Neurocutaneous Diseases Flashcards
(11 cards)
What are Neurocutaneous Disorders?
Neurologic conditions that affect the brain, spine and peripheral nerves with manifestations on the skin
What is Tuberous Sclerosis?
Neurological abnormalities
Skin lesions
Retinal Lesions
Cardiac Rhabdomyomas
What neurologic abnormalities are seen in Tuberous Sclerosis?
Seizures, progressive psychomotor retardation and slowly progressive mental deterioration
What skin changes are seen in Tuberous Sclerosis?
Adenoma Sebaceum: reddened facial nodules
Shagreen Patches: leathery plaques on the trunk
Ash leaf (hypopigmented) patches
What is the strategy for managing Tuberous Sclerosis?
Control Seizures
What is Neurofibromatosis 1 (Von recklinhausen disease)?
Neurofibromas: soft flesh colored lesions attached to peripheral nerves
8th CN tumors
Cutaneous hyperpigmented lesions: Cafe au lait spots
Lisch nodules
Meningioma and Gliomas
What can be done in NF1 to help preserve hearing?
Surgical decompression of CN 8 lesions
What is Neurofibromatosis 2?
Juvenile cataracts: 2 eyes
Bilateral Schwannomas: 2 ears
Meningioma and Ependymoma: 2 brain tumors
What is Sturge-Weber Syndrome?
Port-wine stain of the face
seizures
CNS: homonymous hemianopsia, hemiparesis and mental subnormality
What is seen on Skull X-ray in Sturge-Weber Syndrome?
“Tram-track” Calcification of Angiomas
How do you treat Sturge-weber syndrome?
Control Seizures
