Case of the month: Isaacs' syndrome associated with chronic inflammatory demyelinating polyneuropathy

Odabaşı, Zeki; Joy, Juan L.; Claussen, Gwendolyn C.; Herrera, Guillermo A.; Oh, Shin J.

doi:10.1002/(sici)1097-4598(199602)19:2<210::aid-mus13>3.0.co;2-y

Cited by 31 publications

(5 citation statements)

References 28 publications

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“…It may also be associated with a persistent facial contraction and has been noted to be present for up to 19 years [9,23]. It has been reported with other masses including cerebellar astrocytoma, metastatic tumors and acoustic neuroma.…”

Section: Myokymiamentioning

confidence: 55%

Myokymia and neuromyotonia 2004

Gutmann¹,

Gutmann²

2004

Journal of Neurology

103

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Section: Myokymiamentioning

confidence: 55%

Myokymia and neuromyotonia 2004

Gutmann¹,

Gutmann²

2004

Journal of Neurology

103

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“…IS can be seen in isolation or it can be associated with neoplastic and nonneoplastic processes. 92 Regarding the association of IS with various malignancies, our review showed that there were 14 cases (20% of all patients) in which malignancies were reported before and after the diagnosis of IS (Table 1). As expected, thymoma was the most common tumor reported (n = 7), followed by lymphoma (n = 3), sarcomatoid carcinoma (n = 1) (malignancy that has characteristics of both carcinoma and sarcoma and grows rapidly), spinal cord hemangioblastoma (n = 1), gastric cancer (n = 1), and small-cell carcinoma (n = 1) (Table 1).…”

Section: Resultsmentioning

confidence: 99%

Section: Associated Malignancies and Nonneoplastic Syndromesmentioning

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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“…MMN patients with fasciculations and myokymia and with continuous motor unit activity have been reported . Widespread myokymia, Isaacs syndrome, and continuous motor unit activity have been reported in chronic inflammatory demyelinating polyradiculoneuropathy. In line with these findings, CFS in our patient is thought to be secondary to monofocal motor neuropathy of the median nerve.…”

Section: Discussionmentioning

confidence: 99%

Cramp‐fasciculation syndrome associated with monofocal motor neuropathy

2017

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Monofocal motor neuropathy of the right median nerve was diagnosed. Intravenous immunoglobulin treatment led to significant improvement of symptoms and signs. Although fasciculations and cramps have been reported in multifocal motor neuropathy and are considered supporting criteria for the diagnosis, the occurrence of cramp-fasciculation syndrome as the presenting feature and predominant manifestation in monofocal motor neuropathy, a variant of multifocal motor neuropathy, is unique. Muscle Nerve 56: 828-832, 2017.

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Case of the month: Isaacs' syndrome associated with chronic inflammatory demyelinating polyneuropathy

Cited by 31 publications

References 28 publications

Myokymia and neuromyotonia 2004

Myokymia and neuromyotonia 2004

Systematic Review of the Clinical Characteristics and Management of Isaac Syndrome

Cramp‐fasciculation syndrome associated with monofocal motor neuropathy

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