RESUMEN
This is a report of a young boy with the unusual combination of autonomic dysfunction with locked-in syndrome following multiple shunt revisions for hydrocephalus. A review of the literature on autonomic dysfunction syndrome and the complex clinical picture of the combined syndromes in a pediatric patient are discussed. The marked effectiveness of treatment with carbidopa/levodopa over bromocriptine for both syndromes is noted.
Asunto(s)
Enfermedades del Sistema Nervioso Autónomo/complicaciones , Cuadriplejía/complicaciones , Enfermedades del Sistema Nervioso Autónomo/tratamiento farmacológico , Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Bromocriptina/uso terapéutico , Carbidopa/uso terapéutico , Niño , Dopaminérgicos/uso terapéutico , Agonistas de Dopamina/uso terapéutico , Fiebre/etiología , Humanos , Hidrocefalia/etiología , Hidrocefalia/cirugía , Hipertensión/etiología , Hiperventilación/etiología , Hipotálamo/fisiopatología , Masculino , Mesencéfalo/fisiopatología , Síndrome , Derivación VentriculoperitonealRESUMEN
Locked-in syndrome describes a neurological condition usually associated with infarction of the ventral pons. Such patients are diagnosed with quadriplegia, lower cranial nerve paralysis, and mutism. The patient is alert but communication is severely limited and usually is accomplished by blinking or using vertical eye movements. The rehabilitative management of locked-in syndrome has consisted of preventative and supportive measures. In this study we report two cases of locked-in syndrome which were treated successfully with Sinemet (DuPont Pharmaceuticals, Wilmington, DE). Even though the exact neuropharmacological mechanism is unclear, the dramatic improvement in these cases, as well as in one other published case report, dictates that use of Sinemet should be considered in the management of locked-in syndrome.
