Research progress on risk factors for radiologically isolated syndrome conversion and its clinical management / 中华神经科杂志

Radiologically isolated syndrome (RIS) is considered as an imaging marker of the prodromal stage of multiple sclerosis (MS), and approximately one-third of patients might convert to clinically isolated syndrome or clinically definite multiple sclerosis within 5 years after diagnosis. Although it still remains inconclusive whether or not disease-modifying therapies for RIS need to be initiated, effective identification of prognostic factors for conversion would be of great benefit for early screening of high-risk patients and subsequent clinical precision management. Combined with the latest research progress at home and abroad, this review summarizes the prognostic factors for conversion of RIS to MS from the aspects of demographic characteristics, clinical presentations, imaging manifestations, cerebrospinal fluid and ophthalmological examinations. Given that disease-modifying therapies for RIS are still in clinical research stage and there are no definite recommendations to date, this article expounds the research progress on clinical monitoring, therapeutics and prevention of disease progression, which aims to provide neurologists with reference suggestions on clinical management of RIS.

Changes in circulating follicular helper T cells and regulatory follicular T cells in patients with neuromyelitis optica spectrum disorder / 实用医学杂志

Objective To study the changes and significance of the frequencies of circulating follicular helper T cells (cTfh) and circulating regulatory follicular T cells (cTfr) as well as the cTfh/cTfr ratio in neuromyelitis optica spectrum disorder (NMOSD).Methods The frequencies of cTfh,cTfr and B cells in patients with NMOSD and health controls(HCs) were measured by flow cytometry.Enzyme-linked immunosorbent assay was used to detect the level of IL-21 and AQP4-Ab in patients and HCs.Results The frequencies of cTfh and B cells,the cTfh/cTfr ratio and the plasma level of IL-21 werc significantly higher in the relapsing patients than those in the remitting patients and HCs(P ＜ 0.05),and the cTfr level in the relapsing patients was lower than that in the remitting patients and healthy population (P ＜ 0.05).But no statistical differences were observed in the above indexes between the remitting paticnts and HCs.There was also no significant difference in AQP4-Ab level between the patients with relapse and remission (P ＞ 0.05).The frequency of cTfh in the patients wasc positively correlated with the level of B cells and IL-21(P ＜ 0.05),and the frequency of cTfr was negatively correlated with B cells and IL-21 (P ＜ 0.05).The ratio of cTfh/cTfr was positively correlated with B cell frequency and IL-21 level (P ＜ 0.05).AQP4-Ab level had no correlation with the frequencies of cTfh cells and B cells,cTfh/cTfr ratio and IL-21 concentration (P ＞ 0.05).Conclusion The changes in the frequencies of cTfh and cTfr as well as the imbalanced cTfh/cTfr ratio may promote the activation of humoral immunein NMOSD and participate in the pathogenesis of this disease.

Characteristics of clinical presentation and neuropathology in idiopathic hypereosinophilic syndrome encephalopathy / 中华神经科杂志

Objective To discuss the mechanism and characteristics of clinic presentation and neuropathology in idiopathic hypereosinophilic syndrome (IHES) encephalopathy. Methods IHES encephalopathy was diagnosed by clinical presentations,lab examinations,neurologic images,marrow and brain biopsy,then treated with corticosteroids and continuous follow-up. Results The IHES patient presented progressive limbs weakness and cognitive deficit with elevated eosinophil count. Results of lab examinations and bone marrow biopsy ruled out secondary eosinophilia and clonal eosinophilia such as eosinophilic leukemia.Brain magnetic resonance imaging (MRI) study showed multiple lesions in right frontal lobe, bilateral parietal and occipital lobe, presenting hypointensity in T1 weighted images,hyperintensity in T2 and fluid-attenuated inversion recovery weighed images. Brain biopsy showed proliferation of vascular membrane,small vessels stenosis,ischemia-induced morphological change and necrosis of neurons in the lesions.The patient was continuously treated with corticosteroids,and the situation was stabilized with follow-up.Conclusions IHES encephalopathy should be paid more attention in clinical practice of neurologists for its rarity. Brain vascular damages caused by elevated eosinophil may be an important pathophysiological mechanism of IHES encephalopathy,and corticosteroids or hydroxycarbamide should be used for the treatment.

Clinical analysis of two patients with rhythmic movement disorder / 中华神经科杂志

Objective To investigate the clinical features and the possible pathogenesis of rhythmic movement disorder (RMD) by analyzing 2 patients with RMD and reviewing the literature. Methods By using overnight polysomnogram (PSG) and sleeping video monitoring, the movement patterns, sleep architecture, and sleep quality of 2 patients who met the RMD diagnostic criteria were examined. Results Two male patients were 15-years old. The onset age of patient 1 was 3-years old, and patient 2 was 10-years old. All abnormal movements occurred in sleep, which presented with repetitive, stereotyping and rhythmical movements. Multiple patterns of abnormal sleeping movement were observed in 2 patients: head hypsokinesis, thoracic and waist hyperextension, and pendular movement of bilateral upper extremities. In the sitting position, the patient exhibited kneeling position, and fore-and-aft or lateral rhythmical swing of the upper body accompanied with head-banging. In the prone position, the patient behaved head backward hyperextension, and horizontal and fluctuating pendular movement of the body, which was just like the auto-erotic situation. In the lateral sleep position, the patient supported their head by using the right hand accompanied with fore-and-aft pendular movement of the head and the upper body. These symptoms mentioned above emerged immediately when the patient fell asleep, and continuously existed in all sleep period including non-rapid eye movement and rapid eye movement. All of the symptoms disappeared once the patient woke. The abnormal movement frequency was 0.1-2.0 Hz. In addition, the sleep architecture and quality were severely influenced by RMD in patient 2. Clonazepam might markedly ameliorate the symptoms and sleep quality. Conclusions Multiple abnormal movement patterns may exist in the RMD patients, and these abnormal movements could last during the whole sleep period. PSG and sleeping video monitoring should be undertaken for the suspected RMD patients, which are very useful for the definite diagnosis of RMD.

Restless legs syndrome:23-cases report / 中华神经科杂志

Objective To investigate the clinical features of restless legs syndrome(RLS),its possible pathogenesis.and the effects of benserazide on the patients with RLS.Methods Twenty-three patients who met the primary diagnostic criteria of RLS were retrospectively analyzed.Results Twenty-three middle-aged or elderly patients aged 56 years in average had an average onset age of 52 years.Insomnia and daytime sleepiness were mostly common complains for these patients.Based on the diagnostic criteria of International RLS study group(IRLSSG),the average IRLSSG score was 25,and 16 cases(69%)of the patients were severe(21-30).Polysomnographic examination showed that 18 cases(78%)had periodic limb movement.in which the PLM index of 11 cases(61%)patients Was moderate(25-49).PLM-arousal index of all patients was increased.that of 16 cases(67%)patients being moderate.After treatment by levodopa/benserazide 100/25 mg at bedtime each night for 4 weeks,most of patients'subjective symptoms improved markedly.and the IRLSSG score Was obviously decreased.with an average score of 13,and 5 patients became completely normal.Among patients with periodic legs movement.the PLM index became normal in 5 patients and became mild in others.In addition.the PLM-arousal index in all patients Was markedly decreased.with that of 11 patients becoming normal.The sleep latency of 19 patients became normal.On the other hand,6 patients(26%)had transient headache,nausea,and lethargy.Conclusions Patients with discomfortable feeling of lower extremity which is mitigated after movement.and legs movement during sleep should be check up as early as possible.Benserazide may be considered as an effeetive medication for patients with RLS.

Spinal Clinically Isolated Syndromes:Clinical and MRI Analysis / 实用放射学杂志

Objective To evaluate the clinical manifestations and magnetic resonance imaging(MRI) features of the clinically isolated syndrome(CIS) of the spinal cord.Methods MRI features and expanded disability status scale(EDSS) score in 63 patients with multiple sclerosis(MS) showed early clinical manifestations of spinal CIS were retrospectively analysed.Results 52.9% of MS patients in the early performance was the spinal CIS,88.9% was acute or subacute onset,42.9% of the initial symptoms was isolated sensory dysfunction,and 54.9% had cervical spinal cord involvement.The first MRI positive rate was 91.1% and 35.3% presented with "multifocal" plagues.81.7% of the MRI lesions were not more than two vertebral segments,and 89.0% in the axial diameter of the spinal cord did not exceed 1/2.The number,volume and area of MRI lesions at baseline confirmed the positive correlation with EDSS at diagnosis of MS.Corticosteroid therapy before and after the EDSS score was of a significant difference(P= 0.003).Conclusion Spinal CIS often occurs in cervical spinal cord with acute or subacute onset,and incomplete spinal cord injury.MRI may detect "multifocal" plagues.Quantitative MRI is valuable for the assessment of prognosis.Early intravenous corticosteroid therapy can be an effective way to ease symptoms.

Effect of hyperphosphorylated tau protein on the formation of brain amyloid / 临床神经病学杂志

Objective To explore the effect of hyperphosphorylated tau protein on the formation of brain amyloid.Methods Okadaic acid was injected into lateral ventricle of rats, once a day for eight weeks. The place navigation and spatial probe ability of rats were assessed by Morris water maze. Immunohistochemistry techniques were used to detect the expressions of neurofibrillary tangles and amyloid.Results In the Okadaic acid group, the spatial learning and memory abilities of rats were significantly impaired. The mean incubation period of Morris water maze was longer than control group. The accumulation of neurofibrillary tangles and plenty of ?-amyloid positive cells were detected in hippocampus CA1, CA3 and CA4 regions, dentate gyrus and cortex. Deposition of ?-amyloid was observed in hippocampus CA1 and CA3 regions, dentate gyrus and cortex.Conclusion The hyperphosphorylated tau protein may significantly increase deposition of amyloid in brain.