Patient Education

What is Agenesis of the corpus callosum (ACC)?

Agenesis of the corpus callosum is complete or partial absence of the structure that connects the two hemispheres of the brain - the corpus callosum, which is the largest fibre tract in the central nervous system.It can occur as an isolated condition or in combination with other cerebral abnormalities. These include:

• Arnold-Chiari malformation.
• Dandy-Walker syndrome.
• Andermann's syndrome.
• Schizencephaly (clefts or deep divisions in brain tissue).
• Holoprosencephaly (failure of the forebrain to divide into lobes).

It is one of the most frequent malformations in the brain with a reported incidence ranging between 0.5 and 70 in 10,000 births.

What are signs and symptoms?

There is a wide spectrum of clinical presentation.Those presentations most common to most children with callosal abnormalities include.

• Vision impairment.
• Poor motor co-ordination.
• Delays in motor milestones.
• Low perception of pain.
• Delayed toilet training.
• Early feeding/chewing and swallowing difficulties.
• Seizures.
• Spasticity.

What investigations my doctor will do?

Structural brain abnormalities are diagnosed by MRI or CT scan, prenatal ultrasound, or prenatal MRI.

What is treatment for ACC?

There are no specific treatments for ACC but most patients will require input from a multidisciplinary team. This may include neurologists, neuropsychologists, occupational therapists, physiotherapists, speech and language therapists, pediatricians, geneticists, specific schooling support, and early intervention specialists.

What is Prognosis for ACC?

Prognosis depends on the extent and severity of malformations. It is not possible for the corpus callosum to regenerate or degenerate.

Although some individuals with callosal disorders have average intelligence and lead normal lives, neuropsychological testing reveals subtle differences in higher cortical function compared with individuals of the same age and education without ACC.