Differences in cognitive trajectories of dementia: Comparison Alzheimer's disease dementia and Dementia with Lewy bodies in Korean patient cohort.

BACKGROUND AND PURPOSE: Prediction of the dementia progression is important for patient management. We aimed to investigate the cognitive trajectories of Alzheimer's disease dementia (ADD) and dementia with Lewy bodies (DLB) according to the initial structural change measured by comprehensive visual rating scales (CVRS). Methods We retrospectively included the patients who initially visited the Dementia Clinic of Chonnam National University Hospital between 2010 to 2012. All patients underwent dementia work up including neuropsychological battery (Seoul Neuropsychological Screening Battery, SNSB). We recruited the participant who underwent SNSB annually for three years successively. Total 136 patients of ADD and 63 patients of DLB were included for analyze. We analyzed the decline pattern of cognitive profile according to the initial brain structural changes. Results The general cognitive trajectories between ADD and DLB patients were not different. However, DLB patients showed more rapid decline of cognitive function in language and related function, visual memory function, and frontal executive function. The scores were lower in participants with DLB with lesser atrophy group in attention, visuospatial function, and frontal executive function. In analysis of the cognitive trajectories, the visual memory domain declined rapidly in DLB with lesser atrophy group compared with the ADD with lesser atrophy group. Conclusion We founded that the differences in visual cognitive profile in ADD and DLB patients in serial follow up of neuropsychological tests. It is prominent in the mild structural change group of ADD and DLB.

Deep learning-based personalised outcome prediction after acute ischaemic stroke.

BACKGROUND: Whether deep learning models using clinical data and brain imaging can predict the long-term risk of major adverse cerebro/cardiovascular events (MACE) after acute ischaemic stroke (AIS) at the individual level has not yet been studied. METHODS: A total of 8590 patients with AIS admitted within 5 days of symptom onset were enrolled. The primary outcome was the occurrence of MACEs (a composite of stroke, acute myocardial infarction or death) over 12 months. The performance of deep learning models (DeepSurv and Deep-Survival-Machines (DeepSM)) and traditional survival models (Cox proportional hazards (CoxPH) and random survival forest (RSF)) were compared using the time-dependent concordance index ([Formula: see text] index). RESULTS: Given the top 1 to all 60 clinical factors according to feature importance, CoxPH and RSF yielded [Formula: see text] index of 0.7236-0.8222 and 0.7279-0.8335, respectively. Adding image features improved the performance of deep learning models and traditional models assisted by deep learning models. DeepSurv and DeepSM yielded the best [Formula: see text] index of 0.8496 and 0.8531 when images were added to all 39 relevant clinical factors, respectively. In feature importance, brain image was consistently ranked highly. Deep learning models automatically extracted the image features directly from personalised brain images and predicted the risk and date of future MACEs at the individual level. CONCLUSIONS: Deep learning models using clinical data and brain images could improve the prediction of MACEs and provide personalised outcome prediction for patients with AIS. Deep learning models will allow us to develop more accurate and tailored prognostic prediction systems that outperform traditional models.

A Case Report of Pseudotumor Cerebri Syndrome with a Huge Retroperitoneal Cyst.

Background: Aside from primary pseudotumor cerebri syndrome (PTCS) with an unknown etiology (i.e., idiopathic intracranial hypertension), which typically occurs in association with obesity, several conditions including cerebral venous abnormalities, drug use, and hormonal imbalance may be a secondary cause of PTCS. However, a focal space-occupying lesion outside of the brain as a cause of PTCS has rarely been reported. Case Presentation: A previously healthy 34-year-old man presented with blurred vision for three weeks. The patient had a three-month preceding history of worsening headache. On admission, he was hypertensive (160/90 mmHg) and underweight with a body mass index of 18.4 kg/m2. Fundus examination documented papilledema in both eyes. Neurological examination was unremarkable except for mild nuchal rigidity, and results of routine serologic testing were normal. Gadolinium-enhanced brain magnetic resonance imaging revealed bilateral posterior scleral flattening, suggesting intracranial hypertension. There was no other abnormal brain parenchymal lesion or meningeal enhancement. Cerebrospinal fluid (CSF) assay showed a markedly increased opening pressure (30.0 cmH2O) with normal CSF composition. A tentative diagnosis of PTCS was made based on ophthalmological, neuroradiological, and laboratory findings. During differential diagnosis, abdomen computed tomography demonstrated a huge benign cystic lesion (14.7 × 10.6 × 16.4 cm) in the right retroperitoneal space, which originated from the mesentery and resulted in hydronephrosis and renovascular hypertension due to external compression of the right kidney. Other evaluations were unremarkable. After successful surgical removal of the cyst, clinical symptoms such as headache, blurred vision, and papilledema on fundus examination were markedly improved, and blood pressure was normalized during the three-month follow-up period. Conclusions: A large retroperitoneal cyst that can increase intra-abdominal pressure could be a rare cause of PTCS. Therefore, meticulous evaluation is warranted for patients with PTCS, especially those without known risk factors.

d-dimer Level as a Predictor of Recurrent Stroke in Patients With Embolic Stroke of Undetermined Source.

Background and Purpose: This study aimed to investigate the value of d-dimer levels in predicting recurrent stroke in patients with embolic stroke of undetermined source. We also evaluated the underlying causes of recurrent stroke according to d-dimer levels. Methods: A total of 1431 patients with undetermined source were enrolled in this study and divided into quartiles according to their baseline plasma d-dimer levels. The primary outcome measure was the occurrence of recurrent stroke (ischemic or hemorrhagic) in the year following the stroke event. Results: The risk of recurrent stroke increased significantly with the increasing d-dimer quartile (log-rank P=0.001). Patients in the higher d-dimer quartiles had a higher probability of recurrent embolic stroke because of covert atrial fibrillation, hidden malignancy, or undetermined sources. Most recurrent strokes in Q3 and Q4 were embolic but not in Q1 or Q2. Multivariate analysis revealed that patients in Q3 and Q4 had a significantly increased risk of recurrent stroke compared with those in Q1 (hazard ratio, 3.12 [95% CI, 1.07−9.07], P=0.036; hazard ratio, 7.29 [95% CI, 2.59−20.52], P<0.001, respectively; Ptrend<0.001). Binary analyses showed a significant association between a high d-dimer level above normal range and the risk of recurrent stroke (hazard ratio, 2.48 [95% CI, 1.31−4.70], P=0.005). In subgroup analyses, a high d-dimer level was associated with a significantly higher risk of recurrent stroke in men than in women (P=0.039). Conclusions: Our findings suggest that d-dimer levels can be a useful risk assessment biomarker for predicting recurrent stroke, especially embolic ischemic stroke, in patients with undetermined source.

Association between freezing of gait and bone mineral density in patients with Parkinson's disease.

Parkinson's disease (PD) patients are at risk for developing bone health problems, and freezing of gait (FOG) in PD is associated with a high risk of falling and fracture. This study aimed to determine the association between FOG and bone mineral density (BMD) in patients with PD. We included 148 PD patients. FOG was assessed using the FOG Questionnaire (FOG-Q), and BMD was measured by dual-energy X-ray absorptiometry. Of 148 PD patients, 102 (68.9%) had FOG. PD patients with FOG were older and had longer disease duration, higher levodopa equivalent dose, higher modified Hoehn and Yahr stage, higher Unified PD Rating Scale motor score, higher FOG-Q score, higher total Non-Motor Symptom Scale score, and lower BMD scores in the femoral neck area than those without FOG. Pearson correlation analysis revealed that age, sex, body mass index, and age at onset were significantly correlated with areal BMDs in all areas. FOG-Q scores correlated negatively with areal BMDs in the total hip area and femoral neck, but not with areal BMD in the lumbar spine. Multivariate regression analysis showed that FOG-Q score was significantly correlated with areal BMD in the femoral neck, but not with areal BMDs in the lumbar spine or total hip. FOG in PD patients correlates significantly with BMD in the femoral neck area. Therefore, PD patients with FOG should be screened for osteoporosis.

Cerebellar infarction presenting with isolated positional vertigo: differentiating factors for benign paroxysmal positional vertigo.

BACKGROUND: Isolated central positional vertigo (CPV) due to cerebellar infarction is often difficult to differentiate from benign paroxysmal positional vertigo (BPPV). Here, we aimed to evaluate whether vascular risk factors and serum vitamin D level can differentiate between positional vertigo types. METHODS: A total of 78 consecutive patients were consecutively enrolled from January 2017. All CPV patients had a National Institutes of Health Stroke Scale score of 0 and cerebellar infarctions confirmed by brain MR imaging. Vascular risk factors and serum 25-hydroxyvitamin D levels were compared between the two groups of patients. RESULTS: The proportion of men was higher in the CPV than in the BPPV group (p = 0.004). Atrial fibrillation was common in the CPV group on univariate analysis (p = 0.046). However, there were no independent differentiating factors between the two groups. The proportion of patients according to the number of risk factors was significantly different between the two groups (linear by linear association test, p = 0.02). The mean serum 25-hydroxyvitamin D level did not differ. Also, the proportions of vitamin D insufficiency and deficiency did not differ significantly between the two groups. CONCLUSIONS: Increased number of vascular risk factors including male sex suggested more CPV than BPPV. However, the serum vitamin D level was below the normal range in both groups. Our results demonstrate that serum vitamin D level has little value in the differential diagnosis of positional vertigo. Efforts to identify differentiating factors are warranted, and accumulating evidences including our research may lead to a diagnostic algorithm for isolated positional vertigo.

Inhibitory Neural Network's Impairments at Hippocampal CA1 LTP in an Aged Transgenic Mouse Model of Alzheimer's Disease.

Alzheimer's disease (AD) is a neurodegenerative disorder characterized by a rapid accumulation of amyloid ß (Aß) protein in the hippocampus, which impairs synaptic structures and neuronal signal transmission, induces neuronal loss, and diminishes memory and cognitive functions. The present study investigated the impact of neuregulin 1 (NRG1)-ErbB4 signaling on the impairment of neural networks underlying hippocampal long-term potentiation (LTP) in 5xFAD mice, a model of AD with greater symptom severity than that of TG2576 mice. Specifically, we observed parvalbumin (PV)-containing hippocampal interneurons, the effect of NRG1 on hippocampal LTP, and the functioning of learning and memory. We found a significant decrease in the number of PV interneurons in 11-month-old 5xFAD mice. Moreover, synaptic transmission in the 5xFAD mice decreased at 6 months of age. The 11-month-old transgenic AD mice showed fewer inhibitory PV neurons and impaired NRG1-ErbB4 signaling than did wild-type mice, indicating that the former exhibit the impairment of neuronal networks underlying LTP in the hippocampal Schaffer-collateral pathway. In conclusion, this study confirmed the impaired LTP in 5xFAD mice and its association with aberrant NRG1-ErbB signaling in the neuronal network.

Microbleeds and Outcome in Patients With Acute Ischemic Stroke and Atrial Fibrillation Taking Anticoagulants.

BACKGROUND AND PURPOSE: We investigated the impact of the presence, burden, and location of cerebral microbleeds (CMBs) on the risk of major adverse cerebrovascular and cardiovascular events (MACCE) in patients with acute ischemic stroke and atrial fibrillation treated with oral anticoagulants (OACs). We also examined whether the clinical effect of CMBs differs according to the type of OACs. METHODS: A total of 1742 patients with acute ischemic stroke and atrial fibrillation treated with OACs were enrolled in this cohort study. The primary composite outcome was the occurrence of MACCE (a composite of stroke, acute myocardial infarction, or vascular death) over a 2-year period according to CMB status. RESULTS: CMB presence was significantly associated with the risk of future MACCE (hazard ratio, 1.89 [95% CI, 1.23-2.88]; P=0.003) after adjustment for confounders in patients with acute ischemic stroke and atrial fibrillation taking OACs. Patients with exactly 1 CMB had a similar rate of MACCE compared with those without CMBs (P=0.461). However, patients with multiple CMBs (≥2), particularly high burden CMBs (≥5), had a significantly higher proportion of MACCE. Both CMB-positive groups with lobar and deep CMB had more frequent MACCE than the CMB-negative group, and the rate of MACCE was not different according to CMB location. In patients treated with warfarin, CMB was significantly associated with a risk of MACCE (P=0.002), but not in patients treated with direct OACs (P=0.517). CONCLUSIONS: The study results indicate that the risk of future MACCE increased with increasing CMB burden in patients with AIS and atrial fibrillation taking OACs, while the anatomic location of CMBs did not influence the risk of future MACCE. This risk seemed to be more apparent in patients taking warfarin.

Learning difficulties and auditory processing deficits in a clinical sample of primary school-aged children.

Objective: The current study examined the relationship between learning and auditory processing (AP) ability in a clinical sample of children with and without learning difficulties (LD).Design: A non-randomised, cross-sectional, single measure research design was used.Study sample: The participants consisted of 50 children (7.7-10.8 years) who had been referred for a clinical AP assessment based on having been referred from a school-based AP screening. These children had previously been identified as having (n = 14) or not having (n = 36) LD.Results: Children with LD performed significantly worse than children without LD on frequency patterns with linguistic reports (FPlinR and FPlinL), dichotic digits (DD) and Auditory Word Memory - Forward (ANMF) tests, with significant correlations being observed between these variables and the learning score. The multiple linear regression showed that FPlinR, DDR and ANMF scores explained 50% of the variance in the learning score.Conclusion: The present study's results are most consistent with risk factor models linking AP to learning abilities in children where reduced AP abilities could put children at greater risk for LD. Further investigations into the potential relationships between AP, cognition, speech and language development, and learning ability in children are warranted.

Impact of Visceral Adipose Tissue on Clinical Outcomes After Acute Ischemic Stroke.

Background and Purpose- The purpose of this study was to investigate the association between adiposity using adipose tissue imaging and stroke outcomes in acute ischemic stroke patients treated with intravenous thrombolysis. Methods- A total of 127 patients with acute ischemic stroke treated with intravenous thrombolysis who underwent abdominal computed tomography on admission were enrolled in this prospective cohort study. Patients were grouped according to their visceral adipose tissue (VAT) proportion tertile. The primary outcome was measured using the modified Rankin Scale 3 months after symptom onset. Favorable and excellent outcomes were defined as modified Rankin Scale scores of 0 to 2 and 0 to 1, respectively. Results- As VAT proportion tertile increased, the number of patients exhibiting a favorable or excellent outcome decreased. In the final multivariable analysis after adjustments for confounders, patients in the highest VAT proportion tertile showed a decreased probability of a favorable and excellent outcome compared with those in the lowest tertile (odds ratio=0.18; 95% CI, 0.05-0.60; P=0.005 and odds ratio=0.13; 95% CI, 0.02-0.64; P=0.012, respectively). Obese patients (body mass index ≥25) also showed an excellent outcome compared with nonobese patients (odds ratio=4.88; 95% CI, 1.47-7.85; P=0.011). Among obese patients, those with an excellent outcome presented a significantly lower VAT proportion than those without (38.2% versus 46.1%, P=0.006). Conclusions- Results of this study indicate that low visceral abdominal fat proportion is associated with a favorable and excellent outcome in acute ischemic stroke patients treated with intravenous thrombolysis. Better clinical outcomes in obese patients were also associated with a lower proportion of VAT.

HbA1c (Glycated Hemoglobin) Levels and Clinical Outcome Post-Mechanical Thrombectomy in Patients With Large Vessel Occlusion.

Background and Purpose- We analyzed the relationship between HbA1c (glycated hemoglobin) levels and clinical outcomes in patients with large vessel occlusion treated with mechanical thrombectomy (MT). Methods- A total of 534 patients with acute ischemic stroke (AIS) treated with MT were enrolled in this prospective cohort study. The primary outcome measured was the modified Rankin Scale score at 3 months, according to HbA1c level. High HbA1c levels were defined as a plasma level of HbA1c >6.5%. Favorable outcomes were defined as functional independence, with modified Rankin Scale scores of 0 to 2. Secondary functional outcomes included mortality, early clinical outcomes, and intracranial hemorrhage. Results- The number of patients with a favorable outcome was significantly lower in patients with HbA1c >6.5% than in those with HbA1c ≤6.5% (28.8% versus 42.1%; P=0.006). In multivariate analysis, high HbA1c levels (especially >7.0% HbA1c) were significantly associated with poor functional outcomes 3 months after AIS in patients with large vessel occlusion treated with MT. High HbA1c was also significantly associated with increased mortality and worse early clinical outcomes after AIS in patients treated with MT. Subgroup analyses showed that HbA1c >6.5% was associated with significantly lower odds of functional independence at 3 months after AIS, when comparing the recanalized group with nonrecanalized patients. Conclusions- These results suggest that high HbA1c level is an independent predictor of a poor outcome at 3 months after AIS in patients with large vessel occlusion treated with MT, particularly in those with recanalization, and may augment the risk of mortality and early clinical worsening after AIS.

Impact of Microbleeds on Outcome Following Recanalization in Patients With Acute Ischemic Stroke.

Background and Purpose- We analyzed the association between cerebral microbleeds (CMBs) and clinical outcome in acute ischemic stroke patients and especially in a subgroup of patients with successful recanalization. Methods- A total of 1532 acute ischemic stroke patients treated with intravenous thrombolysis or mechanical thrombectomy were enrolled in this prospective cohort study. The primary outcome was measured using the modified Rankin Scale at 3 months, according to the CMB status based on magnetic resonance imaging at admission. Favorable outcome was defined as functional independence with modified Rankin Scale scores of 0 to 2. Secondary outcomes included the occurrence of symptomatic intracranial hemorrhage. Results- There was no statistically significant association between the presence of CMB and favorable outcome at 3 months when considering all patients (44.3% versus 37.6%; P=0.121). In patients with recanalization, the number of patients with favorable outcomes was significantly higher in the CMB-negative than in the CMB-positive group (57.0% versus 36.0%; P<0.001). In the final multivariate analysis, the presence of CMB, and in particular high CMB burden (≥5), and lobar location, were significantly associated with less favorable 3-month outcomes (odds ratio=0.57; 95% CI, 0.33-0.97; P=0.038) and symptomatic intracranial hemorrhage (odds ratio=3.21; 95% CI, 1.37-7.49; P=0.007) in patients with recanalization. In the analysis of subgroups, a statistically significant interaction was found between CMB presence and recanalization in predicting functional outcomes at 3 months. Conclusions- These results indicate that the presence of CMBs, and especially high burden and lobar location, are independent predictors of poor 3-month clinical outcomes and may increase symptomatic intracranial hemorrhage risk in acute ischemic stroke patients with recanalization. Our findings suggest that CMBs lead to more unfavorable effects in patients with recanalization after large vessel occlusion than in those without recanalization.

Gender-dependent effect of coffee consumption on tremor severity in de novo Parkinson's disease.

BACKGROUND: Coffee consumption represents a negative risk factor for Parkinson's disease (PD) and seems to affect PD motor symptoms. We aimed to investigate the association between coffee consumption and motor symptoms in de novo PD patients. METHODS: In total, 284 patients with de novo PD were included in the current study. Motor and non-motor symptoms were evaluated using various scales. History of coffee consumption was obtained via a semi-structured interview. RESULTS: In total, 204 patients were categorized as coffee drinkers and 80 as non-coffee drinkers. Coffee drinkers were predominantly male and had early symptom onset; in addition, they were younger, reported more years in formal education, and had better motor and non-motor scores than did non-coffee drinkers. After adjustments, coffee drinkers had lower tremor scores than did non-coffee drinkers, and coffee consumption was related to tremors in a dose-dependent manner. These relationships were statistically significant in case of rest tremor but not in case of action tremor. The dose-dependent relationship between coffee consumption and tremor severity was significant only in men. Non-motor symptom scores were not significantly different between coffee drinkers and non-coffee drinkers. CONCLUSIONS: Coffee consumption and tremor severity are inversely related in male patients with de novo PD.

Hearing and Auditory Processing Abilities in Primary School Children with Learning Difficulties.

OBJECTIVES: This study aimed to investigate hearing and auditory processing ability in primary school children with learning difficulties (LD). DESIGN: A nonrandomized, cross-sectional single measure research design was used. A total of 486 children, aged 7.7 to 10.8 years and attending years 3 and 4 in six primary schools, were classified as having an LD (n = 67) or being typically developing (TD, n = 419). This classification was based on a Learning Score generated from their school report results and National Assessment Program - Literacy and Numeracy scores. All children attempted a conventional hearing assessment (CHA) involving pure-tone audiometry, tympanometry, acoustic reflexes (AR), and otoacoustic emissions (OAEs). Children returning pure-tone audiometry results within normal limits also attempted an auditory processing assessment (APA) including dichotic digits (DD) and low-pass filtered speech (LPFS) tests. RESULTS: In children with LD, 21/67 (31.4%) failed the CHA, 20/58 (34.5%) failed the APA, and 32/58 (55.2%) failed the overall hearing assessment (OHA) if they failed either or both CHA and APA. In comparison, in TD children, 55/413 (13.3%) failed the CHA, 52/314 (16.6%) failed the APA, and 86/313 (27.5%) failed the OHA. Proportionally, children with LD were 2.4 times more likely than TD children to fail the CHA, 2.1 times more likely to fail the APA, and 2.0 times more likely to fail the OHA. In children who had completed the OHA, multiple linear regressions showed average AR thresholds, DD scores, and LPFS scores explained 13 to 18% of the variance in the Learning Score. CONCLUSION: The potential for hearing impairment should be investigated in children with LD. These investigations should begin with CHA, and for children returning normal-hearing thresholds, should continue with measures of AR, DD, and LPFS, to ensure these children receive the appropriate auditory support needed to enhance their learning.

Analysis of characteristics affecting instrumental activities of daily living in Parkinson's disease patients without dementia.

Patients with Parkinson's disease (PD) are liable to experience impairment in their activities of daily living (ADL), which include ambulating, eating, dressing, bathing, and personal hygiene. The aim of this study is to assess which clinical characteristics contribute significantly to instrumental ADL (IADL) in PD patients without dementia. We included 106 PD patients in our study, and each patient's motor and non-motor status and basic and instrumental ADL were assessed using the appropriate scales. Of the 106 PD patients, 31 (29.2%) had abnormal Korean IADL (K-IADL) scores. These patients were older and had higher scores in terms of the modified Hoehn and Yahr (mHY) staging scale, Unified Parkinson's Disease Rating Scale (UPDRS) parts II and III, UPDRS part IV motor fluctuation, Beck Depression Inventory (BDI), and total Non-Motor Symptoms assessment scale for PD (NMSS), as well as lower scores in the Mini-Mental State Examination (MMSE). Pearson's correlation analysis showed significant associations between the scores of K-IADL and each of the following characteristics of the patients: age, mHY stage, UPDRS parts II and III, UPDRS part IV motor fluctuation, BDI, total NMSS, and MMSE. Multivariate linear regression analysis showed that the significant clinical characteristics associated with the K-IADL scores were determined to be the UPDRS part II, MMSE, and BDI scores. The results of our study revealed that the cognitive, depression, and motor symptoms were the significant predictors of IADL in PD patients without dementia.

Factors associated with freezing of gait in patients with Parkinson's disease.

BACKGROUND: Freezing of gait (FOG) is a common and debilitating problem in patients with Parkinson's disease (PD). The aim of this study was to estimate the prevalence of FOG, and to identify factors that independently contribute to FOG in patients with PD. METHOD: We included 157 PD patients. FOG was assessed using the FOG Questionnaire (FOG-Q). Patients with or without FOG were defined as item 3 in the FOG-Q. RESULTS: One hundred eleven (70.7%) out of 157 PD patients presented with FOG. Patients with FOG were older, had long disease duration, were taking higher doses of dopaminergic agents, and had higher motor and non-motor scores than those without FOG. Multivariate linear regression analysis showed that high modified Hoehn and Yahr (mHY) stage, Unified PD Rating Scale (UPDRS) part II score, and non-motor symptom assessment scale for PD (NMSS) total score were significant predictors of a high FOG-Q score. Patients with FOG had significantly higher scores for cardiovascular, gastrointestinal tract, urinary, and miscellaneous NMSS domains than those without FOG. CONCLUSIONS: FOG in PD was associated with higher mHY stage, UPDRS part II score, and total NMSS score. Therefore, clinicians should consider non-motor, motor features and activities of daily living states for the proper management of FOG.

The effectiveness of using magnetic resonance imaging in syncope patients visiting an emergency department: A case-control study.

OBJECTIVE: To evaluate the effectiveness of brain magnetic resonance imaging in excluding neurological causes in patients with syncope. METHODS: This retrospective, observational, cohort study was conducted at the Chonnam National University Hospital, Gwangju, South Korea, and comprised medical record of patients with syncope from January 2011 to February 2016. The ratio of abnormal findings, the characteristics of the patients who showed abnormal findings and the relationships between the presence of neurological problem and other clinical factors were analysed. SPSS 18 was used for statistical analysis. RESULTS: Of the 1,045 patients, 142(13.5%) underwent additional magnetic resonance imaging. The results showed that 15(10.6%) patients had abnormal findings indicating neurological problems; of them, 9(60%) showed vascular stenosis, 4(27%) showed cerebral infarction, and 2(13%) showed brain tumours. The neurological problems shown were significantly higher for older patients (p=0.006) and those with the underlying diseases of hypertension (p=0.014) and coronary artery disease (p=0.008). Of these patients in particular, age (p=0.036) and history of coronary artery disease (p=0.029) were significantly associated with abnormal findings in their magnetic resonance imaging. CONCLUSIONS: Although there are no specific neurological examinations or computed tomography findings currently used in patients with syncope in the emergency department, magnetic resonance imaging may be performed to exclude neurological causes in older patients as well as those with a history of coronary artery disease.

MRI measurements of brainstem structures in patients with vascular parkinsonism, progressive supranuclear palsy, and Parkinson's disease.

Magnetic resonance (MR) measurements of brainstem structures have been reported to be useful in differentiating patients with progressive supranuclear palsy (PSP) from those with Parkinson's disease (PD). The aim of this study was to determine whether quantitative measurements of brainstem structures on MR images can help differentiate vascular parkinsonism (VaP) from degenerative parkinsonism (PD and PSP). Areas of the midbrain and pons, and widths of the superior cerebellar peduncle (SCP) and middle cerebellar peduncle (MCP) were measured in 62 patients with PD, 25 patients with PSP (11 probable and 14 possible), and 24 patients with VaP on T 1-weighted MR images. The midbrain-to-pons area ratio (M/P ratio), MCP-to-SCP width ratio (MCP/SCP ratio), and MR parkinsonism index (MRPI; P/M × MCP/SCP) were calculated. The midbrain area and M/P ratio of patients with VaP (104 and 0.22 mm2, respectively) were smaller than those in patients with PD (121 and 0.24 mm2, respectively) and larger than those in patients with PSP (90 and 0.19 mm2, respectively). The MRPI was significantly larger in patients with PSP (13.6) in comparison with those with PD (10.1) and VaP (10.7). However, the MRPI of patients with VaP was not significantly different from patients with PD. Our study showed that MRPI was useful in differentiating PSP from VaP or PD. Thus, MR imaging measurements of brainstem structures may help differentiate patients with VaP from those with PD and PSP.

The Association of Musculoskeletal Pain with Bone Mineral Density in Patients with Parkinson's Disease.

BACKGROUND: Pain and osteoporosis are common in Parkinson's disease (PD), and lower bone mineral density (BMD) or osteoporosis may be associated with an increased risk of reporting to have pain in the general population. The aim of this study was to determine whether there is an association between the pain subtypes and the BMD in patients with PD. METHODS: We included 162 PD patients. Pain was assessed using the patients' descriptions, a structured interview, a detailed neurologic examination, and the Visual Analogue Scale. BMD was measured using dual energy X-ray absorptiometry scans. RESULTS: Of the 162 PD patients, 120 had chronic pain, while 42 reported no pain. The most prevalent type of pain was musculoskeletal, followed by radicular/neuropathic, dystonic, and central. PD patients with musculoskeletal pain had a lower BMD than PD patients without pain. Multivariate regression analysis showed that the low BMD of the lumbar spine, hip, and femoral neck were related to old age, female gender, low MBI, and the presence of musculoskeletal pain. CONCLUSION: PD patients with musculoskeletal pain have low BMD and are at risk for developing osteoporosis. If a PD patient has musculoskeletal pain and other risk factors related to low BMD, clinicians should consider screening for osteoporosis.

A Mild Form of COG5 Defect Showing Early-Childhood-Onset Friedreich's-Ataxia-Like Phenotypes with Isolated Cerebellar Atrophy.

Progressive cerebellar ataxias are rare diseases during childhood, especially under 6 years of age. In a single family, three affected siblings exhibited Friedreich's-ataxia-like phenotypes before 2 years of age. They had progressive cerebellar atrophy, intellectual disability, and scoliosis. Although their phenotypes were similar to those observed in patients with autosomal recessive cerebellar ataxias, other phenotypes (e.g., seizure, movement disorders, ophthalmologic disturbance, cardiomyopathy, and cutaneous disorders) were not noted in this family. Whole-exome sequencing of the family members revealed one potential heterozygous mutation (c.1209delG, NM_181733.2; p.Met403IlefsX3, NP_859422.2) of the gene encoding conserved oligomeric Golgi complex subunit 5 (COG5). The heterozygous deletion at the fifth base in exon 12 of COG5 caused a frameshift and premature stop. Western blotting of COG5 proteins in the skin tissues from an affected proband showed a significantly decreased level of full length COG5 and smaller, aberrant COG5 proteins. We reported a milder form of COG5 defect showing Friedreich's-ataxia-like phenotypes without hypotonia, microcephaly, and short stature that were observed in most patients with COG5 defect.