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.

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.

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.

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.

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.

Thermoregulation is a critical aspect of homeostasis in mammals, ensuring that body temperature remains within a narrow, optimal range. The neural system plays a pivotal role in this process, integrating various signals and orchestrating responses to maintain thermal balance. This review delivers into the mechanisms by which the neural system regulates body temperature, the central and peripheral components involved, and the implications of thermoregulatory dysfunction.

Neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are common causes of optic neuritis, typically involving anti-aquaporin 4 (AQP4) and Myelin oligodendrocyte glycoprotein (MOG) antibodies. It is scarce for both AQP4 and MOG antibodies to be simultaneously positive. Although NMOSD and MOGAD share clinical features, they differ in symptoms, magnetic resonance imaging findings and outcomes. Both conditions are treated with steroids and immunoglobulins but exhibit distinct responses. We report a case of NMOSD which was double seropositive for AQP-4 and MOG antibodies.

Neuropathic pain is defined as pain caused by lesions or diseases of the somatosensory system. Management of neuropathic pain is difficult owing to the heterogeneity of underlying causes and absence of objective standards for diagnosis and assessment of neuropathic pain. In this review, we reviewed the recently published guidelines regarding the pharmacological management of neuropathic pain including the French recommendations and the German Neurological Association guidelines, and the American Neurological Association's guidelines for the management of diabetic polyneuropathy. In general, drugs recommended as the first-line treatment for neuropathic pain include gabapentinoid, serotonin–norepinephrine reuptake inhibitor (SNRI), and tricyclic antidepressants. As the second-line treatment, combination of SNRI or tricyclic antidepressant with gabapentinoid could be recommended. Although tramadol and tapentadol are recommended as the second- or third-line treatment, caution is required in long-term use of these medications due to the side effects including dependence and abuse. Besides efficacy, it is necessary to consider the side effects of the medication and underlying disease of the patient in selecting pharmacological treatment.

Valosin-containing protein (VCP)-related multisystem proteinopathy (MSP1) is a rare genetic disorder marked by abnormal protein accumulation. This study presents the case of a 52-year-old woman with MSP1, showing progressive weakness, gait disturbances, and respiratory muscle weakness over five years. The clinical examination revealed diverse presentations, including neurogenic changes in electrophysiologic study, multifocal fatty changes of muscle, and cognitive impairment with a confirmed VCP gene mutation through genetic testing. Notably, we identified lobulated myofibers in the muscle biopsy, an unusual finding in MSP1. This is the first report of lobulated myofibers in MSP1 with multisystem involvement. Identifying unique muscle biopsy results in suspected MSP1 patients through careful neurological examinations and timely genetic testing may help in early diagnosis and appropriate management.

Background: The brain magnetic resonance imaging (MRI) findings seen in patients with Bell's palsy are abnormal contrast enhancement of affected facial nerves. Previous evaluation included mainly patients who had experienced palsy for several weeks and there are no studies to date on patients with late stage of Bell’s palsy. This study investigated the findings of MRI of Bell's palsy after 3 months of symptom onset to assess how long the enhancement last. Methods: Among the patients with Bell's palsy (idiopathic unilateral facial palsy) who visited the hospital, 9 patients who underwent contrast-enhanced MRI of the internal auditory canal after 3 months of symptom onset were reviewed retrospectively. MRI examination results were investigated along with the patient's clinical symptoms and electrodiagnostic test results. Based on the MRI results, the frequency of abnormal contrast enhancement and contrast-enhanced areas were investigated. Results: 9 patients were included. 6 of them did MRI imaging because of incomplete recovery of facial palsy and the others did because of complication of facial palsy including synkinesis and hemifacial spasm. Time interval between symptom onset and evaluation was 17 months (3-84). Of 9 patients, 2 showed abnormal enhancement of affected nerve and they performed MRI after 5 months and 12 months of symptom onset, respectively. Conclusions Abnormal enhancement of facial nerve in Bell’s palsy could last up to one year. This awareness can be helpful in interpretation of MRI of Bell’s palsy.