Document Sample

                          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

Inpatient Experience
    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
       pediatric/PICU/Heme-Onc/NICU teams

OutPatient Experience
   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
        neurologic examination.
              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
     a. Seizures
              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,
                lamotrigine, topiramate
         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

b. Headache
       1.       Know the clinical characteristics of tension-type and
                migraine headaches
         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,
                including anaerobes
         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
               CNS disorder
        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
              myasthenia gravis

h. Neurodevelopment
        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
              deficit/hyperactivity disorder
        4.    Be aware of the current consensus regarding evaluation of
              global developmental delay

i. Malformations
        1.    Identify the clinical manifestations and plan the diagnostic
              evaluation of spinal dysraphism; know the various types and
              potential complications
        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,
              and complications
        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

k. Miscellaneous
        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
               sinovenous thrombosis
        4.     Recognize the signs and symptoms of and plan treatment for
               Bell palsy
        5.     Know the clinical difference between papilledema and optic
               neuritis (papillitis)
                     Goals Based on Core Competency Teaching


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.


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
       neurologic illness.
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.


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.


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
      religious beliefs.
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
      professional development.
5.    Demonstrate professional respects for superiors, colleagues, students and all
      members of the health care team.


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
      patient care.
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.

Neurology Exam Checklist
         a. Pre and Post Rotation Exam Assessment
                                                                                      YES    NO

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
                       Noon conferences
                       Grand Rounds
                       Case Presentation
                       Literature searches
                       Self study
3. Practice-Based      Clinical teaching rounds         Monthly rotation evaluations
Learning and           Outpatient clinic                Verbal Feedback
Development            Case-related conferences:        Written feedback for presentation
                       AM report
                       Chairman rounds
4. Interpersonal and   Clinical teaching rounds         Monthly rotation evaluations
Communication Skills   Outpatient clinic                Verbal feedback
                       Interactions with students,
                       residents, and ancillary staff
                       Case presentations
                       Effectiveness of charting and
                       clinical notes
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
                       report, conferences
6. Systems-based       Clinical teaching rounds         Monthly rotation evaluations
Practice               Outpatient clinic                Verbal feedback
                       Multi-disciplinary rounds and
                       team meetings
                       Clinical conferences

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
       d. Coma
       e. Headache
       f. Seizures

2) Basic articles, “cheat-sheets” and summary tables, as well as several classic
   background papers.

3) Excellent websites include:
      a. (specifics of essentially all
          neuromuscular symptoms/signs/disorders)
      b. (search for neurogenetic disorders based on
          signs or symptoms)
      c. (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-

  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

  4) Seizures

        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
          Neruol 2008:491-496.
       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
          in Childhood.
       c. Algorithm for Hypotonia and Weakness
       d. Table Etiology of Infantile Hypotonia.

10) Neurodevelopment

       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
          Cerebral Palsy.
       e. Johnson C, Myers, S. Identification and Evaluation of Children with
          Autism Spectrum Disorders. Pediatrics 2007; 120(5): 1183-1215.

11) Malformations
       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.

13) Stroke
        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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