Overview
Isolated focal cortical dysplasia type Ic (FCD type Ic) is a rare malformation of cortical development affecting the brain, specifically the cerebral cortex. It is classified under the International League Against Epilepsy (ILAE) classification of focal cortical dysplasias. FCD type Ic is characterized by abnormal cortical layering (architectural abnormalities) that involves both radial and tangential disruption of cortical lamination, distinguishing it from FCD type Ia (radial dyslamination only) and type Ib (tangential dyslamination only). Unlike FCD types II and III, type Ic does not feature dysmorphic neurons, balloon cells, or association with other principal lesions. The primary clinical manifestation of isolated FCD type Ic is drug-resistant (refractory) epilepsy, which typically presents with focal seizures that may vary in semiology depending on the location of the dysplastic cortex. Seizures may begin in childhood or later, and patients may also experience developmental delay or cognitive difficulties depending on the extent and location of the lesion. Neuroimaging with MRI may show subtle cortical abnormalities, though FCD type I lesions are often difficult to detect on standard imaging, making diagnosis challenging. Treatment primarily involves antiepileptic (antiseizure) medications, although many patients with FCD type Ic have pharmacoresistant epilepsy. In such cases, surgical resection of the dysplastic cortex may be considered and can lead to seizure freedom or significant seizure reduction in selected patients. However, surgical outcomes for FCD type I are generally less favorable compared to FCD type II, partly due to the difficulty in precisely delineating the lesion boundaries. Comprehensive presurgical evaluation including video-EEG monitoring, advanced neuroimaging, and sometimes invasive EEG monitoring is essential for surgical planning.
Also known as:
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Isolated focal cortical dysplasia type Ic.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
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Common questions about Isolated focal cortical dysplasia type Ic
What is Isolated focal cortical dysplasia type Ic?
Isolated focal cortical dysplasia type Ic (FCD type Ic) is a rare malformation of cortical development affecting the brain, specifically the cerebral cortex. It is classified under the International League Against Epilepsy (ILAE) classification of focal cortical dysplasias. FCD type Ic is characterized by abnormal cortical layering (architectural abnormalities) that involves both radial and tangential disruption of cortical lamination, distinguishing it from FCD type Ia (radial dyslamination only) and type Ib (tangential dyslamination only). Unlike FCD types II and III, type Ic does not featur
How is Isolated focal cortical dysplasia type Ic inherited?
Isolated focal cortical dysplasia type Ic follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.