Ataxia-telangiectasia (A-T) is a rare neurodegenerative ataxia syndrome characterized by early childhood onset and autosomal recessive inheritance. The condition results from mutations in the ataxia telangiectasia mutated (ATM) gene located on chromosome 11q22-23 that cause neurological and systemic manifestations. Clinically, A-T is marked by cerebellar ataxia and the development of telangiectasias, dilated capillaries in the oculocutaneous region. As affected children begin to sit and walk, the onset of ataxia is evident through truncal instability.The neurological symptoms associated with A-T include dysarthria, oculomotor apraxia, extrapyramidal symptoms, axonal neuropathy, and cognitive impairment. Beyond the neurological domain, individuals with A-T may experience immunological and endocrine abnormalities and an increased risk of neoplastic disease. Early childhood is a critical period for the emergence of neurological symptoms, with ataxia being the primary observable sign. The management of A-T necessitates an interprofessional team, and this activity aims to guide healthcare professionals in evaluating and improving care for patients with this complex condition.
- Provider:StatPearls, LLC
- Activity Link: https://www.statpearls.com/ArticleLibrary/viewarticle/17937
- Start Date: 2023-09-01 05:00:00
- End Date: 2023-09-01 05:00:00
- Credit Details: AMA PRA Category 1 Credit™️: 1.5 hours
Nursing: 1.5 hours
Pharmacy: 1.5 hours - MOC Credit Details: ABS - 1.5 Point; Credit Type(s): Accredited CME (ABS)
ABPATH - 1.5 Point; Credit Type(s): Lifelong Learning (ABPATH)
ABA - 1.5 Point; Credit Type(s): Lifelong Learning (ABA)
ABIM - 1.5 Point; Credit Type(s): Medical Knowledge (ABIM)
ABS - 1.5 Point; Credit Type(s): Self-Assessment (ABS)
ABP - 1.5 Point; Credit Type(s): Lifelong Learning and Self-Assessment (ABP)
ABOHNS - 1.5 Point; Credit Type(s): Self-Assessment (ABOHNS) - Commercial Support: No
- Activity Type: Enduring Material
- CME Finder Type: Online Learning
- Fee to Participate: Variable
- Measured Outcome: Learner Knowledge, Learner/Team Competence
- Provider Ship: Directly Provided
- Registration: Open to all
- Specialty: Adolescent Medicine, Clinical Pathology, Critical Care Medicine, Dermatopathology, Developmental-Behavioral Pediatrics, General Operative Anesthesia, General Otolaryngology, General Pediatrics, Hospital Medicine, Infectious Disease, Infectious Diseases/Medical Microbiology, Internal Medicine, Medical Oncology, Molecular Genetic Pathology, Neonatal-Perinatal Medicine, Neurocritical Care, Neurodevelopmental Disabilities, Neuropathology (incl. Neuromuscular), Pediatric Anesthesia, Pediatric Critical Care Medicine, Pediatric Emergency Medicine, Pediatric Hematology-Oncology, Pediatric Infectious Diseases, Pediatric Neurology