1.Updates of Tolosa-Hunt Syndrome
Korean Journal of Neuromuscular Disorders 2024;16(2):27-32
Tolosa-Hunt syndrome (THS) is a rare neurological disorder characterized by painful ophthalmoplegia due to idiopathic granulomatous inflammation of the cavernous sinus or orbital apex. This review aims to summarize recent advances in the etiology, diagnosis, and management of THS.
2.Immune Checkpoint Inhibitor-associated Myositis in a Patient with Metastatic Cholangiocarcinoma
Chung Seok LEE ; Soo-Hyun KIM ; Sung Rok LEE ; Young-Chul CHOI ; Hyung Jun PARK
Korean Journal of Neuromuscular Disorders 2024;16(2):43-46
An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.
3.Postherpetic Neuralgia: Current Perspectives and Treatment Strategies
Korean Journal of Neuromuscular Disorders 2024;16(2):33-37
Postherpetic neuralgia (PHN) is a chronic neuropathic pain syndrome that persists for more than three months after the resolution of herpes zoster rash. This condition affects approximately 30% of the population, primarily the elderly and immunocompromised individuals. PHN can be severely debilitating and often proves challenging to treat effectively. Recent advances in prevention and treatment have improved the management of PHN. The introduction of herpes zoster vaccines has shown promise in reducing the incidence of both herpes zoster and PHN. Current treatment guidelines recommend a multimodal approach, with effective therapies including tricyclic antidepressants, topical lidocaine, gabapentin, pregabalin, opioids, and topical capsaicin. This review focuses on optimizing treatment strategies and developing novel therapies to further enhance PHN management.
4.Update of Critical Illness Neuromyopathy
Korean Journal of Neuromuscular Disorders 2024;16(2):21-26
Critical illness neuromyopathy (CINM) is a common yet frequently overlooked complication in intensive care units (ICU). CINM often results in prolonged ventilator dependence and persistent limb weakness, significantly impacting patient recovery and long-term quality of life. CINM can be categorized into two primary subtypes: critical illness polyneuropathy (CIP) and critical illness myopathy (CIM). These conditions frequently arise in the context of severe sepsis, multiple organ failure, or as adverse effects of certain medications used in the ICU. This review aims to provide a concise overview of CINM, focusing on its pathophysiology, diagnostic approaches, and current clinical management strategies.
5.Patterns of Brain Lesions in Adult-Onset Myotonic Dystrophy Type 1: A Quantitative MRI Study
Nam-Hee KIM ; Dong-Eog KIM ; Kyung-Seok PARK ; Soo-Hyun PARK
Korean Journal of Neuromuscular Disorders 2024;16(2):38-42
Background:
Myotonic dystrophy type 1 (DM1) is a multisystemic disorder caused by CTG repeat expansion in the DMPK gene. Brain involvement in DM1 has been reported, but quantitative imaging data is limited. This study aimed to characterize and quantify brain structural abnormalities in DM1 using MRI and DUIH-Image software.
Methods:
Sixteen patients with classic DM1 (10 male, 6 female; age range 17–68 years) underwent genetic testing, neuropsychologic assessment, and brain MRI. FLAIR images were analyzed using DUIH-Image software to quantify brain lesions and create probabilistic lesion maps.
Results:
FLAIR imaging revealed hyperintensities predominantly in periventricular and subcortical white matter. Probabilistic lesion maps highlighted the subcortical white matter near the anterior horn as the most affected site, with additional involvement of the posterior horn, frontal, insular, parietal, and temporal cortices. Neuropsychological testing identified frontal lobe dysfunction as the most common deficit, correlating with brain involvement. No significant correlations were found between lesion load, CTG repeat number, and disease duration.
Conclusions
This study demonstrates significant periventricular and subcortical white matter involvement in DM1, with the anterior subcortical white matter being the most affected site. Frontal lobe dysfunction was a frequent finding. Brain involvement did not correlate with CTG repeat number or disease duration, suggesting complex pathophysiology in DM1-related brain abnormalities.
6.Updates of Tolosa-Hunt Syndrome
Korean Journal of Neuromuscular Disorders 2024;16(2):27-32
Tolosa-Hunt syndrome (THS) is a rare neurological disorder characterized by painful ophthalmoplegia due to idiopathic granulomatous inflammation of the cavernous sinus or orbital apex. This review aims to summarize recent advances in the etiology, diagnosis, and management of THS.
7.Immune Checkpoint Inhibitor-associated Myositis in a Patient with Metastatic Cholangiocarcinoma
Chung Seok LEE ; Soo-Hyun KIM ; Sung Rok LEE ; Young-Chul CHOI ; Hyung Jun PARK
Korean Journal of Neuromuscular Disorders 2024;16(2):43-46
An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.
8.Postherpetic Neuralgia: Current Perspectives and Treatment Strategies
Korean Journal of Neuromuscular Disorders 2024;16(2):33-37
Postherpetic neuralgia (PHN) is a chronic neuropathic pain syndrome that persists for more than three months after the resolution of herpes zoster rash. This condition affects approximately 30% of the population, primarily the elderly and immunocompromised individuals. PHN can be severely debilitating and often proves challenging to treat effectively. Recent advances in prevention and treatment have improved the management of PHN. The introduction of herpes zoster vaccines has shown promise in reducing the incidence of both herpes zoster and PHN. Current treatment guidelines recommend a multimodal approach, with effective therapies including tricyclic antidepressants, topical lidocaine, gabapentin, pregabalin, opioids, and topical capsaicin. This review focuses on optimizing treatment strategies and developing novel therapies to further enhance PHN management.
9.Update of Critical Illness Neuromyopathy
Korean Journal of Neuromuscular Disorders 2024;16(2):21-26
Critical illness neuromyopathy (CINM) is a common yet frequently overlooked complication in intensive care units (ICU). CINM often results in prolonged ventilator dependence and persistent limb weakness, significantly impacting patient recovery and long-term quality of life. CINM can be categorized into two primary subtypes: critical illness polyneuropathy (CIP) and critical illness myopathy (CIM). These conditions frequently arise in the context of severe sepsis, multiple organ failure, or as adverse effects of certain medications used in the ICU. This review aims to provide a concise overview of CINM, focusing on its pathophysiology, diagnostic approaches, and current clinical management strategies.
10.Patterns of Brain Lesions in Adult-Onset Myotonic Dystrophy Type 1: A Quantitative MRI Study
Nam-Hee KIM ; Dong-Eog KIM ; Kyung-Seok PARK ; Soo-Hyun PARK
Korean Journal of Neuromuscular Disorders 2024;16(2):38-42
Background:
Myotonic dystrophy type 1 (DM1) is a multisystemic disorder caused by CTG repeat expansion in the DMPK gene. Brain involvement in DM1 has been reported, but quantitative imaging data is limited. This study aimed to characterize and quantify brain structural abnormalities in DM1 using MRI and DUIH-Image software.
Methods:
Sixteen patients with classic DM1 (10 male, 6 female; age range 17–68 years) underwent genetic testing, neuropsychologic assessment, and brain MRI. FLAIR images were analyzed using DUIH-Image software to quantify brain lesions and create probabilistic lesion maps.
Results:
FLAIR imaging revealed hyperintensities predominantly in periventricular and subcortical white matter. Probabilistic lesion maps highlighted the subcortical white matter near the anterior horn as the most affected site, with additional involvement of the posterior horn, frontal, insular, parietal, and temporal cortices. Neuropsychological testing identified frontal lobe dysfunction as the most common deficit, correlating with brain involvement. No significant correlations were found between lesion load, CTG repeat number, and disease duration.
Conclusions
This study demonstrates significant periventricular and subcortical white matter involvement in DM1, with the anterior subcortical white matter being the most affected site. Frontal lobe dysfunction was a frequent finding. Brain involvement did not correlate with CTG repeat number or disease duration, suggesting complex pathophysiology in DM1-related brain abnormalities.

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