Sensory Conduction Study in Fisher Syndrome: Patterns of Abnormalities and Their Clinical Correlation

Lyu, Rong‐Kuo; Chang, Kuo‐Hsuan; Chu, Chun‐Che; Kuo, Hung‐Chou; Ro, Long‐Sun

doi:10.1159/000347224

Cited by 9 publications

(12 citation statements)

References 24 publications

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“…Several electrophysiological studies have shown evidence of peripheral nerve involvement in FS [3,4,5,6]. Sensory potential amplitude abnormalities are consistent findings in most series including ours [7]. In contrast to GBS, results of motor conduction studies for FS were usually normal or showed mild abnormalities.…”

Section: Introductionsupporting

confidence: 82%

“…A follow-up study 6 months after onset of the disease revealed that only 2 patients did not recover completely. Detailed clinical features of these patients have been described elsewhere [7]. …”

Section: Methodsmentioning

confidence: 99%

“…All these 55 patients underwent nerve conduction studies in our EMG laboratory using standardized procedures [7,9]. Limb temperature was maintained above 32°C in all patients.…”

Section: Methodsmentioning

confidence: 99%

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Motor Conduction Findings in Fisher Syndrome

Lyu

Chu²,

Kuo³

et al. 2013

Eur Neurol

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Objective: To report detailed motor conduction findings on Fisher syndrome (FS). Methods: We retrospectively reviewed motor conduction findings of 55 patients with pure FS and compared them with those obtained from 83 age- and sex-matched healthy volunteers. Results: In the FS group, distal and F-wave latencies of the median, ulnar, peroneal and tibial nerves were all significantly prolonged; motor conduction velocities were slowed in the median, ulnar and peroneal nerves. Conversely, an abnormally low compound muscle action potential amplitude was found only in the median and tibial nerves. Among the four parameters, distal and F-wave latencies were more frequently encountered abnormalities. None of our patients had abnormal temporal dispersion in any motor nerve. Focal involvement of the ulnar nerve across the elbow segment was observed in 6 patients (11%). Of the 55 FS patients, 31 (56%) had abnormal motor conduction results in at least one nerve. Abnormal motor conduction results were noted in 43 and 61% of patients when the tests were performed in the first week and 1 week after onset of FS, respectively (p = 0.238). Conclusion: Our results confirmed that mild motor conduction abnormalities were noted in pure FS, with a pattern similar to those observed in Guillain-Barré syndrome.

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Section: Introductionsupporting

confidence: 82%

Section: Methodsmentioning

confidence: 99%

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Motor Conduction Findings in Fisher Syndrome

Lyu

Chu²,

Kuo³

et al. 2013

Eur Neurol

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“…12 Reaksi antibodi-antigen terjadi pada nodus ranvier secara transien mengganggu proses konduksi. 14 Ikatan autoantibodi pada nodus ranvier ini akan mengaktifkan kaskade komplemen yang akan menghasilkan kompleks yang akan merusak membran saraf. Saluran natrium akan hilang, pelepasan mielin serta pemanjangan nodus ranvier.…”

Section: Konduksi Saraf Motorik Dan F-waveunclassified

“…9 Hasil yang sama juga didapatkan oleh Lyu dkk., (2013) yang menyimpulkan abnormalitas amplitudo SNAP merupakan karakteristik utama gangguan sensorik pada pasien Sindrom Miller-Fisher. 14 Hasil menarik dari studi ini adalah amplitudo SNAP pada ekstremitas atas yang diwakili oleh saraf medianus dan ulnaris memang lebih rendah dibandingkan dengan kontrol yang normal namun tidak demikian dengan amplitudo SNAP saraf suralis yang tidak berbeda dibandingkan dengan kontrol normal. 14 Hasil penelitian oleh Lyu dkk., (2008) dan Sekiguchi dkk., (2013) mendapatkan adanya pola abnormalitas amplitudo SNAP yang lebih banyak terjadi pada ekstremitas atas dibandingkan dengan ekstremitas bawah yang diistilahkan sebagai pola sural sparring.…”

Section: Sensory Nerve Action Potential (Snap)unclassified

Peran Pemeriksaan Elektrofisiologi Dalam Diagnosis Sindrom Miller-Fisher

2020

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Sindrom Miller-Fisher merupakan salah satu varian Sindrom Guillain-Barre yang memiliki gambaran klinis unik dengan trias klasik optalmoplegia, ataksia dan arefleksia. Pemeriksaan penunjang pada Sindrom Miller-Fisher berperan penting karena beberapa kondisi penyakit juga dapat bermanifestasi seperti trias klasik Sindrom Miller-Fisher. Penunjang berupa analisis cairan serebrospinal, pencitraan otak, biomarker antibodi GQ1b telah banyak digunakan untuk menegakkan diagnosis Sindrom Miller-Fisher namun memiliki keterbatasan. Pemeriksaan elektrofisiologi kemudian banyak diteliti karena selain menunjang diagnosis dapat menjelaskan proses penyakit Sindrom Miller-Fisher. Penulisan ini bertujuan untuk membahas peran pemeriksaan elektrofisiologi dalam diagnosis Sindrom Miller-Fisher. Hasil studi menunjukkan bahwa abnormalitas amplitude Sensory Nerve Action Potential (SNAP) dan H-reflex secara konsisten terjadi pada Sindrom Miller-Fisher. Abnormalitas amplitudo SNAP akan membaik dengan cepat dan reversibel sehingga diperlukan juga pemeriksaan elektrofisiologi serial untuk dapat menunjang diagnosis Sindrom Miller-Fisher. Temuan tersebut dapat diperkuat dengan hasil pemeriksaan konduksi saraf motorik, konduksi saraf sensorik dan F-wave yang normal. Kata Kunci: Sindrom Miller-Fisher, Elektrofisiologi, Diagnosis

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Establishment of a new classification system for chronic inflammatory demyelinating polyneuropathy based on unsupervised machine learning

Chang

Lyu

et al. 2022

Muscle and Nerve

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Introduction/Aims: A model for predicting responsiveness to immunotherapy in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) has not been well established. We aimed to establish a new classifier for CIDP patients based on clinical characteristics, laboratory findings, and electrophysiological features. Methods: The clinical, laboratory, and electrophysiological features of 172 treatment-naïve patients with CIDP between 2003 and 2019 were analyzed using an unsupervised hierarchical clustering. The identified pivotal features were used to establish simple classifications using a tree-based model. Results: Three clusters were identified: 1, n = 65; 2, n = 70; and 3, n = 37. Patients in Cluster 1 scored lower on the disability assessment score before treatment. More patients in Clusters 2 (90.0%) fulfilled demyelinating criteria than patients in Cluster 1 (30.8%, p < .001). Cluster 3 had more patients with chronic kidney disease (CKD) (27.0%) and hypoalbuminemia (3.40 g/dL) than did Cluster 2 (CKD: 0%, p < .001; hypoalbuminemia: 4.09 g/dL, p < .001). The responsiveness to pulse steroid therapy was higher in Cluster 2 (70.0%) than in Clusters 1 (31.8%; p = .043) and 3 (25.0%; p = .014). A tree-based model with four pivotal features classified patients in our cohort into new clusters with high accuracy (89.5%). Discussion: The established hierarchical clustering with the tree-based model identified key features contributing to differences in disease severity and response to pulse steroid therapy. This classification system could assist clinicians in the selection of treatments and could also help researchers by clustering patients for clinical treatment trials.

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Sensory Conduction Study in Fisher Syndrome: Patterns of Abnormalities and Their Clinical Correlation

Cited by 9 publications

References 24 publications

Motor Conduction Findings in Fisher Syndrome

Motor Conduction Findings in Fisher Syndrome

Peran Pemeriksaan Elektrofisiologi Dalam Diagnosis Sindrom Miller-Fisher

Establishment of a new classification system for chronic inflammatory demyelinating polyneuropathy based on unsupervised machine learning

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