ABSTRACT
CONTENT: Strumpell-Lorrain disease - or familial spastic paraplegia (FSP) - is a rare hereditary neurological disorder, mainly characterized by variable degrees of stiffness and weakening of the muscles, with cognitive impairment, deafness, and ataxia in the more severe cases. We describe two female siblings with FSP programmed for cholecystectomy and subtotal colectomy, respectively, and also how we dealt with the anesthetic management in both cases and review the literature on this disease in relation to anesthesia.
Subject(s)
Spastic Paraplegia, Hereditary/drug therapy , gamma-Cyclodextrins/therapeutic use , Adult , Female , Humans , Middle Aged , Spastic Paraplegia, Hereditary/genetics , SugammadexABSTRACT
CONTEÚDO: A doença de Strumpell-Lorrain, ou paraparesia espástica familiar (PEF), é uma doença hereditária neurológica rara, caracterizada principalmente por graus variáveis de rigidez e enfraquecimento dos músculos, com comprometimento cognitivo, surdez e ataxia nos casos mais graves. Descrevemos os casos de duas irmãs com PEF, agendadas para colecistectomia e colectomia subtotal, respectivamente. Também descrevemos o manejo anestésico em ambos os casos e revisamos a literatura sobre essa doença em relação à anestesia.
CONTENT: Strumpell-Lorrain disease - or familial spastic paraplegia (FSP) - is a rare hereditary neurological disorder, mainly characterized by variable degrees of stiffness and weakening of the muscles, with cognitive impairment, deafness, and ataxia in the more severe cases. We describe two female siblings with FSP programmed for cholecystectomy and subtotal colectomy, respectively, and also how we dealt with the anesthetic management in both cases and review the literature on this disease in relation to anesthesia.
CONTENIDO: La enfermedad de Strumpell-Lorrain, o paraparesia espástica familiar (PEF), es una enfermedad hereditaria neurológica rara, caracterizada principalmente por grados variables de rigidez y debilitamiento de los músculos, con el compromiso cognitivo, la sordera y la ataxia en los casos más graves. Describimos aquí dos casos de dos hermanas con PEF, citadas para colecistectomía y colectomía subtotal respectivamente. Describimos también el manejo anestésico en ambos casos y revisamos la literatura sobre esa enfermedad con relación a la anestesia.
Subject(s)
Adult , Female , Humans , Middle Aged , Spastic Paraplegia, Hereditary/drug therapy , gamma-Cyclodextrins/therapeutic use , Spastic Paraplegia, Hereditary/geneticsABSTRACT
CONTENT: Strumpell-Lorrain disease--or familial spastic paraplegia (FSP)--is a rare hereditary neurological disorder, mainly characterized by variable degrees of stiffness and weakening of the muscles, with cognitive impairment, deafness, and ataxia in the more severe cases. We describe two female siblings with FSP programmed for cholecystectomy and subtotal colectomy, respectively, and also how we dealt with the anesthetic management in both cases and review the literature on this disease in relation to anesthesia.
Subject(s)
Spastic Paraplegia, Hereditary/drug therapy , gamma-Cyclodextrins/therapeutic use , Adult , Androstanols/antagonists & inhibitors , Anesthesia, General , Cholecystectomy , Colectomy , Female , Humans , Middle Aged , Neuromuscular Blockade , Neuromuscular Nondepolarizing Agents/antagonists & inhibitors , Rocuronium , SugammadexABSTRACT
La cateterización de venas centrales es un proceso rutinario, sobre todo en enfermos graves. Es una técnica en la que están descritas múltiples complicaciones, afortunadamente no muy frecuentes, y en relación con la pericia del que la realiza. El síndrome de Horner es una de estas complicaciones infrecuentes aunque, afortunadamente su porcentaje de recuperación es elevado. Describimos un caso en el que por sospecha clínica, valoración radiológica y recuperación total confirmamos su aparición. Asimismo, se revisa la literatura y se analizan los posibles mecanismos de estos episodios (AU)
The insertion of a central venous catheter is a routine procedure, particularly in critical patients. The complications that have been described are many; fortunately they are rare and skill in performing the procedure bears a relation to their incidence. Horner syndrome is one such rare complication. The recovery rate is fortunately high. We describe a case in which clinical suspicion and radiologic assessment led to recognition of Horner syndrome in this context. Recovery was confirmed. The literature is reviewed, and possible mechanisms to explain these events are discussed (AU)
