General anaesthetics and HSP

Posted - June 2015 in Living with HSP - Management & Treatment News

Neuromuscular blockers a consideration


Have you ever wondered (or has your doctor) if having HSP makes any difference to having a general anaesthetic? This Spanish study describes the cases of two sisters with HSP, at least one of them complicated, who had a general anaesthetic for their surgeries, together with their findings and recommendations.


The Study

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.


The use of neuromuscular blockers is complicated in patients with familial spastic paraplegia. Great care is required at extubation in patients with FSP, particularly if neuromuscular blockers were administered during the operation. If possible, long-acting neuromuscular blockers should be avoided, with routine monitoring of neuromuscular relaxation throughout the operation, using a standard peripheral nerve stimulator 12. A TOF ratio of over 0.9 must be confirmed before awakening, accelerating patient recovery with neostigmine or drugs that selectively bind aminosteroid neuromuscular blockers, such as sugammadex.



The main interest of our study is that both patients were subjected to general anesthesia, with the use of a specific reversal agent for non-depolarizing neuromuscular block, followed by complete recovery and no worsening of the existing neurological disease.


SOURCE: Braz J Anesthesiol. 2013 Jan-Feb;63(1):113-5. doi: 10.1016/S0034-7094(13)70203-4. Copyright © 2013 Elsevier Editora Ltda. All rights reserved. PMID: 23438806 [PubMed – indexed for MEDLINE]


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


Franco-Hernández JA1, Rodríguez LMOrtiz de Landázuri PJHernández AG.


1 Department of Anesthesiology and Resuscitation, Miguel Servet University Hospital, Zaragoza, Spain. [email protected]





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