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Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic recurrent acquired immune-mediated disease of the peripheral nerves that presents with progressive sensory and motor deficits in all four limbs. Cranial nerve involvement is not as common as in Guillain-Barre syndrome, and central nervous system involvement including optic neuritis has rarely been reported in patients with CIDP. We recently experienced a case with classic CIDP involving bilateral facial and trigeminal nerves, right lower cranial nerves, and the right optic nerve.

Background: Intravenous immunoglobulin (IVIG) treatment in Guillain-Barré syndrome (GBS) is relatively easy to administer and safe due to infrequent, non-severe side effects. Adverse effects can include skin reactions, the causes of which have not been fully elucidated. In this study, we investigated skin reactions following IVIG treatment for acute inflammatory neuropathy. We examined the relationship between the clinical results in the neuropathy and the clinical profile of skin reactions. Methods: We retrospectively analyzed patients with skin reactions that developed after IVIG treatment in acute immune-mediated or inflammatory neuropathy [GBS or Miller Fisher syndrome (MFS)] based on the dermatologic evaluation. Results: Ninety-six patients with acute inflammatory neuropathy were treated with IVIG during the study period. Thirteen cases (13.5%), with age at onset ranging from 22–69 years (mean, 50.3 years), developed skin reactions after IVIG treatment. The features accompanying skin reactions were hand eczema, including pompholyx (9/13, 82%), generalized eczematous eruption (2/13, 15.4%), and contact dermatitis, including folliculitis. The electrophysiological classification of 11 patients with classical GBS showed that nine patients (81.8 %) had axonal neuropathy, and one (9.1 %) had acute inflammatory demyelinating polyradiculoneuropathy. Conclusions: Eczematous skin reactions or pompholyx are the most common skin reactions following IVIG treatment in axonal GBS. These reactions were relatively minor and recovered well. Although adverse skin reactions to IVIG were usually benign and the precise mechanism of this cutaneous eruption remains unknown, its occurrence within days of IVIG treatment can be managed effectively if recognized by neurologists.

Facial diplegia (FD) rarely occurs as a regional Guillain-Barré syndrome (GBS) variant. A 70-yearold male presented with bifacial weakness that had started on the left side and extended to the right after several days. He was then treated using steroids and gradually improved. Serum antiganglioside antibody testing revealed positivity for anti-GM1 IgG antibodies. FD can be idiopathic, but it is an uncommon GBS variant. The ganglioside antibody test may increase the possibility of diagnosing isolated FD.

The hypothalamus plays a pivotal role in the pathogenesis of narcolepsy. This study aimed to evaluate the differences in the structural covariance network of thehypothalamus based on volume differences between patients with narcolepsy and healthy controls.We retrospectively enrolled 15 patients with narcolepsy and 19 healthy controls.All subjects underwent three-dimensional T1-weighted imaging using a 3-T magnetic resonance imaging scanner. Hypothalamic subunits were segmented, and the volumes of individual hypothalamic subunits were obtained using the FreeSurfer program. Subsequently, we conducted a structural covariance network analysis of the subunit volumes with graph theory using the BRAPH program in patients with narcolepsy and in healthy controls.There were no significant differences in the volumes of the entire right and left hypothalamus nor in the hypothalamic subunit between patients with narcolepsy and healthy controls. However, we found significant differences in the structural covariance network in the hypothalamus between these groups. The characteristic path length was significantly lower in patients with narcolepsy than in healthy controls (1.698 vs. 2.831, p = 0.001). However, other network measures did not differ between patients with narcolepsy and healthy controls.We found that the structural covariance network of the hypothalamus, as assessed from the subunit volumes of hypothalamic regions using a graph theoretical analysis, is different in patients with narcolepsy compared to healthy controls. These findings may contribute to the understanding of the pathogenesis of narcolepsy.

This study aimed to evaluate the glymphatic system function in patients with obstructive sleep apnea (OSA) compared to healthy controls using diffusion tensor imaging (DTI) with the perivascular space (DTI-ALPS) method. Our hypothesis is that patients with OSA may have glymphatic system dysfunction, which is correlated with OSA severity.We enrolled 24 patients with OSA and 24 healthy controls. All participants underwent DTI magnetic resonance imaging (MRI) using the same 3T MRI scanner, and we calculated the DTI-ALPS index from the DTI. We evaluated the differences in the DTI-ALPS index between patients with OSA and healthy controls. In addition, we conducted a correlation analysis between the DTI-ALPS index and clinical characteristics.The DTI-ALPS index was significantly different between the groups. The DTI-ALPS in patients with OSA was significantly lower than in healthy controls (1.30450 vs. 1.61600, p = 0.0006). Furthermore, the DTI-ALPS index was significantly negatively correlated with the apnea-hypopnea index in sleep stage N (r = -0.427, p = 0.042) and oxygen desaturation index during sleep N (r = -0.497, p = 0.036).We successfully demonstrated glymphatic system dysfunction in patients with OSA. In addition, glymphatic system dysfunction is well correlated with OSA severity, especially during sleep stage N. Thus, these findings can explain the effects of OSA on increased risk of developing dementia and highlight the importance of OSA treatment.

GuillainBarré syndrome (GBS) is the inflammatory neuropathy that affects the myelin and nodal or paranodal areas of peripheral nerves. Immunoglobulin G GM1 antibody is well known as the cause of GBS associated with Campylobacter jejuni infection. However, the relationship between other specific infectious agents and autoantibodies is not yet well elucidated in patients with GBS. Recently we have experienced a case with GBS associated with antiGM1 and phosphatidic acid complex antibody that occurred after Shiga toxinproducing and enterotoxigenic Escherichia coli enteritis.

We aim to evaluate the shear wave velocity (SWV) of the thenar muscle as an adjunct diagnostic tool for carpal tunnel syndrome (CTS).Ninety-two wrists with CTS and 30 control wrists without CTS underwent ultrasonographic evaluation of thenar muscle and median nerve including shear-wave elastography. Cross sectional area (CSA) of medial nerve and SWV of thenar muscle and median nerve were evaluated. CTS patients were assessed for Boston CTS, Padua CTS, modified Hirani grading scores, and nerve conduction study (NCS). SWVs, CSA, and NCS parameters were compared between two groups.The SWVs of thenar muscle and median nerve (p 0.001, respectively), and CSA of median nerve (p 0.001) were more significantly greater in patients with CTS than in controls. The SWV of median nerve was moderately correlated with CSA of median nerve (r = 0.35, p 0.001) and modified Hirani CTS score (r = 0.35, p 0.001). The SWV of thenar muscle was inversely correlated with modified Hirani CTS score (r = -0.21, p = 0.04).The SWV of thenar muscle and median nerve of CTS were significantly increased compared to that of control, and significantly negatively correlated with NCS parameters (modified Hirani CTS score). SWVs may be used as an adjunct diagnostic tool for CTS.

Background We aimed to evaluate the diagnostic accuracy of enzyme-linked immunosorbent assay (ELISA) for anti-muscle specific tyrosine kinase (MuSK) antibody (Ab) in a large cohort of anti-acetylcholine receptor (AChR) Ab-negative generalized myasthenia gravis (MG), and also to investigate clinical contexts for the diagnosis of MuSK MG. Methods A retrospective study of 160 patients with a clinical suspicion of AChR Ab-negative generalized MG was performed. The serum samples were tested for anti-clustered AChR Ab by cell-based assay (CBA), anti-MuSK Ab by ELISA, CBA and/or radioimmunoprecipitation assay (RIPA). Clinical data were compared between anti-MuSK Ab-positive MG and double seronegative (AChR and MuSK) MG groups. Results After excluding non-MG and clustered AChR Ab-positive patients, we identified 89 patients as a cohort of AChR Ab-negative generalized MG. Anti-MuSK Ab was positive by ELISA in 22 (24.7%) patients. While CBA identified five additional anti-MuSK Ab-positive patients, the results of ELISA were mostly consistent with CBA and RIPA with Cohen’s kappa of 0.80 and 0.90, respectively (p Conclusion While confirming the highest sensitivity of CBA for detecting anti-MuSK Ab, our results highlight the clinical pitfalls in making a diagnosis of MuSK MG and may support a diagnostic utility of MuSK-ELISA in clinical practice.

We analyzed the histopathological findings of the clots obtained from patients with acute ischemic stroke by mechanical thrombectomy. We then developed a clinical scoring system for predicting pathogenic causes in patients with undetermined ischemic stroke using these histopathological and the angiographic findings during endovascular treatment. Only cases with the occlusion of the intracranial internal carotid artery or the proximal part of the middle cerebral artery were included in this study. Histopathologic findings of clots were compared and analyzed using the Trial of Org 10,172 in Acute Stroke Treatment (TOAST; large artery atherosclerosis, cardioembolic, and undetermined groups) and angiographic occlusion type (AOT; branching-site occlusion and truncal-type occlusion groups) classification systems. Fifty-two patients had enough clots extracted by mechanical thrombectomy for full histopathologic examination. There was no significant within-group difference in the fraction of components in the thrombi for either the TOAST or AOT system; however, the platelet distribution patterns were different. The large artery atherosclerotic group and truncal-type occlusion group had mostly peripheral patterns, whereas the cardioembolic group, undetermined group and branching-site occlusion group had mostly clustering patterns (p = 0.02 in TOAST classification; p = 0.007 in AOT classification). Patients with scores of 3 or 4 on our new scale had a sensitivity of 93.5% and a specificity of 100% for cardioembolic stroke. The BOCS2 scale, developed using a combination of the TOAST and AOT classification systems, may be helpful as an adjunctive diagnostic tool for identifying cases caused by cardiogenic embolism in patients with undetermined ischemic stroke.

Introduction: Seizures as acute stroke mimics are a diagnostic challenge. Objective: The aim of the study was to characterize the perfusion patterns on perfusion computed tomography (PCT) in patients with seizures masquerading as acute stroke. Methods: We conducted a study on patients with acute seizures as stroke mimics. The inclusion criteria for this study were patients (1) initially presenting with stroke-like symptoms but finally diagnosed to have seizures and (2) with PCT performed within 72 h of seizures. The PCT of seizure patients (n = 27) was compared with that of revascularized stroke patients (n = 20) as the control group. Results: Among the 27 patients with seizures as stroke mimics, 70.4% (n = 19) showed characteristic PCT findings compared with the revascularized stroke patients, which were as follows: (1) multi-territorial cortical hyperperfusion {(73.7% [14/19] vs. 0% [0/20], p = 0.002), sensitivity of 73.7%, negative predictive value (NPV) of 80%}, (2) involvement of the ipsilateral thalamus {(57.9% [11/19] vs. 0% [0/20], p = 0.007), sensitivity of 57.9%, NPV of 71.4%}, and (3) reduced perfusion time {(84.2% [16/19] vs. 0% [0/20], p = 0.001), sensitivity of 84.2%, NPV of 87%}. These 3 findings had 100% specificity and positive predictive value in predicting patients with acute seizures in comparison with reperfused stroke patients. Older age was strongly associated with abnormal perfusion changes (p = 0.038), with a mean age of 66.8 ± 14.5 years versus 49.2 ± 27.4 years (in seizure patients with normal perfusion scan). Conclusions: PCT is a reliable tool to differentiate acute seizures from acute stroke in the emergency setting.

OBJECTIVES The aim of this exploratory study was to investigate the underlying pathomechanisms of migraine with aura (MA) and migraine without aura (MO) in the interictal phase using a connectivity analysis. METHODS We prospectively enrolled patients who were newly diagnosed with migraine. All patients underwent brain MRI, including diffusion tensor imaging and arterial spin labeling perfusion MRI. We analyzed the differences between patients with MA and those with MO in structural connectivity based on diffusion tensor imaging and functional connectivity based on arterial spin labeling perfusion MRI using a graph theoretical analysis. RESULTS We enrolled 58 patients with migraine (11 patients with MA and 47 patients with MO). There were no differences between patients with MA and those with MO in the network measures of global structural connectivity. However, differences in global functional connectivity were found between the two groups. The assortative coefficient was lower in patients with MA than in those with MO (-0.050 vs. -0.012, p = .017). There were no differences in local structural and functional connectivity between patients with MA and those with MO. CONCLUSION We found differences in global functional connectivity between patients with MO and those with MA. The study of MA and MO using a connectivity analysis may shed light on migraine pathophysiology. We suggest it is worthwhile to investigate if changes in functional connectivity may serve as novel biomarkers in MA. In this regard, ASL MRI appears to be valuable in the context of network analysis, but further studies are needed to confirm our findings.

The glymphatic system is a glial cell-dependent waste clearance pathway in the brain that is essential for the maintenance of brain homeostasis. In this study, we evaluated glymphatic system function in patients with juvenile myoclonic epilepsy (JME) compared with healthy controls. Patients with JME and healthy controls were retrospectively enrolled in this study. All the participants underwent brain diffusion tensor imaging (DTI). The “DTI-analysis along the perivascular space (ALPS)”-index was calculated to evaluate the glymphatic system function of the participants. The ALPS-indices of the patients with JME were compared with those of the healthy controls. In addition, the correlations between ALPS-index and the clinical characteristics of the patients with JME were analyzed to validate changes in glymphatic system function. A total of 39 patients with JME and 38 healthy controls were enrolled in this study. The mean ALPS- index of the patients with JME was significantly lower than that of the healthy controls (1.541 vs. 1.653, p = 0.041). ALPS-index was negatively correlated with age in patients with JME (r = -0.375, p = 0.018). However, ALPS-index was not correlated with age at onset, duration of epilepsy, or anti-seizure medication load in patients with JME. This study is the first in which the ALPS method was used to demonstrate that patients with JME have significant glymphatic system dysfunction. The results also show that glymphatic system index is negatively correlated with age in patients with JME, a finding which demonstrates that the glymphatic system function of patients with JME gradually declines with age. The ALPS-index might be a potential biomarker for monitoring glymphatic system function in patients with epilepsy.

Asymmetric clinical presentation in some patients with diabetic polyneuropathy may result from the different vascular environments in both lower limbs. The aim of the study is to determine the association of neuropathy with vascular factors in each lower limb of diabetic patients. A total of 102 patients (204 lower limbs) given a diagnosis of diabetic polyneuropathy were enrolled. The primary end points are sensory nerve action potential (SNAP) amplitude and conduction velocity (CV) of the sural nerve and independent variables are vascular and nonvascular factors. Vascular factors include mean arterial pressure and pulse pressure at the ankle, ankle-brachial index, and arterial stiffness assessed by pulse wave velocity. Nonvascular factors include age, gender, height, body weight, body mass index, total cholesterol, and hemoglobin A1C. Age, hemoglobin A1C, and ankle pulse pressure were inversely correlated with SNAP amplitude of the sural nerve, while no factors were correlated with CV of the sural nerve. Increased arterial stiffness was significant in the limbs group with abnormal SNAP amplitude of the sural nerve, while increased height was significant in the limbs group with abnormal CV of the sural nerve. Vascular factors were more significantly associated with decreased SNAP amplitude rather than decreased CV of the sural nerve in the nerve conduction study of diabetic patients.

Objectives To examine the existence and significance of internal border zone (IBZ) infarcts with accessory lesions in the anteromedial temporal lobe (ATL). Materials and methods IBZ infarcts located at the corona radiata were selected based on diffusion-weighted imaging of 2535 consecutive patients with ischemic stroke and the presence of lesions in the ATL was identified. The Mann–Whitney U test, Student t-test, Pearson χ2 test, or Fisher exact test was used to analyze differences between the IBZ infarct groups with and without accessory lesions in the ATL. Results Thirty-six of 2535 patients (1.4%) had IBZ infarcts. The IBZ group with accessory lesions in the ATL (17 cases, 47.2%) showed a higher portion of occluded middle cerebral arteries than the IBZ group without accessory lesions in the ATL (p = 0.02). The initial National Institutes of Health Stroke Scale score (odds ratio, 2.03; 95% confidence interval, 1.04–3.99; = 0.039) and progression after admission (odds ratio, 25.43; 95% confidence interval, 2.47–261.99; p = 0.007) were independently associated with poor prognosis in patients with IBZ infarcts. There were no differences in the progression rate and clinical outcomes, regardless of the presence of lesions in the ATL. Conclusions Our study suggests the existence of a distinct type of IBZ infarct characterized by accessory lesions in the ATL, which is associated with different arterial features but has a similar clinical course to IBZ infarcts without accessory lesions in the ATL.

The aim of this study was to evaluate the feasibility of using a machine learning approach based on diffusion tensor imaging (DTI) to identify patients with juvenile myoclonic epilepsy. We analyzed the usefulness of combining conventional DTI measures and structural connectomic profiles. This retrospective study was conducted at a tertiary hospital. We enrolled 55 patients with juvenile myoclonic epilepsy. All of the subjects underwent DTI from January 2017 to March 2020. We also enrolled 58 healthy subjects as a normal control group. We extracted conventional DTI measures and structural connectomic DTI profiles. We employed the support vector machines (SVM) algorithm to classify patients with juvenile myoclonic epilepsy and healthy subjects based on the conventional DTI measures and structural connectomic profiles. The SVM classifier based on conventional DTI measures had an accuracy of 68.1% and an area under the curve (AUC) of 0.682. Another SVM classifier based on the structural connectomic profiles demonstrated an accuracy of 72.7% and an AUC of 0.727. The SVM classifier based on combining the conventional DTI measures and structural connectomic profiles had an accuracy of 81.8% and an AUC of 0.818. DTI using machine learning is useful for classifying patients with juvenile myoclonic epilepsy and healthy subjects. Combining both the conventional DTI measures and structural connectomic profiles results in a better classification performance than using conventional DTI measures or the structural connectomic profiles alone to identify juvenile myoclonic epilepsy.

We aimed to compare seroprevalence of anti-myelin oligodendrocyte glycoprotein (MOG) and anti-aquaporin-4 (AQP4) antibodies in Korean adults with inflammatory demyelinating diseases (IDDs) of the central nervous system (CNS), based on a multicenter nationwide database. Sera were analyzed using a live cell–based assay for MOG and AQP4 antibodies. Of 586 Korean adults with IDDs of the CNS, 36 (6.1%) and 185 (31.6%) tested positive for MOG and AQP4 antibodies, respectively. No participant showed double positivity. Seroprevalence of MOG antibodies was about five times lower than that of AQP4 antibodies in a large cohort of Korean adults with IDDs of the CNS.

To compare the diagnostic performance between strain elastography and shear wave elastography (SWE) for the diagnosis of carpal tunnel syndrome (CTS).Between July 2018 and June 2019, 66 consecutive patients with 95 imaged wrists underwent wrist ultrasound, including grayscale ultrasound, strain elastography, and SWE, because of the suspicion of CTS. During wrist ultrasound, the cross-sectional area (CSA), strain ratio, elasticity, and shear wave velocity of each median nerve were measured at the proximal carpal bone level (scaphoid to pisiform). The variables were compared between the normal and CTS groups by using the independent t test, and subgroup analyses were performed using one-way analysis of variance. Receiver operating characteristic (ROC) curves were used to evaluate the diagnostic performance of each variable.CSA, elasticity, and shear wave velocity showed significant intergroup differences (P 0.001, P 0.001, and P = 0.002, respectively). However, the strain ratio showed no statistically significant intergroup difference (P = 0.639). In the subgroup analyses, elasticity showed significantly higher values in the severe group than in the mild and moderate groups (P 0.001 and P = 0.001, respectively). Other parameters showed no significant differences among the different subgroups. The areas under the ROC curve were 0.823 for CSA, 0.772 for elasticity, and 0.779 for shear wave velocity. The differences in the areas under the ROC curve among CSA, elasticity, and shear wave velocity were not statistically significant (all P 0.05).SWE has a good diagnostic value in CTS. In particular, elasticity can discriminate the severe group from the other groups.

Background Although gait disturbances are relatively common symptoms in diabetic polyneuropathy (DPN), few studies have quantitatively analyzed gait and posture in DPN patients. This study aimed to analyze gait and posture quantitatively in DPN patients and to determine the association between clinical and electrophysiological parameters and gait and posture parameters. Methods Sixty-four DPN patients were enrolled in this study. DPN was clinically assessed using the Toronto clinical neuropathy score (TCNS). All participants underwent nerve conduction study (NCS), three-dimensional motion analysis, and static posturography. We evaluate the correlation of gait and posture parameters with electrophysiological and clinical parameters. Results Foot height, step length, and stride length among gait parameters were inversely correlated with the TCNS. Anteroposterior range during eyes-closed and mediolateral distance and range during eyes-open and eyes-closed were inversely correlated with the sensory nerve action potential amplitude in the sural nerve. Mediolateral distance during eyes-open and eyes-closed was correlated with the compound muscle action potential amplitude in the peroneal nerve. Conclusions Gait parameters are associated with clinical parameters, and postural parameters are associated with electrophysiological parameters, particularly sensory NCS. Gait and postural analysis can be a useful tool for assessing the neurological status in DPN patients.

Background The aim of this study is to investigate the alterations of thalamic nuclei volumes and the intrinsic thalamic network in patients with migraine. Methods We enrolled 35 patients with migraine without aura and 40 healthy controls. All subjects underwent three-dimensional T1-weighted imaging. The thalamic nuclei were segmented using the FreeSurfer program. We investigated volume changes of individual thalamic nuclei and analyzed the alterations of the intrinsic thalamic network based on volumes in the patients with migraine. Results Right and left thalamic volumes as a whole were not different between the patients with migraine and healthy controls. However, we found that right anteroventral and right and left medial geniculate nuclei volumes were significantly increased (0.00985% vs. 0.00864%, p = 0.0002; 0.00929% vs. 0.00823%, p = 0.0005; 0.00939% vs. 0.00769%, p p p Conclusions We found significant alterations of thalamic nuclei volumes in patients with migraine compared with healthy controls. These findings might contribute to the underlying pathogenesis of the migraine. Trial registration None.

A 55-year-old postmenopausal and multiparous woman presented to our department with recent memory disturbances associated with rapidly progressive positive Anti-Hu antibodies. She was subsequently diagnosed with anti-Hu antibody-related paraneoplastic limbic encephalitis. Clinical examination and imaging studies revealed a bulky cervical tumor with both parametrial and vaginal fornix extension; biopsy confirmed the tumor as cervical squamous cell carcinoma. In this case, we encountered a patient with anti-Hu-mediated paraneoplastic limbic encephalitis with a subsequent diagnosis of cervical cancer.

Purpose The aim of this study was to investigate the predictive factors and identify the characteristics of the seizure-induced signal changes on MRI (SCM) in patients with first seizures. Methods We conducted a retrospective study of patients with first seizures from March 2010 to August 2014. The inclusion criteria for this study were patients with 1) first seizures, and 2) MRI and EEG performed within 24h of the first seizures. The definition of SCM was hyper-intensities in the brain not applying to cerebral arterial territories. Multivariate logistic regression was performed with or without SCM as a dependent variable. Results Of 431 patients with seizures visiting the ER, 69 patients met the inclusion criteria. Of 69 patients, 11 patients (15.9%) had SCM. Epileptiform discharge on EEG (OR 29.7, 95% CI 1.79–493.37, p=0.018) was an independently significant variable predicting the presence of SCM in patients with first seizures. In addition, the topography of SCM was as follows; i) ipsilateral hippocampus, thalamus and cerebral cortex (5/11), ii) unilateral cortex (4/11), iii) ipsilateral thalamus and cerebral cortex (1/11), iv) bilateral hippocampus (1/11). Moreover, 6 out of 7 patients who underwent both perfusion CT and MRI exhibited unilateral cortical hyperperfusion with ipsilateral thalamic involvement reflecting unrestricted vascular territories. Conclusion There is an association between epileptiform discharges and SCM. Additionally, the involvement of the unilateral cortex and ipsilateral thalamus in SCM and its hyperperfusion state could be helpful in differentiating the consequences of epileptic seizures from other pathologies.

Objectives Orthostatic hypotension (OH) is controversially regarded as the cause of orthostatic dizziness in Parkinson's disease (PD). We sought to evaluate whether cerebral autoregulation is an alternative cause for orthostatic dizziness in PD patients, using transcranial Doppler monitoring during head-up tilting. Methods Forty-five PD patients with dizziness, 13 PD patients without dizziness, and 10 age-matched healthy controls were enrolled. Participants were divided into the following four groups: patients with dizziness and OH (group 1, n = 22), patients with dizziness but no OH (n = 23, group 2), patients without dizziness (n = 11, group 3), and age-matched healthy controls (n = 10, group 4). All participants underwent transcranial Doppler and blood pressure monitoring for 10 minutes during the head-up tilt test. Changes in the cerebral blood flow velocity (CBFV) in the middle cerebral artery and the mean blood pressure (mBP) within 3 minutes after head-up tilting were compared between groups. Results Group 1 showed a significantly higher change in mBP (-16.3 ± 10.8 mmHg) than groups 2 (-2.6 ± 4.9), 3 (-2.2 ± 3.6), or 4 (1.8 ± 6.0) (p Conclusions Our results suggest that cerebral hypoperfusion contributes to dizziness in PD patients despite a lack of OH. Transcranial Doppler monitoring during head-up tilting may be a useful tool for evaluating dizziness in PD patients with or without OH. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:337-342, 2017.

We investigated the potential role of serum procalcitonin in differentiating bacterial meningitis from viral meningitis, and in predicting the prognosis in patients with bacterial meningitis. This was a retrospective study of 80 patients with bacterial meningitis (13 patients died). In addition, 58 patients with viral meningitis were included as the disease control groups for comparison. The serum procalcitonin level was measured in all patients at admission. Differences in demographic and laboratory data, including the procalcitonin level, were analyzed between the groups. We used the mortality rate during hospitalization as a marker of prognosis in patients with bacterial meningitis. Multiple logistic regression analysis showed that high serum levels of procalcitonin (>0.12ng/mL) were an independently significant variable for differentiating bacterial meningitis from viral meningitis. The risk of having bacterial meningitis with high serum levels of procalcitonin was at least 6 times higher than the risk of having viral meningitis (OR=6.76, 95% CI: 1.84-24.90, p=0.004). In addition, we found that high levels of procalcitonin (>7.26ng/mL) in the blood were an independently significant predictor for death in patients with bacterial meningitis. The risk of death in patients with bacterial meningitis with high serum levels of procalcitonin may be at least 9 times higher than those without death (OR=9.09, 95% CI: 1.74-47.12, p=0.016). We found that serum procalcitonin is a useful marker for differentiating bacterial meningitis from viral meningitis, and it is also a potential predicting factor for prognosis in patients with bacterial meningitis.

Background and Purpose Serial nerve conduction studies (NCSs) are recommended for differentiating axonal and demyelinating Guillain-Barré syndrome (GBS), but this approach is not suitable for early diagnoses. This study was designed to identify possible NCS parameters for differentiating GBS subtypes. Methods We retrospectively reviewed the medical records of 70 patients with GBS who underwent NCS within 10 days of symptom onset. Patients with axonal GBS and acute inflammatory demyelinating polyneuropathy (AIDP) were selected based on clinical characteristics and serial NCSs. An antiganglioside antibody study was used to increase the diagnostic certainty. Results The amplitudes of median and ulnar nerve sensory nerve action potentials (SNAPs) were significantly smaller in the AIDP group than in the axonal-GBS group. Classification and regression-tree analysis revealed that the distal ulnar sensory nerve SNAP amplitude was the best predictor of axonal GBS. Conclusions Early upper extremity sensory NCS findings are helpful in differentiating axonal-GBS patients with antiganglioside antibodies from AIDP patients.

Background and Purpose— Fluid-attenuated inversion recovery vascular hyperintensities (FVHs) are seen in some cases with cerebral hemodynamic impairment and collateral flow. Because the worst outcomes of patients with borderzone infarcts were mainly correlated with impaired hemodynamics, the presence of FVH might provide another clue for predicting the prognosis of patients with borderzone infarcts. Methods— We reviewed 1377 consecutive patients with ischemic stroke. Cortical borderzone (CBZ) and internal borderzone infarcts were selected based on diffusion-weighted imaging. FVHs were defined as tubular- or serpentine-shaped hyperintensities in the subarachnoid space. We investigated the clinical significance of FVHs in borderzone-infarcted patients. Results— Among 87 patients with borderzone infarcts, the presence of FVH was observed in 30 (34.5%). We identified 62 patients with CBZ infarcts and 25 patients with internal borderzone infarcts. In the cases with CBZ infarcts, the initial National Institutes of Health Stroke Scale scores and the portions of nonfavorable outcome at 3 months in the FVH(+) group were significantly higher than in the FVH(−) group ( P P Conclusions— The findings of FVH are associated with relatively severe clinical presentation and nonfavorable prognosis in patients with CBZ infarcts, but not in patients with internal borderzone infarcts. The presence of FVH may help to identify CBZ-infarcted patients who require close observation and hemodynamic control.

Obstructive sleep apnea (OSA) is a common sleep disorder characterized by repetitive episodes of apnea and hypopnea during sleep. The symptoms include excessive daytime sleepiness, non-refreshing sleep, daytime fatigue, and decreased concentration, which may cause marked impairment in social or occupational functioning. In addition, the patients with OSA were shown to have an increased risk of cardiovascular disease, stroke, hypertension, diabetes mellitus, depression, and impaired cognitive function. Therefore, an early diagnosis and treatment of OSA are important. The gold standard for the diagnosis of OSA is overnight polysomnography. However, because polysomnography is expensive and has limited availability with complex technical support, it is essential to know the predictive risk factors for OSA. Based on these predictive risk factors, it is possible to determine which patients should have priority for undergoing polysomnography.

Objective: To categorize a syndrome manifesting as prominent acute bulbar palsy (ABP) without limb motor weakness as a variant form of Guillain-Barre syndrome (GBS) and differentiate it from Miller Fisher syndrome (MFS) and pharyngeal-cervical-brachial (PCB) variants. Methods: We analyzed cases of ABP without limb motor weakness based on a dataset containing clinical information and the results of antiganglioside antibodies assays for acute immune-mediated neuropathies. Results: Eleven cases with an age at onset ranging from 18 to 65 years (mean 33.8 years) were identified as ABP-plus syndrome. All of the enrolled cases manifested with ABP as the predominant symptom, and with no limb weakness. The following features accompanied ABP in order of decreasing frequency: ophthalmoplegia (n = 9, 82%), ataxia (n = 9, 82%), and facial palsy (n = 6, 55%). An enzyme-linked immunosorbent assay study disclosed that immunoglobulin G (IgG) anti-GT1a antibodies were the most frequent (n = 11), followed by IgG anti-GQ1b antibodies (n = 6). Conclusions: We propose that ABP-plus syndrome without neck or limb weakness is a variant of GBS that is distinct from the MFS and PCB variants. The presence of IgG anti-GT1a antibodies can explain the relationships between the distinct clinical characteristics and the underlying pathomechanisms.

We aimed to evaluate the efficacy, tolerability, and blood concentration of zonisamide (ZNS) used in daily clinical practice. This was a retrospective study performed at a single epilepsy center and included 149 patients with epilepsy. The efficacy and retention of ZNS for 52 weeks, percentage of patients who were seizure free for 26 weeks, and the 5-year retention rate were analyzed. The tolerability was assessed based on treatment-emergent adverse effects (AEs) and the adverse effect profile (AEP). More than 70% (105/149) of patients were on ZNS polytherapy. The mean dose of ZNS was 300 ± 170.6 mg/day, and the antiepileptic drug (AED) load was 2.0 ± 1.1 (median 1.8, range 0.2–5.3). The retention rate for 52 weeks and the percentage of patients who were seizure-free for 26 weeks were 73% and 42%, respectively. The retention rate decreased with time and reached 43% in 5 years. Younger age of onset was a significant variable affecting retention rate for 52 weeks (p = 0.044), whereas fewer concomitant AEDs were significantly associated with being seizure free for 26 weeks (p = 0.0006). AEs were reported in 24% (36/147) of patients. The number, mechanism of action, or drug load of the AEDs did not predict the development of AEs; however, blood concentration of ZNS was significantly higher in patients with AEs (p = 0.0011) than in those without AEs. ZNS is a well-tolerated and effective AED in daily clinical practice, and several clinical factors may predict the efficacy and tolerability of ZNS.

Objective Neurological progression is a major problem in managing the patients with acute lacunar infarction. The purpose of this was to investigate whether autonomic dysfunction is associated with neurological progression in patients with acute lacunar infarction. Materials and methods The study comprised 60 patients with acute lacunar infarction. All enrolled subjects underwent autonomic function tests including the 30° head-up tilt test, Valsalva test, heart rate response to deep breathing, and sympathetic skin response. The primary endpoint is the neurological progression, and the secondary endpoint is the 3-month outcome. Results Increased initial National Institute of Health stroke scale (NIHSS), decreased time to admission from onset, decreased rise of heart rate in the 30° head-up tilt test, abnormal blood pressure response in the Valsalva test, and decreased rise of systolic blood pressure in stage IV of the Valsalva test are associated with neurological progression of acute lacunar infarction; an abnormal blood pressure response in the Valsalva test is significant in logistic regression analysis of neurological progression. Advanced age, increased initial NIHSS and modified Rankin scale, decreased expiration/inspiration ratio of heart rate to deep breathing, decreased rise of systolic blood pressure in stage IV of the Valsalva test, and neurological progression were associated with an unfavorable 3-month outcome; neurological progression was significant in logistic regression analysis of 3-month outcome. Conclusions An abnormal blood pressure change in the Valsalva test is associated with neurological progression in patients with acute lacunar infarction, and neurological progression can induce an unfavorable 3-month outcome.

OBJECTIVE Increasing evidence has suggested that epilepsy is a network disease. Graph theory is a mathematical tool that allows for the analysis and quantification of the brain network. We aimed to evaluate the influences of duration of epilepsy on the topological organization of brain network in focal epilepsy patients with normal MRI using the graph theoretical analysis based on diffusion tenor imaging. METHODS We prospectively enrolled 66 patients with focal epilepsy (18/66 patients were newly diagnosed) and 84 healthy subjects. All of the patients with epilepsy had normal MRI on visual inspection. All of the subjects underwent diffusion tensor imaging that was analyzed using graph theory to obtain network measures. RESULTS The measures of characteristic path length and small-worldness in the patients with focal epilepsy were significantly decreased, even after multiple corrections (P

Objective Delayed facial palsy (DFP) has often been described during the recovery stage of Fisher syndrome (FS), but the implications of DFP in FS pathophysiology have not been reported previously. The aim of this study was to identify the incidence and clinical course of DFP in FS, and to determine its clinical/pathophysiological implications in FS. Methods About 71 FS patients were enrolled from seven university-based hospitals in Korea. DFP was defined with respect to new development of unilateral or bilateral facial palsies with delayed onset after either the nadir or improvement of initial neurological signs of FS. Results Eleven of the 71 patients (16%) satisfied the definition of DFP. No other cranial palsies developed as a delayed pattern. With the exception of two patients with bulbar involvement, DFP developed after a latent period of upper-cranial neuropathies. Comparison of FS patients without and with DFP revealed no significant clinical, serological, or electrophysiological differences. All except one patient with DFP exhibited a good outcome within 1 month of follow-up. Conclusion DFP was identified as a common and specific phenomenon in FS. Nearly all cases of DFP were developed in a descending manner and were associated with a good outcome, while other cranial neuropathies developed or followed as a sequential pattern. These findings suggest the involvement of so-called “descending reversible paralysis” in the pathophysiology of FS.