Most Common
Most common type of headache in children
Tension headache (second most common = migraine)
Preferred drug for absence seizures
Ethosuximide.
It is not used for any other seizure types and does not reliably treat major motor (convulsive) seizures in patients. Alternatives can include valproate or lamotrigine. Carbamazepine can worsen absence seizures.
Instructing a patient to do *this* for 3-4 minutes usually provokes an absence seizure
Hyperventilate. Can instruct patients to hyperventilate during an EEG to help confirm diagnosis.
What type of fracture is indicated by bilateral orbital ecchymoses (“raccoon eyes”)?
Basilar skull fracture. Basilar skull fractures cause separation of the occipital bone, leading to direct brain injury, disruption of venous structures, and significant bleeding in the posterior fossa.
What is the first-line abortive treatment for migraine headaches?
NSAIDs or acetaminophen
This is the most common neurocutaneous syndrome
NF1
What agent can be taken before pregnancy to reduce the risk of neural tube defects?
Folic Acid
What is classically seen on electroencephalogram in association with typical absence seizures?
Generalized 3 Hz spike wave discharges
An infant presents with sagging shoulder, arm that hangs limp in internal rotation, and a flexed wrist ("waiter's tip position"); which nerve roots are likely injured?
C5-C6, sometimes C7 also involved (triceps reflex will be present if C7 is not involved) [Erb Palsy]
90% of infants will have full recovery within 3 months.
Which anti-seizure medication has an increased risk of teratogenicity vs other ASMs (especially with respect to neural tube defects) and is best avoided in post pubertal adolescent girls?
Valproic acid
The neurocutaneous syndrome in which epilepsy is most frequently seen
Tuberous sclerosis
Treatment for infantile spasms (there are a few treatment options; you will get points for naming one)
ACTH, oral prednisolone, or Vigabatrin (Vigabatrin is recommended if patient has tuberous sclerosis).
Which antibiotic should be avoided with carbamazepine use?
Erythromycin- it elevates carbamazepine levels
A 10-year-old boy presents to the ED with sudden paralysis of his LUE and LLE and slurred speech. During evaluation, MRI of the brain on diffusion-weighted imaging shows restricted diffusion in the arterial territory. MRA shows stenosis of the distal internal carotid artery and collateral vessels of the basal ganglia. What condition predisposes this patient to the described imaging findings?
A) Factor V mutation
B) Klinefelter syndrome
C) Neurofibromatosis type 2
D) Sickle cell disease
D) Sickle cell disease. This child is presenting with signs of a stroke. Moyamoya means puff of smoke in Japanese, which describes the radiological findings found on MRA of either stenosis or occlusion of the distal internal carotid artery and circle of Willis and the presence of collateral vessels of the basal ganglia. Patients with sickle cell disease have a higher risk of moyamoya disease and associated complications of transient ischemic attacks and stroke. Also, as a single risk factor, sickle cell is the most common cause of strokes in children.
5-month-old male infant presents with poor feeding for the past several days. His father indicates he has not been sucking as well and he seems “tired all the time.” He has no fever, respiratory distress, emesis, or rash. He has not stooled for the past 3 days. Born full-term and was gaining weight very well prior to the past week. On exam, he is fussy and globally weak with decreased reflexes. Heart and lung exams are normal. What is the most likely diagnosis?
A) Infant botulism
B) Neonatal myasthenia gravis
C) Prader-Willi syndrome
D) Spinal muscular atrophy type 1
A- botulism. Acute onset of hypotonia, hyporeflexia, and constipation, is suggestive of infant botulism. Constipation can be one of the first and most often overlooked signs. Diagnosis of infant botulism is typically made via stool testing for the toxin. Botulism immune globulin given intravenously is the treatment of choice and should be administered as soon as possible in infants with suspected botulism.
What is the most common cause of chorea in children?
Sydenham chorea (clinical manifestation of acute rheumatic fever)
These anti-epileptics are preferred for treatment of focal seizures
oxcarbazepine, carbamazepine (voltage gated Na+ channel blockers)
A 2-year-old boy presents after having a seizure at home. He recently had URI symptoms and today had a fever to 103°F. Mother says he was stiff and shaking, then his eyes rolled back in his head. The seizure lasted 8 minutes and resolved on its own. In the ED, he is mildly sleepy, but his neurologic exam is now normal. His mother is very worried because she has a nephew with epilepsy. What is the next best course of action?
A) Discharge him with an outpatient neurology referral
B) Discharge him with instructions to receive antipyretics every 4 hours for the next several days
C) Discharge him with supportive care
D) Electroencephalogram
C- DC with supportive care. This patient had a simple febrile seizure. Simple febrile seizure is distinguished from a complex febrile seizure by the patient’s age (typically 6 months to 5 years), duration (< 15 minutes), seizure characteristics (generalized tonic-clonic), and the child being otherwise healthy. Patients with simple febrile seizures can be discharged home with supportive care once their neurologic status has returned to baseline. If febrile seizure lasts more than 30 mins, it is considered febrile status epilepticus. In this case, antiepileptic medications may be required, whereas simple febrile seizures require antiepileptic medications very rarely.
(A) is not necessary because patients with simple febrile seizures do not require neurology evaluation unless the seizures recur frequently or there is evidence of developmental delay.
Discharging the patient with instructions to receive antipyretics every 4 hours for the next several days (B) may make the child more comfortable and may alleviate the mother’s anxiety, but this approach has never been shown to decrease the rate of recurrence of febrile seizures.
An electroencephalogram (D) is not indicated in cases of first-time simple febrile seizures because it offers low yield in this clinical scenario.
A 14-year-old boy presents to the ED after a head trauma. He is asymptomatic and has a normal neurologic exam. MRI of the brain reveals capillary telangiectasias in the pons and middle cerebellar peduncles. Which of the following is recommended?
A) Administration of anti epileptic medication
B) Follow-up imaging in 6 months
C) No intervention
D) Surgical intervention due to risk of bleeding
C-No intervention. Capillary telangiectasias are commonly found incidentally on neuroimaging; they are the result of abnormal embryogenesis of the capillary wall. Often multiple and usually seen in the pons, middle cerebellar peduncles, and dentate nuclei. These lesions do not require intervention as they are not associated with morbidity.
A 2-year-old boy presents to the office with progressive muscle weakness. He started walking at 1 year of age, but in the last 6 months, he seems to be more clumsy, falling more often and having difficulty pushing himself up from the floor to a standing position. He has to use his hands to accomplish this. On exam, calves appear hypertrophic. Creatine kinase levels are grossly elevated. Molecular genetic testing is done to confirm the diagnosis. What other test should be ordered on this patient at the time of diagnosis?
A) Echocardiogram
B) Forced vital capacity
C) Thyroid function
D) Vision
A- echocardiogram. Duchenne muscular dystrophy is an X-linked progressive muscular dystrophy caused by a mutation of the dystrophin gene. Dystrophin is expressed in cardiac, skeletal, and neural tissue. Progressive muscle weakness is noted between 2 to 3 years of age. Muscle weakness is the first sign noted, especially in the proximal lower extremities, which then progresses to distal muscle groups and upper extremities. It is important to obtain an echocardiogram and ECG at the time of diagnosis and annually, as dilated cardiomyopathy is common. Conduction defects and dysrhythmias are seen. Fibrosis of the ventricular wall can also occur. Cardiomyopathy is one cause of mortality in this disease.