RESUMEN
Inclusion body myositis (IBM) is the most common acquired myopathy in people older than 50 years. IBM typically presents with distal upper extremity weakness accompanied by proximal lower extremity muscle weakness. Associated clinical findings include asymmetric weakness, foot drop, and dysphagia. The pathogenesis of IBM is not clear. In this article the authors briefly discuss postulated pathogenic mechanisms. Although no proven pharmacotherapy exists, some promising candidates are discussed.
Asunto(s)
Miositis por Cuerpos de Inclusión , Antiinflamatorios no Esteroideos/uso terapéutico , Anticuerpos Monoclonales/uso terapéutico , Diagnóstico Diferencial , Etanercept , Humanos , Inmunoglobulina G/uso terapéutico , Músculo Esquelético/patología , Músculo Esquelético/fisiopatología , Miositis por Cuerpos de Inclusión/diagnóstico , Miositis por Cuerpos de Inclusión/tratamiento farmacológico , Miositis por Cuerpos de Inclusión/etiología , Polimiositis/diagnóstico , Ensayos Clínicos Controlados Aleatorios como Asunto , Receptores del Factor de Necrosis Tumoral/uso terapéuticoRESUMEN
OBJECT: Many patients develop neurological symptoms related to spinal cord tethering after perinatal repair of myelomeningocele. This is referred to as secondary tethered cord syndrome (STCS). The authors describe their methodology and evaluate the intraoperative utility and postoperative outcomes of electrophysiologically guided untethering for STCS. In addition, the authors describe the use of electrophysiological guidance to identify an "autonomous placode" in the untethering of the cord in STCS. METHODS: The authors retrospectively identified 46 untethering procedures in 38 patients who had undergone perinatal myelomeningocele repair and in whom the index surgery was for tethered cord release at the site of the repair. In all cases, both passive (electromyography) and active (detection of compound muscle action potentials) electrophysiological monitoring was used. The proximity to neural elements was determined based on the current used; eliciting compound muscle action potentials with a Asunto(s)
Electrofisiología/métodos
, Meningomielocele/cirugía
, Defectos del Tubo Neural/cirugía
, Complicaciones Posoperatorias/cirugía
, Potenciales de Acción/fisiología
, Adolescente
, Adulto
, Niño
, Preescolar
, Electromiografía/métodos
, Electromiografía/estadística & datos numéricos
, Humanos
, Lactante
, Persona de Mediana Edad
, Monitoreo Intraoperatorio/métodos
, Defectos del Tubo Neural/etiología
, Procedimientos Neuroquirúrgicos/métodos
, Reoperación
, Estudios Retrospectivos
, Médula Espinal/cirugía
, Resultado del Tratamiento