Resistance to cisatracurium in a patient with MELAS syndrome

Aouad, Marie T.; Gerges, Frederic J.; Baraka, Anis

doi:10.1111/j.1460-9592.2005.01583.x

Cited by 15 publications

(14 citation statements)

References 22 publications

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“…The data regarding neuromuscular blocking agents are mixed, with case reports describing instances of unchanged, increased, or decreased sensitivity to non-depolarizing agents. [75, 79–84]. In general, care should be taken in the titration and monitoring of neuromuscular blockade in patients with mitochondrial disorders due to the possibility of underlying myopathy and the potential for increased drug sensitivity and altered drug metabolism.…”

Section: Special Considerationsmentioning

confidence: 99%

Treatment of Mitochondrial Disorders

Avula

Parikh

Demarest

et al. 2014

Curr Treat Options Neurol

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Opinion statement While numerous treatments for mitochondrial disorders have been suggested, relatively few have undergone controlled clinical trials. Treatment of these disorders is challenging, as only symptomatic therapy is available. In this review we will focus on newer drugs and treatment trials in mitochondrial diseases, with a special focus on medications to avoid in treating epilepsy and ICU patient with mitochondrial disease, which has not been included in such a review. Readers are also referred to the opinion statement in A Modern Approach to the Treatment of Mitochondrial Disease published in Current Treatment Options in Neurology 2009. Many of the supplements used for treatment were reviewed in the previous abstract, and dosing guidelines were provided. The focus of this review is on items not previously covered in depth, and our discussion includes more recently studied compounds as well as any relevant updates on older compounds. We review a variety of vitamins and xenobiotics, including dichloroacetate (DCA), arginine, coenzyme Q10, idebenone, EPI-743, and exercise training. Treatment of epilepsy, which is a common feature in many mitochondrial phenotypes, warrants special consideration due to the added toxicity of certain medications, and we provide a discussion of these unique treatment challenges. Interesting, however, with only a few exceptions, the treatment strategies for epilepsy in mitochondrial cytopathies are the same as for epilepsy without mitochondrial dysfunction. We also discuss intensive care management, building upon similar reviews, adding new dimensions, and demonstrating the complexity of overall care of these patients.

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Section: Special Considerationsmentioning

confidence: 99%

Treatment of Mitochondrial Disorders

Avula

Parikh

Demarest

et al. 2014

Curr Treat Options Neurol

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“…These associated morbidities may increase the risk for perioperative complications. [7][8][9][10][11] Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome patients need anesthesia for diagnostic (muscle biopsy, electrophysiology studies) and therapeutic procedures (cochlear implant, cardiac surgery, pacemaker implantation). Since MELAS is rare (12.5 per 100,000), 12 it is not feasible to perform prospective studies to define anesthetic risk, but potential specific anesthetic concerns have been raised in response to anecdotal reports.…”

Section: Résumémentioning

confidence: 99%

“…For example, controversy exists regarding patient sensitivity to nondepolarizing muscle relaxants (NDMR). [7][8][9][10]13 In addition, the avoidance of lactated intravenous fluids has been advocated because of impaired lactate metabolism. 14,15 As with other mitochondrial disorders, there is the concern of increased susceptibility to malignant hyperthermia (MH).…”

Section: Résumémentioning

confidence: 99%

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Considérations anesthésiques lors d’un syndrome d’encéphalomyopathie mitochondriale avec acidose lactique et épisodes ressemblant à des accidents vasculaires cérébraux: une série de cas

Gurrieri

Kivela

Bojanić

et al. 2011

Can J Anesth/J Can Anesth

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Purpose Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome (MELAS) is a rare inherited mitochondrial disorder associated with severe multiorgan pathology and stress-induced episodes of metabolic decompensation and lactic acidosis. The purpose of this case series is to review the medical records of patients with MELAS who underwent anesthetic care at the Mayo Clinic to observe their perioperative responses to anesthesia and to assess outcomes. Principal findings From September 1997 to October 2010, nine patients with MELAS were identified who underwent 20 general anesthetics, 12 prior to MELAS diagnosis. Debilitating neurologic symptoms involved eight patients, and three patients had substantial cardiac comorbidities. The patients tolerated commonly used anesthetics and muscle relaxants, including succinylcholine. Lactated Ringer's solution was used frequently. One patient was noted to have elevated postoperative serum lactate, but his serum lactate was chronically elevated. Metabolic acidosis was not observed in any patient. Hyponatremia and hyperkalemia, sometimes profound, were observed in seven patients, but these abnormalities also occurred at times remote from surgery. Two patients developed renal dysfunction following cardiac surgery and abdominal surgery for severe sepsis. Conclusion The MELAS patients developed episodes of hyponatremia and hyperkalemia of variable severity unrelated to the timing of surgery, suggesting these patients are prone to major electrolyte disturbances. Given the propensity to develop acid-base disturbances and lactacidemia, it is prudent to review and normalize electrolyte abnormalities and to adjust the anesthetic plan accordingly. Fortunately, the limited data suggest that patients with MELAS tolerate commonly used anesthetic drugs well. RésuméObjectif L'ence´phalomyopathie mitochondriale avec acidose lactique et e´pisodes ressemblant a`des accidents vasculaires ce´re´braux (MELAS) est un trouble mitochondrial he´re´ditaire rare associe´a`une pathologie grave touchant plusieurs organes et des e´pisodes de de´compensation me´tabolique et d'acidose lactique provoque´s par le stress. L'objectif de cette se´rie de cas est d'examiner les dossiers me´dicaux de patients atteints de MELAS et ayant reçu des soins anesthe´siques a`la Clinique Mayo afin d'observer leurs re´ponses pe´riope´ratoires a`l'anesthe´sie et d'e´valuer leurs devenirs. Constatations principales Pour la pe´riode allant de septembre 1997 a`octobre 2010, nous avons identifie´neuf patients atteints de MELAS ayant subi 20 anesthe´sies ge´ne´rales, dont 12 avant que le diagnostic de MELAS n'ait e´te´e´tabli. Les symptômes neurologiques de´bilitants ont touche´huit patients, et trois patients ont manifeste´des

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“…Diseases caused by mitochondrial dysfunctions, such as MELAS syndrome, have clinical manifestations predominantly in organs that require a lot of energy, such as brain, lungs, liver, and kidneys. Patients with the MELAS syndrome have reportedly experienced malignant hyperthermia, hypothermia, and resistance to muscle relaxants or their prolonged effects [2-6]. This case report describes the experiences with total intravenous anesthesia using target-controlled infusion of propofol and remifentanil to a MELAS syndrome patient undergoing a laparoscopic appendectomy.…”

mentioning

confidence: 99%

Total intravenous anesthesia with propofol and remifentanil in a patient with MELAS syndrome -A case report-

Park

Baek

Kang

et al. 2010

Korean J Anesthesiol

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A 23-year-old woman with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) underwent a laparoscopy-assisted appendectomy. MELAS syndrome is a multisystemic disease caused by mitochondrial dysfunction. General anesthesia has several potential hazards to patients with MELAS syndrome, such as malignant hyperthermia, hypothermia, and metabolic acidosis. In this case, anesthesia was performed with propofol, remifentanil TCI, and atracurium without any surgical or anesthetic complications. We discuss the anesthetic effects of MELAS syndrome.

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Resistance to cisatracurium in a patient with MELAS syndrome

Cited by 15 publications

References 22 publications

Treatment of Mitochondrial Disorders

Treatment of Mitochondrial Disorders

Considérations anesthésiques lors d’un syndrome d’encéphalomyopathie mitochondriale avec acidose lactique et épisodes ressemblant à des accidents vasculaires cérébraux: une série de cas

Total intravenous anesthesia with propofol and remifentanil in a patient with MELAS syndrome -A case report-

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