Module 4

Question 1

Age: days - 2 months

Clinical symptoms: Healthy nb with apnea, generalized or focal tonic, or clonic seizures, and occur multiple time/day.
Positive family history of neonatal seizure

Cause: genetic mutations

Prognosis: Remit

Suggested tx: Phenobarbital levetiracetam

Answer 1

Benign Familial Neonatal Convulsions

Question 2

Age: 5th DOL

Clinical symptoms: Healthy nb with generalized or focal tonic or clonic seizures and/or apnea

Cause: unknown

Prognosis: remit

Suggested
tx: Phenobarbital
levetiracetam

Answer 2

Benign Idiopathic Neonatal Sz

Question 3

Age: 5-10 years

Clinical symptoms: Multiple staring episodes. Neurologically normal

Cause: Genetically influenced

Prognosis: excellent remission rate

Suggested tx: ethosuximide

Answer 3

Childhood Absence Epilepsy

Question 4

Age: puberty

Clinical symptoms: Similar to CAE. Absence seizure less frequent and up to 80% have generalized tonic-clonic seizures. Myotonic seizures can be seen

Cause: unknown

Prognosis: persists to adulthood

Suggested tx: ethosuximide

Answer 4

Juvenile Absence Epilepsy (JAE)

Question 5

Age: Adolescence

Clinical symptoms: Initially myoclonic jerks involving the shoulder and arms. Occur on awakening. Can result from fatigue or stress. Can have tonic clonic seizures

Cause: genetics

Prognosis: lifelong

Suggested tx:
Valproate
Lamotrigine
levetiracetam

Answer 5

Juvenile Myoclonic Epilepsy (JME)

Question 6

Age: 3-13 years

Clinical symptoms: Healthy child with focal seizers during sleep that secondarily generalize

Cause: probably genetics

Prognosis: remit in later teens

Suggested tx:
Levetiracetam
oxcarbazepine

Answer 6

Benign childhood epilepsy with centrotemporal spikes (benign rolandic epilepsy)

Question 7

Benign childhood epilepsy with occipital spikes
Age
Clinical symptoms
Cause
Prognosis
Suggested tx

Question 8

Age 7-11 yo

Clinical symptoms: Usually occur I sleep or during nap. Begin with nausea, retching, or vomiting. If a seizure occurs during wakefulness accompanied by behavioral changes. Autonomic features can occur

Cause: probably genetic

Prognosis: remit in later teens

Suggested tx:
Levetiracetam
oxcarbazepine

Answer 8

Benign Occipital epilepsy

Question 9

Age: 4-7 mo

Clinical symptoms: Most common catastrophic epilepsy in children. Infantile spasms, hypsarrhymia on EEG, psychomotor retardation.

Cause: Structural abnormalities and brain injury

Prognosis: Depends of the underlying brain disorder

Suggested tx: ACTH
Vigabatrin

Answer 9

West Syndrome

Question 10

Age: 1-8 years

Clinical symptoms: Catastrophic epilepsy. Intractable mixed seizures, cognitive impairment that deteriorates, slow spike-and-wave EEG. Infantile spasms have previously occurred in up to 30%.

Cause: unknown

Prognosis: Seizures difficult to control and prognosis is poor

Suggested tx: varies

Answer 10

Lennox-Gastaut Syndrome

Question 11

Age: 3mo-5 years

Clinical symptoms: Most common seizure disorder in childhood

Cause: + family history

Prognosis: no long term consequesnces
Suggested tx: treat underlying cause

Answer 11

Febrile Seizures

Question 12

Nutrition for CP

Answer 12

-Children with athetosis may need as much as 50% to 100% more calories to support their constant writhing movements.

• Children with spasticity on the other hand may need fewer calories because of their decreased movements.
• Occasionally oral-motor coordination problems are so severe that a GT is needed, sometimes with fundoplication to prevent reflux and aspiration.
• Feeding clinics are often helpful as feeding therapy, modified positioning during feedings and special feeding devices can help.

Question 13

HA: Indication for prophylactic therapy

Answer 13

is considered when migraines cause a child to miss school regularly and/or when the child suffers severe migraine headaches 2-4 times a month or tension or migraine 3-4 times a week with a clear sense of functional disability.

Question 14

HA: Indication for abortive therapy

Answer 14

abortive meds should be taken at the onset of the headache and in the prescribed dosage and should be available at home, school or work. Importantly, the overuse of analgesics is to be avoided (more than 3 doses per week).

Question 15

Abortive HA medication

Answer 15

First line: Tylenol/Motrin/Naproxen/
Ondansetron (for nausea)
Migraine: Sumatriptan (>12yo) nasal spray or SQ; do not use in basilar-type and hemiplegic migraine or with CV disease, uncontrolled HTN, or if had MAOI in last 2 weeks; use caution in pts with migrain with aura

Question 16

Amitriptyline (migraine use)

Answer 16

Antidepressant, used off label
most commonly used, caution with kids <12;
order EKG if dose >25mg/day
AE: somnolence, dry mouth, dysrhythmia

Question 17

Divalproex sodium (migraine)

Answer 17

Anticonvulsant
AE: weight gain, heartburn, hair loss, dizziness

Question 18

Topiramate (migraine)

Answer 18

anticonvulsant
AE: weight loss, dizziness, irritability, monitor for change in school/cognitive performance

Question 19

cyproheptadine (migraines)

Answer 19

effective in ages 3-12
AE: weight gain, appetite stimulation, sedation (in doses higher than 4-8mg)

Question 20

Propranol (migraine)

Answer 20

may take several weeks-months to be effective
Do not use in children with h/o asthma
use caution in those with depression
AE: low BP, depressive effects, exercise induced asthma

Question 21

Migraines - Indications for neuro-imaging

Answer 21

imaging studies are rarely indicated unless the history suggests intracranial pressure; there is a sudden onset, increased severity or change in headache pattern; the neurologic exam is abnormal or when a complaint of dizziness is accompanied by double vision, a sensation of whirling or confusion.
Neuroimaging must be considered in children whose headaches do not meet specific criteria for a primary headaches syndrome or who have an abnormal physical examination. Neuroimaging should be obtained within a reasonable time period (2-4 weeks) to avoid progression of symptoms or a delay in treatment if an abnormality is found.

Question 22

Arnold Chiari - Type 1

Answer 22

involves the downward elongation (herniation) of the caudal end of the cerebellar vermis through the foramen magnum with frequency of about 0.61% and a female to male ratio 3:1. Can cause headache, neck pain, atrophy and decreased reflexes in the lower extremities, sensory losses and scoliosis.

Question 23

Arnold Chiari Type II

Answer 23

is present in 0.5 to 1 per 1000 children with spina bifida myelomeningocele. The herniation can lead to brainstem and upper cervical cord compression that may ultimately causes necrosis of both structures. Involves same herniation as type I plus an alteration in the shape and development of the medulla. Further symptoms of type II may include hydrocephaly, respiratory distress, syncope, poor feeding, vomiting, dysphagia, tongue paralysis, and cardiopulmonary failure.

Question 24

Bell’s Balsy Mangement

Answer 24

If eyelid closure is incomplete, prescribe methylcellulose eye drops or ocular lubricant to the affected eye several times daily and patch the eye if the child plays outdoors, during active play and when sleeping.

Steroids should be used in newly diagnosed patients (oral prednisone 1mg/kg/day for 1 week then tapered for 1 week; starting within the first 3-5 days).

Question 25

Tethered Cord Assessment and recognition

Answer 25

The caudal end is fixed by a ropelike filum terminale at or below the L2 level. This can cause abnormal stretching and damage to nerve cells, fibers, and blood vessels. Not all tethering leads to clinical symptoms. If symptoms do occur, they manifest as functional deficits to nerves that emanate from the area of the cauda equina. In children, symptoms may include lesions, hairy patches, dimples, or fatty tumors on the lower back, foot and spinal deformities, weakness in the legs, low back pain, scoliosis, and incontinence. Tethered spinal cord syndrome may go undiagnosed until adulthood when pain, sensory and motor problems and loss of bowel and bladder control emerge. Skin changes often seen later such as dimples above the gluteal cleft or within the cleft (dimples at the coccyx are generally benign), spinal hair tufts, a deviated gluteal fold, spinal fatty deposits, midline birthmarks and sacral sinuses or tracts. MRI of the spine is the gold standard for viewing the parenchymal anatomy.

Question 26

FAS clinical findings

Answer 26

Common clinical findings include poor prenatal and postnatal growth, hypotonia, poor coordination, cardiac defects (VSD, ASD), narrow eyes, microphthalmia, large epicanthal folds, microcephaly, small upper jaw, smooth groove in upper lip, thin upper lip and simian creases common. Neurodevelopmental cues include delayed development in 3 or more areas: cognitive, speech, motor, psychosocial.

Question 27

Achondroplasia

Answer 27

- monitor for growth and hydrocephalus - Achondroplasia specific development charts are available - middle ear dysfunction is common - Bowing of legs is common so avoid umbrella stroller, infant carriers, walkers, jumpers, backpack carriers - monitor for OSA - Restrictive pulmonary dz is common - anesthesia risk

Question 28

Campomelic dysplasia

Answer 28

Large head Congenital short and bowed long bones Underdeveloped shoulder blades 11 pairs of ribs instead of 12 Club feet Distinctive facies: Flat face, prominent eyes

Question 29

Angelman Syndrome

Answer 29

- monitor speech and development - icreased r/o seizures, sleep disorders, feeding difficulty

Question 30

Beckwith-Weidemann Syndrome

Answer 30

- anticipate neonatal hypoglycemia - assess for umbilical hernia/omphalacele - monitor for feeding, speech, and breathing problems secondary to macroglossia - monitor for unequal growth - increased r/o certain childhood cancers (Wilm's tumor) - increased r/o renal anomalies