PEDIATRIC NEUROLOGY for PEDIATRIC RESIDENTS
Cesar C. Santos, MD
Chief, Division of Pediatric Neurology
Department of Pediatrics
Georgetown University Hospital
June 9, 2010
1. Pediatric Neurology Elective
2. Learning Goals and Objectives
3. Resident Evaluation
4. Recommended Reading and Resources
I. Pediatric Neurology Elective
Goals and Objectives
Broaden pediatric neurology knowledge-base
Improve the ability to perform a neurologic examination in a pediatric patient
Expose residents to common pediatric neurology problems
Increase the comfort level in managing common pediatric neurologic problems
Understand which patient(s) require referrals to a pediatric neurologist
Encourage pediatric resident’s interest in pediatric neurology
See consults and discuss each consult after formulating an assessment and plan
with the pediatric neurology attending
See active consult patients and write daily progress notes before rounding with the
Coordinate care plan for the consult patients with the general
See patients in the outpatient clinic with the attending on service
For new patients
o Perform history and examination (general and neurologic)
o Review old records and prior studies
o Formulate an assessment and plan
o Discuss the patient with the attending
o Complete the Centricity note
For established patients
o Obtain interval history
o Perform examination (general and neurologic)
o Formulate an assessment and plan
o Discuss with the attending
o Complete Centricity note
II. Learning Goals and Objectives
1. Perform a neurologic history and examination as well as developmental
evaluation of children of all ages.
a. View and study this web site on the neurologic examination up to age 2 ½:
b. See the attached “Summary of the Neurologic Examination”
c. See the attached “cheat sheet” of developmental milestones
2. Understand and employ the concept of anatomical localization of neurologic
symptoms and deficits.
a. Differentiate between upper motor neuron (UMN) and lower motor
neuron (LMN) dysfunction by using the different components of the
1. Differentiate tone from strength
2. Define spasticity
b. Differentiate between UMN vs LMN facial weakness
c. Differentiate sensory deficits due to peripheral nervous system lesion
(radiculopathy, mononeuropathy, brachial plexopathy) vs central nervous
system lesion (recognize cortical sensory loss and spinal sensory level)
d. During the examination of a comatose patient, define, recognize, and
1. Decorticate vs decerebrate posturing
2. Pupillary abnormalities
3. Vestibuloocular reflexes
4. Discuss how these findings help determine structural vs
metabolic causes; hemispheric vs brainstem lesions
5. Know the herniation syndromes
3. Demonstrate knowledge of cardinal manifestations of different neurologic
1. Distinguish seizures from non-epileptic events e.g. jitteriness,
breath-holding spells, night terrors, gastroesophageal reflux,
neonatal sleep myoclonus, pseudoseizures
2. Describe the evaluation and management of new-onset
seizure, and understand epidemiological basis of decision-
making (i.e. risk factors for recurrence)
3. Plan the initial therapy for status epilepticus
4. Know the medications that can be administered rectally to
treat status epilepticus
5. Know the possible etiologies of status epilepticus: infection,
toxin, electrolyte imbalance, drug withdrawal
6. Plan the initial therapy for neonatal seizures
7. Discuss the possible etiologies and evaluation of neonatal
8. Be aware of common side effects of antiseizure medications,
including Phenobarbital, phenytoin, valproic acid,
9. Be aware of treatment options for refractory epilepsy
10. Discuss evaluation and management of febrile seizures
11. Know the risk factors associated with febrile seizures related
to later epilepsy
12. Recognize the epilepsy syndrome of benign rolandic epilepsy
(“benign epilepsy with centrotemporal spikes”)
13. Recognize the epilepsy syndrome of absence epilepsy, its
differential diagnosis, evaluation, and treatment
14. Be able to differentiate absence and complex partial seizures
15. Recognize the characteristic clinical picture of infantile
16. Know the natural history and prognosis of infantile spasms:
risk of developmental delay/regression and later epilepsy
17. Recognize the epilepsy syndrome of juvenile myoclonic
18. Recognize Lennox-Gastaut syndrome
1. Know the clinical characteristics of tension-type and
2. Discuss abortive and prophylactic management of migraine
3. Know the elements of history that characterize a headache
due to increased intracranial pressure and other
signs/symptoms of increased ICP
4. Know the signs and symptoms of a headache that indicate a
need for follow-up with magnetic resonance imaging or CT
c. Altered Consciousness/Trauma
1. Define lethargy, coma
2. Diagnose brain death in children and the persistent vegetative
3. Recognize clinical characteristics of “non-accidental trauma”
or “shaken baby syndrome” and discuss evaluation and
differential diagnosis (e.g. bleeding diathesis, osteogenesis
imperfecta, glutaric aciduria-I)
4. Know the role of pharmacologic therapy in acute spinal cord
or craniocerebral trauma
d. Infectious or Post-Infectious Neurologic Disorders
1. Know the presenting signs, symptoms, and differential
diagnosis of Guillain-Barre syndrome
2. Know the complications of Guillain-Barre syndrome
3. Know the possible presentations (e.g. optic neuritis,
transverse myelitis) and differential diagnosis of ADEM
(acute disseminated encephalomyelitis)
4. Know the presenting signs, symptoms, and differential
diagnosis of cerebellitis (i.e. other causes of acute cerebellar
ataxia and vertigo)
5. Discuss the causes and presentation of congenital intrauterine
infections (TORCH), including herpes and its time of
presentation and mode of transmission
6. Know the 3 most frequent organisms in neonatal meningitis
7. Know the epidemiology, presentation, evaluation, and
treatment of meningoencephalitis. Recognize the CSF
findings in herpes encephalitis
8. Recognize the CSF findings in bacterial (including Lyme)
and viral meningitis. Know the complications of bacterial
9. Know that most abscesses contain multiple organisms,
10. Know that neuroimaging studies should be done before
examination of the CSF in suspected brain abscesses
11. Know the presentation, evaluation, and treatment of infant
12. Know the presentation of Sydenham’s chorea, as well as
relationship to diagnosis of rheumatic fever, and treatment
e. Genetic/Metabolic Syndromes
1. Recognize the historical features indicative of a degenerative
2. Know the typical presentations and diagnosis of Wilson’s
disease and Menkes’ disease
3. Recognize the potential infectious presentation of
4. Recognize the common presentation of a urea cycle defect
5. Recognize the common presentation and evaluation of a
6. Recognize the presentation and mode of transmission of
mitochondrial disorders (e.g. Kearns-Sayre, MERRF,
7. Know the clinical manifestations and diagnosis of Rett
f. Movement Disorders
1. Know the definition, types, and evaluation of cerebral palsy
2. Know the presentation of kernicterus
3. Differentiate between tics and epileptic seizures; know when
to treat tics; understand the diagnostic criteria for Tourette
4. Recognize opsoclonus-myoclonus and discuss possible
etiology and evaluation
g. Neuromuscular Disorders
1. Know the differential diagnosis of weakness and an increased
serum creatine kinase concentration
2. Know the laboratory studies available to diagnose muscle
disease of childhood
3. Know the presentation, diagnosis, and mode of transmission
of Duchenne muscular dystrophy
4. Know the presentation, diagnosis, and treatment of
5. Recognize the presentation and diagnosis of myotonic
6. Recognize the symptoms, signs, and evaluation of
1. Identify primitive reflexes and know age of onset and
2. Understand the definition/purpose of an individualized
educational program (IEP)
3. Know the diagnostic criteria for attention
4. Be aware of the current consensus regarding evaluation of
global developmental delay
1. Identify the clinical manifestations and plan the diagnostic
evaluation of spinal dysraphism; know the various types and
2. Discuss the presentation of Arnold-Chiari Type I and
association with headache
3. Discuss types of cerebral malformations, including cortical
dysplasias (e.g. lissencephaly), Dandy-Walker malformation,
4. Know diagnosis and further evaluation of septo-optic
5. Know diagnosis of fetal alcohol syndrome
j. Neurocutaneous Disorders
1. Know the clinical characteristics of tuberous sclerosis
2. Know the clinical characteristics of neurofibromatosis type I
and potential complications, including neoplasms
3. Know the clinical characteristics and complications of
1. Recognize complications of ventriculoperitoneal shunts
2. Be aware that brain and spinal cord tumors are the 2nd most
common group of neoplasms in children (1st leukemia) and
know the usual location in children (infratentorial)
3. Identify the clinical features of childhood stroke including
4. Recognize the signs and symptoms of and plan treatment for
5. Know the clinical difference between papilledema and optic
Goals Based on Core Competency Teaching
I. PATIENT CARE
1. Interview patients more skillfully.
2. Examine patients more skillfully.
3. Improve neurological localization skills.
4. Accurately diagnose pediatric neurological disorders requiring inpatient
5. Effectively manage the patient with acute neurological illness including
appropriate drug therapy and non-pharmacologic treatments.
6. Define and prioritize pediatric patients' neurological and medical problems.
7. Appropriately select and interpret pertinent laboratory and imaging studies.
8. Improve technical skills like performing lumbar puncture
9. Effectively implement long-term medical care of the pediatric neurology patient.
10. Improve clinical ability to anticipate, prevent, and treat neurological
complications of medical and surgical conditions.
11. Improve efficiency of care in the hospital setting.
II. MEDICAL KNOWLEDGE
1. Improve basic neurological knowledge base.
2. Expand clinical knowledge base regarding common pediatric neurologic problems
requiring inpatient admission. This includes but not limited to AIDP, myasthenia
gravis and exacerbation, multiple sclerosis exacerbation, acute seizures and status
epilepticus, status migrainosus, meningitis/encephalitis, and stroke in children.
3. Improve understanding of evaluation and diagnostic testing for common pediatric
4. Expand knowledge of potential interventions to anticipate and prevent future
complications relative to the patient's illness.
5. Assess and critically evaluate current medical information and scientific evidence
relevant to patient illness.
III. PRACTICE-BASED LEARNING AND IMPROVEMENT
1. Identify and acknowledge gaps in personal knowledge and skills in the care of
hospitalized patients with pediatric neurological illness.
2. Develop and implement strategies for filling in gaps in knowledge and skills.
IV. INTERPERSONAL SKILLS AND COMMUNICATION
1. Communicate effectively with patients and families
2. Communicate effectively with physician colleagues at all levels, especially
regarding effective written communications.
3. Communicate effectively with all ancillary care personnel involved in the care of
the patient to ensure comprehensive and timely care.
4. Present patient information concisely and clearly, verbally and in writing.
5. Teach colleagues and medical students effectively.
1. Demonstrate respect, compassion and integrity when dealing with patients and
2. Demonstrate sensitivity and respect for patients' age, culture, race, gender and
3. Demonstrate a commitment to ethical principles of providing or withholding care,
patient confidentiality and informed consent, and business practices.
4. Demonstrate a commitment to carrying out professional duties including
punctuality, reliability, chart maintenance and independent learning and
5. Demonstrate professional respects for superiors, colleagues, students and all
members of the health care team.
VI. SYSTEMS-BASED PRACTICE
1. Understand and utilize the multidisciplinary resources necessary to care optimally
for patients in the outpatient setting.
2. Collaborate with other members of the health care team to assure comprehensive
3. Use evidence-based, cost-conscious strategies in the care of outpatients.
4. Understand the long-term consequence of patient care in relation to the
individual's socioeconomic status.
III. PEDIATRIC RESIDENT EVALUATION
Neurology Exam Checklist
a. Pre and Post Rotation Exam Assessment
Notation of pertinent general exam findings ____ ____
E.g. HC, dysmorphic features, anterior fontanelle, flattening of occiput, cardiac
abnormalities, hepatosplenomegaly, cutaneous lesions
Notation of Mental Status ____ ____
E.g. Orientation, alertness, language, detailed MS exam if pertinent
Cranial Nerves ____ ____
II: funduscopic exam, visual fields, visual acuity, blink reflex
III, IV, VI: conjugate movements, strabismus, extraocular movements, ptosis
V: sensation, corneal reflex
VII: facial strength
VIII: hearing, nystagmus
IX/X: palate elevation, gag
XI/XII: sternocleidomastoid strength, tongue
Motor ____ ____
Tone (e.g. traction response, horizontal/vertical suspension, limb tone)
Motor development if pertinent
Gait ____ ____
e.g. weight-bearing, toe, heel, tandem walking, Romberg sign
Coordination ____ ____
e.g. dysmetria on reaching for toys, FNF or HKS, rapid alternating movements
Reflexes ____ ____
i.e. DTRs, primitive reflexes e.g. Moro, Tonic neck, Propping, Parachute
Sensory ____ ____
e.g. cries with, localizes, purposefully withdrawals to stim; spinal level;
Dorsal column modalities (vibration, proprioception), LT, temperature
Exam Observed by Attending _________________________ (signature)
Suggestions/Areas to Focus On ___________________________________
Core Competency Teaching and Evaluation for Pediatric Resident rotating on
Pediatric Neurology Inpatient Team or Pediatric Neurology Elective
Competency Educational Activity Evaluation/Assessment/Feedback
1. Patient Care Clinical teaching rounds Neurological Exam Checklist
Outpatient Clinic Monthly rotation evaluations
Multi-disciplinary rounds Verbal feedback
Conferences and Didactics
Subspecialty AM Report
2. Medical Knowledge Clinical teaching rounds Monthly rotation evaluations
Outpatient clinic Verbal feedback
3. Practice-Based Clinical teaching rounds Monthly rotation evaluations
Learning and Outpatient clinic Verbal Feedback
Development Case-related conferences: Written feedback for presentation
4. Interpersonal and Clinical teaching rounds Monthly rotation evaluations
Communication Skills Outpatient clinic Verbal feedback
Interactions with students,
residents, and ancillary staff
Effectiveness of charting and
5. Professionalism Clinical teaching rounds Monthly rotation evaluations
Outpatient clinic Verbal feedback
Interactions with patients,
families, students, residents,
faculty, and ancillary staff
Timely completion of medical
Attendance for rounds, AM
6. Systems-based Clinical teaching rounds Monthly rotation evaluations
Practice Outpatient clinic Verbal feedback
Multi-disciplinary rounds and
IV. RECOMMENDED READING AND RESOURCES
1) Obtain a copy of Clinical Pediatric Neurology, Fenichel. Topics to review prior
to inpatient or outpatient rotation:
a. Neurologic abnormalities of the newborn
b. Ataxia of childhood
c. Movement disorders
2) Basic articles, “cheat-sheets” and summary tables, as well as several classic
3) Excellent websites include:
a. www.neuro.wustl.edu/neuromuscular (specifics of essentially all
b. www.ncbi.nlm.nih.gov/Omim (search for neurogenetic disorders based on
signs or symptoms)
c. www.genetests.org (listings of genetic testing and facilities that perform
them for neurogenetic disorders, also links to reviews of those disorders)
(videos of a pediatric neurologist examining children up to age 2 ½)
4) Pre and post rotation - pediatric neuro exam
5) Textbook References:
Fenichel, Gerald. Clinical pediatric neurology A signs and symptoms approach.
Swaiman, Kenneth. Pediatric neurology Principles and practice.
Zitelli, Basia. Atlas of Pediatric Diagnosis (Neurology Chapter).
6) Contact Numbers:
M. Stella Legarda, MD - pager 202-405-0156
Cesar C. Santos, MD – pager 202-405-2804
Nassim Zecavati, MD – pager 202-405-
CONTENTS OF LEARNING MATERIALS
1) “SUMMARY of the Neurologic Exam”
2) SUMMARY of how to differentiate upper motor neuron and lower motor neuron
3) Cheat-sheet of developmental milestones
a. Friedman MJ. Seizures in Children. Pediatr Clin N Am 2006, 53:257-77.
b. Olson, D. Paroxysmal Events. Differentiating Epileptic Seizures from
Nonepileptic Spells. Consultant for Pediatricians 2008; 461-69.
c. Zupanc ML. Neonatal seizures. Pediatr Clin N Am 2004; 51:961-978.
d. Breningstall GN. Breath-holding spells. Pediatr Neurol 1996;14:91-97.
e. Hirtz D et al. Practice parameter: Evaluating a first nonfebrile seizure in
children. Neurol 2000;55:616-623.
f. Hirtz D et al. Practice parameter: Treatment of the child with a first
unprovoked seizure. Neurol 2003;60:166-175.
g. Tables and summaries: Antiseizure medications by seizure type, AEDs
dosing and side effects, Diastat dosing
h. Algorithm - Status Epilepticus
i. Jarrar RG and Buchhalter JR. Therapeutics in pediatric epilepsy, part 1:
the new antiepileptic drugs and the ketogenic diet. Mayo Clin Proc
j. Buchhalter JR and Jarrar RG. Therapeutics in ped epilepsy, part 2:
epilepsy surgery and vagus nerve stimulation. Mayo Clin Proc
k. Hirtz DG. Febrile seizures. Pediatr Rev 1997;18:5-8.
l. Practice Parameter: the neurodiagnostic evaluation of the child with a first
simple febrile seizure. Pediatr 1996;97:769-772.
m. Baumann RJ and Duffner PK. Treatment of children with simple febrile
seizures: the AAP practice parameter. Pediatr Neurol 2000;23:11-17.
n. Summary of status epilepticus treatment protocol
o. Holmes GL and Riviello JJ. Midazolam and Pentobarital for Refractory
Status Epilepticus. Pediatr Neurol 1999;20:259-264.
p. Riveiello, J et al. Practice Parameter: Diagnostic assessment of a child
with status epilepticus. Neurology 2006; 67: 1542-1550.
q. Hrachovy, R Infantile Epileptic Encephalopathy with Hypsarrhythmia
(Infantile Spasms/West Syndrome). J Clin Neurophys 2003; 20(6):408-
5) Headache, Alteration of Consciousness
a. Hershey, A, et al. Headaches. Curr Opin Pediatr. 2007; 19: 663-669.
b. Lewis, DW, et al. Practice parameter: Evaluation of children and
adolescents with recurrent headaches. Neurol 2002; 59: 490-498.
c. Lewis, DW, et al. Practice parameter: Pharmacological treatment of
migraine headache in children and adolescents. Neurol 2004; 63:2215-
d. Practice parameter: The management of concussion in sports. Neurol
e. Chapter on Coma and its causes.
f. Schneider S and Ashwal S. Determination of brain death in infants and
children. Swaiman K ed, Pediatric neurology Principles and practice.
6) Infectious or Post-Infectious Neurologic Disorders
a. Evans OB and Vedanarayanan V. Guillain-Barre syndrome. Pediatr Rev
b. Jones CT. Childhood autoimmune neurologic diseases of the central
nervous system. Neurol Clin 2003;4:
c. Chapter on Ataxia
d. Wubbel L and McCracken GH. Management of bacterial meningitis:1998.
Pediatr Rev 1998;19:78-84.
e. Bonthius DJ and Karacay B. Meningitis and encephalitis in children: an
update. Neurol Clin 2002;20:
f. Fenichel GM. Assessment: neurologic risk of immunization: report of the
therapeutics and technology assessment subcommittee of the American
Academy of Neurology. Neurol 1999;52:1546-1552.
7) Genetic and Metabolic Syndromes
a. Dagli, A, Zori, R, Heese, B. Testing Strategy for Inborn Errors of
Metabolism in the Neonate. Neoreviews 2008; 9(7):e291-298.
b. Chapter on Psychomotor Retardation and Regression.
8) Movement Disorders
a. Wolf, D, Singer H. Pediatric movement disorders: an update. Curr Opin
b. Dooley, J. Tic Disorders in Childhood. Semin Pediatr Neurol 2006; 13:
9) Neuromuscular Disorders
a. Bodensteiner, J. The Evaluation of the Hypotonic Infant. Semin Pediatr
Neurol 2008; 15:10-20.
b. Chapters of Floppy Infant Syndrome causes and Flaccid Limb Weakness
c. Algorithm for Hypotonia and Weakness
d. Table Etiology of Infantile Hypotonia.
a. Shevell M et al. Practice parameter: evaluation of child with global
developmental delay. Neurol 2003;60:367-380.
b. Palmer FB and Capute AJ . Pediatr Rev 1994;15:473-479.
c. Taft LT. Cerebral palsy. Pediatr Rev 1995;16:411-418
d. Summary, Practice parameter: Diagnostic Assessment of a Child with
e. Johnson C, Myers, S. Identification and Evaluation of Children with
Autism Spectrum Disorders. Pediatrics 2007; 120(5): 1183-1215.
a. Chapter on Disorders of Cranial Volume and Shape.
12) Neurocutaneous Disorders
a. Dahan, D, Fenichel, G, El-Said, R. Neurocutaneous Syndromes. Adolesc
Med 2002;13(3): 495-509.
a. Rivkin MJ and Volpe JJ. Strokes in children. Pediatr Rev 1996;17:265-
b. Table. Suggested diagnostic laboratory evaluation in children who have
arterial ischemic stroke.
c. Seidman, C, Kirkham, F, Pavlakis, S. Pediatric Stroke. Curr Opin Pediatr
2007, 19: 657-662.
14) Hypoxic Ischemic Encephalopathy
a. Roland, E and Hill, A. Clinical Aspects of Perinatal Hypoxic-Ischemic
Brain Injury. Semin Pediatr Neur 1995: 2(1); 57-71.
b. Abend, N and Licht, D. Predicting outcome in children with hypoxic
ischemic encephalopathy. Pediatr Crit Care Med 2008: 9(1); 32-39.
15) Brain Tumors
a. Partap, S and Fisher, P. Updated on new treatments in childhood brain
tumors. Curr Opin Pediatr 2007: 19: 670-674.
b. Pollack IF. Brain tumors in children. New Eng J Med 1994;331:1500-
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