Neurocutaneous syndrome

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Neurocutaneous syndrome (pronounced: noor-oh-kyoo-TAY-nee-uhs SIN-drohm) is a broad term for a group of neurological disorders that cause tumors to grow inside the nervous system. These tumors can be benign or malignant. Neurocutaneous syndromes are typically inherited and involve the skin in some way.

Etymology

The term "Neurocutaneous" is derived from the Greek words "neuron" meaning nerve, and "cutaneous" from the Latin "cutis" meaning skin. The term "syndrome" is from the Greek "syndrome," which means "concurrence of symptoms," or "running together of things."

Types of Neurocutaneous Syndromes

There are several types of neurocutaneous syndromes, including:

  • Neurofibromatosis: This is the most common type of neurocutaneous syndrome. It causes tumors to grow on nerves and produces other abnormalities such as skin changes and bone deformities.
  • Tuberous sclerosis: This condition causes noncancerous tumors to grow in the brain and other vital organs.
  • Sturge-Weber syndrome: This is a neurological disorder indicated at birth by a port-wine stain birthmark on the forehead and upper eyelid of one side of the face.
  • Von Hippel-Lindau disease: This is a rare, genetic multi-system disorder characterized by the abnormal growth of tumors in certain parts of the body.

Symptoms

Symptoms of neurocutaneous syndromes can vary widely in severity and can include seizures, developmental delay, intellectual disability, and skin abnormalities.

Diagnosis and Treatment

Diagnosis of neurocutaneous syndromes often involves a thorough medical history and physical examination, including a detailed skin examination. Treatment is usually focused on managing symptoms and preventing complications, and can include medications, surgery, and physical therapy.

See Also

External links

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