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Anaesthesia and orphan disease

Management of a case of Strumpell–Lorrain disease and review of the literature

Ponsonnard, Sebastien; Damon, Alexandre; Gueye, Edouard Marcel

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European Journal of Anaesthesiology: August 2017 - Volume 34 - Issue 8 - p 562-563
doi: 10.1097/EJA.0000000000000615
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Strumpell–Lorrain disease or hereditary spastic paraplegia (HSP) describes a group of rare and heterogeneous genetic neurological disorders. In Europe, HSP is estimated to affect 1 to 9 per 100 000 individuals.

As of 2008, 41 genes associated with spastic paraplegia have been identified. The main genetic mechanisms underlying the clinical phenotype include membrane trafficking disturbance, impairment of organelle transport, morphogenesis and distribution in neuronal cells and mitochondrial dysfunction.

HSP is a neurodegenerative disorder which affects the corticospinal tract at the lumbar level. For this reason, the clinical picture is characterised mainly by a progressive spasticity of the lower limbs leading to paraparesis or paraplegia affecting several members of the same family. Progression of the disease to the rest of the corticospinal tracts, to peripheral nerves, to the cerebellum or to the brain explains the other additional symptoms. Clinical diagnosis is based on four criteria: exclusion of differential diagnoses, compatible family history, progressive disturbance of gait and corticospinal tract deficits in the lower limbs with hyperreflexia. Spinal cord atrophy is a common finding in HSP.

Anaesthetic management of patients with HSP is unclear. There are no studies published in Pubmed and only seven case reports.1–6 Here, we report the case of a patient with HSP who underwent spinal surgery under general anaesthesia. The patient provided written permission for this case report.

A 64-year-old woman, 78 kg and 1.55 m, diagnosed with HSP at 31 years of age, experienced a rapid deterioration of her symptoms starting in 2008 leading to severe difficulty in walking. Paraparesis was accompanied by unusual lumbar pain with irradiation to the lower right limb. At clinical examination, the muscular deficit was bilateral but localised at the L5 level. This unusual presentation motivated a computed tomography scan which revealed a degenerative stenosis of the lumbar spinal canal and L4–L5 spondylolisthesis. It was decided to perform a decompressive laminectomy under general anaesthesia. The patient had hypertension treated with celiprolol and type 2 diabetes treated with metformin and glicazide; she also took acetaminophen and tramadol for her lumbar pain. She had a history of anaesthesia for a laparoscopy and described a probable prolonged neuromuscular blockade; at this time HSP diagnosis had not yet been made.

Based on reports found in the literature, we decided to perform total intravenous anaesthesia (TIVA; Base Primea, Fresenius Kabi, Bad Homburg vor der Höhe, Germany) with propofol and remifentanil). These two drugs were used at target concentrations using the Schnider and Minto models. Depth of anaesthesia was monitored by bispectral index (BIS). At anaesthesia induction, a 4 μg ml−1 target effect-site concentration of propofol was used with a low BIS, around 24. During the first part of surgery, the propofol target was decreased (2.8 μg ml−1) for a BIS around 45; but the BIS was below 40 during 84 min of surgery (82.3% of time). At the end of surgery, a total of 950 mg were used. Remifentanil was given by continuous infusion with a 5 ng ml−1 target effect-site concentration; at the end of surgery, a total of 950 μg were used. Endotracheal intubation was facilitated by 0.6 mg kg−1 rocuronium. One hundred and two minutes later, at the end of surgery, train of four at adductor pollicis showed four responses with 44% T4/T1; 2 mg kg−1 sugammadex was administered. At the same time, TIVA was stopped. In total, 6 min later T4/T1 value reached 99% and the patient breathed spontaneously. At the 7th minute, she opened her eyes. She was extubated immediately after. She was discharged from the postoperative recovery room 68 min later. Over the next few days, then the next few weeks, she gradually improved and was able to walk with canes.

The choice of neuromuscular blocking agents must be made with extreme care in HSP. The use of succinylcholine is contraindicated in HSP as it may induce hyperkalaemia because of upregulation of nicotinic acetylcholine receptors. In other neurological diseases, like cerebral palsy or lateral amyotrophic sclerosis, the presence of extrajunctional nicotinic receptors could result in resistance to neuromuscular blockade. Currently, nothing in the literature confirms that this is the case in the HSP group. On the contrary, nondepolarising muscle relaxants carry a risk of exaggerated muscle relaxant response, as was the case in our patient history. This is very well illustrated by Dallman,7 who reported recurarisation after a well conducted rocuronium antagonism by prostigmine. Franco-Hernández et al.1 reported the use of sugammadex in two cases because of the presence of moderate neuromuscular block at the end of surgery. After the use of sugammadex, no recurarisation was observed as in our case. Rocuronium appears to be the best choice when muscle relaxation is indicated, because of the possibility of antagonising its effects with sugammadex. Long-acting neuromuscular blockers should be avoided and a train of four ratio over 0.9 must be obtained before extubating. Reversal of neuromuscular blockade with neostigmine may risk recurarisation.

Our patient appeared to be more sensitive to hypnotic drugs; BIS values were low in spite of a low propofol dosage. Was this due to HSP? Five case reports involving the use of general anaesthesia have previously been published.1,3,5,7 Franco-Hernández et al.1 described the case of a 43-year-old woman anaesthetised by TIVA (propofol 4 to 8 mg kg−1.h−1) for a subtotal colectomy and one case using a halogenated agent (sevoflurane with minimum alveolar concentration 0.6 in a 47-year-old woman undergoing cholecystectomy); in these cases, the BIS was monitored and the drug doses seemed to be low in the case involving halogenated anaesthesia. In the other cases, propofol was used for induction and maintenance was carried out with halogenated agents; depth of anaesthesia was not monitored. In all these case reports, no complications from hypnotic or opioid drugs were reported. The problem is that HSP is a heterogeneous group of genetic diseases and there is no certainty that anaesthetic drugs will have the same action in all variants. Anaesthesia depth monitoring may be the best approach in HSP patients.

Some authors prefer regional spinal anaesthesia to general anaesthesia.2,4,6 HSP is not known to increase the toxicity of local anaesthetics, and no complications have been reported.

Some patients may be unaware that they have HSP when undergoing anaesthesia. This idea is reassuring when we meet a patient whose diagnosis of HSP is known. The only advice we can give is to evaluate clinical pharmacodynamics intraoperatively by monitoring depth of anaesthesia and neuromuscular blockade.

Acknowledgements related to this article

Assistance with the letter: none.

Financial support or sponsorship: SP received research funding from Masimo Corp and consulted for Drager Medical SAS.

Conflicts of interest: none.


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© 2017 European Society of Anaesthesiology