Partial Regression of Congenital Brain Stem Glioma and Its Outcome

2021 ◽  
Author(s):  
Sandeep Kadam ◽  
Rajendra Chavan ◽  
Archana Kadam ◽  
Sanjay Patole
Keyword(s):  
Early Intervention ◽  
Brain Stem ◽  
Neurodevelopmental Outcome ◽  
Brain Stem Glioma ◽  
Partial Regression ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Optimal Approach ◽  
And Behavior

AbstractCongenital brain stem gliomas are rare in neonates and are difficult to diagnose given their subtle clinical presentation. They are usually associated with poor prognosis by their location and behavior. However, there are few reports of spontaneous regression of brain stem glioma with favorable long-term outcome. In this article, we reported a case of congenital brain stem glioma with a normal long-term outcome where a wait and watch approach allowed observation of spontaneous partial regression of the tumor with normal neurodevelopmental outcome at 40 months of age. The optimal approach to the management of children with brain stem glioma is difficult to define as, in general, the prognosis is considered poor. Selecting an “early intervention” or “wait and watch” approach depends on the nature, size, and progression of the lesion, and the risk versus benefits of early intervention. The clinical course of our case suggests that a conservative approach may be justified in selected cases as long as the parents have been counselled and regular frequent follow-up is assured.

scholarly journals Neurodevelopmental outcome of babies with hypoxic ischemic encephalopathy

2017 ◽  
Vol 5 (7) ◽  
pp. 3197
Author(s):  
Sirajuddin Nazeer ◽  
Senthilkumar K. ◽  
Thangavel A. ◽  
Uma Maheswari M.
Keyword(s):  
Neurological Outcome ◽  
Neurodevelopmental Outcome ◽  
Hypoxic Ischemic Encephalopathy ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Mri Brain ◽  
Early Markers ◽  
Cdc Grading ◽  
Ischemic Encephalopathy

Background: The aim of the study was to find out the neurodevelopmental outcome of babies with hypoxic ischemic encephalopathy at 6 months of age and to predict early markers of abnormal neurological outcome in those babies.Methods: 50 babies admitted with hypoxic ischemic encephalopathy were enrolled in this prospective study and followed up at 3 and 6 months of age at Mahatma Gandhi Memorial Government Hospital, Trichy. The neurological outcome of the babies was assessed by CDC grading of motor milestones, Trivandrum development screening chart, Amiel Tison angles head circumference and weight measured. USG cranium was done for all the babies and MRI brain was done in babies with abnormal neuro sonogram and abnormal outcome. Vision and hearing were tested clinically.Results: The incidence of abnormal neurological outcome was 14%. The early markers predicting abnormal neurological sequele are identified.Conclusions: Early identification of abnormal neuro behaviour helps in starting early intervention to improve the long term outcome.

scholarly journals Long-term neurodevelopment in children born with esophageal atresia: a systematic review

2021 ◽  
Author(s):  
Camille E van Hoorn ◽  
Chantal A ten Kate ◽  
Andre B Rietman ◽  
Leontien C C Toussaint-Duyster ◽  
Robert Jan Stolker ◽  
...  
Keyword(s):  
Cognitive Development ◽  
Motor Function ◽  
Esophageal Atresia ◽  
Current Knowledge ◽  
Neurodevelopmental Outcome ◽  
Cognitive Outcome ◽  
Term Outcome ◽  
Long Term Outcome ◽  
The One

Summary Background Although the survival rate of esophageal atresia (EA) has increased to over 90%, the risk of functional long-term neurodevelopmental deficits is uncertain. Studies on long-term outcomes of children with EA show conflicting results. Therefore, we provide an overview of the current knowledge on the long-term neurodevelopmental outcome of children with EA. Methods We performed a structured literature search in Embase, Medline Ovid, Web of Science, Cochrane CENTRAL, and Google scholar on November 8, 2020 with the keywords ‘esophageal atresia’, ‘long-term outcome’, ‘motor development’, ‘cognitive development’, and ‘neurodevelopment’. Results The initial search identified 945 studies, of which 15 were included. Five of these published outcomes of multiple tests or tested at multiple ages. Regarding infants, one of six studies found impaired neurodevelopment at 1 year of age. Regarding preschoolers, two of five studies found impaired neurodevelopment; the one study assessing cognitive development found normal cognitive outcome. Both studies on motor function reported impairment. Regarding school-agers, the one study on neurodevelopmental outcome reported impairment. Cognitive impairment was found in two out of four studies, and motor function was impaired in both studies studying motor function. Conclusions Long-term neurodevelopment of children born with EA has been assessed with various instruments, with contrasting results. Impairments were mostly found in motor function, but also in cognitive performance. Generally, the long-term outcome of these children is reason for concern. Structured, multidisciplinary long-term follow-up programs for children born with EA would allow to timely detect neurodevelopmental impairments and to intervene, if necessary.

scholarly journals Clinical and Biochemical Profiles of Filipino Patients with Distal Urea Cycle Disorders Detected by Abnormal Expanded Newborn Screening

2020 ◽  
Vol 54 (4) ◽  
Author(s):  
Michelle E. Abadingo ◽  
Mary Ann R. Abacan ◽  
Mary Anne D. Chiong ◽  
Leniza G. De Castro-Hamoy
Keyword(s):  
Newborn Screening ◽  
Neurodevelopmental Outcome ◽  
Clinical Manifestations ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Expanded Newborn Screening ◽  
Biochemical Profiles ◽  
Cycle Disorders ◽  
Argininosuccinate Synthetase Deficiency

Objective. The study is a retrospective review which provides preliminary data on the correlation between biochemical profiles and initial clinical manifestation of patients diagnosed to have argininosuccinate synthetase deficiency (ASSD) and argininosuccinate lyase deficiency (ASLD) detected by expanded newborn screening (ENBS).Methods. This is a study of five distal UCD patients initially detected by elevated citrulline on ENBS. Medical charts of the patients were reviewed. The initial clinical manifestations of the patients were correlated with results of biochemical tests.Results. There were four cases of ASLD and one case of ASSD reviewed in this study. All cases of ASLD were confirmed by the presence of argininosuccinic acid (ASA) in the urine metabolic screen (UMS). The plasma citrulline level of the ASSD patient is significantly elevated as compared to the ASLD patients (2,690 µmol/L; NV: 10-45 µmol/L). The ASSD patient and one ASLD patient were symptomatic within the first six days of life. Both presented with significantly elevated plasma ammonia, citrulline and glutamine levels. Three ASLD patients were asymptomatic on initial screening.Conclusion. ENBS has shown importance in the early detection and management of ASSD and ASLD. Early initiation of management may prevent hyperammonemic crises. Long term outcome studies are needed to look into the correlation of neurodevelopmental outcome with lifelong accumulation of citrulline and glutamine in ASSD and ASA in ASLD.

The transoral approach to the superior cervical spine

1989 ◽  
Vol 71 (1) ◽  
pp. 16-23 ◽  
Author(s):  
Mark N. Hadley ◽  
Robert F. Spetzler ◽  
Volker K. H. Sonntag
Keyword(s):  
Brain Stem ◽  
Surgical Approach ◽  
Morbidity Rate ◽  
Cervical Cord ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Content Type ◽  
Microsurgical Techniques ◽  
Fine Print

✓ The transoral-transclival surgical approach is the most direct operative approach to pathology ventral to the brain stem and superior spinal cord. In selected patients, this approach is efficacious in the treatment of extradural compressive lesions from the cervicomedullary junction to the C-4 vertebra. The authors have used the transoral surgical approach in treating 53 patients with lesions compressing the ventral extradural brain stem or the cervical cord. The evaluation, management, and long-term outcome of these patients are described (median follow-up time 24 months). The operative morbidity rate in this series was 6%, and the operative mortality rate was zero. The authors review specific features of the transoral procedure, including methods of retraction, microsurgical techniques, and adjunctive measures to avoid cerebrospinal fluid fistulae, that contributed to these good results.

scholarly journals Cord Blood IL-16 Is Associated with 3-Year Neurodevelopmental Outcomes in Perinatal Asphyxia and Hypoxic-Ischaemic Encephalopathy

2017 ◽  
Vol 39 (1-4) ◽  
pp. 59-65 ◽  
Author(s):  
Caroline E. Ahearne ◽  
Ruby Y. Chang ◽  
Brian H. Walsh ◽  
Geraldine B. Boylan ◽  
Deirdre M. Murray
Keyword(s):  
Cord Blood ◽  
Umbilical Cord ◽  
Umbilical Cord Blood ◽  
Roc Curve ◽  
Perinatal Asphyxia ◽  
Neurodevelopmental Outcome ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Prospective Longitudinal

Activation of the inflammatory pathway is increasingly recognized as an important mechanism of injury following neonatal asphyxia and encephalopathy. This process may contribute to the poor prognosis seen in some cases, despite therapeutic hypothermia. Our group has previously identified raised interleukin (IL)-6 and IL-16, measured in umbilical cord blood at birth, to be predictive of grade of hypoxic-ischaemic encephalopathy (HIE). Our aim in this study was to examine the ability of these cytokines to predict the 3-year neurodevelopmental outcome in the same cohort. As part of a prospective, longitudinal cohort study set in a single tertiary maternity unit, term infants with biochemical and clinical evidence of perinatal asphyxia were recruited at birth. Umbilical cord blood was collected and analyzed for IL-6 and IL-16 using a Luminex assay. The neurodevelopmental outcome of these infants was assessed at 3 years using the Bayley Scales of Infant and Toddler Development (Edition 3). Early cord blood measurement of IL-6 and IL-16 and long-term outcome were available in 33/69 infants. Median (IQR) IL-16 differentiated infants with a severely abnormal outcome (n = 6) compared to all others (n = 27), (646 [466-1,085] vs. 383.5 [284-494] pg/mL; p = 0.012). IL-16 levels were able to predict a severe outcome with an area under the receiver-operating characteristic (ROC) curve of 0.827 (95% CI 0.628-1.000; p = 0.014). Levels ≥514 pg/mL predicted a severe outcome with a sensitivity of 83% and a specificity of 81%. IL-16 also outperformed other routine biochemical markers available at birth for the prediction of severe outcome. APGAR scores at 1 and 10 min were also predictive of a severe outcome (p = 0.022 and p = 0.036, respectively). A combination of IL-16 with these clinical markers did not improve predictive value, but IL-16 combined with electroencephalogram grading increased the area under the ROC curve. IL-6 did not show any association with 3-year outcome. This is the first report studying the association of IL-16 measured at birth with long-term outcome in a cohort of neonates with perinatal asphyxia. IL-16 may be an early biomarker of severe injury and aid in the long-term prognostication in infants with HIE.

Long-Term Outcome of Early Intervention for Stuttering

1997 ◽  
Vol 6 (1) ◽  
pp. 51-58 ◽  
Author(s):  
Michelle A. Lincoln ◽  
Mark Onslow
Keyword(s):  
Early Intervention ◽  
Family Member ◽  
Initial Treatment ◽  
Outcome Data ◽  
Preschool Age ◽  
Clinical Population ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Preschool Age Children

This paper reports the long-term outcome of treatment from two previous studies: Onslow, Costa, and Rue (1990), and Onslow, Andrews, and Lincoln (1994). These studies evaluated the effect of a parent-administered, operant, nonprogrammed stuttering treatment (The Lidcombe Programme) when used with preschool-age children who stuttered. The original studies provided outcome data on 4 and 12 children, respectively, until 12 months posttreatment. This study provides outcome data on the children at 2 to 7 years posttreatment. Additionally, data were collected 1 to 4 years posttreatment on a comparable clinical population who received the same treatment. The subjects in this study were 43 children who had been treated for stuttering between 2 and 5 years of age. Parents were requested to make three 10-minute recordings of their child's speech each year for 3 years. They were instructed to record their child talking to a family member at home, talking to a non-family member outside the home, and to make a covert recording of the child speaking to a different family member. Questionnaires were sent to the parents at the same time as the tapes. Near-zero stuttering levels were achieved posttreatment and were maintained in the long-term. These results suggest that preschool-age children treated for stuttering may not need to re-enter treatment for up to 7 years after their initial treatment.

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