Fourteen Unrecorded Species of Agaricales Underw. (Agaricomycetes, Basidiomycota) from the Republic of Korea

Agaricales species form pileate-stipitate fruiting bodies and play important roles in maintaining the terrestrial ecosystem as decomposers, symbionts, and pathogens. Approximately 23,000 Agaricales species have been known worldwide, and 937 species have been recorded in the Republic of Korea. However, most of them were identified solely based on morphological characteristics that often led to misidentifications. The specimens collected from 2018 to 2020 in the Republic of Korea were identified based on phylogenetic analysis of the internal transcribed spacer (ITS) sequences. Their identities were confirmed by microscopic characteristics. As a result, 14 Agaricales species were discovered for the first time in the Republic of Korea. They belonged to nine genera: Agaricus, Calocybe, Cortinarius, Hygrocybe, Inocybe, Lepista, Leucoagaricus, Marasmius, and Psathyrella. Detailed macroscopic and microscopic descriptions were provided to help distinguish these species. The morphological and molecular data provided in this study will serve as reliable references for the identification of Agaricales species.

Extracerebellar Signs and Symptoms in 117 Korean Patients with Early-Stage Spinocerebellar Ataxia

Background@#and Purpose Spinocerebellar ataxias (SCAs) are the most common form of hereditary ataxias. Extracerebellar signs have been well described and are helpful in differentiating the SCA subtypes. However, there are few reports on the early-stage extracerebellar signs in various SCA subtypes. This study explored the clinical and magnetic resonance imaging (MRI) characteristics of early-stage SCAs in the Korean population. @*Methods@#We retrospectively reviewed the medical records of genetically confirmed SCA patients with a disease duration of <5 years. Data on baseline characteristics, extracerebellar signs, and initial MRI findings were organized based on SCA subtypes. @*Results@#This study included 117 SCA patients with a median age at onset of 40.6 years. The family history was positive in 71.8% of the patients, and the median disease duration and the score on the Scale for the Assessment and Rating of Ataxia at the initial visit were 2.6 years and 5.0, respectively. SCA3 was the most prevalent subtype, and oculomotor abnormalities were the most frequent extracerebellar signs in early-stage SCAs. Saccadic slowing was characteristic of SCA2 and SCA7, and gaze-evoked nystagmus was prominent in SCA6. Parkinsonism was relatively frequent in SCA8 and SCA3. Decreased visual acuity was specific for SCA7. Dementia was not an early manifestation of SCAs. Brain MRI revealed a pattern of pontocerebellar atrophy in SCA2 and SCA7, while SCA6 demonstrated only cerebellar cortical atrophy. @*Conclusions@#SCA patients exhibited diverse extracerebellar signs even in the early stage.Specific extracerebellar signs were characteristic of specific subtypes, which could facilitate differential diagnoses of early-stage SCAs.

Extracerebellar Signs and Symptoms in 117 Korean Patients with Early-Stage Spinocerebellar Ataxia

Background@#and Purpose Spinocerebellar ataxias (SCAs) are the most common form of hereditary ataxias. Extracerebellar signs have been well described and are helpful in differentiating the SCA subtypes. However, there are few reports on the early-stage extracerebellar signs in various SCA subtypes. This study explored the clinical and magnetic resonance imaging (MRI) characteristics of early-stage SCAs in the Korean population. @*Methods@#We retrospectively reviewed the medical records of genetically confirmed SCA patients with a disease duration of <5 years. Data on baseline characteristics, extracerebellar signs, and initial MRI findings were organized based on SCA subtypes. @*Results@#This study included 117 SCA patients with a median age at onset of 40.6 years. The family history was positive in 71.8% of the patients, and the median disease duration and the score on the Scale for the Assessment and Rating of Ataxia at the initial visit were 2.6 years and 5.0, respectively. SCA3 was the most prevalent subtype, and oculomotor abnormalities were the most frequent extracerebellar signs in early-stage SCAs. Saccadic slowing was characteristic of SCA2 and SCA7, and gaze-evoked nystagmus was prominent in SCA6. Parkinsonism was relatively frequent in SCA8 and SCA3. Decreased visual acuity was specific for SCA7. Dementia was not an early manifestation of SCAs. Brain MRI revealed a pattern of pontocerebellar atrophy in SCA2 and SCA7, while SCA6 demonstrated only cerebellar cortical atrophy. @*Conclusions@#SCA patients exhibited diverse extracerebellar signs even in the early stage.Specific extracerebellar signs were characteristic of specific subtypes, which could facilitate differential diagnoses of early-stage SCAs.

First Report of Six Macrofungi from Daecheongdo and Socheongdo Islands, Korea

Daecheongdo and Socheongdo Islands are located in the West Sea of Korea, 210 km away from land, and are military border areas very close to North Korea, making them difficult to access. Although the ecosystem of the islands is relatively well preserved due to the lack of accessibility, research on fungi of the regions is insufficient. Therefore, we aimed to investigate indigenous fungi in these geographically and geopolitically constrained regions. A survey of the indigenous fungal diversity of the islands was conducted in 2018. All specimens were identified at the species level based on morphological and molecular analyses. Among them, six macrofungi—namely, Agaricus menieri, Crepidotus praecipuus, Dichomitus squalens, Hortiboletus amygdalinus, Melanoleuca friesii, and Trametes lactinea—were not previously reported in Korea. Considering that the proportion of unrecorded species is high in the survey area and period as well as the number of samples collected, similar research on adjacent islands may be necessary.

Successful Pallidal Stimulation in a Patient with KMT2B-Related Dystonia

Although the KMT2B gene was identified as a causative gene for early-onset generalized dystonia, the efficacy of deep brain stimulation (DBS) in KMT2B-related dystonia has not been clearly elucidated. Here, we describe a 28-year-old woman who developed generalized dystonia with developmental delay, microcephaly, short stature, and cognitive decline. She was diagnosed with KMT2B- related dystonia using whole-exome sequencing with a heterozygous frameshift insertion of c.515dupC (p.T172fs) in the KMT2B gene. Oral medications and botulinum toxin injection were not effective. The dystonia markedly improved with bilateral pallidal DBS (the Burke-Fahn-Marsden Dystonia Rating Scale score was reduced from 30 to 5 on the dystonia movement scale and from 11 to 1 on the disability scale), and she could walk independently. From this case, we suggest that bilateral globus pallidus internus DBS can be an effective treatment option for patients with KMT2B-related generalized dystonia.

Association between phytochemical index and metabolic syndrome

BACKGROUND/OBJECTIVES@#Although previous experimental studies reported the health benefits of foods rich in phytochemicals, few epidemiologic studies have investigated the associations between phytochemicals and metabolic disorders. This study aimed to calculate a phytochemical index (PI) and examine its association with metabolic syndrome in the Korean population. @*SUBJECTS/METHODS@#Data of Korean adults aged ≥ 19 years who participated in the 2008–2016 Korea National Health and Nutrition Examination Surveys were analyzed. The PI was calculated using 24-hour intake recall data regarding whole grains, vegetables, fruits, legumes, nuts and seeds, and soybeans and soy products. Demographic and lifestyle data were obtained using self-administered questionnaires. A multivariable logistic regression was performed to calculate the adjusted odds ratios (ORs) and 95% confidence intervals (CIs) for the prevalence of metabolic syndrome and its components according to PI quintiles. @*RESULTS@#Overall, 31,319 adults were evaluated. Compared with men, women had a higher median PI level (9.96 vs. 13.63) and significantly higher caloric intake levels from most PI components (P < 0.05), except for soy products. After adjusting for multiple confounding variables, participants in the highest PI quintile had significantly lower prevalences of abdominal obesity (OR: 0.90, 95% CI: 0.81–0.99), hyperglycemia (OR: 0.83, 95% CI: 0.74– 0.94), high blood pressure (OR: 0.82, 95% CI: 0.73–0.93), hypertriglyceridemia (OR: 0.84, 95% CI: 0.75–0.94), and metabolic syndrome (OR: 0.78, 95% CI: 0.69–0.88). @*CONCLUSIONS@#Higher intakes of phytochemical-rich foods are associated with a lower prevalence of metabolic dysregulation and consequently, cardiometabolic diseases.

Uncontrolled Blood Pressure in Hypertensive Patients with High Medication Adherence: A Korean Nationwide Population-Based Study / 가정의학회지

Background@#Uncontrolled blood pressure (BP) is relatively common despite treatment with antihypertensive medication. This study aimed to investigate factors associated with uncontrolled systolic BP (SBP) and diastolic BP (DBP) separately in hypertensive patients with high medication adherence in Korea. @*Methods@#This population-based cross-sectional study included 3,236 participants in the Korea National Health and Nutrition Examination Survey from 2013 to 2015. Hypertensive patients with high medication adherence were defined when they stated that they were taking antihypertensive medication daily. “Uncontrolled SBP and DBP” were defined as SBP ≥140 mm Hg and DBP ≥90 mm Hg, respectively. Data were analyzed using logistic regression to determine the association between uncontrolled SBP or DBP and various factors. @*Results@#The prevalence of uncontrolled SBP and uncontrolled DBP was 23.3%, and 9.0%, respectively. In multivariate analysis, uncontrolled SBP was positively associated with older age, female sex, blue-collar occupation or no work (vs. white-collar), presence of a spouse, short sleep duration, frequent heavy alcohol drinking, longer hypertension duration, diabetes, stroke, and missing antihypertensive medication on the examination day, but negatively associated with high stress. Uncontrolled DBP was positively associated with frequent heavy alcohol drinking, but negatively associated with older age, blue-collar occupation or no work (vs. white-collar), and diabetes. @*Conclusion@#After excluding low medication adherence, we identified various factors associated with uncontrolled SBP or DBP in Korean adults with treated hypertension. There were differences between factors associated with uncontrolled SBP or DBP. Separate analysis of SBP and DBP may help understand the factors involved in BP control.

The Effect of Globus Pallidus Interna Deep Brain Stimulation on a Dystonia Patient with the GNAL Mutation Compared to Patients with DYT1 and DYT6

OBJECTIVE: The aim of this study was to investigate the efficacy of globus pallidus interna deep brain stimulation (GPi-DBS) for treating dystonia due to the GNAL mutation. METHODS: We provide the first report of a dystonia patient with a genetically confirmed GNAL mutation in the Korean population and reviewed the literature on patients with the GNAL mutation who underwent GPi-DBS. We compared the effectiveness of DBS in patients with the GNAL mutation compared to that in patients with DYT1 and DYT6 in a previous study. RESULTS: Patients with the GNAL mutation and those with DYT1 had higher early responder rates (GNAL, 5/5, 100%; DYT1, 7/7, 100%) than did patients with DYT6 (p = 0.047). The responder rates at late follow-up did not differ statistically among the three groups (p = 0.278). The decrease in the dystonia motor scale score in the GNAL group was 46.9% at early follow-up and 63.4% at late follow-up. CONCLUSION: GPi-DBS would be an effective treatment option for dystonia patients with the GNAL mutation who are resistant to medication or botulinum toxin treatment.

Self-management levels of diet and metabolic risk factors according to disease duration in patients with type 2 diabetes

BACKGROUND/OBJECTIVES: Metabolic risk factors should be managed effectively in patients with type 2 diabetes mellitus (T2DM) to prevent or delay diabetic complications. This study aimed to compare the self-management levels of diet and metabolic risk factors in patients with T2DM, according to the duration of illness, and to examine the trends in self-management levels during the recent decades. SUBJECTS/METHODS: Data were collected from the Korea National Health and Nutrition Examination Surveys (KNHANES, 1998–2014). In our analysis, 4,148 patients with T2DM, aged ≥ 30 years, were categorized according to the duration of their illness (< 5 years, 5–9 years, and ≥ 10 years). Demographic and lifestyle information was assessed through self-administered questionnaires, and biomarker levels (e.g., fasting glucose level, blood pressure, or lipid level) were obtained from a health examination. Dietary intake was assessed by a 24-recall, and adherence level to dietary guidelines (meal patterns and intake levels of calories, carbohydrates, vegetable/seaweed, sodium, and alcohol) were assessed. Multivariable generalized linear regression and unconditional logistic regression models were used to compare the prevalence rates of hyperglycemia, dyslipidemia, and hypertension according to the duration of patients' illness, accounting for the complex survey design of the KNHANES. RESULTS: In the multivariable adjusted models, patients with a longer duration (≥ 10 years) of T2DM had a higher prevalence of hyperglycemia than those with a shorter duration of T2DM (< 5 years) (odds ratio 2.20, 95% confidence interval 1.61–3.01, P for trend < 0.001). We did not observe any associations of disease duration with the prevalence of hypertension and dyslipidemia. In addition, the adherence levels to dietary recommendations did not significantly differ according to disease duration, except adherence to moderate alcohol consumption. There were significant decreasing trends in the prevalence of hyperglycemia in patients with a duration of illness ≥ 10 years (P for trend = 0.004). CONCLUSION: Although the proportion of patients with adequate control of glucose levels has improved in recent decades, poorer self-management has been found in those with a longer disease duration. These findings suggest the need for well-planned and individualized patient education programs to improve self-management levels and quality of life by preventing or delaying diabetic complications.

Three New Recorded Species of the Physalacriaceae on Ulleung Island, Korea

Most known species in the Physalacriaceae are saprotrophs that grow on decaying leaves and wood, and approximately 21 genera in the Physalacriaceae have been reported worldwide. During an ongoing survey of indigenous fungi in Korea, four specimens belonging to the Physalacriaceae were collected on Ulleung Island. These specimens were identified as three species based on morphological characteristics and molecular analysis of rDNA-internal transcribed spacer sequences. Three species in three genera were shown to be new records in Korea: Hymenopellis orientalis, Paraxerula hongoi, and Ponticulomyces orientalis. The latter two are the first records of these genera in Korea. In this study, we provide detailed morphological descriptions of these species and describe their phylogenetic position within the Physalacriaceae.

The Fungicolous Ascomycetes Genus Hypomyces in Korea

The genus Hypomyces contains fungi that grow on mushrooms, including agarics, boletes, and Aphyllophorales. While 53 Hypomyces species have been reported worldwide, only one was in Korea. In this study, two new Korean species were identified as H. luteovirens and H. tubariicola based on morphology and internal transcribed spacer sequencing.

Occipital Lobe Epilepsy with Hemicrania Epileptica

No abstract available.

A Case of Hypersomnolence with Subclinical Hypothyroidism Treated with Levothyroxine

We present a patient who complained of excessive daytime sleepiness (EDS), which started three years ago. She had no other medical, neurological, and psychiatric disorders. Nocturnal polysomnography did not indicate any sleep disorders, which might cause daytime EDS, such as obstructive sleep apnea. The following multiple sleep latency test was not compatible for narcolepsy. Her laboratory findings were remarkable for subclinical hypothyroidism, although free T4 and T3 were within reference rage, she had elevated thyroid stimulating hormone. After four weeks of levothyroxine treatment, her EDS resolved. The hypersomnolence, as a presenting symptom of subclinical hypothyroidism, was optimally treated after thyroid hormone replacement.