Hye-Rim Shin

Neuronal intranuclear inclusion disease (NIID) is a rare disorder with slowly progressive neurodegeneration. Major clinical presentation is dementia, but some patients can have episodic symptoms. This is a rare case of NIID presented with acute aphasia. A 62-years-old male visited emergency room presenting with aphasia. On brain magnetic resonance imaging, a U-shaped diffusion high signal intensity lesion in bilateral frontal corticomedullary junction was observed. A skin biopsy showed intranuclear cytoplasmic inclusion bodies, so he was diagnosed as NIID.

This case report presents pathological findings in a rare case of cerebral venous thrombosis (CVT) caused by spontaneous intracranial hypotension (SIH) that was treated successfully with mechanical thrombectomy. The etiologies and prognosis of CVT vary, and CVT resulting from SIH is particularly uncommon and challenging to diagnose and manage. In this case, magnetic resonance imaging revealed signs consistent with both SIH and CVT, which contributed to the patient’s worsening weakness. The patient was treated with a combination of endovascular thrombectomy and epidural blood patch, followed by anticoagulation therapy, and recovered without any complications. A pathological analysis of the retrieved thrombus using hematoxylin and eosin staining showed a high proportion of fibrin and platelets, which could shed light on the mechanism of CVT induced by SIH under conditions of low blood flow due to venous engorgement.

This review delves into the complex facets of sleep bruxism (SB), concentrating on its diagnosis, epidemiology, orofacial consequences, comorbidities including sleep disorders, and management strategies. Diverse studies have highlighted the difficulties associated with accurately diagnosing SB, the shifting perceptions surrounding it, variations in prevalence based on age and SB definitions, insights obtained from polysomnography, current knowledge on orofacial impact, and approaches to management. Notably, the association between SB and sleep disorders, especially obstructive sleep apnea, and its possible physiological roles are examined. This review underscores the necessity of a balanced appreciation for SB, accentuating the need for comprehensive evaluations and tailored management strategies. It emphasizes the continual reshaping of SB concepts and the critical need for further research to differentiate between physiological and pathological manifestations of SB, with the goal of optimizing treatment methods.

Rapid eye movement (REM) sleep behavior disorder (RBD) is characterized by dream enactment behavior and REM sleep without atonia and is traumatic for the patient and/or bedpartner. Obstructive sleep apnea (OSA) is a common comorbidity of RBD, and several reports have suggested that severe OSA may aggravate RBD symptoms. This case report discusses a patient with medically intractable RBD combined with OSA, confirmed by 10-year follow-up polysomnography (PSG) data, which improved with nasal continuous positive airway pressure (CPAP) therapy. The clinical symptoms of RBD markedly improved after CPAP treatment. Clinicians should raise the clinical suspicion of OSA in patients with intractable RBD and consider follow-up PSG and OSA treatments.

Rapid eye movement (REM) sleep behavior disorder (RBD) is diagnosed based on a history of dream-enactment behavior and documentation of REM sleep without atonia (RWA) on polysomnography (PSG). RWA can be quantified using various methods. To establish comprehensive and clear PSG criteria for diagnosing RBD, the International RBD Study Group (IRBDSG) has recently published guidelines, which include the use of a video-PSG technical setup, REM sleep staging, RWA scoring, video and audio recording and analyses, and a video-PSG procedure for the diagnosis and identification of the prodromal stages of neurodegenerative diseases. Although the guidelines can identify patients with homogeneous RBD, their applicability in a real-world setting, particularly in Korea, presents challenges. Therefore, the aim of the present study was to evaluate and introduce IRBDSG guidelines and results of Korean sleep experts’ opinion survey for diagnosing RBD based on PSG findings.

Sleep bruxism (SB) is a common disorder that presents as repetitive jaw muscle activity with clenching or grinding of teeth and/or bracing or thrusting of the mandible. Several studies have suggested that SB is associated with obstructive sleep apnea (OSA). However, the effect of treatment with continuous positive airway pressure (CPAP) on SB concomitant with OSA has not been well recognized, except in a few reported cases. Herein, we present three cases of SB with various OSA severities that were successfully treated with CPAP, as indicated by the improvement in their clinical symptoms and bruxism index on polysomnography. Our results suggest that clinicians should raise suspicion of OSA in patients with SB and be aware that patients with SB and concomitant moderate-to-severe OSA can be successfully treated with CPAP.

Yun S, et al. J Korean Med Sci. 2022 Feb;37(8):e61. https://doi.org/10.3346/jkms.2022.37.e61

Shin HR, et al. J Clin Neurol. 2022 Jan;18(1):114-116. https://doi.org/10.3988/jcn.2022.18.1.114