• Title/Summary/Keyword: neurological disease

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Cognitive Outcome of Pediatric Moyamoya Disease

  • Shim, Kyu-Won;Park, Eun-Kyung;Kim, Ju-Seong;Kim, Dong-Seok
    • Journal of Korean Neurosurgical Society
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    • v.57 no.6
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    • pp.440-444
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    • 2015
  • Quality of life is the current trend and issue for the most of human diseases. In moyamoya disease (MMD), surgical revascularization has been recognized as the possible assistance to reduce the neurological insult. However, the progressive nature of the disease has been invincible so far. To improve the quality of life of MMD patients not only the protection from the neurological insult but also the maintenance or improvement of cognitive function is inevitable. For pediatric MMD patients, younger age or longer duration of disease is the key factor among the prognostic factors for bad neurological outcomes. Hence, 'the earlier, the better' is the most precious rule for treatment. Protection from neurological insult is very critical and foremost important to improve cognitive outcome. Clinicians need to know the neuropsychological profile of MMD patients for the care of whole person and make an effort to protect the patients from neurological insults to maintain or improve it.

Impact of Serological and Histological Factors on Neurological Manifestations in Children and Adults with Celiac Disease

  • Niknam, Ramin;Seraj, Seyed Reza;Fattahi, Mohammad Reza;Nejati, Mohammadali;Dehghani, Seyed-Mohsen;Mahmoudi, Laleh
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.24 no.2
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    • pp.197-206
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    • 2021
  • Purpose: Celiac disease (CD) is a common autoimmune disease with extra-intestinal manifestations, including neurological disorders. There are few reports to assess various factors in increasing the chances of developing neurological disorders in CD, so we designed this study. Methods: All patients with CD at any age who had been referred to the Celiac Clinic were evaluated for neurological problems. CD was defined as IgA anti-transglutaminase antibodies (anti-tTG) of 18 IU/mL or higher in serology and Marsh type I or more severe in histopathological evaluation. Logistic regression analysis was used to evaluate the impact of various independent variables on the neurological manifestations. Results: A total of 540 patients enrolled in this study. A 360 (66.7%) of patients were children. A 64.8% and 35.2% were female and male, respectively. Overall, 34.1% of patients had neurological manifestation, including headache, neuropathy, epilepsy, and ataxia. The odds of developing neurological manifestations in children were significantly lower than in adults (odds ratio [OR], 0.66; 95% confidence interval [CI], 0.45-0.96; p=0.03) and in patients with gastrointestinal (GI) symptoms significantly higher than in the group without GI manifestations (OR, 1.77; 95% CI, 1.18-2.63; p=0.005). Other variables, including Marsh classification (OR, 0.44; 95% CI, 0.18-1.11; p=0.08) and anti-tTG levels (OR, 1.00; 95% CI, 0.999-1.001; p=0.59) did not significantly increase the chances of developing neurological disorders. Conclusion: Our study showed that increasing age and the presence of GI symptoms, but not serological and histological findings, could increase the chances of developing neurological diseases in CD patients.

Proline Metabolism in Neurological and Psychiatric Disorders

  • Yao, Yuxiao;Han, Weiping
    • Molecules and Cells
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    • v.45 no.11
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    • pp.781-788
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    • 2022
  • Proline plays a multifaceted role in protein synthesis, redox balance, cell fate regulation, brain development, and other cellular and physiological processes. Here, we focus our review on proline metabolism in neurons, highlighting the role of dysregulated proline metabolism in neuronal dysfunction and consequently neurological and psychiatric disorders. We will discuss the association between genetic and protein function of enzymes in the proline pathway and the development of neurological and psychiatric disorders. We will conclude with a potential mechanism of proline metabolism in neuronal function and mental health.

Three Case Reports of Postural Instability and Gait Difficulty in Parkinson's Disease Patients Treated with Korean and Western Medicine (파킨슨병 환자의 자세 불안정과 보행장애에 대한 한양방 복합치료 3예)

  • Lee, Young-Eun;Lee, Dong-Hyuk;Lee, Ji-Hyun;Lu, Hsu-Yuan;Cho, Seung-Yeon;Park, Jung-Mi;Ko, Chang-Nam;Bae, Hyung-Sup;Park, Seong-Uk
    • Korean Journal of Acupuncture
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    • v.31 no.1
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    • pp.40-47
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    • 2014
  • Objectives : Postural instability and gait difficulty(PIGD) can develop in all Parkinson's disease patients, especially late in the disease course, but does not respond well to conventional dopamine treatment. This study aimed to report three cases of PIGD in Parkinson's disease patients treated with Korean and Western medicine treatment. Methods : We used acupuncture, bee venom acupuncture, herbal medicine and moxibustion to treat patients during hospitalization. They continued Western medication and received rehabilitation treatment. We observed the changes of PIGD using the Unified Parkinson's Disease Rating Scale(UPDRS). Results : After treatment, PIGD symptoms improved and UPDRS scores were decreased. Especially, scores of falling, walking, gait and postural stability subsections related with PIGD were decreased. In addition, general conditions of patients were improved. Conclusions : This study suggests that Korean medicine treatment could be effective in the treatment of PIGD in Parkinson's disease patients.

Ruptured Persistent Trigeminal Artery Aneurysm Associated with Moyamoya Disease - Case Report - (모야모야병을 가진 파열된 지속성 삼차신경동맥 동맥류 - 증례보고 -)

  • Yun, Byung Min;Ahn, Jae Sung;Kim, Joon Soo;Kwon, Yang;Kwun, Byung Duk
    • Journal of Korean Neurosurgical Society
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    • v.30 no.6
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    • pp.769-773
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    • 2001
  • Moyamoya disease is defined as the development of collateral pathways, associated with bilateral chronic progressive stenosis of the carotid fork. Persistent trigeminal artery is the vessel most frequently observed to persist into adult life among persistent carotid-basilar and carotid-vertebral anastomotic vessels. The authors present a man who had a sudden, severe headache and brain CT showed subarachnoid hemorrhage in left interpeduncular and prepontine cistern. Four-vessel angiogram revealed moyamoya disease associated with aneurysm arising from the junction of persistent trigeminal artery aneurysm and basilar artery. As a treatment, coil embolization was tried but it was failed because of anatomical difficulty of aneurysm. The aneurysm was successfully treated with clipping surgery 10 days later. To our knowledge, this is the first case being reported.

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Inherited Metabolic Disorders Involving the Nervous System (신경계 이상을 동반하는 선천성 대사 질환)

  • Jeesuk Yu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.23 no.2
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    • pp.21-30
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    • 2023
  • Inherited metabolic disorders (IMD) are a group of disorders caused by defects in specific biochemical pathways. Up to 85% of IMD display predominantly neurological manifestations by affecting neurodevelopment or causing neurodegeneration. These neurometabolic disorders present with a variety of neurological and non-neurological manifestations. Early diagnosis of IMD is important because some disorders can be treated or improved with specific treatment if detected early. For prompt diagnosis and treatment, it is important to suspect IMD by being familiar with the clinical characteristics, biochemical abnormalities, and characteristic neuroimaging patterns that appear in IMD. Genetic testing, including next-generation sequencing, is also important in diagnosing IMD. During the follow-up of patients with IMD, it is necessary to conduct regular physical and neurological examinations in addition to disease-specific management.

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Sleep Apnea and Sleep Disturbances in Neurological Disorders (신경과 질환에 동반되는 수면무호흡증과 수면의 문제)

  • Hong, Seung-Bong
    • Sleep Medicine and Psychophysiology
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    • v.7 no.2
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    • pp.79-83
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    • 2000
  • Sleep disturbances are frequently associated with neurological disorders. Sleep disorders interfere with rehabilitation of patients with neurological disorders such as stroke and may increase the severity of their symptoms and recurrence rate of stroke. The treatment of sleep apnea syndrome is particularly important in managing patients with cerebral infarction of whom 50-80% have moderate to severe sleep apnea. Sleep apnea produces not only poor quality sleep but also excessive daytime sleepiness, fatigue and lack of energy. Sleep problems frequently found in patients with dementia are sleep-wake cycle abnormality, fragmentation of sleep, nocturnal insomnia, decreased slow wave sleep and REM sleep, and sleep disordered breathing. The management of sleep disturbances is very important for controlling symptoms such as nocturnal wandering and sundowning syndrome in patients with dementia. Parkinson's disease and epilepsy are other neurological disorders that may have sleep disturbances.

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Therapeutic Potential of Active Components from Acorus gramineus and Acorus tatarinowii in Neurological Disorders and Their Application in Korean Medicine

  • Cheol Ju Kim;Tae Young Kwak;Min Hyeok Bae;Hwa Kyoung Shin;Byung Tae Choi
    • Journal of Pharmacopuncture
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    • v.25 no.4
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    • pp.326-343
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    • 2022
  • Neurological disorders represent a substantial healthcare burden worldwide due to population aging. Acorus gramineus Solander (AG) and Acorus tatarinowii Schott (AT), whose major component is asarone, have been shown to be effective in neurological disorders. This review summarized current information from preclinical and clinical studies regarding the effects of extracts and active components of AG and AT (e.g., α-asarone and β-asarone) on neurological disorders and biomedical targets, as well as the mechanisms involved. Databases, including PubMed, Embase, and RISS, were searched using the following keywords: asarone, AG, AT, and neurological disorders, including Alzheimer's disease, Parkinson's disease, depression and anxiety, epilepsy, and stroke. Meta-analyses and reviews were excluded. A total of 873 studies were collected. A total of 89 studies were selected after eliminating studies that did not meet the inclusion criteria. Research on neurological disorders widely reported that extracts or active components of AG and AT showed therapeutic efficacy in treating neurological disorders. These components also possessed a wide array of neuroprotective effects, including reduction of pathogenic protein aggregates, antiapoptotic activity, modulation of autophagy, anti-inflammatory and antioxidant activities, regulation of neurotransmitters, activation of neurogenesis, and stimulation of neurotrophic factors. Most of the included studies were preclinical studies that used in vitro and in vivo models, and only a few clinical studies have been performed. Therefore, this review summarizes the current knowledge on AG and AT therapeutic effects as a basis for further clinical studies, and clinical trials are required before these findings can be applied to human neurological disorders.

Clinical Study for Characteristics of Heart Rate Variability in Stroke Patients (중풍 환자의 심박변이도 특성에 대한 임상적 연구)

  • Lee, ln-whan;Shin, Ae-sook;Kim, Na-hee;Kim, Hye-mi;Shim, So-ra;Kim, Min-kyung;Cho, Seung-yeon;Na, Byung-jo;Jung, Woo-sang;Moon, Sang-kwan;Park, Jung-mi;Ko, Chang-nam;Cho, Ki-ho;Kim, Young-suk;Bae, Hyung-sup;Park, Seong-uk
    • The Journal of the Society of Stroke on Korean Medicine
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    • v.11 no.1
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    • pp.1-8
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    • 2010
  • Objectives : We designed this study to investigate difference of heart rate variability(HRV) according to sex, age, acute or chronic phase, category of stroke, national institute of health stroke scale(NIHSS), lesion of stroke. Methods : 64 subjects were recruited from the patients admitted to the department of oriental medicine at East-West Neo Medical Center, Kyung Hee University from 1 September 2009 to 31 August 2010. We compared heart rate(HR), standard deviation of all normal P-P intervals(SDNN), low frequency(LF), high frequency(HF), LF/HF ratio. Results and Conclusions : 1. LF/HF ratio is significantly different between over-70 and below-70 of age. 2. SDNN is significantly different between acute and chronic stroke patients. 3. In sex, category of stroke, national institute of health stroke scale(NIHSS), lesion of stroke, there are no significantly different among the any values of heart rate variability(HRV).

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Neurological Outcome of Patients with Late-onset Ornithine Transcarbamylase Deficiency (지발형 오르니틴 트랜스카바미라제 결핍증 환자들의 신경학적 예후)

  • Jang, Kyung Mi;Hwang, Su-Kyeong
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.22 no.1
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    • pp.15-20
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    • 2022
  • The most common urea cycle disorder is ornithine transcarbamylase deficiency. More than 80 percent of patients with symptomatic ornithine transcarbamylase deficiency are late-onset, which can present various phenotypes from infancy to adulthood. With no regards to the severity of the disease, characteristic fluctuating courses due to hyperammonemia may develop unexpectedly, and can be precipitated by various metabolic stressors. Late-onset ornithine transcarbamylase deficiency is not merely related to a type of genetic variation, but also to the complex relationship between genetic and environmental factors that result in hyperammonemia; therefore, it is difficult to predict the prevalence of neurological symptoms in late-onset ornithine transcarbamylase deficiency. Most common acute neurological manifestations include psychological changes, seizures, cerebral edema, and death; subacute neurological manifestations include developmental delays, learning disabilities, intellectual disabilities, attention-deficit/hyperactivity disorder, executive function deficits, and emotional and behavioral problems. This review aims to increase awareness of late-onset ornithine transcarbamylase deficiency, allowing for an efficient use of biochemical and genetic tests available for diagnosis, ultimately leading to earlier treatment of patients.