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1.  Depression and Anxiety in People with Epilepsy 
Many recent epidemiological studies have found the prevalence of depression and anxiety to be higher in people with epilepsy (PWE) than in people without epilepsy. Furthermore, people with depression or anxiety have been more likely to suffer from epilepsy than those without depression or anxiety. Almost one-third of PWE suffer from depression and anxiety, which is similar to the prevalence of drug-refractory epilepsy. Various brain areas, including the frontal, temporal, and limbic regions, are associated with the biological pathogenesis of depression in PWE. It has been suggested that structural abnormalities, monoamine pathways, cerebral glucose metabolism, the hypothalamic-pituitary-adrenal axis, and interleukin-1b are associated with the pathogenesis of depression in PWE. The amygdala and the hippocampus are important anatomical structures related to anxiety, and γ-aminobutyric acid and serotonin are associated with its pathogenesis. Depression and anxiety may lead to suicidal ideation or attempts and feelings of stigmatization. These experiences are also likely to increase the adverse effects associated with antiepileptic drugs and have been related to poor responses to pharmacological and surgical treatments. Ultimately, the quality of life is likely to be worse in PWE with depression and anxiety than in PWE without these disorders, which makes the early detection and appropriate management of depression and anxiety in PWE indispensable. Simple screening instruments may be helpful for in this regard, particularly in busy epilepsy clinics. Although both medical and psychobehavioral therapies may ameliorate these conditions, randomized controlled trials are needed to confirm that.
doi:10.3988/jcn.2014.10.3.175
PMCID: PMC4101093  PMID: 25045369
epilepsy; depression; anxiety
2.  Dual Antiplatelet Therapy after Noncardioembolic Ischemic Stroke or Transient Ischemic Attack: Pros and Cons 
Dual antiplatelet therapy simultaneously blocks different platelet activation pathways and might thus be more potent at inhibiting platelet activation and more effective at reducing major ischemic vascular events compared to antiplatelet monotherapy. Aspirin plus clopidogrel dual therapy is now the standard therapy for patients with acute coronary syndrome and for those undergoing percutaneous coronary intervention. However, dual antiplatelet therapy carries an increased risk of bleeding. Patients with ischemic stroke or transient ischemic attack (TIA) are generally older and likely to have a fragile cerebrovascular bed, which further increases the risk of systemic major bleeding events and intracranial hemorrhage. Clinical trials and meta-analyses suggest that in comparison to antiplatelet monotherapy, dual antiplatelet therapy initiated early after noncardioembolic ischemic stroke or TIA further reduces the rate of recurrent stroke and major vascular events without significantly increasing the rate of major bleeding events. In contrast, studies of long-term therapy in patients with noncardioembolic ischemic stroke or TIA have yielded inconsistent data regarding the benefit of dual antiplatelet therapy over monotherapy. However, the harm associated with major bleeding events, including intracranial hemorrhage, which is generally more disabling and more fatal than ischemic stroke, is likely to increase with dual antiplatelet therapy. Physicians should carefully assess the benefits and risks of dual antiplatelet therapy versus antiplatelet monotherapy when managing patients with ischemic stroke or TIA.
doi:10.3988/jcn.2014.10.3.189
PMCID: PMC4101094  PMID: 25045370
ischemic stroke; TIA; dual antiplatelet therapy
3.  T2 Relaxometry Using 3.0-Tesla Magnetic Resonance Imaging of the Brain in Early- and Late-Onset Restless Legs Syndrome 
Background and Purpose
Previous T2 relaxometry studies have provided evidence for regional brain iron deficiency in patients with restless legs syndrome (RLS). Measurement of the iron content in several brain regions, and in particular the substantia nigra (SN), in early- and late-onset RLS patients using T2 relaxometry have yielded inconsistent results. In this study the regional iron content was assessed in patients with early- and late-onset RLS using magnetic resonance imaging (MRI), and compared the results with those in controls.
Methods
Thirty-seven patients with idiopathic RLS (20 with early onset and 17 with late onset) and 40 control subjects were studied using a 3.0-tesla MRI with a gradient-echo sampling of free induction decay and echo pulse sequence. The regions of interest in the brain were measured independently by two trained analysts using software known as medical image processing, analysis, and visualization. The results were compared and a correlation analysis was conducted to investigate which brain areas were related to RLS clinical variables.
Results
The iron index in the SN was significantly lower in patients with late-onset RLS than in controls (p=0.034), while in patients with early-onset RLS there was no significant difference. There was no significant correlation between the SN iron index of the late-onset RLS group and clinical variables such as disease severity.
Conclusions
Late-onset RLS is associated with decreased iron content in the SN. This finding supports the hypothesis that regional brain iron deficiency plays a role in the pathophysiology of late-onset RLS.
doi:10.3988/jcn.2014.10.3.197
PMCID: PMC4101095  PMID: 25045371
restless legs syndrome; T2 relaxometry; red nucleus; substantia nigra; iron
4.  Sleep Problems Associated with Behavioral and Psychological Symptoms as Well as Cognitive Functions in Alzheimer's Disease 
Background and Purpose
It has been shown that sleep problems in Alzheimer's disease (AD) are associated with cognitive impairment and behavioral problems. In fact, most of studies have founded that daytime sleepiness is significantly correlated with cognitive decline in AD. However, a few studies have also shown that nighttime sleep problems are associated with cognitive function and behavioral symptoms in AD. Accordingly, the aim of this study was to evaluate the effects of nighttime sleep on cognition and behavioral and psychological symptoms of dementia (BPSD) in AD.
Methods
The study population comprised 117 subjects: 63 AD patients and 54 age- and sex-matched non-demented elderly subjects. Detailed cognitive functions and behavioral symptoms were measured using the Seoul Neuropsychological Screening Battery (SNSB) and the Korean version of the Neuropsychiatric Inventory (NPI-K). Sleep characteristics were evaluated using the Korean version of the Pittsburgh Sleep Quality Index (PSQI-K). The correlations between PSQI-K and SNSB scores and between PSQI-K and NPI-K scores were analyzed.
Results
In AD patients, sleep latency was found to be negatively correlated with praxis (p=0.041), Rey-Osterrieth Complex Figure Test (RCFT) immediate recall (p=0.041), and RCFT recognition (p=0.008) after controlling for age and education, while sleep duration and sleep efficiency were positively correlated with praxis (p=0.034 and p=0.025, respectively). Although no significant correlation was found between PSQI-K and NPI-K scores, sleep disturbance and total PSQI-K scores were found to be significantly associated with apathy/indifference in AD.
Conclusions
Sleep problems such as prolonged sleep duration, sleep latency, and poor sleep efficiency in AD patients were correlated with cognitive dysfunction, and especially frontal executive and visuospatial functions, and BPSD. These findings suggest that treatment of nighttime sleep problems might improve cognition and behavioral symptoms in AD patients.
doi:10.3988/jcn.2014.10.3.203
PMCID: PMC4101096  PMID: 25045372
sleep; cognition; behavioral symptoms; Alzheimer's disease
5.  Validation of a Korean Version of the Insomnia Severity Index 
Background and Purpose
The purposes of this study were to standardize and validate a Korean version of the Insomnia Severity Index (ISI-K), and to evaluate its clinical usefulness.
Methods
We translated the ISI into Korean and then translated it back into English to check its accuracy. The 614 patients with sleep disorders who were enrolled in this study comprised 169 with primary insomnia, 133 with comorbid insomnia, and 312 with obstructive sleep apnea. All subjects underwent one night of polysomnography (PSG) and completed the Korean versions of both the Pittsburgh Sleep Quality Index (PSQI-K) and the Epworth Sleepiness Scale, as well as the ISI-K. The ISI-K was compared to these sleep scales and various PSG sleep parameters.
Results
The internal consistency the ISI-K total score was confirmed by a Cronbach's alpha of 0.92, and the item-to-total-score correlations (item-total correlations) ranged from 0.65 to 0.84, suggesting adequate reliability. The correlation between the ISI-K total score and PSQI-K was 0.84, which suggested adequate convergent validity. Low-to-moderate correlations were obtained between the ISI-K total score and PSG-defined sleep parameters: 0.22 for sleep onset latency, 0.38 for wake after sleep onset, and 0.46 for sleep efficiency. A cutoff score of 15.5 on the ISI-K was optimal for discriminating patients with insomnia. The test-retest scores over a 4-week interval with 34 subjects yielded a correlation coefficient of 0.86, suggesting excellent temporal stability.
Conclusions
The findings of this study show that the ISI-K is a reliable and valid instrument for assessing the severity of insomnia in a Korean population.
doi:10.3988/jcn.2014.10.3.210
PMCID: PMC4101097  PMID: 25045373
sleep; insomnia; reliability; validity
6.  Emergency Medical Care of Multiple Sclerosis Patients: Primary Data from the Mount Sinai Resource Utilization in Multiple Sclerosis Project 
Background and Purpose
There has been no systematic analysis of emergency department (ED) utilization in the multiple sclerosis (MS) population. We investigated the acute-care needs of MS patients using ED as a route for entry into healthcare services.
Methods
ED visits made by MS patients were identified. Data extracted included demographics, medical/neurological history, and workup/management in the ED.
Results
The Mount Sinai ED received 569 visits from 224 MS patients during a 3-year period, of whom 33.5% were covered by Medicaid and 12.9% were uninsured. Patients with an Expanded Disability Status Scale score of ≥6 accounted for 54%, 50.5% of relapsing remitting MS patients were being treated with disease-modifying therapies, and 74.5% of the ED visits were non-neurological. Patients with mild-to-moderate MS were more likely to present to the ED for issues directly related to MS such as acute exacerbations, while those with severe MS presented more often due to medical issues indirectly related to MS, such as urinary tract infections (p<0.0001).
Conclusions
Most MS patients seeking ED care suffer from acute non-neurological problems. The MS patients presenting to the ED tended to be underinsured, had high levels of disability, and were undertreated with disease-modifying therapies. The acute-care needs of MS patients evolve over the disease course, as do the resources that must be utilized in providing emergency care across the spectrum of MS severity. Understanding the characteristics, problems, and needs of MS patients utilizing the ED is an important step in improving care in this population from both clinical and public health perspectives.
doi:10.3988/jcn.2014.10.3.216
PMCID: PMC4101098  PMID: 25045374
multiple sclerosis; underinsured; emergencies; healthcare access; utilization
7.  Distribution of Cerebral Microbleeds Determines Their Association with Impaired Kidney Function 
Background and Purpose
Cerebral microbleeds (CMBs) are associated with various pathologies of the cerebral small vessels according to their distribution (i.e., cerebral amyloid angiopathy or hypertensive angiopathy). We investigated the association between CMB location and kidney function in acute ischemic stroke patients.
Methods
We enrolled 1669 consecutive patients with acute ischemic stroke who underwent gradient-recalled echo brain magnetic resonance imaging. Kidney function was determined using the estimated glomerular filtration rate (eGFR). CMBs were classified into strictly lobar, strictly nonlobar (i.e., only deep or infratentorial), and a combination of both lobar and nonlobar. Multinomial logistic regression analyses were used to determine the factors associated with the existence of CMBs according to their location.
Results
The patients were aged 66±12 years (mean±standard deviation), and 61.9% (1033/1669) of them were male. CMBs were found in 27.0% (452/1669) of the patients. The stroke subtypes of small-artery occlusion and cardioembolism occurred more frequently in those with strictly nonlobar CMBs (10.8%) and strictly lobar CMBs (48.8%), respectively. The mean eGFR was lower in the strictly nonlobar CMBs group (72±28 mL/min/1.73 m2) and the both lobar and nonlobar CMBs group (72±25 mL/min/1.73 m2) than in the no-CMBs group (86±29 mL/min/1.73 m2). Multivariate multinomial logistic regression revealed that eGFR <60 mL/min/1.73 m2 was independently related to strictly nonlobar CMBs (odds ratio=2.63, p=0.001).
Conclusions
Impaired kidney function is associated with strictly nonlobar CMBs. Our findings indicate that the distribution of CMBs should be considered when evaluating their relationships or prognoses.
doi:10.3988/jcn.2014.10.3.222
PMCID: PMC4101099  PMID: 25045375
cerebral microbleed; estimated glomerular filtration rate; chronic renal failure
8.  Abnormal Brain Activity Changes in Patients with Migraine: A Short-Term Longitudinal Study 
Background and Purpose
Whether or not migraine can cause cumulative brain alterations due to frequent migraine-related nociceptive input in patients is largely unclear. The aim of this study was to characterize longitudinal changes in brain activity between repeated observations within a short time interval in a group of female migraine patients, using resting-state functional magnetic resonance imaging.
Methods
Nineteen patients and 20 healthy controls (HC) participated in the study. Regional homogeneity (ReHo) and functional interregional connectivity were assessed to determine the focal and global features of brain dysfunction in migraine. The relationship between changes in headache parameters and longitudinal brain alterations were also investigated.
Results
All patients reported that their headache activity increased over time. Abnormal ReHo changes in the patient group relative to the HC were found in the putamen, orbitofrontal cortex, secondary somatosensory cortex, brainstem, and thalamus. Moreover, these brain regions exhibited longitudinal ReHo changes at the 6-week follow-up examination. These headache activity changes were accompanied by disproportionately dysfunctional connectivity in the putamen in the migraine patients, as revealed by functional connectivity analysis, suggesting that the putamen plays an important role in integrating diverse information among other migraine-related brain regions.
Conclusions
The results obtained in this study suggest that progressive brain aberrations in migraine progress as a result of increased headache attacks.
doi:10.3988/jcn.2014.10.3.229
PMCID: PMC4101100  PMID: 25045376
resting state; migraine; longitudinal study; brain functional abnormality
9.  Chronic Daily Headache in Korea: Prevalence, Clinical Characteristics, Medical Consultation and Management 
Background and Purpose
Chronic daily headache (CDH) is a commonly reported reason for visiting hospital neurology departments, but its prevalence, clinical characteristics, and management have not been well documented in Korea. The objective of this study was to characterize the 1-year prevalence, clinical characteristics, medical consultations, and treatment for CDH in Korea.
Methods
The Korean Headache Survey (KHS) is a nationwide descriptive survey of 1507 Korean adults aged between 19 and 69 years. The KHS investigated headache characteristics, sociodemographics, and headache-related disability using a structured interview. We used the KHS data for this study.
Results
The 1-year prevalence of CDH was 1.8% (95% confidence interval, 1.1-2.5%), and 25.7% of the subjects with CDH met the criteria for medication overuse. Two-thirds (66.7%) of CDH subjects were classified as having chronic migraine, and approximately half of the CDH subjects (48.1%) reported that their headaches either substantially or severely affected their quality of life. Less than half (40.7%) of the subjects with CDH reported having consulted a doctor for their headaches and 40.7% had not received treatment for their headaches during the previous year.
Conclusions
The prevalence of CDH was 1.8% and medication overuse was associated with one-quarter of CDH cases in Korea. Many subjects with CDH do not seek medical consultation and do not receive appropriate treatment for their headaches.
doi:10.3988/jcn.2014.10.3.236
PMCID: PMC4101101  PMID: 25045377
chronic daily headache; chronic migraine; epidemiology; headache; migraine
10.  Analysis of Dosage Mutation in PARK2 among Korean Patients with Early-Onset or Familial Parkinson's Disease 
Background and Purpose
There is some controversy regarding heterozygous mutations of the gene encoding parkin (PARK2) as risk factors for Parkinson's disease (PD), and all previous studies have been performed in non-Asian populations. Dosage mutation of PARK2, rather than a point mutation or small insertion/deletion mutation, was reported to be a risk factor for familial PD; dosage mutation of PARK2 is common in Asian populations.
Methods
We performed a gene-dosage analysis of PARK2 using real-time polymerase chain reaction for 189 patients with early-onset PD or familial PD, and 191 control individuals. In the case of PD patients with heterozygous gene-dosage mutation, we performed a sequencing analysis to exclude compound heterozygous mutations. The association between heterozygous mutation of PARK2 and PD was tested.
Results
We identified 22 PD patients with PARK2 mutations (11.6%). Five patients (2.6%) had compound heterozygous mutations, and 13 patients (6.9%) had a heterozygous mutation. The phase could not be determined in one patient. Three small sequence variations were found in 30 mutated alleles (10.0%). Gene-dosage mutation accounted for 90% of all of the mutations found. The frequency of a heterozygous PARK2 gene-dosage mutation was higher in PD patients than in the controls.
Conclusions
Heterozygous gene-dosage mutation of PARK2 is a genetic risk factor for patients with early-onset or familial PD in Koreans.
doi:10.3988/jcn.2014.10.3.244
PMCID: PMC4101102  PMID: 25045378
Parkinson's disease; PARK2; gene-dosage change; risk factor
11.  Diffusion Tensor Tractography Analysis of the Corpus Callosum Fibers in Amyotrophic Lateral Sclerosis 
Background and Purpose
Involvement of the corpus callosum (CC) is reported to be a consistent feature of amyotrophic lateral sclerosis (ALS). We examined the CC pathology using diffusion tensor tractography analysis to identify precisely which fiber bundles are involved in ALS.
Methods
Diffusion tensor imaging was performed in 14 sporadic ALS patients and 16 age-matched healthy controls. Whole brain tractography was performed using the multiple-region of interest (ROI) approach, and CC fiber bundles were extracted in two ways based on functional and structural relevance: (i) cortical ROI selection based on Brodmann areas (BAs), and (ii) the sulcal-gyral pattern of cortical gray matter using FreeSurfer software, respectively.
Results
The mean fractional anisotropy (FA) values of the CC fibers interconnecting the primary motor (BA4), supplementary motor (BA6), and dorsolateral prefrontal cortex (BA9/46) were significantly lower in ALS patients than in controls, whereas those of the primary sensory cortex (BA1, BA2, BA3), Broca's area (BA44/45), and the orbitofrontal cortex (BA11/47) did not differ significantly between the two groups. The FreeSurfer ROI approach revealed a very similar pattern of abnormalities. In addition, a significant correlation was found between the mean FA value of the CC fibers interconnecting the primary motor area and disease severity, as assessed using the revised Amyotrophic Lateral Sclerosis Functional Rating Scale, and the clinical extent of upper motor neuron signs.
Conclusions
Our findings suggest that there is some degree of selectivity or a gradient in the CC pathology in ALS. The CC fibers interconnecting the primary motor and dorsolateral prefrontal cortices may be preferentially involved in ALS.
doi:10.3988/jcn.2014.10.3.249
PMCID: PMC4101103  PMID: 25045379
amyotrophic lateral sclerosis; motor neuron disease; corpus callosum; diffusion tensor imaging; tractography; cortical parcellation
12.  Mutation analysis of SPAST, ATL1, and REEP1 in Korean Patients with Hereditary Spastic Paraplegia 
Background and Purpose
Hereditary spastic paraplegia (HSP) is a genetically heterogeneous group of neurodegenerative disorders that are characterized by progressive spasticity and weakness of the lower limbs. Mutations in the spastin gene (SPAST) are the most common causes of HSP, accounting for 40-67% of autosomal dominant HSP (AD-HSP) and 12-18% of sporadic cases. Mutations in the atlastin-1 gene (ATL1) and receptor expression-enhancing protein 1 gene (REEP1) are the second and third most common causes of AD-HSP, respectively.
Methods
Direct sequence analysis was used to screen mutations in SPAST, ATL1, and REEP1 in 27 unrelated Korean patients with pure and complicated HSP. Multiplex ligation-dependent probe amplification was also performed to detect copy-number variations of the three genes.
Results
Ten different SPAST mutations were identified in 11 probands, of which the following 6 were novel: c.760A>T, c.131C>A, c.1351_1353delAGA, c.376_377dupTA, c.1114A>G, and c.1372A>C. Most patients with SPAST mutations had AD-HSP (10/11, 91%), and the frequency of SPAST mutations accounted for 66.7% (10/15) of the AD-HSP patients. No significant correlation was found between the presence of the SPAST mutation and any of the various clinical parameters of pure HSP. No ATL1 and REEP1 mutations were detected.
Conclusions
We conclude that SPAST mutations are responsible for most Korean cases of genetically confirmed AD-HSP. Our observation of the absence of ATL1 and REEP1 mutations needs to be confirmed in larger series.
doi:10.3988/jcn.2014.10.3.257
PMCID: PMC4101104  PMID: 25045380
hereditary spastic paraplegia; SPAST; ATL1; REEP1; Korea
13.  Anti-Ma2 Paraneoplastic Encephalitis in Association with Recurrent Cervical Cancer 
Background
Paraneoplastic neurological syndromes are rare, and although they are frequently associated with gynecological malignancies, cervical cancer is a rare cause. The symptoms of anti-Ma2 encephalitis are diverse and often present prior to the diagnosis of malignancy.
Case Report
We report a case of a 37-year-old woman with a history of cervical cancer presenting with unexplained weight gain and vertical supranuclear gaze palsy. Magnetic resonance imaging of the brain revealed lesions within the bilateral hypothalami and midbrain. Anti-Ma2 antibodies were eventually found in the serum, prompting a search for malignancy. Recurrent metastatic cervical cancer was found in the retroperitoneal lymph nodes.
Conclusions
This is the first report of cervical cancer in association with anti-Ma2 encephalitis, and highlights the need for a high degree of suspicion in patients with a cancer history presenting with neurological symptoms. The symptoms associated with anti-Ma2 encephalitis are diverse and typically precede the diagnosis of cancer in patients, and should trigger a search for an underlying malignancy.
doi:10.3988/jcn.2014.10.3.262
PMCID: PMC4101105  PMID: 25045381
paraneoplastic syndromes; cervical cancer; anti-Ma2 encephalitis
14.  Role of High-Resolution Magnetic Resonance Imaging in the Diagnosis of Primary Angiitis of the Central Nervous System 
Background
Primary angiitis of the central nervous system (PACNS) is a rare disorder and is often difficult to diagnose due to the lack of a confirmatory test. PACNS can generally be diagnosed based on typical angiographic findings. We describe herein a patient diagnosed with PACNS despite the presence of normal findings on conventional angiography.
Case Report
A 44-year-old man with a recent history of ischemic stroke in the right posterior cerebral artery territory developed acute-onset vertigo. Diffusion-weighted imaging revealed an acute infarction within the left posterior inferior cerebellar artery. His medical history was unremarkable except for hyperlipidemia; the initial examination revealed mild gait imbalance. During the 10 days of hospital admission, the patient experienced four recurrent ischemic strokes within the posterior circulation territory (occipital lobe, pons, and cerebellum). He was diagnosed with recurrent cerebral infarctions due to PACNS. The basilar artery exhibited no demonstrable luminal stenosis, but there were direct imaging signs of central nervous system angiitis including wall thickening and contrast enhancement. High-dose intravenous steroid therapy followed by oral prednisolone was administered. There was no further stroke recurrence and follow-up imaging of the arterial walls showed normalization of their characteristics.
Conclusions
The present case emphasizes the importance of wall imaging in the diagnosis and treatment of PACNS.
doi:10.3988/jcn.2014.10.3.267
PMCID: PMC4101106  PMID: 25045382
ischemic stroke; magnetic resonance imaging; vasculitis; inflammation
15.  Opsoclonus-Myoclonus Syndrome Associated with Mumps Virus Infection 
Background
Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder that is characterized by involuntary eye movements and myoclonus. OMS exhibits various etiologies, including paraneoplastic, parainfectious, toxic-metabolic, and idiopathic causes. The exact immunopathogenesis and pathophysiology of OMS are uncertain.
Case Report
We report the case of a 19-year-old male who developed opsoclonus and myoclonus several days after a flu-like illness. Serological tests revealed acute mumps infection. The findings of cerebrospinal fluid examinations and brain magnetic resonance imaging were normal. During the early phase of the illness, he suffered from opsoclonus and myoclonus that was so severe as to cause acute renal failure due to rhabdomyolysis. After therapies including intravenous immunoglobulin, the patient gradually improved and had fully recovered 2 months later.
Conclusions
This is the first report of OMS associated with mumps infection in Korea. Mumps infection should be considered in patients with OMS.
doi:10.3988/jcn.2014.10.3.272
PMCID: PMC4101107  PMID: 25045383
opsoclonus; myoclonus; mumps virus
19.  Long-Term Outcomes of Hemispheric Disconnection in Pediatric Patients with Intractable Epilepsy 
Background and Purpose
Hemispherectomy reportedly produces remarkable results in terms of seizure outcome and quality of life for medically intractable hemispheric epilepsy in children. We reviewed the neuroradiologic findings, pathologic findings, epilepsy characteristics, and clinical long-term outcomes in pediatric patients following a hemispheric disconnection.
Methods
We retrospectively studied 12 children (8 males) who underwent a hemispherectomy at Asan Medical Center between 1997 and 2005. Clinical, EEG, neuroradiological, and surgical data were collected. Long-term outcomes for seizure, motor functions, and cognitive functions were evaluated at a mean follow-up of 12.7 years (range, 7.6-16.2 years) after surgery.
Results
The mean age at epilepsy onset was 3.0 years (range, 0-7.6 years). The following epilepsy syndromes were identified in our cohort: focal symptomatic epilepsy (n=8), West syndrome (n=3), and Rasmussen's syndrome (n=1). Postoperative histopathology of our study patients revealed malformation of cortical development (n=7), encephalomalacia as a sequela of infarction or trauma (n=3), Sturge-Weber syndrome (n=1), and Rasmussen's encephalitis (n=1). The mean age at surgery was 6.5 years (range, 0.8-12.3 years). Anatomical or functional hemispherectomy was performed in 8 patients, and hemispherotomy was performed in 4 patients. Eight of our 12 children (66.7%) were seizure-free, but 3 patients with perioperative complications showed persistent seizure. Although all patients had preoperative hemiparesis and developmental delay, none had additional motor or cognitive deficits after surgery, and most achieved independent walking and improvement in daily activities.
Conclusions
The long-term clinical outcomes of hemispherectomy in children with intractable hemispheric epilepsy are good when careful patient selection and skilled surgical approaches are applied.
doi:10.3988/jcn.2014.10.2.101
PMCID: PMC4017012  PMID: 24829595
seizure; hemispherectomy; hemispherotomy; psychomotor outcomes
20.  Idiopathic Small Fiber Neuropathy: Phenotype, Etiologies, and the Search for Fabry Disease 
Background and Purpose
The etiology of small fiber neuropathy (SFN) often remains unclear. Since SFN may be the only symptom of late-onset Fabry disease, it may be underdiagnosed in patients with idiopathic polyneuropathy. We aimed to uncover the etiological causes of seemingly idiopathic SFN by applying a focused investigatory procedure, to describe the clinical phenotype of true idiopathic SFN, and to elucidate the possible prevalence of late-onset Fabry disease in these patients.
Methods
Forty-seven adults younger than 60 years with seemingly idiopathic pure or predominantly small fiber sensory neuropathy underwent a standardized focused etiological and clinical investigation. The patients deemed to have true idiopathic SFN underwent genetic analysis of the alpha-galactosidase A gene (GLA) that encodes the enzyme alpha-galactosidase A (Fabry disease).
Results
The following etiologies were identified in 12 patients: impaired glucose tolerance (58.3%), diabetes mellitus (16.6%), alcohol abuse (8.3%), mitochondrial disease (8.3%), and hereditary neuropathy (8.3%). Genetic alterations of unknown clinical significance in GLA were detected in 6 of the 29 patients with true idiopathic SFN, but this rate did not differ significantly from that in healthy controls (n=203). None of the patients with genetic alterations in GLA had significant biochemical abnormalities simultaneously in blood, urine, and skin tissue.
Conclusions
A focused investigation may aid in uncovering further etiological factors in patients with seemingly idiopathic SFN, such as impaired glucose tolerance. However, idiopathic SFN in young to middle-aged Swedish patients does not seem to be due to late-onset Fabry disease.
doi:10.3988/jcn.2014.10.2.108
PMCID: PMC4017013  PMID: 24829596
etiology; Fabry disease; idiopathic; impaired glucose tolerance; small fiber neuropathy
21.  Clinical and Electrophysiologic Responses to Acetylcholinesterase Inhibitors in MuSK-Antibody-Positive Myasthenia Gravis: Evidence for Cholinergic Neuromuscular Hyperactivity 
Background and Purpose
Patients with muscle-specific tyrosine kinase (MuSK) antibody (MuSK-Ab)-positive myasthenia gravis (MG) show distinct responses to acetylcholinesterase inhibitors (AChEIs). Although clinical responses to AChEIs in MuSK-Ab MG are reasonably well known, little is known about the electrophysiologic responses to AChEIs. We therefore investigated the clinical and electrophysiologic responses to AChEIs in MuSK-Ab-positive MG patients.
Methods
We retrospectively reviewed the medical records and electrodiagnostic findings of 17 MG patients (10 MuSK-Ab-positive and 7 MuSK-Ab-negative patients) who underwent electrodiagnostic testing before and after a neostigmine test (NT).
Results
The frequency of intolerance to pyridostigmine bromide (PB) was higher in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (50% vs. 0%, respectively; p=0.044), while the maximum tolerable dose of PB was lower in the former (90 mg/day vs. 480 mg/day, p=0.023). The frequency of positive NT results was significantly lower in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (40% vs. 100%, p=0.035), while the nicotinic side effects of neostigmine were more frequent in the former (80% vs. 14.3%, p=0.015). Repetitive compound muscle action potentials (R-CMAPs) developed more frequently after NT in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (90% vs. 14.3%, p=0.004). The frequency of a high-frequency-stimulation-induced decrement-increment pattern (DIP) was higher in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (100% vs. 17.7%, p=0.003).
Conclusions
These results suggest that MuSK-Ab-positive MG patients exhibit unique and hyperactive responses to AChEIs. Furthermore, R-CMAP and DIP development on a standard AChEI dose may be a distinct neurophysiologic feature indicative of MuSK-Ab-positive MG.
doi:10.3988/jcn.2014.10.2.119
PMCID: PMC4017014  PMID: 24829597
myasthenia gravis; acetylcholinesterase inhibitor; muscle-specific tyrosine kinase; repetitive compound muscle action potential
22.  Obsessive-Compulsive Symptoms and Their Impacts on Psychosocial Functioning in People with Epilepsy 
Background and Purpose
Obsessive-compulsive symptoms (OCS) in people with epilepsy (PWE) have not been studied systematically. We evaluated the severity, predictors, and psychosocial impact of OCS in PWE.
Methods
We recruited PWE who visited our epilepsy clinic and age-, gender-, and education-matched healthy controls. Both PWE and healthy controls completed the Maudsley Obsessional-Compulsive Inventory (MOCI), which measures OCS. PWE also completed the Beck Depression Inventory (BDI) and the Quality of Life in Epilepsy Inventory-31 (QOLIE-31). We examined the severity of OCS in PWE relative to healthy controls. Predictors of OCS and the QOLIE-31 score were measured by regression analyses. A path analysis model was constructed to verify interrelations between the variables.
Results
The MOCI total score was significantly higher in PWE than in healthy controls (p=0.002). OCS were found in 20% of eligible patients. The strongest predictor of the MOCI total score was the BDI score (β=0.417, p<0.001), followed by EEG abnormality (β=0.194, p<0.001) and etiology (β=0.107, p=0.031). Epileptic syndrome, the side of the epileptic focus, and action mechanisms of antiepileptic drugs did not affect the MOCI total score. The strongest predictor of the QOLIE-31 overall score was the BDI score (β=-0.569, p<0.001), followed by seizure control (β=-0.163, p<0.001) and the MOCI total score (β=-0.148, p=0.001). The MOCI total score directly affected the QOLIE-31 overall score and also exerted indirect effects on the QOLIE-31 overall score through seizure control and the BDI score.
Conclusions
OCS are more likely to develop in PWE than in healthy people. The development of OCS appears to elicit psychosocial problems directly or indirectly by provoking depression or uncontrolled seizures.
doi:10.3988/jcn.2014.10.2.125
PMCID: PMC4017015  PMID: 24829598
obsessive-compulsive symptom; MOCI; predictor; epilepsy; quality of life; depression
23.  Association between Ischemic Stroke and Vascular Shear Stress in the Carotid Artery 
Background and Purpose
Vascular shear stress is essential for maintaining the morphology and function of endothelial cells. We hypothesized that shear stress in the internal carotid artery (ICA) may differ between patients with ischemic stroke and healthy control subjects.
Methods
ICA shear stress was calculated in 143 controls and 122 patients with ischemic stroke who had a normal ICA or an ICA with <50% stenosis. The stroke group included patients who presented with a first-ever or recurrent ischemic stroke but excluded cardioembolic stroke and uncertain etiologies. Of the 122 patients, 107 (87.7%) and 15 (12.3%) patients were categorized as first-ever and recurrent stroke, respectively.
Results
Carotid diameters were significantly larger, and both peak-systolic and end-diastolic velocities were significantly lower in patients with ischemic stroke than in controls (all p values <0.05). Mean values of peak-systolic and end-diastolic shear stress in both ICAs were significantly lower in patients with ischemic stroke in models that adjusted for age, sex, and vascular risk factors (p for trend <0.05). The ICA shear stress was lowest in patients with recurrent stroke or the subtype of small-vessel occlusion. Higher peak-systolic and end-diastolic shear stresses in both ICAs were independently and negatively associated with ischemic stroke after adjusting for potential confounders (all p values <0.05).
Conclusions
ICA shear stresses were significantly lower in patients with ischemic stroke than in control subjects. Future studies should attempt to define the causal relationship between carotid arterial shear stress and ischemic stroke.
doi:10.3988/jcn.2014.10.2.133
PMCID: PMC4017016  PMID: 24829599
carotid artery; hemodynamics; ischemic stroke; shear stress
24.  Antioxidant Effects of Statins in Patients with Atherosclerotic Cerebrovascular Disease 
Background and Purpose
Oxidative stress is involved in the pathophysiological mechanisms of stroke (e.g., atherosclerosis) and brain injury after ischemic stroke. Statins, which inhibit 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase, have both pleiotropic and low-density lipoprotein (LDL)-lowering properties. Recent trials have shown that high-dose statins reduce the risk of cerebrovascular events. However, there is a paucity of data regarding the changes in the oxidative stress markers in patients with atherosclerotic stroke after statin use. This study evaluated changes in oxidative stress markers after short-term use of a high-dose statin in patients with atherosclerotic stroke.
Methods
Rosuvastatin was administered at a dose of 20 mg/day to 99 patients who had suffered an atherosclerotic stroke and no prior statin use. Blood samples were collected before and 1 month after dosing, and the serum levels of four oxidative stress markers-malondialdehyde (MDA), oxidized LDL (oxLDL), protein carbonyl content (PCO), and 8-hydroxy-2'-deoxyguanosine (8-OHdG)-were evaluated to determine the oxidation of MDA and lipids, proteins, and DNA, respectively, at both of those time points.
Results
The baseline levels and the degrees of reduction after statin use differed among the oxidative stress markers measured. MDA and PCO levels were associated with infarct volumes on diffusion-weighted imaging (r=0.551, p<0.05, and r=0.444, p=0.05, respectively). Statin use decreased MDA and oxLDL levels (both p<0.05) but not the PCO or 8-OHdG level. While the reduction in MDA levels after statin use was not associated with changes in cholesterol, that in oxLDL levels was proportional to the reductions in cholesterol (r=0.479, p<0.01), LDL (r=0.459, p<0.01), and apolipoprotein B (r=0.444, p<0.05).
Conclusions
The impact of individual oxidative stress markers differs with time after ischemic stroke, suggesting that different oxidative markers reflect different aspects of oxidative stress. In addition, short-term use of a statin exerts antioxidant effects against lipid peroxidation via lipid-lowering-dependent and -independent mechanisms, but not against protein or DNA oxidation in atherosclerotic stroke patients.
doi:10.3988/jcn.2014.10.2.140
PMCID: PMC4017017  PMID: 24829600
atherosclerosis; ischemic stroke; statin; oxidative stress; cholesterol
25.  Validity of Korean Versions of the Multiple Sclerosis Impact Scale and the Multiple Sclerosis International Quality of Life Questionnaire 
Background and Purpose
Assessment of the health-related quality of life (HRQoL) is important in clinical evaluations of multiple sclerosis (MS) patients for quantifying the impact of illness and treatment on their daily lives. Although MS-specific HRQoL instruments have been used internationally, there are no data regarding HRQoL instruments specifically designed for patients with MS in Korea. The objective of this study was to determine the reliability and validity of the Korean Multiple Sclerosis Impact Scale (MSIS-29) and the Multiple Sclerosis International Quality of Life (MusiQoL) questionnaire.
Methods
Fifty-six patients with MS were recruited from June 2009 to February 2010 at the National Cancer Center in Korea. The original English versions of the MSIS-29 scale and the MusiQoL questionnaire were translated into Korean and evaluated for their acceptability, reliability, and validity.
Results
The patients wereaged 36.5±8.6 years (mean±SD; range, 20-56 years). Their score on the Expanded Disability Status Scale was 2.0±1.9 (mean; range, 0-7.5), and their disease duration was 5.2±4.7 years (mean±SD; range, 1-24 years). The Korean versions of the MSIS-29 and MusiQoL questionnaires showed satisfactory psychometric properties, including construct validity (item-internal consistencies of 0.59-0.95 and 0.59-0.92, respectively; item-discriminant validities of 95-100% and 93.8-100%), internal consistency (Cronbach's alpha coefficients of 0.96-0.97 and 0.77-0.96), reliability (intraclass correlation coefficients of 0.78-0.90 and 0.50-0.93), unidimensionality (Loevinger scalability coefficients of 0.70-0.78 and 0.63-0.90), and acceptability. External validity testing indicated the presence of significant correlations between similar aspects of the two questionnaires.
Conclusions
The Korean translated versions of the MSIS-29 and MusiQoL questionnaires demonstrated reliability and validity for measuring HRQoL in Korean patients with MS.
doi:10.3988/jcn.2014.10.2.148
PMCID: PMC4017018  PMID: 24829601
multiple sclerosis; health-related quality of life; MSIS-29; MusiQoL

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