Recent genetic studies have revealed that hemimegalencephaly (HME) is a multi-system disorder associated with germline or mosaic variants within the PI3K-mTOR-GATOR1 signaling pathways. Patients with HME typically develop drug-resistant epilepsy necessitating extensive evaluation, hemispherectomy, and long-term management. We describe the role of a multidisciplinary team (MDT) for the diagnosis and management of recent patients with HME at UCLA who underwent hemispherectomy.
View Article and Find Full Text PDFPurpose: To evaluate the clinical characteristics and management of patients with an anteriorly and nasally inserted superior oblique tendon.
Design: Retrospective interventional case series.
Methods: Institutional clinical records between 2020 and 2024 were retrospectively reviewed for 9 consecutive patients in whom the superior oblique (SO) tendon was inserted anterior to the equator and nasal to the superior rectus muscle.
Study Objectives: Trauma-related nightmares (TRNs) are a hallmark symptom of PTSD and are highly correlated with PTSD severity and poor sleep quality. Given the salience and arousal associated with TRNs, they might be an effective target for imaginal exposures during Prolonged Exposure (PE) therapy. As a first step in this line of research, the current study compared participants' emotional reactivity during recollection of TRNs to their recollection of the index traumatic event.
View Article and Find Full Text PDFPurpose: To assess the utility of 3D, tablet-based, glasses-free Accurate STEReotest (ASTEROID) in children compared with the Titmus test.
Methods: Children aged 5-13 years were enrolled in a single-center, nonrandomized, observational comparison study and analyzed by age (5-7 vs 8-13 years) and visual acuity (20/25 or better in both eyes vs abnormal). Each participant underwent both the ASTEROID and Titmus stereoacuity tests.
J Binocul Vis Ocul Motil
July 2024
Incomitant hypotropia in thyroid eye disease can be difficult to manage, especially in the presence of orthotropia with fusion in down gaze and reading position. Recessing the affected ipsilateral inferior rectus muscle may result in an undesirable downgaze diplopia secondary to a hypertropia in downgaze. Various surgical techniques have been described to manage this potential complication including asymmetric recession of both inferior rectus muscles, posterior myoscleropexy operation, and the Scott recess/resect procedure of the contralateral inferior rectus.
View Article and Find Full Text PDFPrior research has shown PTSD treatment leads to reductions in cardiovascular reactivity during trauma recall, but the extent to which such reductions are associated with changes in PTSD symptoms is less clear. Moreover, such relationships have not been investigated in a cognitively focused PTSD treatment. To examine changes in cardiovascular reactivity to the trauma memory in patients receiving cognitive processing therapy (CPT), CPT with a written trauma account, and a written account only condition.
View Article and Find Full Text PDFBackground: Posttraumatic stress disorder (PTSD) and sleep disturbance are highly comorbid and repetitive negative thinking (RNT) is associated with both sleep disturbance and PTSD. However, few studies have examined the association between RNT and sleep disturbance in individuals exposed to trauma, with and without PTSD.
Method: Associations between trait-level and trauma-related RNT, insomnia, and sleep quality were investigated in a trauma-exposed MTurk ( = 342) sample.
Purpose: To review the published literature assessing the clinical utility of genetic testing in individuals with infantile nystagmus syndrome (INS), defined as binocular conjugate nystagmus and onset prior to 6 months of age, with or without associated findings.
Methods: A literature search was last conducted in October 2022. The results were limited to articles published in English.
Purpose: To review the published literature on the use of levodopa/carbidopa to augment the treatment of amblyopia.
Methods: Literature searches for English language studies were last conducted in October 2022 in the PubMed database with no date restrictions. The combined searches yielded 55 articles, of which 23 were reviewed in full text.
Background: Although significant progress has been made in improving the rate of survival for pediatric optic pathway gliomas (OPGs), data describing the methods of diagnosis and treatment for OPGs are limited in the modern era. This retrospective study aims to provide an epidemiological overview in the pediatric population and an update on eye care resource utilization in OPG patients using big data analysis.
Methods: Using the OptumLabs Data Warehouse, 9-11 million children from 2016 to 2021 assessed the presence of an OPG claim.
Crouzon Syndrome is a genetic craniosynostosis disorder associated with a high risk of ophthalmologic sequelae secondary to structural causes. However, ophthalmologic disorders due to intrinsic nerve aberrations in Crouzon Syndrome have not been described. Optic pathway gliomas (OPGs) are low grade gliomas that are intrinsic to the visual pathway, frequently associated with Neurofibromatosis type 1 (NF-1).
View Article and Find Full Text PDFPurpose: This literature review aims to investigate the potential association between strabismus and mental illness among children.
Materials: The search was conducted in the PubMed and Google Scholar databases using a wide range of search terms related to strabismus, mental disorders, psychiatric illness, childhood, and adolescence.
Results: Eleven published studies were included in this review.
Arch Soc Esp Oftalmol (Engl Ed)
July 2023
Introduction: Recurrent exotropia is common following surgery for monocular large angle constant sensory exotropia. Surgery is usually limited to operations on the affected eye. Simultaneous oblique weakening surgery may enhance the effect of the horizontal rectus muscles surgery by decreasing the abducting forces.
View Article and Find Full Text PDFPTSD is associated with deficits in synthesis of progesterone metabolites such as allopregnanolone and pregnanolone that potently facilitate gamma-amino-butyric acid (GABA) effects at GABA receptors. These neurosteroids modulate neuronal firing rate, regional brain connectivity, and activation of amygdala-mediated autonomic nervous system, hypothalamic-pituitary-adrenal axis, and behavioral reactions to unconditioned and conditioned threat. They also play critical roles in learning and memory processes such as extinction and extinction retention and inhibit toll-like receptor activation of intracellular pro-inflammatory pathways.
View Article and Find Full Text PDFFor patients with a complete, chronic abducens nerve palsy and resulting abduction deficit, a transposition procedure is often the procedure of choice. One such transposition procedure involves transposing the superior rectus (SR) and inferior rectus (IR) laterally without disinserting or splitting either muscle. While effective, this procedure - like many transposition procedures - carries with it the risk of induced torsional or vertical misalignment.
View Article and Find Full Text PDFPurpose: To review the literature on the efficacy of surgical procedures to improve visual acuity (VA) in patients with infantile nystagmus syndrome (INS).
Methods: Literature searches were last conducted in January 2022 in the PubMed database for English-language studies with no date restrictions. The combined searches yielded 354 abstracts, of which 46 were reviewed in full text.