Flashcards in Peds III Deck (60)
What is cerebral palsy?
Static motor encephalopathy due to an insult to the developing brain in the first 3 years of life
True or false: kids with cerebral palsy usually have other neurologic problems, like seizures, developmental delay, cognitive delay, etc.
What is the usual presentation of cerebral palsy?
Peripheral hypertonia and axial hypotonia
What are the four classifications of cerebral palsy?
What is the spastic hemiplegia form of cerebral palsy? Which limbs are usually involved? What causes it?
One side of the body is affected, and usually the arm more than the leg.
Cause by a unilateral perinatal stroke
What is the spastic diplegic form of cerebral palsy? What causes it?
Both legs are only affected. Usually seen in premature infants with periventricular leukomalacia
What is the spastic quadriplegia form of cerebral palsy?
All extremities are affected
What is the dyskinetic form of cerebral palsy? What causes it?
What are the skin problems that can occur with CP kids?
What are the GI problems that can occur with CP kids?
What are the orthopedic problems that can occur with CP kids?
What are the respiratory problems that can occur with CP kids?
Chronic lung disease
What are the neurological problems that can occur with CP kids?
Vision and hearing deficits
What is the treatment for CP kids?
Individualized multidisciplinary team
What is the incidence of migraines in kids?
What gender is usually affected more with migraines in childhood/adolescence?
What are the characteristics of migraines?
Recurrent headaches accompanied by n/v and photophobia
What aggravates and alleviates the HA with migraines?
-Physical activity worsens
-Sleep cures it
Are migraines usually bilateral or unilateral in children?
Bilateral, but progresses unilaterally as they grow older
What is the most common type of migraine in children?
Migraine without aura
What are the complicated type of migraines? (4)
-Basilar migraine (vertigo)
-Ophthalmoplegic migraine (oculomotor palsy)
What should always be obtained with complicated migraines in children?
What is the treatment for migraines in children?
OTC NSAIDs, or triptans if severe
What is the antiemetic medication of choice for migraines in children?
What is the basilar form of migraine?
Migraine accompanied by vertigo,diplopia, nystagmus and/or dysarthria
What is the prophylactic treatment for migraines? (lifestyle + drug)
What are the side effects of cyproheptadine? (2)
Increased appetite and somnolence
Who usually gets topiramate for their migraines?
Obese children and children with epilepsy
What are the side effects of topiramate? (3)
What are the side effects of amitriptyline?
What should be started prior to prescribing amitriptyline for migraine headaches? Why?
Can cause QT prolongation
What is the role of propranolol in treating migraines in kids?
Contraindicated in asthma
What are neurocutaneous syndromes?
Group of neurological disorders with skin lesions
What are the three most common neurocutaneous syndromes?
-Sturge Weber syndrome
What is the inheritance pattern and cause of Neurofibromatosis?
-Defect in neurofibromin protein, which normally negatively regulates Ras oncogene signal transduction
What are the 7 diagnostic criteria for NF1?
1. 6+ cafe au lait spots
2. axillary or inguinal freckling
3. 2 neurofibromas or 1 complex neurofibroma
4. Optic glioma
5. 2 lisch nodules
6. Sphenoid dysplasia
7. first degree relative with NF1
How many cafe au lait spots are needed to diagnose NF1? how big should they be pre and post puberty?
6 or more
More than 5 mm pre
more than 15 mm post
How many axillary/inguinal freckling are needed to diagnose NF1?
2 or more
How many neurofibromas are needed to diagnose NF1?
2 neurofibromas or 1 complex neurofibroma
What is the optic finding that is needed to diagnose NF1?
How many lisch nodules are needed to diagnose NF1?
What are the bony abnormalities that are diagnostic for NF1? (2)
-Long bone abnormalities
How many first degree relatives are needed to diagnose NF1?
1 first degree
How many criteria are needed (of the 7) to diagnose NF1?
2 of the 7
What is the inheritance pattern of tuberous sclerosis?
AD, but most patients have de novo mutations
What is the genetic defect in tuberous sclerosis? What renal pathology can this cause?
TSC is caused by a mutation of either of two genes, TSC1 and TSC2, which code for the proteins hamartin and tuberin, respectively. These proteins act as tumor growth suppressors, agents that regulate cell proliferation and differentiation
What are the skin manifestations of tuberous sclerosis? (2)
-Hypomelanotic macules (ash leaf)
-facial angiofibromas (like facial acne)
What are the CNS findings of tuberous sclerosis? (3)
-Giant cell astrocytomas
What are the eye findings of tuberous sclerosis?
What are the heart findings of tuberous sclerosis?
What are the lungs findings of tuberous sclerosis? When does this usually present?
Presents in adulthood
What is Lymphangioleiomyomatosis (LAM)?
A rare, progressive, systemic disease that typically results in cystic lung destruction and predominantly affects women, especially during childbearing years
What is the prognosis for kids with cardiac rhabdomyomas 2/2 tuberous sclerosis?
What are the cognitive outcomes of tuberous sclerosis?
Depends on the severity of the seizure disorder--infantile spasms may be associated with worse cognitive prognosis
What is the inheritance pattern of sturge-Weber syndrome?
What is sturge weber syndrome?
Non-inherited disorder characterized by development of angiomas in the brain, skin, and eyes
What is the usual presentation of sturge-weber syndrome? What age?
Port wine stain affecting V1 and V2 unilaterally
Usually obvious at birth
What are the CNS findings of sturge-weber syndrome? (4)
-Cognitive delay of various degrees
What are the eye manifestations of Sturge-Weber syndrome?