The prevalence of developmental delay (DD) in children worldwide is still on the rise, and its causes are complex and diverse, causing varying degrees of impact on the development of the sensory nervous system in the brain of children. Sensory integration training is a treatment method that applies sensory stimulation and comprehensive activities. It is currently widely used in children with DD. This therapy can effectively improve the development and integration of the sensory system in children with DD. It also enhances the individual’s ability to adapt to external stimuli, promoting their development in language, motor, and cognitive functions. This article explores the relevant mechanisms of sensory integration training and DD, as well as the current status and effects of its application in DD children, in order to promote the development and progress of related research fields.
Severe psychomotor developmental delay resulting from early postnatal (within 3 months) seizures can be diagnosed as Early-Infantile Developmental and Epileptic encephalopathies (EIDEE). Its primary etiologies include structural, hereditary, metabolic and etc. The main pathogenesis may be related to the inhibition of normal physiological activity of the brain by abnormal electrical activity and the damage of the brain neural network. Ohtahara syndrome and Early Myoclonic Encephalopathy (EME) are typical types of EIDEE. The principle of treatment is to improve the cognitive and developmental function by controlling frequent seizures. When the seizure is difficult to control with drugs, surgical evaluation should be performed as soon as possible, and surgical treatment is the first choice for patients suitable for surgery. The types of surgery can be divided into excision surgery, dissociation surgery, neuromodulation surgery and etc. The current status of surgical treatment of EIDEE was described, and the curative effect of surgical treatment was explored, so as to help clinicians choose appropriate treatment methods.
ObjectiveTo analyze the causes of unrelieved epilepsy thoroughly in children with isolated focal cortical dysplasia (FCD) based on MRI.MethodsRetrospective analysis of MRI and clinical data of 21 children with isolated FCD during July 2014 to January 2018, which confirmed by pathology and unrelieved thoroughly after operation performed, the pathological types and MRI signs were analyzed, as well as the frequency of different MRI signs in FCD of each pathological type. Analyzed the possible factors of surgical failure.ResultsAmong the 21 cases, there were 15 males and 6 females, with an average age of (5.7±0.3) years and an average course of disease of (3.4±0.5) years.MRI signs of this part of the children were mainly manifested by blurred focal gray matter boundaries, abnormal cortical structure changes (thickening and/or thinning), transmantle signs (abnormal cone signals extending from subcortical white matter to the ventricle) and abnormal gray matter signals, which were similar to MRI signs of FCD with satisfactory postoperative epilepsy control. 17 cases (80.9%) appeared epileptic discharge after operation in the EEG monitoring area 2 weeks to 6 months, FCD type I and type Ⅱ accounted for 35.3%, 64.7% respectively. During intraoperative EEG monitoring, no epileptiform discharge was observed in the transmantle sign region in 6 cases, and the region was retained, and only the surrounding abnormal discharge cortex was removed, complete removal of the tansmantle sign and surrounding abnormal discharge area was performed in 2 cases, and different degrees of epileptic epilepsy were observed in both methods.ConclusionMRI signs of isolated FCD with unrelieved epilepsy after operation were nonspecific, there were still epilepsy of varying degrees after all epileptogenic lesions have been removed, the cause may be related to potential epileptic factors.
ObjectiveTo recognize and carry out early diagnosis for Cockayne syndrome (CS) as it is an extremely rare auto-recessive genetic syndrome characterized by multiple symptoms including growth failure and impaired development of the nervous system. MethodsHere we reported a case of typical CS with an unusual appearance. The 19-year-old young male patient was referred to West China Hospital on December 24th 2012. We analyzed the clinical characteristics of the patient and followed the literature review to help improve the knowledge on CS for clinicians. ResultsThe patient's parents were cousins. Laboratory data showed that lipoprotein profile, blood glucose and electrolytes, liver and renal function, as well as hormones (thyroxin, para-thyroxin, growth hormones, adrenocorticotropic hormone, corticosteroid) were all within normal limit. Electronic hearing examination showed moderate neural hearing loss. CT scan indicated multiple intracranial calcifications. The patient was definitely diagnosed with CS. He received nutritional support and symptomatic treatment but discharged due to lack of effective treatment. ConclusionCS is a progressive multisystem disorder characterized by a specific cellular defect in transcription-coupled repair. Typical features include developmental delay and impaired development of the nervous system. Typical clinical manifestations and imaging changes are helpful for clinical diagnosis of CS. Genotyping is necessary for patients with CS. Unfortunately, there is no ideal treatment for CS. Most of the patients with CS have poor prognosis.
【Abstract】 Objective To evaluate the flexibil ity of the treatment of osteoarthritis secondary to acetabular dysplasiaby total hip arthroplasty (THA) , in which the acetabular component is placed in the true acetabulum and femoral osteotomy is not performed. Methods From January 1999 to December 2005, 35 THA procedures were performed in 32 patients with 35 hips, including 6 males with 7 hips and 26 females with 28 hips, with the average age of 53 years (ranging from 28 years to 72 years). On the basis of Crowe classification, type I included 10 patients with 11 hips, type II included 14 patients with 15 hips, type III included 5 patients with 6 hips, and type IV included 3 patients with 3 hips. All patients experienced severe pain and dysfunction. In 19 cases, the leg length discrepancy was from 3 cm to 6 cm. The Harris score was 41.49 ± 10.13 before the operation. In all procedures, the soft tissue was released entirely and the acetabular component was placed in the true acetabulum, but femoral osteotomy was not performed. Results The average operation time of unilateral THA was 50 minutes. All patients were given transfusion from 2 U to 4 U. All incisions healed at the first stage. After the operation, the leg was lengthened 2-6 cm, and the two legs were equally long. The follow-up lasted for 12 to 60 months. The Harris score was 84.71 ± 9.34 after the operation, showing statistically significant differece (P lt; 0.05). According to cl inical outcomes and X-ray films, no dislocation, femoral fracture, femoral or sciatic nerve palsy was detected. Conclusion It is effective to use THA procedures for osteoarthritis secondary to acetabular dysplasia. If the soft tissue is released entirely, the leg will be lengthened 4-6 cm without nerve palsy.
Objective To investigate the spatial and temporal regulation effect of VEGF on human fetal retinal vascularization and angiogenesis. Methods The posterior segmental retinas from 54 human fetuses of the 9th week to the 40th week were studied by immunohistodhemistry standing for the expressions of VEGF and PCNA. Results 1. The distribution of VEGF espression was spiking and the peaks were during the 9th-13th and around the 26th week. 2. PCNA immunoreactivity was localized in spindle cells and vascular endothelial cells. The expression level was fluctuated during the developmental process. The peaks were during the 9th-13th and around the 21st week. In these periods, the spindle cells kept proliferating and differentiating, and remodelled subsequently to form the inner side retinal vessels. From the 26th or 34th week, the PCNA immununoreactivity is fully expressed in the vascular endothelial cells of the inner and outer margin of inner nuclear layer(INL) and kept to full terms. 3. Significant positive correlation were shown between the content of VEGF in the retina and that of PCNA in spindle cells and vascular endothelial cells(r=0.736,p＜0.01). Conclusion VEGF was positively involved in modulating human fetal retinal vascularization and angiogenesis. (Chin J Ocul Fundus Dis,1999,15:12-15)