Limited information is known about neuropsychological outcomes in Alexander disease, a rare leukodystrophy. Two pediatric cases are summarized. Case 1 (evaluations at 6, 7, 9, and 12 years of age) represents Type I Alexander disease with associated seizures. Case 2 (evaluations at 12, 13, and 16 years of age) represents Type II Alexander disease without additional complications. Case 1 experienced declines in intellectual functioning, visual motor skills, receptive vocabulary, verbal memory, and academic achievement. Case 2 experienced variable neurocognitive change and academic functioning, with average word reading and spelling. Verbal memory also remained intact. Taken together, individuals with Alexander disease may experience cognitive decline to variable degrees. Type I Alexander disease, associated with earlier onset and additional neurological complications, may presage greater cognitive decline than Type II. Due to variability in functioning over time, it is critical to follow individuals across development to make recommendations for educational and treatment planning.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8532242 | PMC |
| http://dx.doi.org/10.1177/2329048X211048614 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Advances in the Treatment of Neonatal Coarctation of the Aorta.
Pediatrics
January 2025
Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, Germany.
Coarctation of the aorta (CoA) is a potentially life-threatening congenital and obstructive anomaly of the distal aortic arch. After constriction of the ductus arteriosus, neonates may develop critical CoA in the isthmus area and present with severe left ventricular dysfunction or even cardiac failure. Low cardiac output and abdominal hypoperfusion (distal to the coarctation) may lead to metabolic derangements and clinical deterioration.
View Article and Find Full Text PDFAssociation of gut microbiota and gut metabolites and adverse outcomes in biliary atresia: A longitudinal prospective study.
Hepatol Commun
November 2024
Paediatric Liver, GI and Nutrition Centre and Mowatlabs, King's College Hospital, London, UK.
Background: The Kasai portoenterostomy (KPE) aims to re-establish bile flow in biliary atresia (BA); however, BA remains the commonest indication for liver transplantation in pediatrics. Gut microbiota-host interplay is increasingly associated with outcomes in chronic liver disease. This study characterized fecal microbiota and fatty acid metabolites in BA.
View Article and Find Full Text PDFPEG-PLGA nanoparticles deposited in and .
J Pharm Anal
December 2024
Institute of Infectious Disease and Infection Control, Jena University Hospital, Jena, 07747, Germany.
In our prior research, polymer nanoparticles (NPs) containing tobramycin displayed robust antibacterial efficacy against biofilm-embedded () and (. ) cells, critical pathogens in cystic fibrosis. In the current study, we investigated the deposition of a nanoparticulate carrier composed of poly(d,l-lactic--glycolic acid) (PLGA) and poly(ethylene glycol)--PLGA (PEG-PLGA) that was either covalently bonded with cyanine-5-amine (Cy5) or noncovalently bound with freely embedded cationic rhodamine B (RhB), which served as a drug surrogate.
View Article and Find Full Text PDFImpact of structured surveillance of patients with esophageal cancer following surgical resection with curative intent.
Surg Oncol
December 2024
Department of Internal Medicine, University Hospital Cologne, Faculty of Medicine of University of Cologne, Kerpener Str. 62, 50937, Cologne, Germany; CIO Cologne, University Hospital Cologne, Center for Integrated Oncology Aachen Bonn Cologne Duesseldorf (CIO ABCD), Kerpener Str. 62, 50937, Cologne, Germany. Electronic address:
Background: Esophageal cancer (EC) is a disease with a poor prognosis. While treatment options have been improved, there is no consensus for surveillance strategies following therapy with curative intent. As the incidence of EC is rising and a large fraction of patients will experience disease recurrence, the need for evidence-based treatment and optimal surveillance is evident.
View Article and Find Full Text PDFComprehensive histopathological analysis of gastric cancer in European and Latin America populations reveals differences in PDL1, HER2, p53 and MUC6 expression.
Gastric Cancer
January 2025
Department of Medical Oncology, Hospital Clinico Universitario, INCLIVA, Biomedical Research Institute, University of Valencia, Avenida Menendez Pelayo nro 4 accesorio, Valencia, Spain.
Introduction: Gastric cancer (GC) burden is currently evolving with regional differences associated with complex behavioural, environmental, and genetic risk factors. The LEGACy study is a Horizon 2020-funded multi-institutional research project conducted prospectively to provide comprehensive data on the tumour biological characteristics of gastroesophageal cancer from European and LATAM countries.
Material And Methods: Treatment-naïve advanced gastroesophageal adenocarcinoma patients were prospectively recruited in seven European and LATAM countries.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!