Considerations for advanced training in behavioral child neurology
Shafali S. Jeste, MD,
Sandra L. Friedman, MD and
David K. Urion, MD
From the Departments of Neurology (S.S.J., D.K.U.) and Developmental Medicine (S.L.F.), Children's Hospital Boston, Harvard Medical School, Boston, MA.
Address correspondence and reprint requests to Dr. Shafali Spurling Jeste, Children's Hospital, Boston, Department of Neurology, 300 Longwood Avenue, Fegan 11, Boston, MA 02115 shafali.jeste{at}childrens.harvard.edu.
In this article, we advocate for advanced training for childneurologists in behavior and development in order to facilitatethe investigation of childhood behavioral and neurodevelopmentaldisabilities, with autism serving as a model disorder. We explorethe current training options and then propose alternative subspecialtytraining options that focus on behavior and development, withappreciation that most developmental disabilities are not staticencephalopathies but, rather, dynamic processes representingthe influence of genetics and environment on neural circuitry.
Both the incidence and recognition of developmental disabilitiesare growing, with an estimated 9 million children and adolescentsaffected with autism spectrum disorders, global developmentaldelay, or mental retardation.1 Due in large part to increasedawareness and earlier diagnosis, the incidence of autism hasrisen substantially in the past decade, with current estimatesbeing 1 in 150 children.2 As evidenced by the increase in NIHfunding and media coverage, autism has become a major publichealth issue. Extensive efforts are being made to understandits neurobiology in order to create informed therapies.
As a new generation of child neurologists is trained, the autismfield is turning heavily toward advanced neuroimaging and genetics.Such technological advances, while invaluable, must be matchedwith sophisticated phenotyping grounded in behavior and development.We submit that well-trained child neurologists with a solidfoundation in behavioral neurology would be well-equipped todevelop this type of rigorous phenotyping. Coupling clinicalobservation with an understanding of functional neuroanatomy,behavior, and normal brain development, child neurologists coulddefine phenotypes informed by functional neural networks, which,in turn, would enable us to design effective therapies for thesechildren.
In this article, we advocate for advanced training for childneurologists in behavioral child neurology in order to facilitatethe investigation of childhood behavioral and developmentaldisabilities, with autism serving as a model disorder.
The concept of approaching autism from a neurologic perspectiveis by no means a novel one. In the 1970s, Damasio and Maurer3took a traditional, lesion-based approach in their analysisof the behavioral and motor disturbances in children with autism.Based on their observations, they wrote a seminal paper in Archivesof Neurology entitled "A neurological model for childhood autism."3
An elegant example of their approach lies in their account ofthe motor disturbances, characterized as "dystonia, bradykinesiaand hyperkinesias and involuntary movements," many of which,they concluded, seemed rooted in the striatum and its connectionsto the frontal lobe. They provided critical observation thatthe areas affected "constitute the entire area of terminationof the dopaminergic neurons arising in the mesencephalon."3
Since the localization efforts of Maurer and Damasio, severalclinicians and scientists have investigated autism from a childneurology perspective. Through their work, and that of theircontemporaries, we have learned much more about the neurologiccomorbidities of autism, such as epilepsy, and have better definedautistic regression as distinguished from Landau Kleffner syndrome.We also have learned that several neurologic disorders are associatedwith a higher incidence of autism, such as tuberous sclerosiscomplex and fragile X,4 and that behavioral syndromes, suchas obsessive compulsive disorder and attention deficit hyperactivitydisorder, occur at higher rates in children with autism.5 Since2000, 30 original articles about autism have been publishedin Neurology®, and over 200 in the Journal of Child Neurologyand Pediatric Neurology.
Clearly, the precedent has been set for child neurologists totake initiative in the investigation and care of autism spectrumdisorders, but our training must expand upon these efforts.We propose 3 areas of investigation in which child neurologistscan make contributions to this field: 1) creation of neurologicallybased endophenotypes; 2) analysis of early behavioral markersthat precede a formal diagnosis; and 3) understanding of thedevelopmental trajectory of autism, focusing on late life sequelae.
Several examples of neurologic endophenotypes can be given.First, neuropathologic and imaging studies have indicated abnormalitiesin cerebellar volume and structure in children with autism.6In this subgroup, one could ask whether there is evidence ofhypotonia, ataxia, or speech impairments attributable to cerebellarcircuits. Second, per Maurer and Damasio, one could ask whetherchildren with more prominent movement disorders show evidenceof abnormalities in dorsal striatal circuitry as visualizedin functional or structural imaging. Perhaps dopamine receptoror transporter genes show mutations that could further refinethis clinical profile. Finally, one could ask whether childrenwith autism with specific EEG abnormalities share a common behavioralphenotype, thereby providing insight into common aberrant neuralcircuitry.
The 2 other areas of investigation (early markers and late sequelae)speak to the importance of understanding autism from a developmentalperspective. Autism is not a static encephalopathy, but, rather,a dynamic process that begins in early brain development andcontinues throughout childhood and possibly later. Observationalstudies reveal that children with autism can exhibit deficitsin social interaction, language, and motor skills in the firstyear of life, well before a formal diagnosis is made.7 Adultswith autism often cannot live or work independently, and havea high rate of comorbid psychiatric and behavioral disorders.Furthermore, early childhood language ability is an importantpredictor of independent functioning in adults with autism.8This dynamic process is substantiated by studies showing dysregulationof brain growth based on head circumference measurements inchildren with autism.9
Child neurologists who can understand behavior in the contextof development can investigate autism across a lifetime, fromearly infancy into adulthood. Through this process, one couldask questions about early precursors of autism, predictors ofspecific phenotypes, and association of early behavioral andneural markers with late life prognosis. This approach couldfacilitate the creation of interventions that might modify thedevelopmental progression of autism.
We are using autism as a model disorder, but these suggestionscould easily be applied to other, later-onset neurobehavioraldisorders, such as schizophrenia, in which early childhood behavioralor cognitive markers may exist.
Currently, child neurology training consists of 2 years of generalpediatrics, 1 year of adult neurology, and 2 years of childneurology. There is a required elective in child and adolescentpsychiatry, and most programs offer electives in neuropathologyand neuroimaging. While residents gain some exposure to behavioraldisorders through outpatient clinics and didactics, there isno formal training in behavioral disorders, psychopathology,or development. In order to gain formal advanced training inbehavioral disorders, child neurologists must pursue a fellowshipin adult behavioral neurology, for which there is an AmericanBoard of Psychiatry and Neurology (ABPN) board certification.
There also exists an accredited Neurodevelopmental Disabilities(NDD) residency which focuses on the longitudinal managementof patients with neurodevelopmental disorders. This 6-year residencyprogram was created through joint efforts of the American Boardof Pediatrics and the ABPN and consists of 2 years of pediatrics,1 year of adult neurology, 18 months of clinical child neurologyand developmental disabilities, and 18 months of "clinical andbasic science" including electives in child psychiatry, neurosurgery,neurorehabilitation, and dedicated research time. Residentsare eligible for certification in Pediatrics, Neurology withSpecial Competence in Child Neurology, and NeurodevelopmentalDisabilities.10 This training is limited to 7 established NDDprograms across the country (www.acgme.org).
We envision 2 viable options for training in behavior and development.The ABPN currently provides no mechanism for child neurologiststo integrate into the NDD training. Therefore, one advancedtraining option would be to allow those who have completed theirchild neurology residency to spend 12–18 months in anNDD program completing its training requirements, with focuson research and clinical electives relevant to developmentaland behavioral disorders. This option would be particularlyrelevant for those who decide to pursue this area of interestafter beginning child neurology training. These neurologistscould sit for both Neurology and NDD boards.
An alternative option would be to create an accredited fellowshipin behavioral child neurology, ultimately with board certification.A 1-year fellowship in behavioral child neurology would includea clinical, didactic, and research component, promoting theunderstanding of behavior from a developmental perspective.Clinical focus would be placed on children with behavioral anddevelopmental disabilities. As with NDD training, fellows wouldbe given clinical instruction in advanced neuropsychologicalassessments. Didactics would include lectures on normal childdevelopment, psychopathology, childhood behavioral disorders,and psychopharmacology. Advanced instruction in neuroimaging,electrophysiology, or genetics would be provided based on anindividual's interests.
Both of these training options would differ from the DevelopmentalBehavioral Pediatric fellowship training, as the latter is apediatrics subspecialty not focused on neurology or neuroscience.Some of the behavioral issues emphasized in the DBP training,such as encopresis and attachment disorders, are not basic toneurology training and would not be a focus in our proposedtraining paths.
Finally, we emphasize that all child neurology trainees wouldbenefit from some additional training in neurodevelopmentaldisorders, particularly as these disorders become more widelyrecognized and diagnosed. Our proposed training options wouldprovide added benefits to all child neurology residents becauseof stronger available clinical programs and faculty involvementin development and behavior within child neurology programs.
It is time for child neurologists to make scientific contributionsto this growing field of neurobehavioral and developmental disorders,with rigorous training opportunities to be able to do so. Armedwith knowledge of development, behavior, and clinical neurology,behavioral child neurologists and their NDD counterparts canmake innovative, clinically relevant contributions to the assessmentand care of children with developmental disabilities and, inthe process, regain expertise in the neurology of behavior.
Dr. Jeste serves as an editorial team member for the Neurology®Resident and Fellow Section. Dr. Urion has received travel expensesand honoraria for other activities not funded by industry; isan editor of the Journal of Religion, Disability, and Health;receives royalties from publication of Compassion as a SubversiveActivity (Rowman and Littlefield, 2007); and served as an expertwitness for a Cleveland Clinic defendant. Dr. Friedman has receivedtravel expenses and speaker honorarium for activities not fundedby industry.
Disclosure: Author disclosures are provided at the end of thearticle.
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