Fellowship In Pediatric Neurology

Pediatric neurology is an upcoming pediatric sub-specialty and there are few institutes which offer training in this sub-specialty. Objective of the course is to provide broad understanding of the principles of Neurology particularly pediatric neurology. Fellows will be trained in Skills in the clinical diagnosis, and management of common conditions in pediatric neurology by relevant current therapeutic methods. Candidate will learn the skills to perform and interpret investigative procedures related to child neurology, take independent decisions in emergency situations and perform required procedures in Pediatric neurology and manage complications.Fellows will gain competence in intensive care with practical knowledge of working with resuscitation and monitoring equipment, to critically appraise published literature, interpret data and to broaden his/her knowledge by keeping abreast with modern developments in pediatric neurology, ability to search online, use information technology to his/her advantage and critically evaluate medical literature and draw his/her own conclusion.

Basic knowledge of allied and general clinical disciplines to ensure appropriate and timely referral and ability to conduct research. Fellows will be paid stipend during the one year training period and there is no fees for this program.

Preliminary comments:

Specific areas that need to be addressed because many feel inadequately prepared in these
Areas: behavioral and developmental disorders (ASD and CP and mimickers thereof, psychosomatic disorders, disorders of higher cortical function, neuropsychological testing).

The Medicolegal interface will affect all trainees, so education about and exposure to the many aspects of this interface are essential (expert witness, risk minimization, confidentiality issues, interaction with hostile patients and their attorneys, etc).

A. Clinical

The clinical content of the curriculum should facilitate learning clinical child neurology in the broadest sense. That is, trainees should be exposed to and be responsible for patients with a comprehensive and representative variety of neurological disorders. There should be a concerted effort to correlate clinical information with relevant applied and basic science information.

1. Patient-oriented approach to neurological disorders

a. History taking

The patient history is the cornerstone of diagnosing and treating neurological disorders. Many such disorders are not observed by the physician because episodes are intermittent or complaints are subjective. Furthermore, the circumstances preceding and following certain events are important for fully understanding the event or symptom. Therefore, the child neurologist must be a consummate historian.

b. Neurological and developmental examination

Many neurological symptoms are accompanied by signs observable to the careful examiner. In fact, patients are often unaware of physical abnormalities that yield clues to a diagnosis. Thus, a careful physical and neurological examination is an essential extension of the history that must never be overlooked or downplayed. The neurological examination is often more sensitive than any ancillary diagnostic procedure in localizing lesions and determining diagnoses. Alsoa cerfully conducted neurodevelopmental assessment helps chart the further progress of investigations and helps in counseling the parents properly. Complete familiarity with the following is essential:

1) Higher cortical function (normal, confusion, delirium, dementia)
2) Cranial nerves
3) Motor function\
4) Sensation
5) Reflex function
6) Gait and stance
7) Special circumstances

a) The comatose patient
b) The psychiatric patient
8) Developmentally appropriate application of above principles to infants and children of all ages

c. Lesion (anatomic) localization and pathophysiological correlation

The logical result of a careful history and physical examination, lesion localization is of the utmost importance in leading the clinician toward a reasonable differential diagnosis and formulation of a plan for ancillary diagnostic procedures, if necessary.

Understanding normal neurophysiology is essential to explaining the basis of disease. At minimum, working knowledge of the anatomy, connectivity, and physiology of the following is essential:

1) Motor system (motor unit and corticospinal tract)

a) Differentiate between disorders causing weakness, in coordination, and involuntary movements
b) Differentiate between upper motor neuron and lower motor neuron dysfunction by using the distribution of weakness, muscle bulk, muscle tone, muscle strength, fasciculations, sensory changes, and reflex changes
c) List the components of the motor unit
d) Compare and contrast the common LMN clinical syndromes involving motor neuron, peripheral nerve, neuromuscular junction, and muscle by symptoms of weakness, muscle tone, muscle strength, fasciculations, sensory changes, reflex changes, and muscle enzymes.
e) Differentiate between the common UMN syndromes of hemiparesis, paraparesis, and quadriparesis by distribution and define and discuss the pathophysiology of:

Spasticity

Superficial and deep reflexes

Tone

Apraxia

Ataxia

f) Differentiate between UMN and LMN facial weakness (Bell palsy)

2) Basal ganglia

Define and differentiate involuntary movements:
Tremor (resting, postural, action)
Spasticity vs. rigidity
Hyperkinetic movement disorders
Chorea, athetosis, hemiballismus
Dystonia
Myoclonus
Tics
Hypokinetic movement disorders

3) Cerebellum

Discuss the clinical findings and pathophysiology for midline vs. hemispheric cerebellar disorders:
Define ataxia, dysmetria, dysdiadochokinesia, titubation, rebound
Define wide-based gait and discuss anatomical localization

4) Sensory pathways

a) Differentiate between central and peripheral sensory disorders by distribution, modalities affected, associated findings, and the presence or absence of pain

b) Describe the following sensory disorders and discuss localization:

Cortical sensory loss
Hemihyperesthesia
Thalamic pain
Sensory level
Brown-Sequard syndrome
Dissociated sensory loss

c) Describe these peripheral sensorimotor disorders and discuss localization:

Radiculopathy
Mononeuropathy
Anesthesia, hypesthesia, paresthesia, dyesthesia

5) Cranial nerves

6) Hypothalamus and pituitary

7) Limbic system

8) Cerebral cortex

9) Visual system

Localize the lesion causing:

Homonymous hemianopsia (congruent, non-congruent)

Bitemproal field defect
Superior and inferior qadrantanopsia
Central scotoma Enlarged blind spot Afferent papillary defect

b) Differentiate between papilledema and optic neuritis

c) Describe innervation and action of each of the extraocular muscles

d) Describe nystagmus (jerk, pendular)
Evaluate ptosis, including Horner’s syndrome

10) Auditory system

11) Autonomic system

12) Cerebrospinal fluid pathways

13) Neurophysiology of the above systems including understand CNS neurotransmission; neuromuscular transmission; muscle contractile processes; neuronal excitation, inhibition, and release; cortical activation and inhibition; seizure productio

Concepts essential to the Pediatric neurology trainee

1. Brain and spinal cord development / embryology
2. CNS plasticity
3. Normal infant and child development
4. Genetic principles
   a. Mendelian genetics
   b. Molecular genetics
   c. Dysmorphology / syndrome recognition
   d. Chromosomal disorders
   e. Mitochondrial disorders
5. Brain death and the persistent vegetative state in infants and
   children

d. Formulation of differential diagnosis

The goal of obtaining a thorough history, performing a detailed physical examination, and localizing the lesion is establishing a differential diagnosis. This carefully – prepared list of diagnostic possibilities directs the clinician toward a rational plan for using ancillary diagnostic procedures, if necessary, to include or exclude specific disorders on the differential list.

e. Evaluation and management plan

Treating patients with neurological disorders is the primary goal of a practicing clinical pediatric neurologist. The trainee should learn the appropriate standard of care for neurological disorders and should constantly be vigilant for evolution of thinking and practice regarding treating these disorders. This requires continuous learning, and it requires sufficient practical experience with patients (including explaining these concepts of diagnosis and treatment to patients and to their families).

In addition to being thoroughly competent in the art and science of history and physical examination in formulating an evaluation and management plan, the trainee should also e completely familiar with the indications, techniques, contraindications, and risks of the following neurodiagnostic tests:

a) Lumbar puncture
b) EEG
c) CT
d) MRI and MRA
e) EMG and NCS
f) Visual, auditory, brainstem and somatosensory evoked potentials
g) Cerebral and spinal angiography
h) Nerve and muscle biopsy

f. Specific symptoms (the patient-oriented approach)

1) Paroxysmal disorders

Distinguish:

• seizures from syncope
• jitteriness from seizures
• seizures and epilepsy
• simple from complex febrile seizures

Understand international classification of seizures and epilepsies
List common causes of seizures in:

neonates
infants
older children

e) Describe routine evaluation and treatment indications in new onset seizure

g) Define status epilepticus

• outline initial evaluation and management
• list medications and doses to treat status

h) Sleep disorders

• define parasomnias, narcolepsy, cataplexy, and sleep apnea

2) Coma and altered consciousness

Describe major disease categories that cause lethargy and coma (e.g., metabolic, infectious, traumatic, vascular etc.)

3) Increased intracranial pressure

a) Describe differences between communicating and noncommunicating hydrocephalus and give etiologic examples of each
b) Discuss side effects of ventriculoperitoneal and ventriculoatrial shunts
c) Describe the most common brain tumors in children
d) Discuss the presentation of supratentorial and infratentorial brain tumors
e) List the most common organisms causing bacterial meningitis in neonates and children
f) List factors commonly predisposing to pyogenic brain abscess in children
g) Discuss Idiopathic raised ICP in children
h) Discuss metabolic and toxic causes of increased ICP
i) Discuss treatment of acute and chronic increased ICP

4) Ataxia and other gait disorders

Discuss differential diagnosis, evaluation, and management of acute and subacute ataxia in children

5) Movement disorders

a) Discuss differential diagnosis of chorea,
b) List medications that can cause movement disorders
c) Define Tourette syndrome, comorbid associations, and treatment

6) Headache

a) Describe the headache features (onset, location, character, duration, precipitants, associated symptoms, and family history) of migraine, and other headaches
b) Be familiar with the International Classification of Headache criteria
c) List indications and medications for headache treatment

7) Intellectual disability

a) Discuss normal motor and cognitive development
b) Discuss consequences of tobacco, alcohol, and other commonly abused drugs (marijuana, cocaine and heroin)
c) Discuss evaluation and treatment
d) Discuss common manifestations of neurofibromatosis and tuberous sclerosis

8) Cognitivel and motor regression

Be familiar with :

a) Lysosomal storage disorders
b) Peroxisomal disorders
c) Mitochondrial disorders
d) Aminoacidopathies
e) Organic acidopathies
f) Disorders of carbohydrate metabolism
g) Chromosomal disorders
h) Dysmorphic syndrome

9) Weakness (including peripheral, central, and weakness caused by cranial nerve
dysfunction)

Be familiar with these peripheral nervous system disorders:

a) Spinal muscular atrophies
b) Duchenne muscular dystrophy
c) Myasthenia gravis
d) Acute inflammatory demyelinating polyneuropathy
e) Peripheral neuropathy (hereditary and non-hereditary)

Be familiar with the following central causes of weakness in children:

a) Stroke
b) Cerebral palsy
c) Leukodystrophies
d) Discuss significance of sacral dimple, hairy patch, port wine stain

Be familiar with disorders of cranial nerve function:

a) Discuss causes of facial weakness and evaluation and treatment of Bell’s palsy

10) Disorders of sensation (including somatosensory, discriminative, position,
vibration, smell, hearing and taste; peripheral and central causes)

a) Discuss evaluation of child with hearing loss
b) Discuss evaluation of vertigo

11) Visual disorders

a) Discuss congenital nystagmus and spasmus nutans
b) List causes for congenital cataracts
c) Describe several causes of acquired ophthalmoplegia
d) Discuss the meaning of optic atrophy
e) Discuss causes of strabismus

12) Hearing disorders

13) Abnormalities of head growth

a) Discuss causes and evaluation of macrocephaly and microcephaly
b) Discuss craniosynostosis

14) Disorders unique to newborn infants

15) Learning disorders and disorders of higher cognitive function

a) List common causes of learning disabilities
b) Discuss approach to a child with

• delayed speech
• impaired attention
• poor academic performance

16) Speech and language disorders
17) Behavioral disorders
18) Genetic Disorders

2. Categories of disease and specific disorders

a. Neurological disorders of childhood

Common disorders including various types seizures and epilepsy syndromes, non-epileptic paroxysmal disorders, headache, learning/developmental/cognitive disorders, disorders causing intellectual disability, neuromuscular disorders, acute encephalopathies, infections of the nervous system, disorders of the term and preterm infant, neurotrauma, complications of systemic disease (heart, kidney, lung, liver etc), and neurogenetic and neurometabolic disorders, etc should make up the bulk of patients  More common disorders should be proportionately represented, but attempts should be made to familiarize the trainee with less common disorders to prepare him or her for the consultative role as a child neurology specialist. Case conferences, clinico-pathological correlations, and reading should be a major adjunct to seeing patients and should expand the trainee’s knowledge of both common and less familiar disorders. The following list of specific disorders is in reality a list of categories of specific disorders. No attempt will be made to name specific disorders; as such a list would be exhaustive and would invariably omit disorders as important as the ones listed. The trainee, however, is expected to be exposed to an exhaustive number of different specific disorders representing the broad spectrum of conditions seen in a child neurology practice.

• Specific categories of disorders
• Disorders of brain and spinal cord development
• Disorders unique to infants (neonatal neurology)
• Infections involving the nervous system
• Cerebrovascular disorders
• Cerebral palsy
• Syndromes associated with mental retardation
• Chromosomal disorders affecting the nervous system
• Metabolic and neurodegenerative diseases
• Nutritional and toxin-associated disorders of the nervous system
• Neurocutaneous syndromes
• Neoplasms of the nervous system
• Neuroendocrine disorders
• Seizures, epilepsy, and epilepsy syndromes
• Nonepileptic paroxysmal disorders
• Sleep disorders
• Movement disorders
• Headache
• Neuromuscular diseases
• Disorders of the autonomic nervous system
• Disorders of learning and behaviour
• Spinal cord disorders
• Neurological complications of systemic disease
• Brain injury, brain death, coma and the persistent vegetative state

3. Clinical electives

a. Neuro-ophthalmology
b. Neurosurgery
c. Neuropsychology / Psychiatry
d. Neuroradiology
e. Child psychiatry / Neuropsychiatry / Neuropsychology
f. Pediatric Neurorehabilitation
g. Neurogenetic

Basic neurosciences

1. Neuroanatomy
2. Neurophysiology
3. Neuropathology
4. Neuropharmacology / Neurochemistry

Applied neurology and neurosciences

1. EEG / Evoked potentials
2. EMG/NCS; muscle biopsy, nerve biopsy
3. Neuroradiology

Essential to child neurology training is acquiring basic science information as it applies to the nervous system and becoming familiar with the “classical” approach to clinical neurology, which is best, acquired by seeing adult patients. So, the first 6 months of training should be devoted to the following disciplines:

Neuroanatomy / Neuropathology* – ongoing

Primary early emphasis on basic microanatomy with some clinic pathological correlations and brain cutting. Combining these two disciplines is a suggestion that could be modified to fit the university and departmental structures.

Neurophysiology / EEG / EMG* /PSG – ongoing

Primary early emphasis in the first month on basic neurophysiology / theory with introduction to applications using these techniques. This introduction will be supplemented later in the curriculum, and could be further enhanced with fellowship training.

Neuropharmacology / Neurochemistry – ongoing

The above list of basic science disciplines is the foundation for understanding neurology. A series of formal rotations in electives or “selectives” designed to provide detailed exposure to neuroanatomy, neurophysiology, and neuropathology will be included in the first 6 months of the curriculum. Neuropharmacology and neurochemistry might be learned best through formal, regularly scheduled didactic seminars that make up a part of the ongoing conference schedule suggested as an integral part of the one-year curriculum. Basic science exposure should begin early in the trainee’s one-year curriculum to enhance understanding of diseases affecting the nervous system. Correlating basic science and clinical information would be a desirable method for learning, so early exposure to clinicopathologic, clinico-physiologic and clinico-anatomic correlations would be ideal. Such correlations could occur with actual and hypothetical patients. The above time recommendations are suggestions and could be modified to fit what is most compatible with the local university and departmental structures.

Clinical adult neurology – 1 month

Clinical Pediatric neurology (5 months)

In patient
– Primary neurology service
– Consultation

• Outpatient

• Clinic

– Child neurology –2 day per week
• Emergency department consultation

While caring for patients, the trainee should also be exposed to all pertinent ancillary diagnostic procedures, including lumbar puncture, EEG/evoked potentials, EMG/NCS, neuro-CT/MRI/angiography/ultrasonography.

Because continuity of clinical care is essential to learning and to the practice of medicine, the child neurology trainee will also have regular continuity clinics in child neurology during the first 6 months of training and during the second 6 months of training areas requiring a team approach to the patient (pediatric rehabilitation medicine, developmental/behavioral pediatrics).

The ultimate goal of this curriculum is to train child neurologists for the practice of pediatric neurology. Essential to this purpose are expertise in all aspects for clinical child neurology itself and proficiency in performing and interpreting necessary ancillary diagnostic procedures, including lumbar puncture, EEG/evoked potentials, EMG/NCS, and possibly neuroimaging studies.

• Inpatient – inpatient experiences should not preclude outpatient activity
• Routine
• Intensive care
• Newborn nursery
• Neonatal intensive care
• Outpatient – except for intensive electives, should occur concomitantly with in patient experience
• Emergency department (evaluating acutely ill children with neurologic symptoms and signs) – this is not viewed as an outpatient clinic for routine, non-urgent complaints
• General child neurology continuity clinic – 1/2 days per week
•  Clinic requiring or benefiting from a team approach to patient management – ½ day clinics once or twice per month

1. Neuromuscular disease
2. Neurorehabilitation
3. Developmental disorders
4. Behavior disorders / child psychiatry / neuropsychology

Participation in outpatient clinics should not preclude ongoing involvement in inpatient and consultative child neurology activities. In other words, the clinics (behavioral/developmental/rehabilitative/neuromuscular, etc) should not dilute the remainder of the clinical child neurology experience. These outpatient activities should enhance – not dilute – the rest of the experience. They should be performed in addition to not in place of the other activities. If, however, an inpatient activity on the general child neurology service is too busy to allow time for these other areas, special attention should be given to provide time away from inpatient responsibilities for the trainee to obtain essential experience in these outpatient areas.