1 Describe the physical findings in Bell’s palsy

Bell’s palsy refers to peripheral facial nerve weakness on one side of the face, including inability to wrinkle the forehead on the affected side, inability to close the affected eye, and flattening of the affected nasolabial fold. Bell’s palsy may also involve hyperacusis in the affected ear (noises sound excessively loud) because the site of injury is thought to be in the facial canal and may involve other branches of C7—including those to the stapedius muscle, which dampens sound waves.

2 What is the definition of status epilepticus?

Seizures that are continuous for 30 minutes or longer or that are repetitive with the patient not regaining consciousness are defined as status epilepticus.

Tharp BR: Clinical features and complications of status epilepticus in children, 2006. Available at www.uptodate.com.

3 What is Todd’s paralysis?

Todd’s paralysis refers to paresis or paralysis of one or more areas of the body that is transient, usually disappearing within 24 hours following a seizure.

4 A child is having an acute seizure. You know that IV access will not be possible quickly. What are your options?

Wiznitzer M: Buccal midazolam for seizures. Lancet 366:182–183, 2005.

5 List the drugs commonly used to stop seizures acutely in the emergency department (ED)

See Table 32-1.

6 Describe the characteristics of absence (petit mal) seizures

Absence seizures usually develop in school-age children before puberty. The seizures are characteristically of abrupt onset with a brief loss of consciousness. Most are less than 30 seconds in duration. Children may appear to be staring or may have eye blinking or rhythmic nodding. The child does not fall, although he or she may drop things. These seizures are often easily precipitated by having the child hyperventilate.

7 What are infantile spasms?

Infantile spasms are characteristic seizures usually beginning when a baby is 4–7 months old. The seizures are usually of sudden onset and generalized, with bilateral and symmetric contraction of the muscles in the neck, trunk, and extremities. The initial contraction typically lasts < 2 seconds and is then followed by a sustained contraction of 2–10 seconds. Patients with infantile spasms have a poor developmental prognosis, and 65–90% are developmentally delayed at the time of diagnosis.

Glaze DG: Management and prognosis of infantile spasms, 2006. Available at www.uptodate.com.

8 A 16-year-old boy presents after an early-morning generalized tonic clonic seizure. On history, he describes occasional, brief, jerking movements in the morning that make teeth brushing and hair combing difficult. These jerking movements resolve and do not recur later in the day. What does this symptom pattern suggest?

Juvenile myoclonic epilepsy most commonly presents between 12 and 18 years of age with brief, bilateral flexor jerking movements of the arms. The seizures can be precipitated by sleep deprivation, alcohol ingestion, and awakening from sleep. Patients may have generalized tonic clonic seizures but are normal neurologically.

Yu KT: Approach to the child with seizures. In Osborn LM, Dewitt TG, First LR, Zenel JA (eds): Pediatrics. St. Louis, Elsevier Mosby, 2005, pp 752–756.

9 How are seizures distinguished from breath-holding spells?

Involuntary muscular contractions occur in both, although they are more prominent with seizures. In breath-holding spells, these contractions are due to transient cerebral hypoperfusion. With breath-holding spells, a precipitating event causes the child to cry or be angry or frustrated, and thus hold his or her breath or perform the Valsalva maneuver. Seizures usually do not have an obvious precipitating event. Breath-holding spells are usually brief, and may feature a short period of sleepiness afterwards. Seizures may be brief, but suspicion for a seizure disorder occurs when the episode lasts > 1 minute or if the postictal period is prolonged. Finally, breath-holding spells usually occur in children 6 months to 4 years of age; episodes outside these limits are more suspicious for seizures.

10 What are the indications for urgent computed tomography (CT) after a seizure?

Magnetic resonance imaging (MRI) is the preferred study in all cases but is usually not immediately available in the emergency setting. Patient stability may also be a factor, as CT is typically faster and often does not require sedation.

Hirtz D, Ashwal S, Berg A, et al: Practice parameter: Evaluating a first nonfebrile seizure in children. Neurology 55:616–623, 2000.

11 What are the indications for nonurgent MRI after a seizure?

MRI testing for the preceding indications avoids unnecessary radiation exposure from CT.

Hirtz D, Ashwal S, Berg A, et al: Practice parameter: Evaluating a first nonfebrile seizure in children. Neurology 55:616–623, 2000.

12 What is the incidence of febrile seizures? When do they occur?

About 3–4% of all children have a febrile seizure; most occur in children between the ages of 6 months and 5 years.

American Academy of Pediatrics. Available at www.aap.org/patiented/febrileseizures.htm.

13 What makes a febrile seizure “simple” or “complex”?

A simple febrile seizure has all of the following characteristics:

A complex febrile seizure has at least one of the following characteristics:

14 What is the recurrence risk for a febrile seizure?

Approximately 30%. This number varies by age at onset of first febrile seizure.

15 Is the risk of recurrence increased if the initial febrile seizure is complex?

No. Children at greater risk of recurrence may have one or more of the following features:

Berg AT, Shinnar S, Darefsky AS, et al: Predictors of recurrent febrile seizures. A prospective cohort study. Arch Pediatr Adolesc Med 151:371–378, 1997.

16 Is the risk of epilepsy increased in children with febrile seizures?

Yes, but to a small degree. The risk of epilepsy in the general population of children without febrile seizures is 0.5%, which increases to 2–4% in children with febrile seizures. If there is a family history of epilepsy, and if the child has a neurologic disorder or developmental delay, then the risk of a subsequent afebrile seizure increases to 10%.

Waruiru C, Appleton R: Febrile seizures: An update. Arch Dis Child 89:751–756, 2004.

17 What tests should be performed on children after a simple febrile seizure?

There is no need for brain imaging studies or electroencephalography after a simple febrile seizure. Laboratory testing or radiography should be performed as appropriate for the diagnosis and management of the cause of the fever.

American Academy of Pediatrics, Provisional Committee on Quality Improvement, Subcommittee on Febrile Seizures: Practice Parameter: The neurodiagnostic evaluation of the child with a first simple febrile seizure. Pediatrics 97:769–772, 1996.

18 When should you consider a lumbar puncture in a child with a febrile seizure?

American Academy of Pediatrics, Provisional Committee on Quality Improvement, Subcommittee on Febrile Seizures: Practice Parameter: The neurodiagnostic evaluation of the child with a first simple febrile seizure. Pediatrics 97:769–772, 1996.

19 In a child with suspected meningitis, what are some contraindications to performing an immediate lumbar puncture?

In these cases, antibiotic therapy may be initiated presumptively and lumbar puncture delayed.

Cronan K, Wiley J: Lumbar puncture. In Henretig F, King C (eds): The Textbook of Pediatric Emergency Medicine Procedures. Philadelphia, Lippincott Williams & Wilkins, 2008, p 507.

20 What is an impact seizure?

An impact seizure is a brief, generalized seizure occurring immediately at the time of injury. The seizure activity is thought to be due to traumatic depolarization of neurons on impact, and these children do not have a higher incidence of epilepsy.

21 How is a hyponatremic seizure managed?

Hyponatremic seizures are often managed with hypertonic saline to transiently raise plasma sodium levels by 5–10 mEq to stop the seizure. The dose is 2–4 mL/kg body weight of 3% sodium chloride (0.5 mEq/mL), given rapidly and intravenously.

22 What are some differentiating features of Guillain-Barré syndrome and transverse myelopathy?

See Table 32-2

Table 32-2 Guillain-BarrÉ Syndrome vs. Tranverse myelopathy

* A level below which the patient has absence of sensation.

From Gorelick MH, Blackwell CD: Neurologic emergencies. In Fleisher GR, Ludwig S, Henretig FM [eds]: Textbook of Pediatric Emergency Medicine, 5th ed. Philadelphia, Lippincott William & Wilkins, 2006, pp 759–781.

23 What is the Miller Fisher variant of Guillain-Barré syndrome?

Ophthalmoplegia, areflexia, and ataxia.

24 What simple grooming measure should be performed on any patient with acute onset of ascending paralysis?

Combing the hair. Tick paralysis can present very similarly to Guillain-Barré syndrome, although the rate of progression is faster and tick paralysis is accompanied by ataxia (not present in Guillain-Barré syndrome). Removal of the tick can bring about rapid improvement, and the tick is often found serendipitously.

Felz MW, Smith CD, Swift TR: A six-year-old girl with tick paralysis. N Engl J Med 342:90–94, 2000.

25 A 2-month-old baby with a history of hypotonia presents in acute respiratory distress. He has abdominal respirations and appears to be tiring out. In preparing for intubation, what skeletal muscle relaxant is relatively contraindicated?

Succinylcholine. In children with undiagnosed skeletal myopathy, concern is raised about the use of succinylcholine and the potential for ventricular dysrhythmias and cardiac arrest from hyperkalemia.

26 What is the initial symptom seen in infantile botulism?

Infantile botulism commonly presents with constipation for days to weeks before lethargy, feeding difficulties, diminished reflexes, weakness, hypotonia, and diminished gag reflex appear.

27 How is the diagnosis of infant botulism made?

The diagnosis is largely clinical and relies on the elimination of other causes of lethargy and weakness. Clostridium botulinum toxin may be identified in the stool. Brief, small, abundant motor action potentials are characteristic on electromyogram.

American Academy of Pediatrics: Clostridial infections. In Pickering LK (ed): 2003 Red Book: Report of the Committee on Infectious Diseases, 26th ed. Elk Grove Village, IL, American Academy of Pediatrics, 2006, pp 257–260.

28 Which antibiotics should be avoided in infant botulism?

The aminoglycosides, because they may potentiate the neuromuscular blockade and lead to respiratory arrest.

29 How would you differentiate a migraine headache from that due to pseudotumor cerebri?

Both conditions present with headache, although headache due to pseudotumor cerebri may be worse in the morning, while migraine symptoms are often relieved by sleep. Nausea, vomiting, and visual problems may also occur with both, and the neurologic examination may be normal. However, papilledema is seen in most cases of pseudotumor cerebri and is not present in migraine. Perform CT or MRI to rule out other causes of elevated intracranial pressure. If imaging does not demonstrate a mass lesion, perform a lumbar puncture to measure opening pressure, which will be elevated in pseudotumor cerebri.

30 Describe the weakness in myasthenia gravis

Bilateral ptosis and ophthalmoplegia are the most common manifestations of myasthenia gravis. The weakness is variable, and the specific muscles affected may vary from examination to examination. Those with generalized weakness may report a history of worsening throughout the day or with continued activity. In the ED, easy fatigability of muscle strength may be demonstrated.

Gorelick MH, Blackwell CD: Neurologic emergencies. In Fleisher GR, Ludwig S, Henretig FM (eds): Textbook of Pediatric Emergency Medicine, 5th ed. Philadelphia, Lippincott Williams & Wilkins, 2006, pp 759–781.

31 Which electrolyte abnormality is responsible for periodic paralysis?

Periodic paralysis can be due to hypokalemia or hyperkalemia. Paralytic episodes may occur after strenuous exercise. Persistent weakness is possible.

32 Acute cerebellar ataxia is most commonly attributed to which viral illness?

Varicella. Acute cerebellar ataxia typically occurs within 10 days of a viral illness and is felt to be a parainfectious or postinfectious phenomenon. The onset of ataxia is acute, and nystagmus and slurred speech can occur, but most children are otherwise normal. Recovery typically takes a few weeks, and residual neurologic deficits are possible.

33 What is “milkmaid’s hand”?

Milkmaid’s hand is found in patients with chorea. The child cannot maintain a continuous grasp when asked to squeeze the examiner’s fingers, but instead performs intermittent squeezing as if he or she were “milking” the finger. These low-amplitude, jerking movements are characteristic of chorea.

34 What is the most common cause of acquired chorea in children?

Sydenham’s chorea, which is a manifestation of rheumatic fever.

35 In uncal (unilateral transtentorial) herniation, is pupillary dilation present on the same side as the increased intracranial pressure or the opposite side?

The pupil dilates on the same side where the temporal lobe causes the uncus to bulge into the tentorial notch.

36 Why does the pupil dilate in uncal herniation?

Direct pressure on the oculomotor nerve (cranial nerve III) causes the ipsilateral pupil to dilate.

37 How do brain abscesses occur?

38 Why is the diagnosis of sphenoid sinusitis often difficult?

The sphenoid sinuses are located posterior to the ethmoid sinuses, and the usual physical examination techniques, such as percussion and transillumination, are not useful. Headache is usually severe, but can mimic migraine and is often located on top of the head, behind the eyes or at the back of the neck. Painful paresthesias may occur in the trigeminal nerve distribution. Eighty percent of pediatric patients with intracranial complications of sinusitis have sphenoid involvement.

Saitoh A, Beall B, Nizet V: Fulminant bacterial meningitis complicating sphenoid sinusitis. Pediatr Emerg Care 19:415–417, 2003.

39 What are the neurologic complications of sinusitis?

40 Where are pediatric brain tumors most commonly located?

Posterior fossa tumors account for 50% of brain tumors in children of all ages, and are the most common location for brain tumors in children ages 1–8 years. This explains why gait disturbances are common in children with brain tumors.

41 Brain tumors in children commonly present with which ocular nerve palsy?

The sixth nerve (abducens) is commonly affected in children with posterior fossa brain tumors. In some, the inability to perform lateral rectus motion may be the sole manifestation of the tumor. The sixth nerve lies close to the cerebellum, fourth ventricle, and pons, which may explain its involvement in childhood brain tumors.

Rheingold SR, Lange BJ: Oncologic emergencies. In Fleisher GR, Ludwig S, Henretig FM (eds): Textbook of Pediatric Emergency Medicine, 5th ed. Philadelphia, Lippincott Williams & Wilkins, 2006, pp 1239–1274.

42 What is acute demyelinating encephalomyelitis (ADEM)?

ADEM is an inflammatory demyelinating disease that presents in children and young adults. It is an uncommon illness usually occurring in the winter and spring. In many cases it follows an upper respiratory tract infection. Children present with multifocal neurologic signs. Symptoms last for 2–4 weeks.

Murthy SN, Faden HS, Cohen ME, Bakshi R: Acute disseminated encephalomyelitis in children. Pediatrics 110:e21, 2002.

43 What is the differential diagnosis of ADEM?