Treatment Overview

A diagnosis of Cortical Dysplasia (CD) means that a small area of the brain’s outer layer, the cortex, developed abnormally before birth. While dysplasia itself is stable, it often creates neurons that are highly susceptible to sudden, abnormal electrical discharges, leading to recurrent seizures (epilepsy). Living with this condition can cause significant stress, particularly because the seizures are often frequent and resistant to standard treatments, impacting independence, safety, and development, especially in children. 

Treatment is critical to minimize the frequency of seizures, reduce the risk of injury, and protect cognitive function and quality of life. The strategy requires a specialized approach, as the success of traditional medication is typically lower than in other forms of epilepsy. The primary goal is achieving seizure freedom, or at least a significant reduction in seizure severity, by calming the overactive neurons in the abnormal brain tissue (American Association of Neurological Surgeons, 2023). 

Overview of treatment options for Cortical Dysplasia 

The treatment approach for epilepsy caused by Cortical Dysplasia is a two-step process. Anti-seizure medications (ASMs) are always the initial strategy, used to try and suppress the electrical activity originating from the dysplastic lesion. 

However, Cortical Dysplasia is one of the most common causes of refractory epilepsy, meaning the seizures persist despite trials of two or more appropriate ASMs. Because of this high likelihood of drug resistance, procedures and surgical interventions play a prominent and essential role. Studies show that when medication fails, surgical removal of the dysplastic tissue offers the highest probability of long-term seizure freedom. 

Medications used for Cortical Dysplasia 

Since the seizures associated with Cortical Dysplasia are typically focal (starting in one small area), the choice of medication often focuses on drugs effective for this seizure type, though broad-spectrum medications are also widely used. 

1. Newer Broad-Spectrum ASMs: These drugs are often used first due to generally favorable side-effect profiles. Examples include levetiracetam and lamotrigine. They are effective at calming the overall irritability of the brain’s electrical signals. 

2. Sodium Channel Blockers: These are specifically useful for focal seizures. Medications like carbamazepine or oxcarbazepine help stabilize nerve impulses arising from the dysplastic area. 

3. Rescue Medications: These are fast-acting benzodiazepines, sometimes administered nasally or rectally, that are used as “rescue therapy” to stop a prolonged seizure (status epilepticus) or a cluster of seizures. 

Patients should anticipate a lengthy process of dose adjustment and drug switching, as finding the combination that works best while minimizing side effects can take time. Complete seizure control using medication alone is achieved in a minority of CD patients, meaning the care team must prepare for the possibility of exploring surgical options. 

How these medications work 

Anti-seizure medications (ASMs) function by stabilizing the highly excitable nerve cells in and around the dysplastic tissue. Seizures occur when there is an abnormal rush of electrical activity in the brain. 

The drug classes work in different ways to prevent this surge. Some drugs block the flow of ions, such as sodium or calcium, into the neurons. By doing so, they reduce the rate at which the nerve cell can fire electrical impulses, acting like a damper on the system. Other medications enhance the function of GABA, which is the brain’s main inhibitory (calming) neurotransmitter. Boosting GABA effectively increases the “brake” on electrical activity, making it harder for the abnormal dysplastic neurons to trigger a widespread seizure. 

Side effects and safety considerations 

Side effects from ASMs are common and often include dizziness, drowsiness, fatigue, or mild cognitive slowing. These usually improve after the body adjusts to the medication. However, more serious reactions can occur. 

Some medications, particularly lamotrigine, require a very slow introduction to prevent a serious, potentially dangerous rash. Patients on certain drugs like carbamazepine may require regular blood tests to monitor levels and check liver function. Because ASMs affect brain chemistry, subtle mood changes, irritability, or increased anxiety should always be reported to the care provider. Patients must seek immediate medical care if they experience a high fever accompanied by a rash or if a seizure lasts longer than five minutes (MedlinePlus, 2022). 

Since everyone’s experience with the condition and its treatments can vary, working closely with a qualified healthcare provider helps ensure safe and effective care. 

References 

  1. American Association of Neurological Surgeons. https://www.aans.org 
  1. MedlinePlus. https://medlineplus.gov 
  1. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov 
  1. Mayo Clinic. https://www.mayoclinic.org 

Medications for Cortical Dysplasia

These are drugs that have been approved by the US Food and Drug Administration (FDA), meaning they have been determined to be safe and effective for use in Cortical Dysplasia.

Found 4 Approved Drugs for Cortical Dysplasia

SIrolimus

Brand Names
Rapamune, Hyftor, Fyarro

SIrolimus

Brand Names
Rapamune, Hyftor, Fyarro
Form: Solution, Injection, Tablet, Gel
Method of administration: Oral, Intravenous, Topical
FDA approval date: July 01, 2001
Classification: mTOR Inhibitor Immunosuppressant
FYARRO ™ is indicated for the treatment of adult patients with locally advanced unresectable or metastatic malignant perivascular epithelioid cell tumor (PEComa). FYARRO is an mTOR inhibitor indicated for the treatment of adult patients with locally advanced unresectable or metastatic malignant perivascular epithelioid cell tumor (PEComa). ( 1 )

Everolimus

Brand Names
Torpenz, Afinitor Disperz, Afinitor, Zortress

Everolimus

Brand Names
Torpenz, Afinitor Disperz, Afinitor, Zortress
Form: Tablet
Method of administration: Oral
FDA approval date: March 31, 2009
Classification: mTOR Inhibitor Immunosuppressant
TORPENZ (everolimus) tablets are a kinase inhibitor indicated for the treatment of: Postmenopausal women with advanced hormone receptor-positive, HER2-negative breast cancer in combination with exemestane after failure of treatment with letrozole or anastrozole.

MethylPREDNISolone

Brand Names
Solu-Medrol MethylPREDNISolone, Solu-Medrol, Medrol

MethylPREDNISolone

Brand Names
Solu-Medrol MethylPREDNISolone, Solu-Medrol, Medrol
Form: Injection, Tablet
Method of administration: Oral, Intravenous, Intramuscular
FDA approval date: October 24, 1957
Classification: Corticosteroid
When oral therapy is not feasible, and the strength, dosage form, and route of administration of the drug reasonably lend the preparation to the treatment of the condition, the intravenous or intramuscular use of Methylprednisolone Sodium Succinate for Injection, USP, is indicated as follows: Allergic states Control of severe or incapacitating allergic conditions intractable to adequate trials of conventional treatment in asthma, atopic dermatitis, contact dermatitis, drug hypersensitivity reactions, perennial or seasonal allergic rhinitis, serum sickness, transfusion reactions. Dermatologic diseases Bullous dermatitis herpetiformis, exfoliative erythroderma, mycosis fungoides, pemphigus, severe erythema multiforme (Stevens-Johnson syndrome). Endocrine disorders Primary or secondary adrenocortical insufficiency (hydrocortisone or cortisone is the drug of choice; synthetic analogs may be used in conjunction with mineralocorticoids where applicable; in infancy, mineralocorticoid supplementation is of particular importance), congenital adrenal hyperplasia, hypercalcemia associated with cancer, nonsuppurative thyroiditis. Gastrointestinal diseases To tide the patient over a critical period of the disease in regional enteritis (systemic therapy) and ulcerative colitis. Hematologic disorders Acquired (autoimmune) hemolytic anemia, congenital (erythroid) hypoplastic anemia (Diamond-Blackfan anemia), idiopathic thrombocytopenic purpura in adults (intravenous administration only; intramuscular administration is contraindicated), pure red cell aplasia, selected cases of secondary thrombocytopenia. Miscellaneous Trichinosis with neurologic or myocardial involvement, tuberculous meningitis with subarachnoid block or impending block when used concurrently with appropriate antituberculous chemotherapy. Neoplastic diseases For the palliative management of leukemias and lymphomas. Nervous System Acute exacerbations of multiple sclerosis; cerebral edema associated with primary or metastatic brain tumor, or craniotomy. Ophthalmic diseases Sympathetic ophthalmia, uveitis and ocular inflammatory conditions unresponsive to topical corticosteroids. Renal diseases To induce diuresis or remission of proteinuria in idiopathic nephrotic syndrome or that due to lupus erythematosus. Respiratory diseases Berylliosis, fulminating or disseminated pulmonary tuberculosis when used concurrently with appropriate antituberculous chemotherapy, idiopathic eosinophilic pneumonias, symptomatic sarcoidosis. Rheumatic disorders As adjunctive therapy for short-term administration (to tide the patient over an acute episode or exacerbation) in acute gouty arthritis; acute rheumatic carditis; ankylosing spondylitis; psoriatic arthritis; rheumatoid arthritis, including juvenile rheumatoid arthritis (selected cases may require low-dose maintenance therapy). For the treatment of dermatomyositis, temporal arteritis, polymyositis, and systemic lupus erythematosus.

Kenalog

Brand Names
Lidolog, Mlk F3, Mlk F1, Pro-C-Dure 6, Bupivilog

Kenalog

Brand Names
Lidolog, Mlk F3, Mlk F1, Pro-C-Dure 6, Bupivilog
Form: Kit
Method of administration: Intra-articular, Epidural, Topical, Intramuscular, Infiltration
FDA approval date: January 16, 2014
Intramuscular Where oral therapy is not feasible, injectable corticosteroid therapy, including triamcinolone acetonide injectable suspension is indicated for intramuscular use as follows: Allergic states: Control of severe or incapacitating allergic conditions intractable to adequate trials of conventional treatment in asthma, atopic dermatitis, contact dermatitis, drug hypersensitivity reactions, perennial or seasonal allergic rhinitis, serum sickness, transfusion reactions. Dermatologic diseases: Bullous dermatitis herpetiformis, exfoliative erythroderma, mycosis fungoides, pemphigus, severe erythema multiforme (Stevens-Johnson syndrome). Endocrine disorders: Primary or secondary adrenocortical insufficiency (hydrocortisone or cortisone is the drug of choice; synthetic analogs may be used in conjunction with mineralocorticoids where applicable; in infancy, mineralocorticoid supplementation is of particular importance), congenital adrenal hyperplasia, hypercalcemia associated with cancer, nonsuppurative thyroiditis. Gastrointestinal diseases: To tide the patient over a critical period of the disease in regional enteritis and ulcerative colitis. Hematologic disorders: Acquired (autoimmune) hemolytic anemia, Diamond-Blackfan anemia, pure red cell aplasia, selected cases of secondary thrombocytopenia. Miscellaneous: Trichinosis with neurologic or myocardial involvement, tuberculous meningitis with subarachnoid block or impending block when used with appropriate antituberculous chemotherapy. Neoplastic diseases: For the palliative management of leukemias and lymphomas. Nervous system: Acute exacerbations of multiple sclerosis; cerebral edema associated with primary or metastatic brain tumor or craniotomy. Ophthalmic diseases: Sympathetic ophthalmia, temporal arteritis, uveitis and ocular inflammatory conditions unresponsive to topical corticosteroids. Renal diseases: To induce diuresis or remission of proteinuria in idiopathic nephrotic syndrome or that due to lupus erythematosus. Respiratory diseases: Berylliosis, fulminating or disseminated pulmonary tuberculosis when used concurrently with appropriate antituberculous chemotherapy, idiopathic eosinophilic pneumonias, symptomatic sarcoidosis. Rheumatic disorders: As adjunctive therapy for short-term administration (to tide the patient over an acute episode or exacerbation) in acute gouty arthritis; acute rheumatic carditis; ankylosing spondylitis; psoriatic arthritis; rheumatoid arthritis, including juvenile rheumatoid arthritis (selected cases may require low-dose maintenance therapy). For the treatment of dermatomyositis, polymyositis and systemic lupus erythematosus. Intra-Articular The intra-articular or soft tissue administration of triamcinolone acetonide injectable suspension is indicated as adjunctive therapy for short-term administration (to tide the patient over an acute episode or exacerbation) in acute gouty arthritis, acute and subacute bursitis, acute nonspecific tenosynovitis, epicondylitis, rheumatoid arthritis, synovitis of osteoarthritis.
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