínas de Señalización Intracelular , Glicoproteínas de Membrana , Narcolepsia/diagnóstico , Biomarcadores/análisis , Proteínas Portadoras/líquido cefalorraquídeo , Niño , Diagnóstico Diferencial , Antígenos HLA-DQ/análisis , Cadenas beta de HLA-DQ , Antígenos HLA-DR/análisis , Cadenas HLA-DRB1 , Humanos , Masculino , Neuropéptidos/líquido cefalorraquídeo , Orexinas , Polisomnografía , Sueño REMRESUMEN
We measured cerebrospinal fluid (CSF) hypocretin-1 levels in 11 patients with narcolepsy-cataplexy, five with narcolepsy without cataplexy and 12 with idiopathic hypersomnia (IHS). All patients were Japanese. As reported in Caucasian patients, undetectable or very low hypocretin-1 levels were observed in most (9 out of 11) Japanese narcolepsy--cataplexy patients. Our hypocretin-deficient narcoleptics included three prepubertal cases within few months after the disease onset. All nine hypocretin-deficient patients were human leuckocyte antigen (HLA) DR2 positive, while two who had normal CSF hypocretin-1 levels were HLA DR2 negative. In contrast, none of the narcolepsy without cataplexy and IHS subjects had undetectable low levels. Low CSF hypocretin-1 is therefore very specific for HLA DR2 positive narcolepsy-cataplexy, and the deficiency is likely to be established at the early stage of the disease.
