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Journal of Clinical Neurology

  to  Present  ISSN: 1738-6586

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A Novel GFAP Mutation in Late-Onset Alexander Disease Showing Diffusion Restriction.

Tai Seung NAM ; Jeeyoung OH ; Michael LEVY ; Kyung Wook KANG ; Seok Yong CHOI ; Myeong Kyu KIM

Journal of Clinical Neurology.2017;13(4):426-428. doi:10.3988/jcn.2017.13.4.426

No abstract available.
Alexander Disease* ; Diffusion*

Alexander Disease* ; Diffusion*

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Repetitive Questioning and Writing in a Patient with Transient Global Amnesia.

Dae Lim KOO ; Jin Ah KIM ; Hyunwoo NAM

Journal of Clinical Neurology.2017;13(4):424-425. doi:10.3988/jcn.2017.13.4.424

No abstract available.
Amnesia, Transient Global* ; Humans ; Writing*

Amnesia, Transient Global* ; Humans ; Writing*

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Hemiatrophy of the Tongue with Contralateral Hemiparesis in a Patient with Multifocal Acquired Demyelinating Sensory and Motor Neuropathy.

Susumu IKENOSHITA ; Satoshi YAMASHITA ; Tetsuro SAKAMOTO ; Yohei MISUMI ; Yukio ANDO

Journal of Clinical Neurology.2017;13(4):422-423. doi:10.3988/jcn.2017.13.4.422

No abstract available.
Humans ; Paresis* ; Tongue*

Humans ; Paresis* ; Tongue*

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A History of Falls is Associated with a Significant Increase in Acute Mortality in Women after Stroke.

Emma J FOSTER ; Raphae S BARLAS ; Adrian D WOOD ; Joao H BETTENCOURT-SILVA ; Allan B CLARK ; Anthony K METCALF ; Kristian M BOWLES ; John F POTTER ; Phyo K MYINT

Journal of Clinical Neurology.2017;13(4):411-421. doi:10.3988/jcn.2017.13.4.411

BACKGROUND AND PURPOSE: The risks of falls and fractures increase after stroke. Little is known about the prognostic significance of previous falls and fractures after stroke. This study examined whether having a history of either event is associated with poststroke mortality. METHODS: We analyzed stroke register data collected prospectively between 2003 and 2015. Eight sex-specific models were analyzed, to which the following variables were incrementally added to examine their potential confounding effects: age, type of stroke, Oxfordshire Community Stroke Project classification, previous comorbidities, frailty as indicated by the prestroke modified Rankin Scale score, and acute illness parameters. Logistic regression was applied to investigate in-hospital and 30-day mortality, and Cox proportional-hazards models were applied to investigate longer-term outcomes of mortality. RESULTS: In total, 10,477 patients with stroke (86.1% ischemic) were included in the analysis. They were aged 77.7±11.9 years (mean±SD), and 52.2% were women. A history of falls was present in 8.6% of the men (n=430) and 20.2% of the women (n=1,105), while 3.8% (n=189) of the men and 12.9% of the women (n=706) had a history of both falls and fractures. Of the outcomes examined, a history of falls alone was associated with increased in-hospital mortality [odds ratio (OR)=1.33, 95% confidence interval (CI)=1.03–1.71] and 30-day mortality (OR=1.34, 95% CI=1.03–1.73) in women in the fully adjusted models. The Cox proportional-hazards models for longer-term outcomes and the history of falls and fractures combined showed no significant results. CONCLUSIONS: The history of falls is an important factor for acute stroke mortality in women. A previous history of falls may therefore be an important factor to consider in the short-term stroke prognosis, particularly in women.
Accidental Falls* ; Classification ; Comorbidity ; Female ; Hospital Mortality ; Humans ; Logistic Models ; Male ; Mortality* ; Prognosis ; Prospective Studies ; Stroke*

Accidental Falls* ; Classification ; Comorbidity ; Female ; Hospital Mortality ; Humans ; Logistic Models ; Male ; Mortality* ; Prognosis ; Prospective Studies ; Stroke*

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Early-Onset LMNA-Associated Muscular Dystrophy with Later Involvement of Contracture.

Younggun LEE ; Jung Hwan LEE ; Hyung Jun PARK ; Young Chul CHOI

Journal of Clinical Neurology.2017;13(4):405-410. doi:10.3988/jcn.2017.13.4.405

BACKGROUND AND PURPOSE: The early diagnosis of LMNA-associated muscular dystrophy is important for preventing sudden arrest related to cardiac conduction block. However, diagnosing early-onset Emery-Dreifuss muscular dystrophy (EDMD) with later involvement of contracture and limb-girdle muscular dystrophy type 1B is often delayed due to heterogeneous clinical presentations. We aimed to determine the clinical features that contribute to a delayed diagnosis. METHODS: We reviewed four patients who were recently diagnosed with LMNA-associated muscular dystrophy by targeted exome sequencing and who were initially diagnosed with nonspecific or other types of muscular dystrophy. RESULTS: Certain clinical features such as delayed contracture involvement and calf hypertrophy were found to contribute to a delayed diagnosis. Muscle biopsies were not informative for the diagnosis in these patients. CONCLUSIONS: Genetic testing of single or multiple genes is useful for confirming a diagnosis of LMNA-associated muscular dystrophy. Even EDMD patients could experience the later involvement of contracture, so clinicians should consider early genetic testing for patients with undiagnosed muscular dystrophy or laminopathy.
Biopsy ; Contracture* ; Delayed Diagnosis ; Diagnosis ; Early Diagnosis ; Exome ; Genetic Testing ; Humans ; Hypertrophy ; Muscular Dystrophies* ; Muscular Dystrophies, Limb-Girdle ; Muscular Dystrophy, Emery-Dreifuss

Biopsy ; Contracture* ; Delayed Diagnosis ; Diagnosis ; Early Diagnosis ; Exome ; Genetic Testing ; Humans ; Hypertrophy ; Muscular Dystrophies* ; Muscular Dystrophies, Limb-Girdle ; Muscular Dystrophy, Emery-Dreifuss

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Effectiveness of Cognitive Behavioral Therapy for Caregivers of People with Dementia: A Systematic Review and Meta-Analysis.

Oh Young KWON ; Hyeong Sik AHN ; Hyun Jung KIM ; Kun Woo PARK

Journal of Clinical Neurology.2017;13(4):394-404. doi:10.3988/jcn.2017.13.4.394

BACKGROUND AND PURPOSE: Caregivers endure tremendous physical, emotional, and financial burdens while caring for people with dementia. The current study aimed to estimate the effectiveness of cognitive behavioral therapy (CBT) for caregivers of people with dementia (CGPWD). METHODS: Studies in the MEDLINE, EMBASE, Cochrane Library, Web of Science, and SCOPUS databases were screened. Studies with a randomized controlled design and which produced CBT outcomes for CGPWD were included in this study, and we investigated these outcomes. RESULTS: The screening of abstracts of 263 studies resulted in 12 randomized controlled trials being included in this study. The mean age of the CGPWD ranged from 51.5 to 66.2 years. The caregiver role was most frequently adopted by a female spouse or daughter. CBT for the CGPWD resulted in positive effects on various conditions, including depression, anxiety, stress, and dysfunctional thoughts. Depression was the most commonly evaluated condition, and the Center for Epidemiologic Studies Depression Scale (CES-D) was most frequently used as an inventory for depressive symptoms. The mean differences between the baseline and postintervention CES-D scores were compared between the CBT-intervention and control groups. The CES-D score decreased significantly more in the CBT-intervention group than in the control group. The difference in pooled mean differences between the two groups was −4.98. CONCLUSIONS: CBT is an efficient intervention tool for reducing the various emotional burdens experienced by CGPWD. This meta-analysis found that CBT significantly improved the depressive symptoms of CGPWD.
Anxiety ; Caregivers* ; Cognitive Therapy* ; Dementia* ; Depression ; Epidemiologic Studies ; Female ; Humans ; Mass Screening ; Nuclear Family ; Spouses

Anxiety ; Caregivers* ; Cognitive Therapy* ; Dementia* ; Depression ; Epidemiologic Studies ; Female ; Humans ; Mass Screening ; Nuclear Family ; Spouses

7

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Functional Connectivity of the Hippocampus in Early- and vs. Late-Onset Alzheimer's Disease.

Kee Hyung PARK ; Young NOH ; Eun Jung CHOI ; Hyungsik KIM ; Sohyun CHUN ; Young Don SON

Journal of Clinical Neurology.2017;13(4):387-393. doi:10.3988/jcn.2017.13.4.387

BACKGROUND AND PURPOSE: Early-onset Alzheimer's disease (EOAD) and late-onset Alzheimer's disease (LOAD) have different clinical and neuroimaging characteristics, but memory decline is usually present in both types. However, there have been few functional studies focused on the hippocampus in Alzheimer's disease. We therefore investigated the functional connectivity between the hippocampus and other brain regions using resting-state fMRI and compared the findings between EOAD and LOAD. METHODS: We recruited 13 patients with EOAD and 19 patients with LOAD at the early disease stage. Twenty-one young controls and ten old controls were also recruited. Each participant completed a standardized neuropsychological battery of tests and underwent T1-weighted structural MRI. fMRI data were acquired during the resting state using 3-T MRI. The functional connectivity to the hippocampus was calculated based on automated anatomical labeling templates. RESULTS: The functional connectivity from the hippocampus to other brain regions differed between patients with EOAD and LOAD. The LOAD patients showed decreased hippocampal connectivity to cortical regions, such as to the middle temporal cortex, orbitofrontal cortex, postcentral cortex, supramarginal cortex, and rolandic operculum. In contrast, EOAD patients showed smaller functional changes of the cortical regions connected to the hippocampus, such as the middle frontal cortex. CONCLUSIONS: EOAD and LOAD patients exhibited different hippocampal connectivity. The memory decline in EOAD may be due to brain areas other than the hippocampus.
Alzheimer Disease* ; Brain ; Frontal Lobe ; Hippocampus* ; Humans ; Magnetic Resonance Imaging ; Memory ; Neuroimaging ; Prefrontal Cortex ; Temporal Lobe

Alzheimer Disease* ; Brain ; Frontal Lobe ; Hippocampus* ; Humans ; Magnetic Resonance Imaging ; Memory ; Neuroimaging ; Prefrontal Cortex ; Temporal Lobe

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Femur Fractures in Parkinsonism: Analysis of a National Sample Cohort in South Korea.

Soo Jeong AN ; Seung Hwan LEE ; Seo Young LEE ; Jae Woo KWON ; Seung Joon LEE ; Young Ju KIM

Journal of Clinical Neurology.2017;13(4):380-386. doi:10.3988/jcn.2017.13.4.380

BACKGROUND AND PURPOSE: Falling with a femur fracture is a serious event that negatively affects the quality of life of elderly individuals as well as patients with parkinsonism. This study investigated the association between parkinsonism and femur fracture and compared the risk of femur fracture between subjects with and without parkinsonism. METHODS: This study examined a population-based matched cohort constructed using the National Sample Cohort data set, which comprises approximately one million subscribers to medical insurance and aid in South Korea. Subjects with parkinsonism during 2003–2013 were identified as the exposed group, and up to five individuals matched for age, sex, and index years were identified as the controls for each parkinsonism subject. The risk of femur fracture for parkinsonism was evaluated using Cox regression. RESULTS: The incidence of femur fracture according to age, sex, and body mass index varied significantly between subjects with parkinsonism and controls (p<0.001). The presence of parkinsonism was associated with a higher risk of femur fractures for males [hazard ratio (HR)=2.85, 95% confidence interval [CI]=1.87–4.34], subjects younger than 65 years (HR=2.89, 95% CI=1.64–5.11), and underweight subjects (HR=3.90, 95% CI=1.82–8.35). The adjusted HR for femur fracture with parkinsonism was highest within 2 years of the disease diagnosis (HR=3.10, 95% CI=2.12–4.53). CONCLUSIONS: Our study found that the presence of parkinsonism is more strongly related to femur fracture in males, and increases the influence of traditional risk factors on femur fracture. It is necessary to consider how factors associated with the amount of ambulatory activity–even in an early diagnosed state–can play an important role in femur fracture in subjects with parkinsonism.
Accidental Falls ; Aged ; Body Mass Index ; Cohort Studies* ; Dataset ; Diagnosis ; Femur* ; Humans ; Incidence ; Insurance ; Korea* ; Male ; Parkinsonian Disorders* ; Quality of Life ; Risk Factors ; Thinness

Accidental Falls ; Aged ; Body Mass Index ; Cohort Studies* ; Dataset ; Diagnosis ; Femur* ; Humans ; Incidence ; Insurance ; Korea* ; Male ; Parkinsonian Disorders* ; Quality of Life ; Risk Factors ; Thinness

9

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Effects of Antiepileptic Drugs on the Carotid Artery Intima-Media Thickness in Epileptic Patients.

Qilun LAI ; Chunhong SHEN ; Yang ZHENG ; Yinxi ZHANG ; Yi GUO ; Meiping DING

Journal of Clinical Neurology.2017;13(4):371-379. doi:10.3988/jcn.2017.13.4.371

BACKGROUND AND PURPOSE: It has been reported that taking antiepileptic drugs (AEDs) may increase the risk of atherosclerosis. We performed a meta-analysis to evaluate the carotid artery intima-media thickness (CA-IMT) as a surrogate factor for atherosclerosis in epileptic patients. METHODS: We searched NCBI (PubMed), ISI Web of Knowledge, EMBASE, and the Cochrane Library databases for studies of the association between AEDs and CA-IMT in epileptic patients. A random-effects meta-analysis was used to pool results across studies. RESULTS: Fifteen studies involving 1,775 epileptic patients were included in the analysis. The overall CA-IMT was significantly larger among users of AEDs [mean difference (MD)=0.09 mm, 95% confidence interval (CI)=0.06–0.12 mm). When stratified by age, the MD was similar in adult patients (MD=0.09 mm, 95% CI=0.06–0.13 mm), but no significant difference was observed in children (MD=0.03 mm, 95% CI=0.00–0.07 mm). Regarding specific AEDs, monotherapy with carbamazepine (CBZ) or valproic acid (VPA) was associated with a larger CA-IMT, while phenytoin monotherapy was not and the result for lamotrigine was inconclusive. CONCLUSIONS: This study suggests that using AEDs is associated with the CA-IMT in patients with epilepsy, particularly for adult patients. In particular, CBZ and VPA may be related to a significant increase in CA-IMT.
Adult ; Anticonvulsants* ; Atherosclerosis ; Carbamazepine ; Carotid Arteries* ; Child ; Epilepsy ; Humans ; Phenytoin ; Valproic Acid

Adult ; Anticonvulsants* ; Atherosclerosis ; Carbamazepine ; Carotid Arteries* ; Child ; Epilepsy ; Humans ; Phenytoin ; Valproic Acid

10

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Comparison of Somatic and Sudomotor Nerve Fibers in Type 2 Diabetes Mellitus.

Eun Hee SOHN ; Kyu Sang SONG ; Ju Yeon LEE ; Ae Young LEE

Journal of Clinical Neurology.2017;13(4):366-370. doi:10.3988/jcn.2017.13.4.366

BACKGROUND AND PURPOSE: The objective of this study was to find a sensitive method for the early detection of diabetic polyneuropathy (DPN) and determine the relationship between the functions of somatic and autonomic small nerve fibers in DPN. METHODS: Patients with type 2 diabetes mellitus and DPN based on clinical symptoms, signs, intraepidermal nerve fiber density (IENFD), and findings in the quantitative sudomotor axon reflex test (QSART) were enrolled retrospectively. Neurological examinations and nerve conduction studies were performed on all patients. Heart-rate variability during deep breathing (DB ratio) and the Valsalva maneuver (Valsalva ratio) were used to quantify the cardiovagal function. Patients were divided into two groups: 1) normal nerve conduction, defined as small-fiber neuropathy (SFN) and 2) abnormal nerve conduction, defined as mixed-fiber neuropathy. RESULTS: In total, 82 patients were enrolled (age: 60.7±10.7 years, mean±SD). A decreased IENFD was the most frequent abnormality across all of the patients, followed by abnormalities of the QSART and cardiovagal function. A decreased IENFD was more sensitive than the QSART, DB ratio, and Valsalva ratio for detecting diabetic SFN. The DB ratio was significantly correlated with the duration of diabetes mellitus and clinical symptoms and signs. There was no correlation between the IENFD and the findings of the QSART for the distal leg. CONCLUSIONS: Measuring the IENFD was a more sensitive method than the QSART for the early detection of DPN. The degree of involvement of the somatic small nerve fibers and sudomotor nerve fibers was independent in DPN.
Axons ; Diabetes Mellitus ; Diabetes Mellitus, Type 2* ; Diabetic Neuropathies ; Humans ; Leg ; Methods ; Nerve Fibers* ; Neural Conduction ; Neurologic Examination ; Reflex ; Respiration ; Retrospective Studies ; Valsalva Maneuver

Axons ; Diabetes Mellitus ; Diabetes Mellitus, Type 2* ; Diabetic Neuropathies ; Humans ; Leg ; Methods ; Nerve Fibers* ; Neural Conduction ; Neurologic Examination ; Reflex ; Respiration ; Retrospective Studies ; Valsalva Maneuver

Country

Republic of Korea

Publisher

Korean Neurological Association

ElectronicLinks

http://synapse.koreamed.org/LinkX.php?code=0145JCN

Editor-in-chief

Sang-Ahm Lee

E-mail

jcn@neuro.or.kr

Abbreviation

J Clin Neurol

Vernacular Journal Title

ISSN

1738-6586

EISSN

2005-5013

Year Approved

2008

Current Indexing Status

Currently Indexed

Start Year

Description

The JCN aims to publish the cutting-edge research from around the world. The JCN covers clinical and translational research for physicians and researchers in the field of neurology. Encompassing the entire neurological diseases, our main focus is on the common disorders including stroke, epilepsy, Parkinson's disease, dementia, multiple sclerosis, headache, and peripheral neuropathy. Any authors affiliated with an accredited biomedical institution may submit manuscripts of original articles, review articles, case reports, and letters to the Editor. The JCN will allow clinical neurologists to enrich their knowledge of patient management, education, and clinical or experimental research, and hence their professionalism.

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