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.
- 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 production
Concepts essential to the Pediatric neurology trainee
- Brain and spinal cord development / embryology
- CNS plasticity
- Normal infant and child development
- Genetic principles
a. Mendelian genetics
b. Molecular genetics
c. Dysmorphology / syndrome recognition
d. Chromosomal disorders
e. Mitochondrial disorders
- 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
- 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
- Clinical electives
a. Neuro-ophthalmology
b. Neurosurgery
c. Neuropsychology / Psychiatry
d. Neuroradiology
e. Child psychiatry / Neuropsychiatry / Neuropsychology
f. Pediatric Neurorehabilitation
g. Neurogenetic
Basic neurosciences
- Neuroanatomy
- Neurophysiology
- Neuropathology
- Neuropharmacology / Neurochemistry
Applied neurology and neurosciences
- EEG / Evoked potentials
- EMG/NCS; muscle biopsy, nerve biopsy
- Neuroradiology
