Effectiveness of immunotherapy in a CASPR2 and LGI1 antibody-positive elderly patient with Isaacs’ syndrome: a case study

Ye, Zhinan; Jin, Yingying; Xu, Hao; Qiao, Xinyu; Yu, Huili; Shenglong, Xu

doi:10.1007/s13760-020-01446-8

Cited by 5 publications

(3 citation statements)

References 6 publications

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“…Spontaneous remission is rare in autoimmune neurologic disorders. Therefore, immunomodulatory treatment is needed (e.g., with IVIg/plasmapheresis/corticosteroids or immunosuppressive therapies) in refractory case [ 5 , 7 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

Anti-CASPR2 antibody-associated limbic encephalitis in a patient with a squamous cell carcinoma of the throat

et al. 2023

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Section: Discussionmentioning

confidence: 99%

Anti-CASPR2 antibody-associated limbic encephalitis in a patient with a squamous cell carcinoma of the throat

et al. 2023

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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%

“…16,31 Nine cases tested positive for antibodies against contactin-associated protein-like 2 (CASPR2) (Table 2). 36,46,75,88,89,94…”

Section: Clinical Characteristics and Management Of Ismentioning

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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Cytokines/chemokines and immune checkpoint molecules in anti-leucine-rich glioma-inactivated 1 encephalitis

Qiao

Zhang

et al. 2022

Neurol Sci

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Effectiveness of immunotherapy in a CASPR2 and LGI1 antibody-positive elderly patient with Isaacs’ syndrome: a case study

Cited by 5 publications

References 6 publications

Anti-CASPR2 antibody-associated limbic encephalitis in a patient with a squamous cell carcinoma of the throat

Anti-CASPR2 antibody-associated limbic encephalitis in a patient with a squamous cell carcinoma of the throat

Systematic Review of the Clinical Characteristics and Management of Isaac Syndrome

Cytokines/chemokines and immune checkpoint molecules in anti-leucine-rich glioma-inactivated 1 encephalitis

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