Upbeat nystagmus has been described in bilateral lesions of the medulla, the ventral tegmentum, the anterior cerebellar vermis, the adjacent brachium conjunctivum, and the midbrain. Imbalance of the vertical vestibulo- ocular reflex (VOR) favoring the downward VOR activity would therefore result in upbeat nystagmus. We report a patient with transient upbeat nystagmus due to unilateral pontine infarction that may have disrupted bilateral upward VOR pathways running in the ventral tegmental tracts.
Humans ; Infarction* ; Mesencephalon ; Reflex ; Running

Herpes simplex encephalitis (HSE) is the most common type of viral encephalitis and a fourteen day administration of acyclovir is well-known as the treatment of choice for HSE. Occasionally HSE relapses, but rarely with acyclovir treatment. We report a case of relapsing HSE after treatment with acyclovir for 14 days. Our case suggests that patients with progressive high signal intensities in diffusion-weighted brain MRIs might need longer antiviral therapy over 14 days for preventing the relapse of herpes simplex encephalitis.
Acyclovir ; Brain ; Encephalitis, Herpes Simplex* ; Encephalitis, Viral ; Herpes Simplex* ; Humans ; Magnetic Resonance Imaging ; Recurrence

Enchondromas are common benign bone tumors of the hand and are typically located in the medullary cavity of the metaphysis of the tubular bones. Typical radiological findings reveal cartilage lobules with ring and arc calcifications and entrapped normal medullary fat. The regression of an existing enchondroma, in which the cartilage lobules are replaced by fatty marrow, is considered unusual. Only a few studies have been reported on long tubular bones. Herein, we report an unusual case of a 73-year-old man with an enchondroma in the hand. In this case, a histologic appearance of the enchondroma exhibited loss of matrix mineralization, which corresponded to replacement by marrow fat revealed by magnetic resonance imaging.

Background: and Purpose The wearing-off (WO) phenomenon is the most common motor complication in advanced Parkinson’s disease (PD), but its identification remains challenging. The 9- and 19-item Wearing-off Questionnaires (WOQ-9 and WOQ-19) are self-assessment tools for motor and nonmotor symptoms that are widely used for WO screening. We produced Korean versions of the WOQ-19 and WOQ-9 (K-WOQ-19 and K-WOQ-9) and investigated their validity and reliability. Methods: We used the translation–back translation method to produce K-WOQ-19 and KWOQ-9, which were self-administered by 124 patients with PD. We conducted in-depth 10-minute interviews for confirming the presence of the WO phenomenon, and then stratified the participants into groups with and without WO. Diagnostic accuracy was assessed by analyzing receiver operating characteristic curves. Concurrent validity was assessed using the Movement Disorder Society–Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) and the Hoehn and Yahr stage with Spearman’s rank correlation analysis. Reliability was assessed based on test–retest Cohen’s kappa (κ) values and intraclass correlation coefficients (ICCs). Results: The optimal cutoff scores on the K-WOQ-19 and K-WOQ-9 for WO screening were 4 and 2, respectively. The test–retest ICCs of K-WOQ-19 and K-WOQ-9 were 0.943 and 0.938, respectively. Nineteen of the combined 20 items in K-WOQ-19 and K-WOQ-9 showed moderate-to-substantial agreement (κ=0.412–0.771, p<0.001). The scores on the translated scales were significantly correlated with MDS-UPDRS IV scores. Conclusions K-WOQ-19 and K-WOQ-9 are reliable and valid tools for detecting WO, with optimal cutoff scores of 4 and 2, respectively.

Ludwig’s angina is a rapidly progressive and gangrenous cellulitis in the submandibular, sublingual spaces, and neck. It is characterized by an elevated tongue and laryngeal edema, resulting in airway compromise and, rarely, in sudden death. The most common cause is an odontogenic infection of a molar tooth. Infection can spread to the superior mediastinum and buccal area through anatomical structures. Herein, we report the sudden death of a 45-year-old male who was not diagnosed with Ludwig’s angina. He died in the emergency room 20 minutes after loss of consciousness. Intubation was impossible owing to severe laryngeal edema. The C-reactive protein level was 33.81 mg/dL. On autopsy, a diffuse light green abscess of the submandibular space was detected, along with severe edema of the epiglottis, aryepiglottic fold, and vocal cords. Subsequently, we confirmed that the airway obstruction was caused by Ludwig’s angina based on a literature review. In conclusion, this report emphasizes the need to identify the infection source and perform a detailed dissection according to the anatomical structure in the autopsy procedure for Ludwig’s angina.

BACKGROUND: Seizure can be triggered by the non-ketotic hyperglycemia (NKH). Recently we analysed 18 cases of NKH induced seizure to identify the causes for NKH, seizure types, prognosis, and the differences of clinical presentation between the patient with chronic brain structural lesion (CBSL) and the patient without. METHODS: Eighteen patients with NKH induced seizure were selected from the database. Data regarding brain images, clinical symptoms, co-morbid illnesses, blood laboratories, and prognosis were collected. Patients were divided into two groups according to the presence of CBSL. RESULTS: The patients with CBSL showed more generalized tonic-clonic seizure (GTCS) than without. Focal seizures in this group appeared to be originated from the pre-existing lesion in many situations. The poor compliance to anti-diabetic treatment and physical stresses were most common causes for NKH. One year seizure remission without anti-epileptic drug treatment was achieved in 17 of 18 patients. CONCLUSIONS: The patients with CBSL might have more GTCS than without. The impairment of inhibitory mechanism surrounding the focal irritative zone might be one of plausible explanation for this phenomenon. The prognosis was favorable. Further large studies are required.
Brain ; Compliance ; Humans ; Hyperglycemia ; Prognosis ; Seizures

Background: Because persistent fever often occurs in adrenal insufficiency, it might be confused with infectious diseases. This study aimed to identify clinical characteristics and risk factors of patients with adrenal insufficiency and fever. Methods: All adult patients (n = 150) admitted to a tertiary care hospital in South Korea and diagnosed with adrenal insufficiency between 1 March 2018, and 30 June 2019, were recruited. Patients were excluded if they had: 1) proven structural problems in the adrenal or pituitary gland; 2) a history of chemotherapy within 6 months prior to the diagnosis of adrenal insufficiency; and 3) other medical conditions that may cause fever. Results: Among the included patients, 45 (30.0%) had fever at the time of the diagnosis of adrenal insufficiency. The mean C-reactive protein level was higher (11.25 ± 8.54 vs. 4.36 ± 7.13 mg/dL) in patients with fever than in those without fever. A higher proportion of patients with fever changed antibiotics (33.3% vs. 1.0%). On multivariate logistic regression analysis, female sex (odds ratio [OR], 0.32) lowered the risk of adrenal insufficiency with fever, while a history of surgery within 6 months (OR, 4.35), general weakness (OR, 7.21), and cough (OR, 17.29) were significantly associated with that. Conclusion The possibility of adrenal insufficiency should be considered in patients with fever of unknown origin, especially those with risk factors.

Background: Because persistent fever often occurs in adrenal insufficiency, it might be confused with infectious diseases. This study aimed to identify clinical characteristics and risk factors of patients with adrenal insufficiency and fever. Methods: All adult patients (n = 150) admitted to a tertiary care hospital in South Korea and diagnosed with adrenal insufficiency between 1 March 2018, and 30 June 2019, were recruited. Patients were excluded if they had: 1) proven structural problems in the adrenal or pituitary gland; 2) a history of chemotherapy within 6 months prior to the diagnosis of adrenal insufficiency; and 3) other medical conditions that may cause fever. Results: Among the included patients, 45 (30.0%) had fever at the time of the diagnosis of adrenal insufficiency. The mean C-reactive protein level was higher (11.25 ± 8.54 vs. 4.36 ± 7.13 mg/dL) in patients with fever than in those without fever. A higher proportion of patients with fever changed antibiotics (33.3% vs. 1.0%). On multivariate logistic regression analysis, female sex (odds ratio [OR], 0.32) lowered the risk of adrenal insufficiency with fever, while a history of surgery within 6 months (OR, 4.35), general weakness (OR, 7.21), and cough (OR, 17.29) were significantly associated with that. Conclusion The possibility of adrenal insufficiency should be considered in patients with fever of unknown origin, especially those with risk factors.

Enchondromas are common benign bone tumors of the hand and are typically located in the medullary cavity of the metaphysis of the tubular bones. Typical radiological findings reveal cartilage lobules with ring and arc calcifications and entrapped normal medullary fat. The regression of an existing enchondroma, in which the cartilage lobules are replaced by fatty marrow, is considered unusual. Only a few studies have been reported on long tubular bones. Herein, we report an unusual case of a 73-year-old man with an enchondroma in the hand. In this case, a histologic appearance of the enchondroma exhibited loss of matrix mineralization, which corresponded to replacement by marrow fat revealed by magnetic resonance imaging.

Background: and Purpose The wearing-off (WO) phenomenon is the most common motor complication in advanced Parkinson’s disease (PD), but its identification remains challenging. The 9- and 19-item Wearing-off Questionnaires (WOQ-9 and WOQ-19) are self-assessment tools for motor and nonmotor symptoms that are widely used for WO screening. We produced Korean versions of the WOQ-19 and WOQ-9 (K-WOQ-19 and K-WOQ-9) and investigated their validity and reliability. Methods: We used the translation–back translation method to produce K-WOQ-19 and KWOQ-9, which were self-administered by 124 patients with PD. We conducted in-depth 10-minute interviews for confirming the presence of the WO phenomenon, and then stratified the participants into groups with and without WO. Diagnostic accuracy was assessed by analyzing receiver operating characteristic curves. Concurrent validity was assessed using the Movement Disorder Society–Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) and the Hoehn and Yahr stage with Spearman’s rank correlation analysis. Reliability was assessed based on test–retest Cohen’s kappa (κ) values and intraclass correlation coefficients (ICCs). Results: The optimal cutoff scores on the K-WOQ-19 and K-WOQ-9 for WO screening were 4 and 2, respectively. The test–retest ICCs of K-WOQ-19 and K-WOQ-9 were 0.943 and 0.938, respectively. Nineteen of the combined 20 items in K-WOQ-19 and K-WOQ-9 showed moderate-to-substantial agreement (κ=0.412–0.771, p<0.001). The scores on the translated scales were significantly correlated with MDS-UPDRS IV scores. Conclusions K-WOQ-19 and K-WOQ-9 are reliable and valid tools for detecting WO, with optimal cutoff scores of 4 and 2, respectively.