Anesthetic experience using total intravenous anesthesia in a patient with Isaacs' syndrome -A case report-

Kim, Young Mi; Lee, Sang Hoon; Han, Cheol Sig; Choi, Eun Mi; Choi, Young-Sun; Chung, Moon Sang

doi:10.4097/kjae.2013.64.2.164

Cited by 12 publications

(6 citation statements)

References 13 publications

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“…Since previous reports have shown an increase in acetylcholine release in these patients and tolerance of dtubocurarine has been demonstrated in vitro, we can predict a tolerance for non-depolarizing muscle relaxants in patients with Isaacs' syndrome [7,8]. Some reports have suggested that general anesthesia in patients with Isaacs' syndrome does not exacerbate neuromuscular disorders such as myotonia and involuntary muscular contraction [9][10][11]. However, respiratory complications caused by respiratory muscle weakness and laryngeal muscle myotonia can result from anesthesia [9][10][11].…”

Section: Discussionmentioning

confidence: 80%

“…Some reports have suggested that general anesthesia in patients with Isaacs' syndrome does not exacerbate neuromuscular disorders such as myotonia and involuntary muscular contraction [9][10][11]. However, respiratory complications caused by respiratory muscle weakness and laryngeal muscle myotonia can result from anesthesia [9][10][11]. Furthermore, patients with Isaacs' syndrome are often comorbid with myasthenia gravis.…”

Section: Discussionmentioning

confidence: 99%

“…In this case, the patient had strongly suspected complications with myasthenia gravis, and we had concerns about increasing the sensitivity of non-polarizing muscle relaxants. As a result, we performed general anesthesia using total intravenous anesthesia to avoid prolonged neuromuscular blockade [9]. She received DFPP four times before surgery, so the specific symptoms were improved, and the pharmacokinetics of muscle relaxant was almost normal.…”

Section: Discussionmentioning

confidence: 99%

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Painful myokymia after surgery in a patient with Isaacs’ syndrome: a case report

2020

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Background: Isaacs' syndrome is a peripheral nerve hyperexcitability syndrome and rare acquired channel disease. The symptoms (myokymia, neuromyotonia, and muscle spasm) emerge even during sleep. This report describes the anesthetic management, especially neuromuscular blocking drugs and postoperative pain, in a case of Isaacs' syndrome. Case presentation: A 63-year-old woman with Isaacs' syndrome underwent elective laparoscopic distal gastrectomy under general anesthesia without epidural anesthesia. She received double filtration plasmapheresis four times to alleviate symptoms before surgery. To avoid a prolonged neuromuscular blockade, we performed total intravenous anesthesia and titrated muscle relaxant with neuromuscular monitoring. Anesthetic management was performed without any problems. However, pain management after surgery proved difficult as she experienced severe pain due to myokymia. Conclusions: Despite attempts to minimize symptoms, severe postoperative pain associated with myokymia occurred. Continuous regional anesthesia should be considered to treat pain from abnormal discharge in the central nervous system in Isaacs' syndrome.

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Section: Discussionmentioning

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Painful myokymia after surgery in a patient with Isaacs’ syndrome: a case report

2020

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“…Muscle cramp, slow relaxation following muscle contraction, and excessive sweating (hyperhidrosis) are the main symptoms of Isaacs' syndrome [3] [6] [9]. Patients almost always develop persistent muscle contraction, which is often worse following exercise myokimia, generalized, and fasciculations are also common [10] [11].…”

Section: Discussionmentioning

confidence: 99%

Case Report: A 72 Years Old Man with Isaacs’ Syndrome: A Rare Entity with Different Outcomes

Saeed¹,

Mohammed²,

Saeed³

2016

WJNS

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Neuromyotonia is a neuromuscular hyperexitability disorder characterized by muscle stiffness caused by continuous muscle fiber activity. It is an immune mediated disorder with elevated antibody level against presynaptic, voltage gated potassium channels, either as isolation or as a paraneoplastic process. Symptoms usually include muscle twitching during rest (myokymia), cramps, peudomyotonia (delayed relaxation), increased sweating, and sometimes motor weakness. In this case report, we present a seventy-two years old man who presented with pain in both thighs for one month. It gradually became worse to involve feet and chest. His brain CT scan showed features of brain atrophy. EMG showed fasciculation along neuromyotonic discharges with characteristic wave in frequency and amplitude typical of Isaacs' syndrome. Potassium channel antibodies were very high. Diagnosis of Isaacs' syndrome was made. He was followed up for two months with treatment by three-day course of methyl prednisolone followed by oral steroid and methotrexate with much improvement. This is the first case of Isaacs' syndrome in Kurdistan.

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“…and disagreement was resolved by consensus. This left us with a total of 61 case reports and 4 case series 14,16,30–91 (Fig. 1).…”

Section: Methodsmentioning

confidence: 99%

Systematic Review of the Clinical Characteristics and Management of Isaac Syndrome

Al-Chalabi,

Hegde,

Moore

et al. 2023

Journal of Clinical Neuromuscular Disease

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Objectives: Isaac syndrome (IS) is a condition characterized by peripheral nerve hyperexcitability caused by voltage-gated potassium channel (VGKC)-complex antibodies. Muscle twitching, stiffness, hypertrophy, and dysautonomic characteristics, such as hyperhidrosis, are common manifestations. The syndrome can be autoimmune or paraneoplastic, with thymoma being a common cause of paraneoplastic IS. Furthermore, this condition could be handed down from one generation to another. However, there is limited information regarding outcomes, relapses, associated syndromes, associated malignancies (other than thymoma), and treatment options. Despite its rarity, there remains a need for effective management strategies for patients with IS. To address this gap, we conducted a systematic review to summarize the most common and effective treatments of IS in immunomodulatory agents and symptomatic medications, as well as to describe outcomes, relapses, and associated malignancies. Altogether, this review serves to guide clinical practice recommendations for IS and highlight areas for further research. Methods: We used the Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol to conduct a systematic review of cases reposted through the PubMed and Google Scholar databases. The terms “Isaac Syndrome” and “Acquired Neuromyotonia” were used. The Joanna Briggs Institute's critical appraisal tool was used to evaluate the quality of the included studies. Results: We identified 61 case reports and 4 case series, comprising a total of 70 patients with IS (mean age at onset: 42.5 ± 18 years, and 69% were males). Fourteen cases reported relapses. Thymoma was the most common malignancy associated with IS, followed by lymphoma. Among various serum antibodies, voltage-gated potassium channel-complex antibodies were the most reported antibodies elevated in IS (reported in 38 patients and elevated in 21 patients [55.2%]), followed by acetylcholine ganglionic receptor antibodies, which were reported in 30% of patients (n = 21) and were elevated in 5 cases. The most common electromyography findings were myokymic discharges (n = 22), followed by fasciculations (n = 21) and neuromyotonia (n = 19). For treatment, combining anticonvulsants such as carbamazepine with immunotherapy therapy showed the best results in controlling the symptoms. Among immunotherapy therapies, the combination of plasma exchange plus intravenous high-dose steroids achieved the best results in the acute treatment of IS ([n = 6], with improvement noted in 83.3% [n = 5] of cases). Among the symptomatic treatments with anticonvulsants, carbamazepine was the most efficacious anticonvulsant in treatment of IS, with an average effective dosing of 480 mg/day (carbamazepine was used in 32.3% of acute treatment strategies [n = 23], with improvement noted in 73.9% [n = 17] of cases). Conclusions: IS a rare neuromuscular syndrome that tends to affect middle-aged men. These patients should be screened for thymoma and other malignancies such as lymphomas. The management of IS symptoms can be challenging, but based on our review, the combination of multiple immunosuppressives such as IV steroids and plasmapheresis with anticonvulsants such as carbamazepine seems to achieve the best results.

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Anesthetic experience using total intravenous anesthesia in a patient with Isaacs' syndrome -A case report-

Cited by 12 publications

References 13 publications

Painful myokymia after surgery in a patient with Isaacs’ syndrome: a case report

Painful myokymia after surgery in a patient with Isaacs’ syndrome: a case report

Case Report: A 72 Years Old Man with Isaacs’ Syndrome: A Rare Entity with Different Outcomes

Systematic Review of the Clinical Characteristics and Management of Isaac Syndrome

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