Introduction to Pediatric Neurology b.

Normal values in newborn

Dr. de Sagun July 27, 2006 differ from infants and older
child
Outline: c. ICP can be determined
Approach to a Neurologic Disease d. Bacteriologic tests on the fluid
Congenital CNS Malformation would help in management
Increase intracranial pressure e. C/I in cases with lesions at the
Paroxysmal Disorders site of lumbar puncture
Psychomotor Retardation
CSF Analysis
Pediatric Neuro History Character of fluid: clear, colorless
Chief complaint Cell count: RBC, WBC, differential
HPI count (neutrophils, lymphocytes,
System Review blast cells)
Past Personal History Protein level
Gestational history Sugar level and ratio to blood
Birth history: asphyxia sugar
Immunization Gram stain, culture and antibiotic
sensitivity
Developmental history
IgG values
Past medical history
Viral studies
Family history
General PE 2. EEG
a. Indicated in seizures and
Diagnostic Approach to a Neurologic paroxysmal disorders
Disease b. Determination of brain death
Questions to answer 3. Electrodiagnostic tests
1. Does the child have a neurologic a. EMG
disorder?
A concentric needles is
2. If so, where is the site of the lesion, or
electrode inserted into
does it involve all part of the brain to
the muscle records the
an equal degree? Lateralize, if
action potentials
possible.
generated by muscle
3. What is the lesion? Check course of
activity
the illness (acute, subacute, static or
Indicated in lower motor
remitting)
neuron disease
b. NCV
Nature of the Illness
Determines the
1. Congenital: must have started early conduction rate of motor
on in the age nerves by stimulating the
2. Infectious: acute or accompanied by nerve at 2 points and
fever, dramatic onset recording the latency
3. Developmental: developmental delays between each stimulus
come in and muscle contraction
4. Neoplastic: gradual but progressive Distinguishes between
manifestation of symptoms demyelination and axonal
5. Vascular: acute, reach the peak of degeneration
symptoms immediately 4. Evoked potentials
6. Immunologic a. Visual evoked potential
7. Degenerative (VEP/VER)
8. Nutritional Useful to establish
9. Metabolic vision in children who are
10. Paroxysmal thought to be visually
handicapped or blind
Neurodiagnostic tests b. Brainstem evoked potentials
1. Lumbar puncture & CSF analysis (BAER)
a. Indicated in CNS infections
 Checks the integrity of
the auditory system
5. Neuroimaging
a. Cranial ultrasound
Least invasive, uses
fontanels and opened
sutures as windows
Helpful in perinatal
asphyxia, monitoring
ventricular size
b. CT Scan
Very useful in
delineation of intracranial
structures; utilizes
radiation
Procedure of choice in
acute encephalopathy,
trauma, subarachnoid
bleed, intracranial
calcifications
c. MRI
Does not use radiation;
delineates intracranial
structures
Gray-white matter
delineation is excellent
hence it is useful in
degenerative diseases,
intractable seizures and
unexplained
developmental disabilities
Helpful in spinal cord
lesions
Inferior to CT scan in
intracranial calcifications
and acute hemorrhage
6. Other tests
a. Muscle or Nerve biopsy: for
accurate diagnosis of type of
muscle disorders and nerve
disorders
b. Cerebral angiography: for
vascular disorders
c. Neuropsychological testing
d. Metabolic tests in the urine
and blood
e. Antiepileptic drug assay
f. Muscle enzymes
Congenital Disorders of the Nervous
System
Malformations of the brain and spinal
cord form during the NS development
Intrinsic and extrinsic factors play a role
Etiology of malformations is not known
in 60% of cases
Understanding the normal embryonic
embryonic development is vital for the
understanding and classification of
abnormalities
Common Causes of CNS Malformations
Teratogens
o Physical agents: trauma,
hyperthermia, radiation
o Infectious agents: rubella, HSV,
CMV, mumps, varicella,
treponema, toxoplasma
o Maternal illnesses
o Maternal toxin and drug
exposure
Genetic Conditions
Congenital Defects and the Embryonic
Stages
I. Neural tube formation
Chiari syndrome
Cranioschisis (anterior neuropore)
Anencephaly
Encephalocoele
Meningocoele
Raschischis (posterior neuropore)
Meningocoele
Myelomeningocoele
Spina bifida
II. Segmentation and Cleavage
Basilar impression
Holoprosencephaly
III. Sulcation, Proliferation, Neural Migration,
Organization
Callosal agenesis
Heterotopias
Macrogyria/Microgyria
Neuronal migration defects
Cerebral anomalies
Lissencephaly
Schizencephaly
IV. Myelination
White matter hypoplasia
V. Mesodermal Development
Craniosynostosis
Fibrous dysplasia
NEURAL TUBE DEFECTS (DYSRAPHISMS)
Spina bifida occulta
Midline defect of the vertebral bodies
without protrusion of the spinal cord or
meninges
Usually asymptomatic
Usually at level L5 and S1
No abnormality of the spinal cord,
meninges or nerve roots
 Occasionally with other developmental
disabilities such as syringomyelia,
tethered cord
A dermal sinus tract or opening may be
a cause of recurrent meningitis
Meningocele
Meninges herniate through a defect in
the posterior vertebral arches
Spinal cord usually normal
Fluctuant mass seen in the midline
Surgery if with leaking CSF or if only
thin skin covering
CT scan or MRI prior to surgery
Myelomeningocele
Most severe form of dysraphism
Spinal cord and meninges protrude into
the defect
Etiology: unknown
o Drugs, nutritional, genetics
have been implicated
Clinical manifestation
o Dysfunction of many organs as
skin, gut, skeleton, peripheral
nervous system
May be associated with hydrocephalus
(Chiari Type 2)
Management: multidisciplinary
Encephaloceles
Affect the skull through a midline bony
defect (cranium bifidum)
Cranial meningocele
Cranial encephalocele may be
associated with other defects as
aqueductal stenosis, Dandy-Walker
malformation, or Chiari malformation
Anencephaly
Primitive brain with connective tissues,
hypoplastic pituitary, absent cerebrum &
cerebellum
Death in several days
DISORDERS OF NEURONAL MIGRATION
Lissencephaly or Agyria
Absence of cerebral convolutions and
poorly formed sylvian fissure with
appearance of a 3-4 month fetal brain
With a 4-layered cortex instead of 6
Clinical manifestations
o Failure to thrive, MR, seizure
disorder, chromosomal
features (Miller-Dieker
Syndrome)
o CT and MRI: smooth brain
Schizencephaly
Unilateral or bilateral clefts with the
cerebral hemispheres due to an
abnormality in morphogenesis
Clinical manifestations
o MR, seizures, microcephaly,
spastic paresis
Porencephaly
Presence of cyst or cavities within the
brain
Clinical manifestations
o Often associated with other
brain malformations as
microcephaly, MR, optic
atrophy, seizures
Cysts usually unilateral; usually do not
communicate with a fluid-filled cavity
Holoprosencephaly
Results from defective cleavage
Patients may have a single ventricle,
other malformations
Mortality is high
Microcephaly
Head size > 3SD below the mean for
age and sex
Types
o Primary: genetic
o Secondary: non-genetic
Hydrocephalus
Results from conditions with impaired
circulation and absorption of the CSF or
increased production by a choroid plexus
papilloma
Types
o Obstructive or non-
communicating
o Non-obstructive or
communicating
Clinical manifestations
o Enlarging head with big
fontanel, wide sutures,
prominent scalp veins and
setting sun signs
o Long tract signs
o Irritability, lethargy, vomiting
o Gradual change in personality;
deterioration in academic
performance
Diagnosis
o Complete PE and NE
o Ultrasound
 o MRI/CT scan

Craniosynostosis
Premature closure of the cranial sutures
o Primary: due to abnormalities
in the skull development
o Secondary: failure of brain
growth and expansion
Cause: Primary – genetic abnormalities
Diagnosis: skull x-ray

MANAGEMENT APPROACH
1. Complete history and PE
2. Identify the cause
3. Neurodiagnostics
4. Management: multidisciplinary team
5. Family support

Transcribed by: Jobern Hipol

Slides courtesy of: Faye delos Santos

“All endings are also beginnings. We

just don’t know it at the time…”
– Mitch Albom “The Five People You Meet in
Heaven”