Related Experiment Video

Updated: May 30, 2026

Evaluation of Hemisphere Lateralization with Bilateral Local Field Potential Recording in Secondary Motor Cortex of Mice
07:03

Evaluation of Hemisphere Lateralization with Bilateral Local Field Potential Recording in Secondary Motor Cortex of Mice

Published on: July 31, 2019

Laterality of brain and ocular lesions in Aicardi syndrome

Michelle T Cabrera1, Bryan J Winn, Travis Porco

  • 1Department of Ophthalmology, University of California, San Francisco, California, USA. mimitcabrera@gmail.com

Pediatric Neurology
|August 10, 2011
PubMed

Insights

This study introduces a new scoring system for Aicardi syndrome, detailing ocular and brain lesion severity. Findings reveal significant brain lesion asymmetry, correlating with microphthalmos in affected children.

Area of Science:

  • Pediatric Neurology
  • Ophthalmology
  • Medical Imaging

Background:

  • Aicardi syndrome is a rare genetic disorder affecting primarily females, characterized by a triad of agenesis of the corpus callosum, infantile spasms, and retinal abnormalities.
  • Previous studies on Aicardi syndrome have been limited by small sample sizes, hindering comprehensive analysis of lesion laterality and severity.

Purpose of the Study:

  • To establish a novel severity scoring system for ocular and brain lesions in Aicardi syndrome.
  • To assess the sidedness and severity of ocular and brain lesions using magnetic resonance imaging (MRI).
  • To investigate correlations between lesion laterality and specific ophthalmologic findings.

Main Methods:

  • A cohort of 26 female patients diagnosed with Aicardi syndrome was analyzed.
  • Dilated ophthalmologic examinations and brain MRI scans were performed for all participants.
  • New scoring systems were devised to quantify the severity of ocular and brain MRI lesions, focusing on asymmetry.

Main Results:

  • Ocular abnormalities included optic nerve coloboma (42%), severe lacunae (36%), and microphthalmos (27%).
  • Brain MRI revealed significant asymmetry in lesion distribution, with 58% of subjects showing side-predominant lesions, more frequently on the right.
  • A significant correlation was observed between the sidedness of brain disease and the presence of microphthalmos (P=0.02).

Conclusions:

  • The developed scoring system effectively quantifies Aicardi syndrome-associated ocular and brain lesions.
  • Brain lesion asymmetry is a common feature in Aicardi syndrome, with a notable right-sided predilection.
  • The findings support a link between neurological and ophthalmological manifestations, underscoring the importance of comprehensive assessment in Aicardi syndrome.

More Related Videos

Symmetric Bihemispheric Postmortem Brain Cutting to Study Healthy and Pathological Brain Conditions in Humans08:29

Symmetric Bihemispheric Postmortem Brain Cutting to Study Healthy and Pathological Brain Conditions in Humans

Published on: December 18, 2016

Related Concept Videos

Lateralization01:28

Lateralization

Brain lateralization refers to the division of mental processes and functions between the two hemispheres of the brain, a phenomenon that optimizes neural efficiency and underpins complex abilities in humans. This specialization allows each hemisphere to perform tasks where it has a comparative advantage, facilitating more refined cognitive capabilities across different domains.
Visual Agnosia01:12

Visual Agnosia

Visual agnosia is a condition characterized by the inability to recognize visually presented objects despite having normal vision. For instance, a person with visual agnosia can describe the shape and color of an object but cannot identify or name it. This impairment does not affect their visual field, acuity, color vision, brightness discrimination, language, or memory. An example of this condition in a social setting is someone at a dinner party asking for "that silver thing with a round end"...
Aneurysm II: Clinical Manifestations and Diagnostic Studies01:21

Aneurysm II: Clinical Manifestations and Diagnostic Studies

Thoracic, aortic arch and abdominal aneurysms are significant vascular conditions that can present with various clinical manifestations and lead to serious complications. Understanding these manifestations and the appropriate diagnostic studies is essential for effective management and treatment.Thoracic Aortic AneurysmsThoracic aortic aneurysms often remain asymptomatic until they reach a size that impinges on adjacent structures. They typically cause deep, diffuse chest pain that radiates to...
Secondary Spinal Cord Injury llI: Pathophysiology01:25

Secondary Spinal Cord Injury llI: Pathophysiology

Early Ischemia and Ionic ImbalanceWithin minutes of spinal cord injury, a secondary cascade begins, progressing over hours to weeks. Vascular damage reduces blood flow, causing ischemia and mitochondrial dysfunction. ATP depletion leads to ion pump failure, membrane depolarization, sodium influx, potassium efflux, and water accumulation, resulting in cellular swelling. Increased intracellular calcium further disrupts mitochondria and accelerates cellular injury.Excitotoxicity and Neuronal...
Cerebral Edema ll: Pathophysiology01:22

Cerebral Edema ll: Pathophysiology

Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...
Hemorrhagic Stroke ll: Pathophysiology01:29

Hemorrhagic Stroke ll: Pathophysiology

A hemorrhagic stroke develops when a cerebral blood vessel ruptures, allowing blood to escape into the surrounding brain tissue, as in intracerebral hemorrhage (ICH), or into the subarachnoid space, as in subarachnoid hemorrhage (SAH). Because the skull is a rigid compartment, the sudden presence of extravascular blood rapidly increases intracranial pressure and compresses adjacent neural structures, leading to immediate tissue injury and impaired cerebral perfusion.Mass Effect and Primary...