Background: EEG could be normal or atypical in spite of suggestive clinical features and positive measles Ab of SSPE cases which could have typical EEG pattern after Benzodiazepine. Objectives: The purpose of the pres...Background: EEG could be normal or atypical in spite of suggestive clinical features and positive measles Ab of SSPE cases which could have typical EEG pattern after Benzodiazepine. Objectives: The purpose of the present study was to find out the necessity of administration of benzodiazepine during EEG recording of SSPE cases as well as to compare the efficacy of diazepam and midazolam in eliciting EEG pattern. Methodology: This double blind, parallel, single centered, non-randomized clinical trial was conducted in the Department of Pediatric Neurology at National Institute of Neurosciences, Dhaka, Bangladesh from July 2014 to June 2015 for a period of 1 (one) year. All the clinical and investigational suspected cases of sub-acute Sclerosing Panencephalitis (SSPE) children in both sexes were included as study population. Others neurodegenerative diseases including Wilson’s disease were excluded from this study. Patients were divided into two groups named as group A who were given diazepam and the other group B was given midazolam in IV during EEG recording. The clinical outcomes were measured and were recorded in a pre-designed data sheet. Result: The characteristic typical periodic slow wave complex (PSWC) was found only in 8 (30.8%) patients among the 26 (100.0%) before intervention with benzodiazepines. The remaining 18 (69.2%) had non-typical PSWC of which 10 (38.5%) were normal, 3 (11.5%) with atypical PSWC and 5 (19.2%) were with other EEG findings. After intervention with benzodiazepines, 23 (88.5%) had shown typical PSWC and only 3 (11.5%) had non-typical PSWC. Among the typical PSWC cases after intervention, 8 (30.8%) had normal EEG initially, 5 (19.2%) had other EEG finding, 2 (7.7%) had non-typical PSWC and 8 (30.8%) had typical PSWC from the beginning. Of the 3 (11.5%) of the non-typical PSWC of intervention group, 2 (7.7%) had shown no changes in EEG from the beginning and 1 (3.8%) had shown other EEG finding. The difference between before and after intervention was actually statistically extremely significant (p 0.05). Conclusion: The role of benzodiazepine is very obvious in eliciting the typical EEG pattern in SSPE patients which has shown the characteristic PSWC in EEG in most cases.展开更多
<strong>Background:</strong><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> Cerebral palsy is the m...<strong>Background:</strong><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> Cerebral palsy is the most common chronic motor disability that begins in childhood with the predominant motor abnormality</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> that</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> is spasticity</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">.</span></span></span><span><span><span style="font-family:""> <b><span style="font-family:Verdana;">Objective:</span></b><span style="font-family:Verdana;"> The purpose of the present study was to compare the efficacy of oral baclofen and oral tizanidine in reducing spasticity in cerebral palsy patients. </span><b><span style="font-family:Verdana;">Methodology: </span></b><span style="font-family:Verdana;">This randomized controlled trial was conducted from January 2010 to December 2011 and it was carried out in the Out Patient Department (OPD) of Pediatrics at Dhaka Medical College Hospital (DMCH), Dhaka, Bangladesh and Center for Neurodevelopment and Autism in Children (CNAC) of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. </span><b><span style="font-family:Verdana;">Results</span></b><span style="font-family:Verdana;">: A total number of 70 cerebral palsy children were recruited for this study of which 35 patients were in </span><span style="font-family:Verdana;">Tizanidine</span><span style="font-family:Verdana;"> group and 35 patients were in </span><span style="font-family:Verdana;">baclofen</span><span style="font-family:Verdana;"> group. </span><span style="font-family:Verdana;">Furthermore</span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">,</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;"> 70 cerebral palsy children were taken as control who were treated with </span><span style="font-family:Verdana;">placebo</span><span style="font-family:Verdana;">. Tizanidine had superior efficacy in reducing tone in spastic cerebral palsy over baclofen measured by using </span><span style="font-family:Verdana;">Modified</span><span style="font-family:Verdana;"> Ashworth scale in </span><span style="font-family:Verdana;">different</span> <span style="font-family:Verdana;">time period</span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">s</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;"> from AS score-3 to score-2 (p < 0.05). Tizanidine was also superior in joint angle improvement in spastic cerebral palsy measured by </span><span style="font-family:Verdana;">physician</span><span style="font-family:Verdana;"> rating scale from AS score-3 to score-2 (crouch p < 0.0001) and foot contact from AS score-3 to score-2 (p < 0.0001);but no statistically significant improvement in gross motor function. </span><span style="font-family:Verdana;">Adverse</span><span style="font-family:Verdana;"> effect </span><span style="font-family:Verdana;">w</span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">as</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;"> more in </span><span style="font-family:Verdana;">baclofen</span><span style="font-family:Verdana;"> group. </span><b><span style="font-family:Verdana;">Conclusion</span></b><span style="font-family:Verdana;">: For reducing generalized spasticity with regards to muscle tone, joint angle </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> improvement in gait in cerebral palsy patients, tizanidine has superior efficacy and less adverse effects than baclofen.</span></span></span></span>展开更多
<strong>Background:</strong><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> The epileptic encephalo...<strong>Background:</strong><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> The epileptic encephalopathies collectively</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">exact an immense personal, medical, and financial toll on</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">the affected children, their families, and</span></span></span><span><span><span style="font-family:""> </span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;">the healthcare system.</span><b><span style="font-family:Verdana;"> Objective:</span></b><span style="font-family:Verdana;"> This study was aimed to delineate the clinical spectrum of patients with Epileptic encephalopathies (EEs) and classify them under various epileptic syndromes. </span><b><span style="font-family:Verdana;">Methods:</span></b><span style="font-family:Verdana;"> This was a cross-sectional study that was carried out in the department of Neurophysiology of the National Institute of Neurosciences and Hospital, Bangladesh from July 2016 to June 2019.</span></span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">Children with recurrent seizures which w</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">ere </span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;">difficult to control and associated with developmental arrest or regression in absence of a progressive brain pathology were considered to be suffering from EE. Children under 12 years of age fulfilling the inclusion criteria were enrolled in the study. These patients were evaluated clinically and Electroencephalography (EEG) was done in all children at presentation. Based on the clinical profile and EEG findings the patients were categorized under various epileptic syndromes according to International League Against Epilepsy (ILAE) classification 2010.</span><b><span style="font-family:Verdana;"> Results:</span></b><span style="font-family:Verdana;"> A total of 1256 children under 12 years of age were referred to the Neurophysiology Department. Among them, 162</span></span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">(12.90%) fulfilled the inclusion criteria. Most of the patients were male (64.2%) and below 1 year (37.7%) of age. The majority (56.8%) were delivered at the hospital and 40.1% had a history of perinatal asphyxia. Development was age-appropriate before the onset of a seizure in 38.9% of cases. Most (53.7%) of the patients had seizure onset within 3 months of age. Categorization of Epileptic syndromes found that majority had West Syndrome (WS)</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">(37.65%) followed by Lennox-Gastaut syndrome (LGS) (22.22%), Otahara syndrome (11.73%), Continuous spike-and-wave during sleep (CSWS) (5.66%), Myoclonic astatic epilepsy (MAE)</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">(4.94%), Early myoclonic encephalopathy (EME) (3.7%), Dravet</span></span></span><span><span><span style="font-family:""> </span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;">syndrome (3.7%) and Landau-Kleffner syndrome (LKS) (1.23%). 9.26% of syndromes were unclassified. </span><b><span style="font-family:Verdana;">Conclusion:</span></b><span style="font-family:Verdana;"> EEG was found to be a useful tool in the evaluation of Epileptic encephalopathies. The clinico-electroencephalographic features are age-related. Their recognition and appropriate management are critical.</span></span></span></span>展开更多
Antiphospholipid syndrome(APS)or Hughes syndrome is an acquired thromboinflammatory disorder.Clinical criteria of APS diagnosis are large-and small-vessel thrombosis as well as obstetric problems;laboratory criteria a...Antiphospholipid syndrome(APS)or Hughes syndrome is an acquired thromboinflammatory disorder.Clinical criteria of APS diagnosis are large-and small-vessel thrombosis as well as obstetric problems;laboratory criteria are the presence of antiphospholipid antibodies(lupus anticoagulant,anticardiolipin antibodies and anti-β2-glycoprotein-1).The presence of at least 1 clinical and 1 laboratory criterion allows definitive diagnosis of APS.Primary APS is diagnosed in patients without features of connective tissue disease;secondary APS is diagnosed in patients with clinical signs of autoimmune disease.A high frequency of catastrophic APS as well as a high tendency to evolve from primary APS to secondary syndrome during the course of lupus and lupus-like disease is a feature of pediatric APS.The most characteristic clinical presentation of APS in the pediatric population is venous thrombosis,mainly in the lower limbs,and arterial thrombosis causing ischemic brain stroke.Currently,no diagnostic criteria for pediatric APS exist,which probably results in an underestimation of the problem.Similarly,no therapeutic procedures for APS specific for children have yet been established.In the present literature review,we discussed data concerning APS in children and its role in cerebrovascular diseases,including pediatric arterial ischemic stroke,migraine and cerebral venous thrombosis.展开更多
An 8-year follow-up study of the birth cohort of babies enrolled in the Wess ex controlled trial of universal newborn screening (UNS) for permanent childhood hearing impairment (PCHI) was undertaken to establish wheth...An 8-year follow-up study of the birth cohort of babies enrolled in the Wess ex controlled trial of universal newborn screening (UNS) for permanent childhood hearing impairment (PCHI) was undertaken to establish whether UNS would increas e the proportion of all true cases of PCHI in children aged 7-9 years who are r eferred early. The proportion referred before 6 months of age increased from 11 of 35 (31%) children with true PCHI born during periods without UNS to 23 of 31 (74%) born during periods with UNS (difference 43%, 95%CI 19-60). UNS leads to early referral of PCHI.展开更多
Neuronal surface antibody syndromes(NSAS)encompass a variety of disorders associated with“neuronal surface antibodies”.These share clinical and neuroradiological features that pose challenges related to their recogn...Neuronal surface antibody syndromes(NSAS)encompass a variety of disorders associated with“neuronal surface antibodies”.These share clinical and neuroradiological features that pose challenges related to their recognition and treatment.Recent epidemiological studies show a clear predominance for the glutamate-N-methyl-D-aspartate receptor encephalitis in both adults and pediatric population.Despite this,the overall NSAS’s incidence remains underestimated,and diagnosis persists to be not always easy to achieve.Based on current literature data,in this paper the authors propose a diagnostic pathway to approach and treat pediatric NSAS.An autoimmune etiology can be suggested through the integration of clinical,immunological,electrophysiological and neuroradiological data.On that basis,a target treatment can be started,consisting of corticosteroids and intravenous immunoglobulin or plasma exchange as a first-line immunotherapy,followed by second-line drugs including rituximab,cyclophosphamide or mycophenolate mophetil,if the case.In children a prompt diagnosis and a targeted treatment may lead to a better clinical outcome.Nevertheless further studies are required to assess the need of more tailored treatments according to long-term outcome findings and prognostic factors in different NSAS.展开更多
文摘Background: EEG could be normal or atypical in spite of suggestive clinical features and positive measles Ab of SSPE cases which could have typical EEG pattern after Benzodiazepine. Objectives: The purpose of the present study was to find out the necessity of administration of benzodiazepine during EEG recording of SSPE cases as well as to compare the efficacy of diazepam and midazolam in eliciting EEG pattern. Methodology: This double blind, parallel, single centered, non-randomized clinical trial was conducted in the Department of Pediatric Neurology at National Institute of Neurosciences, Dhaka, Bangladesh from July 2014 to June 2015 for a period of 1 (one) year. All the clinical and investigational suspected cases of sub-acute Sclerosing Panencephalitis (SSPE) children in both sexes were included as study population. Others neurodegenerative diseases including Wilson’s disease were excluded from this study. Patients were divided into two groups named as group A who were given diazepam and the other group B was given midazolam in IV during EEG recording. The clinical outcomes were measured and were recorded in a pre-designed data sheet. Result: The characteristic typical periodic slow wave complex (PSWC) was found only in 8 (30.8%) patients among the 26 (100.0%) before intervention with benzodiazepines. The remaining 18 (69.2%) had non-typical PSWC of which 10 (38.5%) were normal, 3 (11.5%) with atypical PSWC and 5 (19.2%) were with other EEG findings. After intervention with benzodiazepines, 23 (88.5%) had shown typical PSWC and only 3 (11.5%) had non-typical PSWC. Among the typical PSWC cases after intervention, 8 (30.8%) had normal EEG initially, 5 (19.2%) had other EEG finding, 2 (7.7%) had non-typical PSWC and 8 (30.8%) had typical PSWC from the beginning. Of the 3 (11.5%) of the non-typical PSWC of intervention group, 2 (7.7%) had shown no changes in EEG from the beginning and 1 (3.8%) had shown other EEG finding. The difference between before and after intervention was actually statistically extremely significant (p 0.05). Conclusion: The role of benzodiazepine is very obvious in eliciting the typical EEG pattern in SSPE patients which has shown the characteristic PSWC in EEG in most cases.
文摘<strong>Background:</strong><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> Cerebral palsy is the most common chronic motor disability that begins in childhood with the predominant motor abnormality</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> that</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> is spasticity</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">.</span></span></span><span><span><span style="font-family:""> <b><span style="font-family:Verdana;">Objective:</span></b><span style="font-family:Verdana;"> The purpose of the present study was to compare the efficacy of oral baclofen and oral tizanidine in reducing spasticity in cerebral palsy patients. </span><b><span style="font-family:Verdana;">Methodology: </span></b><span style="font-family:Verdana;">This randomized controlled trial was conducted from January 2010 to December 2011 and it was carried out in the Out Patient Department (OPD) of Pediatrics at Dhaka Medical College Hospital (DMCH), Dhaka, Bangladesh and Center for Neurodevelopment and Autism in Children (CNAC) of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. </span><b><span style="font-family:Verdana;">Results</span></b><span style="font-family:Verdana;">: A total number of 70 cerebral palsy children were recruited for this study of which 35 patients were in </span><span style="font-family:Verdana;">Tizanidine</span><span style="font-family:Verdana;"> group and 35 patients were in </span><span style="font-family:Verdana;">baclofen</span><span style="font-family:Verdana;"> group. </span><span style="font-family:Verdana;">Furthermore</span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">,</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;"> 70 cerebral palsy children were taken as control who were treated with </span><span style="font-family:Verdana;">placebo</span><span style="font-family:Verdana;">. Tizanidine had superior efficacy in reducing tone in spastic cerebral palsy over baclofen measured by using </span><span style="font-family:Verdana;">Modified</span><span style="font-family:Verdana;"> Ashworth scale in </span><span style="font-family:Verdana;">different</span> <span style="font-family:Verdana;">time period</span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">s</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;"> from AS score-3 to score-2 (p < 0.05). Tizanidine was also superior in joint angle improvement in spastic cerebral palsy measured by </span><span style="font-family:Verdana;">physician</span><span style="font-family:Verdana;"> rating scale from AS score-3 to score-2 (crouch p < 0.0001) and foot contact from AS score-3 to score-2 (p < 0.0001);but no statistically significant improvement in gross motor function. </span><span style="font-family:Verdana;">Adverse</span><span style="font-family:Verdana;"> effect </span><span style="font-family:Verdana;">w</span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">as</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;"> more in </span><span style="font-family:Verdana;">baclofen</span><span style="font-family:Verdana;"> group. </span><b><span style="font-family:Verdana;">Conclusion</span></b><span style="font-family:Verdana;">: For reducing generalized spasticity with regards to muscle tone, joint angle </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> improvement in gait in cerebral palsy patients, tizanidine has superior efficacy and less adverse effects than baclofen.</span></span></span></span>
文摘<strong>Background:</strong><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> The epileptic encephalopathies collectively</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">exact an immense personal, medical, and financial toll on</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">the affected children, their families, and</span></span></span><span><span><span style="font-family:""> </span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;">the healthcare system.</span><b><span style="font-family:Verdana;"> Objective:</span></b><span style="font-family:Verdana;"> This study was aimed to delineate the clinical spectrum of patients with Epileptic encephalopathies (EEs) and classify them under various epileptic syndromes. </span><b><span style="font-family:Verdana;">Methods:</span></b><span style="font-family:Verdana;"> This was a cross-sectional study that was carried out in the department of Neurophysiology of the National Institute of Neurosciences and Hospital, Bangladesh from July 2016 to June 2019.</span></span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">Children with recurrent seizures which w</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">ere </span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;">difficult to control and associated with developmental arrest or regression in absence of a progressive brain pathology were considered to be suffering from EE. Children under 12 years of age fulfilling the inclusion criteria were enrolled in the study. These patients were evaluated clinically and Electroencephalography (EEG) was done in all children at presentation. Based on the clinical profile and EEG findings the patients were categorized under various epileptic syndromes according to International League Against Epilepsy (ILAE) classification 2010.</span><b><span style="font-family:Verdana;"> Results:</span></b><span style="font-family:Verdana;"> A total of 1256 children under 12 years of age were referred to the Neurophysiology Department. Among them, 162</span></span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">(12.90%) fulfilled the inclusion criteria. Most of the patients were male (64.2%) and below 1 year (37.7%) of age. The majority (56.8%) were delivered at the hospital and 40.1% had a history of perinatal asphyxia. Development was age-appropriate before the onset of a seizure in 38.9% of cases. Most (53.7%) of the patients had seizure onset within 3 months of age. Categorization of Epileptic syndromes found that majority had West Syndrome (WS)</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">(37.65%) followed by Lennox-Gastaut syndrome (LGS) (22.22%), Otahara syndrome (11.73%), Continuous spike-and-wave during sleep (CSWS) (5.66%), Myoclonic astatic epilepsy (MAE)</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">(4.94%), Early myoclonic encephalopathy (EME) (3.7%), Dravet</span></span></span><span><span><span style="font-family:""> </span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;">syndrome (3.7%) and Landau-Kleffner syndrome (LKS) (1.23%). 9.26% of syndromes were unclassified. </span><b><span style="font-family:Verdana;">Conclusion:</span></b><span style="font-family:Verdana;"> EEG was found to be a useful tool in the evaluation of Epileptic encephalopathies. The clinico-electroencephalographic features are age-related. Their recognition and appropriate management are critical.</span></span></span></span>
文摘Antiphospholipid syndrome(APS)or Hughes syndrome is an acquired thromboinflammatory disorder.Clinical criteria of APS diagnosis are large-and small-vessel thrombosis as well as obstetric problems;laboratory criteria are the presence of antiphospholipid antibodies(lupus anticoagulant,anticardiolipin antibodies and anti-β2-glycoprotein-1).The presence of at least 1 clinical and 1 laboratory criterion allows definitive diagnosis of APS.Primary APS is diagnosed in patients without features of connective tissue disease;secondary APS is diagnosed in patients with clinical signs of autoimmune disease.A high frequency of catastrophic APS as well as a high tendency to evolve from primary APS to secondary syndrome during the course of lupus and lupus-like disease is a feature of pediatric APS.The most characteristic clinical presentation of APS in the pediatric population is venous thrombosis,mainly in the lower limbs,and arterial thrombosis causing ischemic brain stroke.Currently,no diagnostic criteria for pediatric APS exist,which probably results in an underestimation of the problem.Similarly,no therapeutic procedures for APS specific for children have yet been established.In the present literature review,we discussed data concerning APS in children and its role in cerebrovascular diseases,including pediatric arterial ischemic stroke,migraine and cerebral venous thrombosis.
文摘An 8-year follow-up study of the birth cohort of babies enrolled in the Wess ex controlled trial of universal newborn screening (UNS) for permanent childhood hearing impairment (PCHI) was undertaken to establish whether UNS would increas e the proportion of all true cases of PCHI in children aged 7-9 years who are r eferred early. The proportion referred before 6 months of age increased from 11 of 35 (31%) children with true PCHI born during periods without UNS to 23 of 31 (74%) born during periods with UNS (difference 43%, 95%CI 19-60). UNS leads to early referral of PCHI.
文摘Neuronal surface antibody syndromes(NSAS)encompass a variety of disorders associated with“neuronal surface antibodies”.These share clinical and neuroradiological features that pose challenges related to their recognition and treatment.Recent epidemiological studies show a clear predominance for the glutamate-N-methyl-D-aspartate receptor encephalitis in both adults and pediatric population.Despite this,the overall NSAS’s incidence remains underestimated,and diagnosis persists to be not always easy to achieve.Based on current literature data,in this paper the authors propose a diagnostic pathway to approach and treat pediatric NSAS.An autoimmune etiology can be suggested through the integration of clinical,immunological,electrophysiological and neuroradiological data.On that basis,a target treatment can be started,consisting of corticosteroids and intravenous immunoglobulin or plasma exchange as a first-line immunotherapy,followed by second-line drugs including rituximab,cyclophosphamide or mycophenolate mophetil,if the case.In children a prompt diagnosis and a targeted treatment may lead to a better clinical outcome.Nevertheless further studies are required to assess the need of more tailored treatments according to long-term outcome findings and prognostic factors in different NSAS.
