Search

Xiaoyun Liu,1,* Liling Yang,1,* Yuxiang Han,1 Jingjing Xu,2 Zaiying Pang,1 Yifeng Du,1 Yabo Feng,1,* Youting Lin1,* 1Department of Neurology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan City, Shandong Province, 250021, People’s Republic of China; 2Department of Geriatrics, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan City, Shandong Province, 250021, People’s Republic of China*These authors contributed equally to this workCorrespondence: Yabo Feng; Youting LinDepartment of Neurology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, No. 324, Jingwu Road, Huaiyin Zone, Jinan City, Shandong Province, 250021, People’s Republic of ChinaTel/Fax +86-531‐68776354Email fyb-sd@hotmail.com; youtinglin2019@126.comObjective: The purpose of this study was to illustrate the electrophysiological features of sleep disturbances in patients with anti-leucine-rich glioma-inactivated protein 1 (anti-LGI1) encephalitis in both active and recovery stages.Methods: Retrospectively filed video electroencephalogram (VEEG) and polysomnography (PSG) data in 24 patients with anti-LGI1 encephalitis were analyzed in comparison with that in 20 individuals without sleep disorders as control group.Results: Sleep efficiency (SE) and total sleep time involving REM and NREM sleep were significantly decreased in patients with anti-LGI1 encephalitis during the active stage compared to that during the recovery stage and in the control group. Imbalanced sleep structure was found, demonstrated by elevated N1, decreased N3 and REM components, as well as abnormal N2 structure characterized with significantly lower spindle duration and density during the active stage. These findings were independent of the presence of nocturnal episodic events or sleep hyperkinetic movements (HMs). HMs were present in 11/23 patients throughout NREM and REM sleep (nonspecific in sleep stages) during the active stage. During the recovery stage, SE and sleep structures were dramatically improved, including the percentage of N3 and REM sleep, spindle duration and density. Ten of 11 patients with HMs were followed up. HMs were totally remitted in 3 patients and still persistent in 1, while evolved into REM sleep behavior disorder (RBD) in 4 with comorbid periodic limb movement syndrome (PLMS) in 3/4, and only PLMS in 2.Conclusion: Sleep disturbances were remarkable and intrinsic features in active anti-LGI1 encephalitis, marked by overall disruptions of both NREM and REM sleep, as well as the presence of HMs, which tend to evolve into RBD or PLMS during the recovery stage. Long-term follow-up with PSG is needed, especially for those patients with severe sleep disturbances during the active phase.Keywords: anti-leucine-rich glioma-inactivated protein 1 encephalitis, video electroencephalogram, polysomnography, sleep disturbances, REM sleep behavior disorder, periodic limb movement in sleep

Knee instability as a symptom of ligament injury usually only receives attention when it causes pain or impacts patients’ mobility in China. In this study both the physical and psychosocial impact of an innovative approach to treatment, Half-cut Wood Training, was examined. Twenty individuals with knee instability who received Halfcut Wood Training (Intervention group) and twenty two individuals with knee instability who did not receive Half-cut Wood Training (as Control group) participated in the study. The electric WIFI based HBF-306 was used to collect the anthropometry and biomedical data. Symptom severity was assessed by a doctor and through response to a specific question. Half-cut Wood Training was used as the intervention for the knee instability patients, and the effects among pre-intervention, post-intervention and control group on self-esteem, self-efficacy, quality of life and reductions in knee instability were assessed and compared to patients in the control group. The Half-cut Wood Training has significantly improved the physical health, self-esteem, self-efficacy, legs and feet functioning of patients in the intervention group compared with control group. Moreover, the Symptom were decreased (F = 15.47, P < 0.001), and the doctor reported knee function was improved (F = 36.20, P < 0.001). The subgroups including number of sessions of attending the Half-cut Wood Training had effects on the reduction of symptoms and duration of training time more than one month has effect on the improvement of physical health. Half-cut Wood Training provides beneficial means of improving knee function, self-esteem, self-efficacy, and physical health of the quality of life of patients with the knee instability.

Purpose Late-onset epilepsy due to autoimmune dysfunction has been reported. However, definitive diagnosis requires positive antibody results. As a result, patients with negative antibody results, but presenting with classical manifestation of autoimmune epilepsy, may be managed as suspected cases. In this study, we aim to isolate and profile the concentration of cytokines/chemokines in the cerebrospinal fluid (CSF) and the serum to ascertain if they could act as alternative diagnostic biomarkers. Patients and methods Twenty patients aged ≥50 years were considered in this study. Ten patients were diagnosed with suspected autoimmune epilepsy (sAE) based on clinic manifestation, electroencephalogram, magnetic resonance imaging, and with negative antibody results of the serum and the CSF. The equivalent control group exhibited neurological disorders due to non-inflammatory pathologies. Serum and CSF were analyzed for cytokines/chemokines concentration, including interleukin (IL)-6, IL-10, IL-17, chemokine (C-X-C motif) ligand (CXCL)12 and CXCL13, as well as high-mobility group box protein 1 (HMGB1) and B cell activation factor (BAFF)). Results The CSF levels of IL-6, IL-17, HMGB1, and CXCL12 were significantly higher in the sAE group than in the control group. There was no difference in the CSF levels of IL-10, CXCL13 and BAFF. The serum levels of HMGB1 and CXCL12 were elevated in the sAE group compared with the control group, and there was no statistical difference in the serum levels of IL-6, IL-10, IL-17, CXCL13, and BAFF between the two groups. Conclusion Our study shows that cytokines/chemokines may act as alternative biomarkers for diagnosis of sAE. The activation of both HMGB1/CXCL12-mediated immunity and T helper cells 17 (Th17) cells may be playing a central role in the pathogenesis of sAE. We suggest that cytokines/chemokines be treated as adjuvant biomarkers, instead of solely relying on antibody screening test. However, a larger cohort in a prospective approach is required to validate our findings.

Purpose The purpose of this study was to characterize the spectrum of motor events in patients with acute anti-leucine-rich glioma-inactivated protein 1 (anti-LGI1) encephalitis through video-electroencephalogram (VEEG) recordings. Method We collected data retrospectively from 16 patients diagnosed with anti-LGI1 encephalitis who had completed VEEG recording during hospitalization. Results VEEG monitoring lasted a median of 11.0 h (range 4.5∼20). Fourteen types of seizures were recorded in 9 patients (56.3 %). Eight of the 14 types of seizures demonstrated typical ictal EEG evolution (including 2 subclinical seizures), 3/14 demonstrated EEG electrodecremental events (EDE) at onset but without further evolution, and 3/14 could be only judged by analyzing semiology. FBDS was recorded in 6 patients (37.5 %), and all these attacks were followed by epileptic seizures. Simple hyperkinetic movements (HMs), such as jerk-like or twisting movements, were found in 8 (50 %) patients, and 6 of them had complex HMs, such as manipulating movements or mimics of daily activities, during sleep. Conclusions 1. Atypical seizures, for instance, seizures without EEG evolution, are not rare but likely to be overlooked. 2. FBDS is closely linked with epileptic seizures, revealing FBDS to be a part of epileptic attacks. 3. HMs could expand the spectrum of motor manifestations, overlapping with sleep disorders. 4. The high prevalence of these motor events might be due to the disrupted cortical-subcortical network, which is critical in motor control and sleep.

Background Although antibody-mediated immune responses are considered pathogenic and responsible for neural injury in anti-leucine-rich glioma-inactivated protein 1 (anti-LGI1) encephalitis, previous studies have indicated that cytokines and chemokines might play roles in the pathogenic process by serving as B cell enhancers. In this study, we detected the profiles of cytokines and chemokines in the cerebral fluid (CSF) and serum of patients with anti-LGI1 encephalitis to identify potential biomarkers. Methods Sixteen patients diagnosed with anti-LGI1 encephalitis and nine patients diagnosed with noninflammatory neurologic disorders were included in the study. Cytokines and chemokines including IL-6, IL-10, IL-17, CXCL12, CXCL13, BAFF and HMGB1 in serum and CSF were measured. Results The serum and CSF levels of CXCL13 were significantly higher in patients with anti-LGI1 encephalitis (36.32±34.71 pg/mL and 2.23±2.41 pg/mL, respectively) than in controls (10.84±5.02 pg/mL and 0.34±0.21 pg/mL, respectively). There was no significant difference in serum or CSF levels of IL-6, IL-10, IL-17, CXCL12, BAFF and HMGB1 between the two groups. Conclusion CXCL13 is a potential biomarker of active inflammation in anti-LGI1 encephalitis. The distinctive response of cytokines and chemokines might be closely linked to the mechanisms underlying this condition.

Purpose Late-onset epilepsy due to autoimmune dysfunction has been reported. However, definitive diagnosis requires positive antibody results. As a result, patients with negative antibody results, but presenting with classical manifestation of autoimmune epilepsy, may be managed as suspected cases. In this study, we aim to isolate and profile the concentration of cytokines/chemokines in the cerebrospinal fluid (CSF) and the serum to ascertain if they could act as alternative diagnostic biomarkers. Patients and Methods Twenty patients aged ≥50 years were considered in this study. Ten patients were diagnosed with suspected autoimmune epilepsy (sAE) based on clinic manifestation, electroencephalogram, magnetic resonance imaging, and with negative antibody results of the serum and the CSF. The equivalent control group exhibited neurological disorders due to non-inflammatory pathologies. Serum and CSF were analyzed for cytokines/chemokines concentration, including interleukin (IL)-6, IL-10, IL-17, chemokine (C-X-C motif) ligand (CXCL)12 and CXCL13, as well as high-mobility group box protein 1 (HMGB1) and B cell activation factor (BAFF)). Results The CSF levels of IL-6, IL-17, HMGB1, and CXCL12 were significantly higher in the sAE group than in the control group. There was no difference in the CSF levels of IL-10, CXCL13 and BAFF. The serum levels of HMGB1 and CXCL12 were elevated in the sAE group compared with the control group, and there was no statistical difference in the serum levels of IL-6, IL-10, IL-17, CXCL13, and BAFF between the two groups. Conclusion Our study shows that cytokines/chemokines may act as alternative biomarkers for diagnosis of sAE. The activation of both HMGB1/CXCL12-mediated immunity and T helper cells 17 (Th17) cells may be playing a central role in the pathogenesis of sAE. We suggest that cytokines/chemokines be treated as adjuvant biomarkers, instead of solely relying on antibody screening test. However, a larger cohort in a prospective approach is required to validate our findings.

Background Painful ophthalmoplegia can be caused by various etiologies, and broad differential diagnosis is needed. Carotid-cavernous fistula (CCF) is a rare cause of painful ophthalmoplegia, and early diagnosis is quite difficult. Case presentation Here, we present a case of paroxysmal painful ophthalmoplegia caused by CCF. The episodic symptoms were nonstereotypical and lasted minutes to hours. Magnetic resonance imaging (MRI) and computed tomography angiography (CTA) results were normal, which confounded efforts to determine a diagnosis. Subsequently, digital subtraction angiography (DSA) revealed a posterior-draining CCF. The CCF was treated at an early stage without residual symptoms. Conclusions We propose that symptoms could be relapsing or remitting during an early stage of CCF and that posterior-draining CCF is prone to misdiagnosis due to atypical manifestations. Normal CTA results cannot exclude carotid-cavernous fistula, and DSA should be performed once CCF is suspected.

Temporal lobe epilepsy is characterized by spontaneous recurrent seizures (SRS) and associated with behavioral problems. However, the molecular mechanisms underlying these problems are not yet clear. In this study, kainic acid (KA) was systemically administered to immature male Wistar rats to induce SRS. The behavior of the immature rats was evaluated with a water maze, elevated-plus mazes, and open field tests. The expression patterns of synaptophysin, SNAP-25, and synaptotagmin 1 (Syt 1) were examined by reverse-transcriptase polymerase chain reaction (RT-PCR) and Western blot analysis. KA-treated rats with SRS demonstrated learning and memory deficits, reduced anxiety, and increased locomotor activity, compared with placebo-treated rats and KA-treated rats without SRS. No neuronal cell loss was observed in the hippocampus 6 weeks after exposure to KA. However, RT-PCR and Western blot analyses revealed decreased synaptophysin, SNAP-25, and Syt 1 expression in KA-treated rats with SRS. Synaptophysin, SNAP-25, and Syt1 expression levels were found to be positively correlated with learning and memory but negatively correlated with anxiety and locomotor activity. These data suggested that SRS may induce changes in synaptophysin, SNAP-25, and Syt1 expression and may be functionally related to SRS-induced behavioral deficits.

In this study, we demonstrated a decreased level of mitochondrial DNA (mtDNA) with a large number of oxidized bases in hippocampi of rats with epilepsy induced by pilocarpine. In order to verify the underlying mechanism of mtDNA impairment, we detected the response of antioxidant defense system and mitochondrial base excision repair (mtBER) pathway. Superoxide dismutase2 (SOD-2) and glutathione (GSH) were significantly decreased in the experimental group, manifesting a decreased capacity of scavenging free radicals. Mitochondrial base excision repair (mtBER) pathway, which is the main repair pathway for the removal of oxidative base modifications, displayed unbalanced expression in epileptic group. DNA polymerasegamma (polgamma) increased, while apurinic/apyrimidinic endonuclease (APE1), one of mtBER initiators, decreased in mitochondria in the chronic phase of epileptogenesis. In conclusion, mtDNA was impaired during chronic recurrent seizures, whereas the endogenous antioxidants and the mtBER pathway failed to respond to the elevated mtDNA damage.