Uso de sugamadex em doença de Strumpell-Lorrain: relato de dois casos

José Antonio Franco-Hernández; Luis Munoz Rodríguez; Pilar Jubera Ortiz de Landázuri; Alejandra Garcia Hernández

doi:10.1590/S0034-70942013000100010

Clinical Information

Uso de sugamadex em doença de Strumpell-Lorrain: relato de dois casos

Use of sugammadex in Strumpell-Lorrain disease: a report of two cases

José Antonio Franco-Hernández; Luis Munoz Rodríguez; Pilar Jubera Ortiz de Landázuri; Alejandra Garcia Hernández

http://dx.doi.org/10.1590/S0034-70942013000100010 Braz J Anesthesiol, vol.63, n1, p.116-118, 2013

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Resumo

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.

Palavras-chave

BLOQUEADOR MUSCULAR, Rocurônio, DOENÇAS, Raras, Paraplegia Espástica Hereditária

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.

Keywords

Spastic Paraplegia, Hereditary, Anesthesia, Neuromuscular blockade

References

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Uso de sugamadex em doença de Strumpell-Lorrain: relato de dois casos

Use of sugammadex in Strumpell-Lorrain disease: a report of two cases

José Antonio Franco-Hernández; Luis Munoz Rodríguez; Pilar Jubera Ortiz de Landázuri; Alejandra Garcia Hernández

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