The effect of the potassium channel blocker 4-aminopyridine(4-AP) on spontan eous upbeat nystagmus (UBN) was investigated with the search coil technique duri ng fixation in different gaze positions and smooth pursuit ...The effect of the potassium channel blocker 4-aminopyridine(4-AP) on spontan eous upbeat nystagmus (UBN) was investigated with the search coil technique duri ng fixation in different gaze positions and smooth pursuit in a patient before a nd after ingestion of 10 mg 4-AP. UBN was reduced from 8.6 deg/s to 2.0 deg/s b y 4-AP causing subjective relief from distressing oscillopsia, and impaired upw ard smooth pursuit was restored(gains: before medication 0.38; after medication 0.86). In the dark, UBN was slightly stronger and not affected by 4-AP. We prop ose that 4-AP improved the function of cerebellar pathways that mediate gaze ho lding and smooth pursuit by intensifying the excitability of cerebellar Purkinje cells.展开更多
Background: We report an unusual case of Wernicke’s encephalopathy presenting with transient upbeat nystagmus that changed to a persistent downbeat nystagmus . Case: A 27-year-old man presented with upbeat nystagmus....Background: We report an unusual case of Wernicke’s encephalopathy presenting with transient upbeat nystagmus that changed to a persistent downbeat nystagmus . Case: A 27-year-old man presented with upbeat nystagmus. Three months earlie r, he had been diagnosed with Wernicke’s encephalopathy after fasting for a mon th. Observations: This diagnosis was supported by his symptoms (ataxia, a confus ed state). Clinical recovery followed thiamine therapy. His upbeat nystagmus had linear slow phases with average amplitude and frequency (±SD) during fixation straight ahead of 2.8±0.7°and 4.6±2.2Hz. respectively. Two months later, the primary position upbeat nystagmus had diminished and down beat nystagmus (0.9 + 0.5°and 3.2±0.7 Hz on average) for a 20°downward gaze had developed. Then, 8 months later, he showed only downbeat nystagmus, which obeyed Alexander’s law. His primary position downbeat nystagmus was completely suppressed by clonazepam, a γ-aminobutyric acid (GABA) agonist. Conclusions: Owing to an underlying cen tral vestibular imbalance, even after the recovery of acute neurological symptom s, Wernicke’s encephalopathy can be complicated by persistent downbeat nystagmu s, which can be treated by a GABA agonist.展开更多
患儿 女，6岁。主因发作性双眼上视4年，于2017年11月就诊于宣武医院。患儿入院前4年无明显诱因出现发作性双眼上视，表现为清醒期突发低头伴双眼上视，目光呆滞，每次持续5~10 s。发作时伴或不伴身体发软，但不摔倒。每天发作10余次，...患儿 女，6岁。主因发作性双眼上视4年，于2017年11月就诊于宣武医院。患儿入院前4年无明显诱因出现发作性双眼上视，表现为清醒期突发低头伴双眼上视，目光呆滞，每次持续5~10 s。发作时伴或不伴身体发软，但不摔倒。每天发作10余次，发热、劳累或仰卧位时多发。发作间期一般状态好。外院脑电图提示痫样放电，诊断为癫痫，后左乙拉西坦3 ml 2次/d，丙戊酸钠4 ml 2次/d，硝西泮1 mg 2次/d，托吡酯5 mg 2次/d，均无效或者短暂有效，10余天后又恢复原有发作频率。故逐渐减停抗癫痫药物，仅剩左乙拉西坦口服液2 ml 1次/d。展开更多
AIM:To investigate the effect of acetyl-L-carnitine(ALCAR)on cell viability,morphological integrity,and vascular endothelial growth factor(VEGF)expression in human retinal pigment epithelium(ARPE-19)cells using a hypo...AIM:To investigate the effect of acetyl-L-carnitine(ALCAR)on cell viability,morphological integrity,and vascular endothelial growth factor(VEGF)expression in human retinal pigment epithelium(ARPE-19)cells using a hypoxic model.METHODS:In the first set of experiments,the optimal CoCl_(2) dose was determined by exposing ARPE-19 cell cultures to different concentrations.To evaluate the effect of ALCAR on cell viability,five groups of ARPE-19 cell culture were established that included a control group,a sham group(200μM CoCl_(2)),and groups that received 1,10 and 100 mM doses of ALCAR combined with 200μM CoCl_(2),respectively.The cell viability was measured by MTT assay.The morphological characteristics of cells were observed by an inverted phase contrast microscope.The levels of VEGF and HIF-1α secretion by ARPE-19 cells were detected by enzyme linked immunosorbent assay(ELISA)assay.RESULTS:ARPE-19 cells were exposed to different doses of CoCl_(2) in order to create a hypoxia model.Nevertheless,when exposed to a concentration of 200μM CoCl_(2),a notable decrease in viability to 83% was noted.ALCAR was found to increase the cell viability at 1 mM and 10 mM concentrations,while the highest concentration(100 mM)did not have an added effect.The cell viability was found to be significantly higher in the groups treated with a concentration of 1 mM and 10 mM ALCAR compared to the Sham group(P=0.041,P=0.019,respectively).The cell viability and morphology remained unaffected by the greatest dose of ALCAR(100 mM).The administration of 10 mM ALCAR demonstrated a statistically significant reduction in the levels of VEGF and HIF-1α compared with the Sham group(P=0.013,P=0.033,respectively).CONCLUSION:The findings from the current study indicate that ALCAR could represent a viable therapeutic option with the potential to open up novel treatment pathways for retinal diseases,particular relevance for age-related macular degeneration(AMD).However,to fully elucidate ALCAR’s application potential in retinal diseases,additional investigation is necessary to clearly define the exact mechanisms involved.展开更多
文摘The effect of the potassium channel blocker 4-aminopyridine(4-AP) on spontan eous upbeat nystagmus (UBN) was investigated with the search coil technique duri ng fixation in different gaze positions and smooth pursuit in a patient before a nd after ingestion of 10 mg 4-AP. UBN was reduced from 8.6 deg/s to 2.0 deg/s b y 4-AP causing subjective relief from distressing oscillopsia, and impaired upw ard smooth pursuit was restored(gains: before medication 0.38; after medication 0.86). In the dark, UBN was slightly stronger and not affected by 4-AP. We prop ose that 4-AP improved the function of cerebellar pathways that mediate gaze ho lding and smooth pursuit by intensifying the excitability of cerebellar Purkinje cells.
文摘Background: We report an unusual case of Wernicke’s encephalopathy presenting with transient upbeat nystagmus that changed to a persistent downbeat nystagmus . Case: A 27-year-old man presented with upbeat nystagmus. Three months earlie r, he had been diagnosed with Wernicke’s encephalopathy after fasting for a mon th. Observations: This diagnosis was supported by his symptoms (ataxia, a confus ed state). Clinical recovery followed thiamine therapy. His upbeat nystagmus had linear slow phases with average amplitude and frequency (±SD) during fixation straight ahead of 2.8±0.7°and 4.6±2.2Hz. respectively. Two months later, the primary position upbeat nystagmus had diminished and down beat nystagmus (0.9 + 0.5°and 3.2±0.7 Hz on average) for a 20°downward gaze had developed. Then, 8 months later, he showed only downbeat nystagmus, which obeyed Alexander’s law. His primary position downbeat nystagmus was completely suppressed by clonazepam, a γ-aminobutyric acid (GABA) agonist. Conclusions: Owing to an underlying cen tral vestibular imbalance, even after the recovery of acute neurological symptom s, Wernicke’s encephalopathy can be complicated by persistent downbeat nystagmu s, which can be treated by a GABA agonist.
文摘患儿 女，6岁。主因发作性双眼上视4年，于2017年11月就诊于宣武医院。患儿入院前4年无明显诱因出现发作性双眼上视，表现为清醒期突发低头伴双眼上视，目光呆滞，每次持续5~10 s。发作时伴或不伴身体发软，但不摔倒。每天发作10余次，发热、劳累或仰卧位时多发。发作间期一般状态好。外院脑电图提示痫样放电，诊断为癫痫，后左乙拉西坦3 ml 2次/d，丙戊酸钠4 ml 2次/d，硝西泮1 mg 2次/d，托吡酯5 mg 2次/d，均无效或者短暂有效，10余天后又恢复原有发作频率。故逐渐减停抗癫痫药物，仅剩左乙拉西坦口服液2 ml 1次/d。
文摘AIM:To investigate the effect of acetyl-L-carnitine(ALCAR)on cell viability,morphological integrity,and vascular endothelial growth factor(VEGF)expression in human retinal pigment epithelium(ARPE-19)cells using a hypoxic model.METHODS:In the first set of experiments,the optimal CoCl_(2) dose was determined by exposing ARPE-19 cell cultures to different concentrations.To evaluate the effect of ALCAR on cell viability,five groups of ARPE-19 cell culture were established that included a control group,a sham group(200μM CoCl_(2)),and groups that received 1,10 and 100 mM doses of ALCAR combined with 200μM CoCl_(2),respectively.The cell viability was measured by MTT assay.The morphological characteristics of cells were observed by an inverted phase contrast microscope.The levels of VEGF and HIF-1α secretion by ARPE-19 cells were detected by enzyme linked immunosorbent assay(ELISA)assay.RESULTS:ARPE-19 cells were exposed to different doses of CoCl_(2) in order to create a hypoxia model.Nevertheless,when exposed to a concentration of 200μM CoCl_(2),a notable decrease in viability to 83% was noted.ALCAR was found to increase the cell viability at 1 mM and 10 mM concentrations,while the highest concentration(100 mM)did not have an added effect.The cell viability was found to be significantly higher in the groups treated with a concentration of 1 mM and 10 mM ALCAR compared to the Sham group(P=0.041,P=0.019,respectively).The cell viability and morphology remained unaffected by the greatest dose of ALCAR(100 mM).The administration of 10 mM ALCAR demonstrated a statistically significant reduction in the levels of VEGF and HIF-1α compared with the Sham group(P=0.013,P=0.033,respectively).CONCLUSION:The findings from the current study indicate that ALCAR could represent a viable therapeutic option with the potential to open up novel treatment pathways for retinal diseases,particular relevance for age-related macular degeneration(AMD).However,to fully elucidate ALCAR’s application potential in retinal diseases,additional investigation is necessary to clearly define the exact mechanisms involved.
