Cortical dysplasia is a congenital abnormality where the neurons in an area of the brain failed to migrate in the proper formation in utero. Occasionally neuron

Neuron

A neuron is an electrically excitable cell that processes and transmits information by electrical and chemical signaling. Chemical signaling occurs via synapses, specialized connections with other cells. Neurons connect to each other to form networks. Neurons are the core components of the nervous...

s will develop that are larger than normal in certain areas. This causes the signals sent through the neurons in these areas to misfire, which sends an incorrect signal. It is commonly found near the cerebral cortex

Cerebral cortex

The cerebral cortex is a sheet of neural tissue that is outermost to the cerebrum of the mammalian brain. It plays a key role in memory, attention, perceptual awareness, thought, language, and consciousness. It is constituted of up to six horizontal layers, each of which has a different...

 and is associated with seizures. Medication is used to treat the seizures that may arise due to cortical dysplasia. Increasingly, surgery has become a viable treatment option for appropriate candidates.

Focal cortical dysplasia is a common cause of intractable epilepsy

Epilepsy

Epilepsy is a common chronic neurological disorder characterized by seizures. These seizures are transient signs and/or symptoms of abnormal, excessive or hypersynchronous neuronal activity in the brain.About 50 million people worldwide have epilepsy, and nearly two out of every three new cases...

 in children and is a frequent cause of epilepsy in adults. All forms of focal cortical dysplasia lead to disorganization of the normal structure of the cerebral cortex. Focal cortical dysplasia associated with enlarged cells is known as FCDIIB. The enlarged cells are called balloon cells for their large elliptical shape, displaced nucleus, and lack of dendrite

Dendrite

Dendrites are the branched projections of a neuron that act to conduct the electrochemical stimulation received from other neural cells to the cell body, or soma, of the neuron from which the dendrites project...

s or axon

Axon

An axon is a long, slender projection of a nerve cell, or neuron, that conducts electrical impulses away from the neuron's cell body or soma....

s. The developmental origin of balloon cells is unknown although they are believed to be derived from neuronal or glial progenitor cells. Balloon cells are similar in structure to giant cells in the disorder tuberous sclerosis complex. Other large cells known as cytomegalic neurons or dysplastic neurons exhibit an enlarged cell body but clear axons and dendrites. It is widely hypothesized that balloon cells and dysplastic neurons contribute to seizures in patients with cortical dysplasia.

Treatment

No specific treatment is required for cortical dysplasia, and all treatment is aimed at the resulting symptoms (typically seizures). When a cortical dysplasia is a cause of epilepsy, then seizure medications (anticonvulsants) are a first line treatment. If anticonvulsants fail to control seizure activity, neurosurgery may be an option to remove or disconnect the abnormal cells from the rest of the brain (depending on where the cortical dysplasia is located and the safety of the surgery relative to continued seizures). Neurosurgery can range from removing an entire hemisphere (hemispherectomy

Hemispherectomy

Hemispherectomy is a very rare surgical procedure where one cerebral hemisphere is removed or disabled. This procedure is used to treat a variety of seizure disorders where the source of the epilepsy is localized to a broad area of a single hemisphere of the brain...

), a small lesionectemy, or multiple transections to try and disconnect the abnormal tissue from the rest of the brain (multiple subpial transections). Physical therapy

Physical therapy

Physical therapy , often abbreviated PT, is a health care profession. Physical therapy is concerned with identifying and maximizing quality of life and movement potential within the spheres of promotion, prevention, diagnosis, treatment/intervention,and rehabilitation...

 should be considered for infants and children with muscle weakness. Educational therapy

Educational Therapy

Educational Therapy is a form of therapy used to treat individuals with learning differences, disabilities, and challenges. This form of therapy offers a wide range of intensive interventions that are designed to remediate learning problems. These interventions are individualized and unique to the...

 is often prescribed for those with developmental delays, but there is no complete treatment for the delays.

Foundations

The Hemispherectomy Foundation

Hemispherectomy Foundation

The Hemispherectomy Foundation is a 501 non-profit organization founded to provide a support structure for children, and the families of children who have had or are preparing to have hemispherectomy brain surgery. The foundation also provides scholarships for college, trade schools, and summer...

was formed in 2008 to assist families with children who have Cortical Dysplasia and other conditions that require hemispherectomy.