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Agenesis of the Corpus Callosum

Description

There are a group of disorders of the corpus callosum. These are birth defects in which there are problems with the connection between the left and the right side of the brain (termed the corpus callosum).

  • Agenesis: partial or complete absence of the corpus callosum
  • Dysgenesis: malformation of the corpus callosum
  • Hypogenesis: a partial formation of the corpus callosum
  • Hypoplasia: underdevelopment of the corpus callosum

This can occur by itself or in combination with other brain formation problems or syndromes.

Causes

For many children, we cannot identify a single cause. In these cases, there are likely multiple. These factors may include genetics, metabolic disorders, and structural interruptions. 

Known causes of disorders of the corpus callosum include

  • Chromosomal defects that affect fetal brain development
  • Certain viral infections that a mother has while pregnant
  • Exposure of the unborn baby to certain toxins (like alcohol) or medications
  • Abnormal brain development caused by cysts

Diagnosis 

Diagnosis is made by viewing the brain. This is with MRI, CT scan, or prenatal or post-natal ultrasound. 

Associated Developmental and Learning Issues

Children with disorders of the corpus callosum have a range of learning abilities and disabilities. The level of difficulty depends on the number of brain structure problems and the presence of other syndromes. Some children have mild learning problems. Others have severe intellectual disabilities.

Other developmental skills and abilities often go along with cognitive abilities. The more brain structure problems a child has, the more likely they are to have slow development of speech, motor skills, and social skills. Some children who learn well may have more “hidden” disabilities related to how they learn. For these children, specialized evaluation of their learning strengths and weaknesses will be important to support their success. 

For children with primary disorders of the corpus callosum (without other syndromes or brain formation problems), it is common to experience the following:

  • Cognitive disabilities including learning problems through intellectual disabilities
  • Problems with abstract thinking and problem solving
  • Low muscle tone and poor motor coordination
  • Attention Deficit Disorder
  • Social immaturity

Associated Medical Conditions

Many children with Disorders of the corpus callosum have other health complications. It is important that these are recognized and treated. A child with disorders of the corpus callosum who is physically healthy is more likely to do well in school and in other treatments. This contributes to more independence, better daily functioning, and better quality of life. The type and severity of health problems is often dependent upon whether the disorder of the corpus callosum is part of syndrome or something that occurred by itself. If the child has a syndrome, it is important to understand the health issues associated with that syndrome. 

  • Acrocallosal syndrome
  • Aicardi syndrome
  • Arnold-Chiari malformation
  • Andermann syndrome
  • Dandy-Walker syndrome
  • Differences in the appearance of the face, including wide space between the eyes, and hypertelorism (differences in appearance of the skin around the eyes)
  • Fetal Alcohol syndrome
  • Hearing problems
  • Holoprosencephaly (failure of the forebrain to divide into lobe)
  • Hydrocephalus
  • Microcephaly (small head)
  • Midline facial defects
  • Poor muscle tone and coordination
  • Schizencephaly (clefts or deep divisions in brain tissue)
  • Seizures
  • Shapiro syndrome
  • Trisomy 13
  • Trisomy 18
  • Vision problems

Associated Behavioral Conditions

  • ADHD
  • Insomnia and other sleep problems

Online Medical Reviewers:

Related Services

  • Behavior Treatment Services - Provides assessment and short-term treatment for children and teens with developmental delay or disability and challenging behaviors.
  • Community Consultation Program - Provides technical assistance, training, and continuing education to schools, community and state agencies that provide services to children with learning and behavioral challenges.
  • Crisis Intervention Program - Provides services to individuals with a developmental or intellectual disability living in Monroe County with significant behavioral difficulties.
  • Pediatric Feeding Disorders Program - Provides assessment and treatment for children who have difficulty eating related to food selectivity, food refusal, and disruptive mealtime behavior.

URMC Collaborations

  • Child Neurology - Assures children have the evaluation and treatment they need for seizures or other neurologic issues.
  • Dental Department - Helps children maintain good oral health.
  • Pediatric Gastroenterology - Evaluates and offers suggestions for treatment for problems with slow bowel motility, constipation, or gastroesophageal reflux (GER).
  • Pediatric Neurosurgery - Provides care for children with CP who have problems with too much fluid in the brain or require a shunt to drain fluid (ventriculoperitoneal (VP) shunt). Dr. Howard Silberstein is the pediatric and adult neurosurgeon who provides care for both children and adults with cerebral palsy when needed.
  • Pediatric Orthopaedics - Monitors and treats bone and muscle issues.
  • Pediatric Sleep Medicine Service - Evaluates and treats sleep problems.
  • Physical Medicine and Rehabilitation - Provides outpatient evaluation and intense and targeted therapy programs for children and teens.
  • Speech Pathology Department - Meets the needs of children who have difficulties with speech, communication, oral-motor control, and feeding/swallowing.

Resources

You can find resources for agenesis of the corpus callosum in our Resource Directory!