REM sleep behavior disorder is parasomnia characterized by unpleasant dreams and dream-enactment behaviors associated with excessive electromyography activity in REM sleep. This may appear idiopathic or secondary to other neurological or medical conditions. REM sleep behavior disorder, which appears to be idiopathic, most often implies the possibility of later neurodegenerative diseases due to synucleinopathy, so accurate diagnosis is important in predicting prognosis. For the diagnosis of REM sleep behavioral disorder, REM sleep without atonia, which appears in the polysomnography, is essential. Obstructive sleep apnea, trauma-related sleep disorders, and vigorous periodic leg movements during sleep are known as diseases that show dream enactment behavior in elderly patients. Considering that it may be accompanied by other sleep disorders that can mimic REM sleep behavioral disorders, it is important to differentiate sleep
Purpose: The purpose of study is to estimate the number of chronic disease patients and medical care expenditure at the time baby-boomers belong to 65 years old aging population, and compare with current 65 year-old aging population. Methods: Analysis method used an estimating formula devised by the researcher and estimated the number of chronic disease patients and medical care expenditure of each generation. Results: When comparing the estimated number of chronic diseases patients of each generation, 40.6% of the first generation, 76.4% of the second generation, 95.2% of third generation are expected to get chronic disease. When comparing each generation's total medical care expenditure, based on the estimated number of chronic diseases patients of each generation, the second generation( 1,206,251,224 thousand won) showed higher than other generation. This study compared the number of chronic disease patients and medical care expenditure between the second generation of the elderly and current elder generation. As a result, the second generation patients was higher than the fourth generation in high blood pressure, diabetes, psychological and behavioral disorder, and neurological diseases whereas the fourth generation is only high the number of patients in heart disease. As for total medical care expenditure, the second generation paid more in high blood pressure, psychological and behavioral disorder while the fourth generation in neurological disease and heart disease. Conclusion: It is desired that considering the number of chronic disease patients and medical care expenditure of baby boomers accounting for 14.6% of total population, in-depth follow-up study is carried out that inquires into what are issues with a current chronic disease management project, what business is needed in order to manage these issues, and how to fund to cover increasing medical care expenditure.
Hereditary spastic paraplegia is a not common inherited neurological disorder with heterogeneous clinical expressions. ALDH18A1 (located on 10q24.1) gene-related spastic paraplegias (SPG9A and SPG9B) are rare metabolic disorders caused by dominant and recessive mutations that have been found recently. Autosomal recessive hereditary spastic paraplegia is a common and clinical type of familial spastic paraplegia linked to the SPG11 locus (locates on 15q21.1). There are different symptoms of spastic paraplegia, such as muscle atrophy, moderate mental retardation, short stature, balance problem, and lower limb weakness. Our first proband involves a 45 years old man and our second proband involves a 20 years old woman both are affected by spastic paraplegia disease. Genomic DNA was extracted from the peripheral blood of the patients, their parents, and their siblings using a filter-based methodology and quantified and used for molecular analysis and sequencing. Sequencing libraries were generated using Agilent SureSelect Human All ExonV7 kit, and the qualified libraries are fed into NovaSeq 6000 Illumina sequencers. Sanger sequencing was performed by an ABI prism 3730 sequencer. Here, for the first time, we report two cases, the first one which contains likely pathogenic NM_002860: c.475C>T: p.R159X mutation of the ALDH18A1 and the second one has likely pathogenic NM_001160227.2: c.5454dupA: p.Glu1819Argfs Ter11 mutation of the SPG11 gene and also was identified by the whole-exome sequencing and confirmed by Sanger sequencing. Our aim with this study was to confirm that these two novel variants are direct causes of spastic paraplegia.
Objectives : The author tried to find out reasons why and how hysteria(and conversion disorder) patient numbers, which were so prevalent even a few decades ago, have decreased and the phenotype of symptoms have changed. Methods : The number of visiting patients diagnosed with conversion disorder and their phenotype of symptoms were investigated through chart reviews in a psychiatric department of a University hospital for the last 12 years. Additionally, the characteristics of conversion disorder patients visiting the emergency room for last 2 years were also reviewed. Those results were compared with previous research results even if it seemed to be an indirect comparisons. The research relied on Briquet P. and Charcot JM's established factors of the vicissitudes of hysteria(and conversion disorder) which has been the framework for more than one hundred and fifty years since hysteria has been investigated. Results : The author found decreased numbers and changes of the phenotype of the hysteria patients(and conversion disorder) over the last several decades. The decreased numbers and changes of the symptoms of those seemed to be partly due to several issues. These issues include the development of the diagnostic techniques to identify organic causes of hysteria, repeated changes to the symptom descriptions and diagnostic classification, changes of the brain nervous functions in response to negative emotions, and the influence of human evolution. Conclusions : The author proposed that the evolutionary brain discord reaction theory explains the causes of disappearance of and changes to symptoms of hysteria(conversion disorder). Most patients with hysteria(conversion disorder) have been diagnosed in the neurological department. For providing more appropriate treatment and minimizing physical disabilities to those patients, psychiatrists should have a major role in cooperating not only with primary care physicians but with neurologists. The term 'hysteria' which had been used long ago should be revived and used as a term to describe diseases such as somatic symptom disorder, functional neurological symptoms, somatization, and somatoform disorders, all of which represent almost the same vague concept as hysteria.
Journal of the Korean Academy of Child and Adolescent Psychiatry
/
v.12
no.1
/
pp.149-156
/
2001
After the fact that obsessive-compulsive symptoms or tic symptoms are common in Sydenham's chorea which is a sequale of rheumatic fever produced by group A beta-hemolytic streptococcus was reported, the association between group A beta-hemolytic streptococcus and a subgroup of obsessivecompulsive disorder(OCD) or tic disorder has been attentioned. This subgroup shared a unique clinical course, characterized by an abrupt onset of symptoms and/or dramatic exacerbations. And this subgroup was distinguished by pre-pubertal onset of symptoms, neurological abnormalities(choreiform movements and a unique pattern of motoric hyperactivity), as well as by relapsing and remitting symptom course. Acronym PANDAS(pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection) was used to denote a subgroup of OCD or tic disorder patients with these clinical characteristics. Then, there was a report suggesting that PANDAS category include some of attention-deficit/hyperactivity disorder(ADHD) and were two case reports of anorexia nervosa and body dysmorphic disorder with characteristics of PANDAS. This case is a patient who developed normally until age of 7, but after pharyngeal inflammation with high fever, he showed disturbance of cognition, social relationship, and language and communication, as well as tic symtoms and abnormal movement on face, hand, and foot. We report this case with review of literatures, because we think that this case belongs to the PANDAS category. Based on this observation, we suggest that PANDAS category include some of childhood disintegrative disorder as well as OCD, tic disorder, and ADHD.
Kim, Young-Hwa;So, Hyang-Sook;Lee, Eun-Joo;Ko, Eun
Korean Journal of Adult Nursing
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v.20
no.3
/
pp.489-499
/
2008
Purpose: The purpose of this study was to assess the importance and contribution of 9 nursing outcomes and their indicators that could be applied to cerebrovascular patients. Methods: Data were collected from 175 neurosurgical nurses working at two university affiliated hospitals and five secondary hospitals located in Gwang-ju. The Fehring method was used to estimate outcome content validity(OCV) and outcome sensitivity validity(OSV) of nursing outcomes and their indicators. Stepwise regression was used to evaluate relationship between outcome and its indicators. Results: The core outcomes identified by the OCV were Tissue Perfusion: Cerebral, Nutritional Status, Neurological Status, and Wound Healing: Primary Intention, whereas highly supportive outcomes identified by the OSV were Oral Health, Self-Care: ADL, and Nutritional Status. All the critical indicators selected for Fehring method were not included in stepwise regression model. By stepwise regression analysis, the indicators explained outcomes from 19% to 52% in importance and from 21% to 45% in contribution. Conclusion: This study identified core and supportive outcomes and their indicators which could be useful to assess the physical status of cerebrovascular patients. Further research is needed for the revision and development of nursing outcomes and their indicators at neurological nursing area.
Purpose: Epilepsy is the most common neurological disorder in childhood. Hospital nurses, who are the first to recognize seizures in epilepsy patients in the ward environment, possess expertise related to epilepsy and play a central role in epilepsy management. The purpose of this study was to develop an algorithm-based education program and to improve nurses' knowledge and self-efficacy related to providing nursing care to children with epilepsy. Methods: The education program consisted of lectures on the definition, cause, classification, diagnosis, treatment, and nursing of epilepsy based on a booklet, as well as practice using an algorithm for nursing interventions when a child experiences a seizure. Twenty-seven nurses working at pediatric neurological wards and a pediatric emergency room participated in the education program. The data were analyzed using descriptive statistics and the paired t-test. Results: Nurses' knowledge and self-efficacy showed a statistically significant improvement after participation in the education program on nursing care for children with epilepsy. Conclusion: The application of this education program for hospital setting is expected to improve nurses' capability to care for children with epilepsy, thereby contributing to a higher quality of nursing.
Journal of the Korean Academy of Child and Adolescent Psychiatry
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v.30
no.3
/
pp.127-131
/
2019
Many neurologic disorders manifest as psychiatric symptoms. Anti-N-Methyl-D-Aspartate (NMDA) receptor encephalitis is an autoimmune disease of the brain characterized by numerous neurological and psychiatric features. Despite being rare, its prevalence is rapidly increasing and early management is critical in ensuring successful and sustainable recovery. Therefore, the illness should be considered as a differential diagnosis when clinically assessing patients. This report presents a case of a female child who was hospitalized for acute psychiatric manifestations, which was later confirmed as anti-NMDA receptor encephalitis. She recovered relatively successfully after combined neurological and psychiatric treatment. This report provides information on the clinical course of early onset anti-NMDA receptor encephalitis, including treatment strategy and prognosis.
Journal of mucopolysaccharidosis and rare diseases
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v.5
no.1
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pp.1-7
/
2021
Gaucher disease (GD, OMIM #230800 OMIM#230800) is a rare, autosomal recessive inherited metabolic disorder caused by mutation in GBA1 encoding the lysosomal enzyme, glucocerebrosidase. The deficiency of glucocerebrosidase leads to an accumulation of its substrate, glucosylceramide in macrophages of various tissues. Common clinical manifestations include cytopenia, splenomegaly, hepatomegaly, and bone lesions. The phenotype of GD is classified into three clinical categories: Type 1 (non-neuronopathic) is characterized by involvements on the viscera, whereas types 2 and 3 (neuronopathic) are associated with not only visceral symptoms but also neurological impairment, either severe in type 2 or variable in type 3. A diagnosis of GD can be confirmed by demonstrating the deficiency of acid glucocerebrosidase activity in leukocytes. Mutations in the GBA1 should be identified as they may be of prognostic value in some cases. Biomarkers including Chitotriosidase, CCL18, and glucosylsphingosine (lyso-GL1) are useful in diagnosis and treatment monitoring. Currently available disease-specific treatment in Korea consists of intravenous enzyme replacement therapy and substrate reduction therapy. For enhancing long-term prognosis, the onset of Parkinson's disease and Lewy body dementia, or the occurrence of a blood disease or cancer (hepatocellular carcinoma) should be monitored in older patients. The development of new strategies that can modify the neurological phenotype are expected, especially in Asia including Korea, where the prevalence of neuronopathic GD is relatively higher than that in western countries.
Park, Jae-Hyung;Yun, Young-Ju;Park, Jae-Hyun;Paeck, Eun-Kyung
The Journal of Pediatrics of Korean Medicine
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v.24
no.2
/
pp.1-12
/
2010
Objectives Taking detailed patient history helps earlier diagnosis and treatment of developmental disability. In this study we analyzed the clinical questionnaire to find out the clinical characteristics of those with five-retardation, five-limpness, or five-stiffness. Methods The data was collected from 484 children under the age of six who have visited H oriental medicine clinic for developmental delay. The clinical questionnaire was filled out by their parents and the data was analyzed statistically. Results 436 children showed symptoms of five-retardation, 90 children suffered from five-stiffness, 54 children showed five-limpness and 7 children suffered from five-stiffness and five-limpness complex. Generally, boys had higher chance to show disease symptoms than the girls (2.32:1) and 40 children (8.26%) reported family history of developmental disability. Cerebral palsy ranks the most common familial disease, followed by developmental delay, mental retardation, autistic disorder and language disorder. Among the children we have studied, 285 children (63.19%) showed delayed unassisted walk while 192 children (42.57%) had language disorder. Also, 138 children (28.51%) had both walk and language disorders. The children in this study also showed delayed toilet training and half of them had little stranger anxiety when they were infants. It was also found that 120 children (24.79%) experienced epilepsy. This study reaffirmed that low birth weight, premature birth, and suffocation are major risks causing neurological damage. Conclusions They had history which including family history, problems at birth, epilepsy, face recognition, muscle tone disorder, delayed walking without assistance, language ability, and toilet training.
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