RESUMO
Dermatomyositis is an acquired disease characterised by symmetric predominantly proximal muscle weakness of the arms and legs, and misery. It may be associated with myalgia and there is often a characteristic rash. The mainstay of therapy is corticosteroids. Recently efficacy of intravenous immunoglobulin (IVIg) in chronic refractory dermatomyositis was reported. Because corticosteroids can cause serious side effects, we treated a seven-year-old girl suffering from dermatomyositis with IVIg as initial therapy. After two courses of IVIg infusions at a dose of 0.4 g/kg/day for five consecutive days, the patient made a rapid and complete recovery. This case shows that IVIg may be effective as initial therapy in patients with dermatomyositis. Whether IVIg is really a better treatment than corticosteroids should be investigated in a randomised study.
Assuntos
Dermatomiosite/terapia , Imunoglobulinas Intravenosas/uso terapêutico , Biópsia , Criança , Dermatomiosite/patologia , Feminino , Humanos , Músculos/patologiaAssuntos
Distúrbios do Sono por Sonolência Excessiva/imunologia , Antígenos HLA-DR/análise , Hiperfagia/imunologia , Adolescente , Criança , Diagnóstico Diferencial , Suscetibilidade a Doenças , Distúrbios do Sono por Sonolência Excessiva/diagnóstico , Humanos , Hiperfagia/diagnóstico , Masculino , Narcolepsia/diagnóstico , SíndromeRESUMO
Two boys, aged 12 and 13 years, showed relapsing periods of somnolence and excessive eating, starting after a viral illness. One of them also showed periodic disturbance of sexual impulse control. The symptomatic periods were followed by symptom-free intervals in a highly characteristic pattern. This gave the clue to the diagnosis Kleine-Levin syndrome. The cause of this syndrome is unknown, in some cases a relationship between infectious disease or traumatic brain damage has been postulated. A dysfunction of the hypothalamus and associated structures is suspected. The syndrome has a rather favourable prognosis. The symptoms can be relieved by amphetamines, methylphenidate and probably also by lithium carbonate.