Subcortical laminar heterotopia

Subcortical Laminar Heterotopia
Synonyms	Band Heterotopia
Pronounce	N/A
Specialty	N/A
Symptoms	Seizures, Developmental delay
Complications	Intellectual disability
Onset	Congenital
Duration	Lifelong
Types	N/A
Causes	Genetic mutation
Risks	Family history
Diagnosis	MRI, Genetic testing
Differential diagnosis	N/A
Prevention	N/A
Treatment	Anticonvulsants, Supportive care
Medication	N/A
Prognosis	N/A
Frequency	Rare
Deaths	N/A

Subcortical Laminar Heterotopia (SLH), also known as Band Heterotopia, is a rare neurological disorder characterized by the presence of bands of gray matter located beneath the cerebral cortex. This condition is a type of neuronal migration disorder, where neurons fail to migrate to their proper position during brain development.

Signs and Symptoms

Individuals with subcortical laminar heterotopia often present with seizures and varying degrees of developmental delay. The severity of symptoms can range from mild to severe, depending on the extent of the heterotopia. Some individuals may also experience intellectual disability.

Causes

Subcortical laminar heterotopia is primarily caused by mutations in the DCX gene or the LIS1 gene, which are involved in neuronal migration. These genetic mutations can be inherited in an X-linked dominant pattern, particularly affecting females, or can occur de novo.

Diagnosis

Diagnosis of subcortical laminar heterotopia is typically made through magnetic resonance imaging (MRI), which reveals the characteristic bands of gray matter. Genetic testing can confirm mutations in the DCX or LIS1 genes.

Treatment

There is no cure for subcortical laminar heterotopia, but treatment focuses on managing symptoms. Anticonvulsants are commonly used to control seizures. Supportive care, including physical therapy, occupational therapy, and speech therapy, can help improve quality of life.

Epidemiology

Subcortical laminar heterotopia is a rare condition, with a higher prevalence in females due to its X-linked inheritance pattern. The exact frequency is unknown, but it is considered a rare disorder.

See Also

• Neuronal migration disorder
• Gray matter heterotopia
• Lissencephaly

External Links

• [Genetics Home Reference on Subcortical Laminar Heterotopia](https://ghr.nlm.nih.gov/condition/subcortical-laminar-heterotopia)
• [National Organization for Rare Disorders (NORD) on Band Heterotopia](https://rarediseases.org/rare-diseases/band-heterotopia/)