Pediatric Neurology

Pediatric Neurology
Topics
* Lecture 1
o Clinical duties of neurologists and pediatric neurologists
o Neonatal and developmental neurobiology (normal development and congenital malformations of the CNS)
* Lecture 2
o Developmental disorders (cerebral palsy, mental retardation)
o The Floppy Infant: Hypotonia

Developmental Milestones
CATEGORY EXAMPLE
Gross motor sitting, walking
Fine motor manipulate toys, utensils
Personal-social play, imitate housework
Language speech acquisition
Normal Motor Function
Abnormal Motor Function
Developmental Case #1
Physical Findings
CT Scan
Pathology and Pathophysiology
Diagnosis
* Obstructive hydrocephalus, due to stenosis of the cerebral aqueduct
* Other congenital causes
o Chiari malformation
o Dandy-Walker malformation
* Acquired causes
o Post-meningitis
o Post-traumatic
o Tumor
Hydrocephalus Treatment
Prognosis
* Cognitive and motor handicaps
* Complications of shunt system
o Shunt failure
o Shunt infection
* Epilepsy
Developmental Case #2
Fetal Ultrasound
Fetal Autopsy
Diagnosis
Developmental Pathology
Prognosis
Related Conditions
Encephalocele
Anencephaly
Prevention:
Folic Acid

Pediatric Neurology.ppt

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CNS Malformations

CNS Malformations
By:SCOTT KULICH, M.D., Ph.D.
RAFAEL MEDINA-FLORES, M.D.
RONALD L. HAMILTON, M.D.
Division of Neuropathology

Neural tube defects
Anencephaly
Failure of closure of the anterior neuropore
Common malformation
Frog-like facies
Area cerebrovasculosa
Underdeveloped hypothalamus
Adrenal cortical hyperplasia
Multifactorial-Folic acid supplementation
Anencephaly
SPINA BIFIDA
* myelomenigocele occulta meningocele
* Sacral dimple: dermal sinus track with spina bifida
Myelomeningocele
* Herniation of malformed cord + meninges through vertebral defect
* Usually associated with Arnold-Chiari and hydrocephalus
* Lumbosacral level most common
Chiari II (Arnold Chiari)
* Cerebellar tonsillar herniation
* Small posterior fossa
* Extension of medulla below foramen magnum
* Kinking of medulla (Z-formation)
* Beaking of the quadrigeminal plate
* Hydrocephalus
* Myelomeningocele
* Cerebellar tonsillar herniation
* Small posterior fossa
* Extension of medulla below foramen magnum
* Kinking of medulla (Z-formation)
* Beaking of the quadrigeminal plate
* Hydrocephalus
* Myelomeningocele
Chiari I Malformation
Dandy-Walker Malformation
* Dandy-Walker syndrome
o Agenesis of cerebellar vermis
o cystic dilatation of 4th venticle
o enlargement of posterior fossa
o Variable clinical manifestations
o Hypothesized to result from arrest of cerebellar development prior to the 3rd month

CEREBELLAR MALFORMATIONS: VERMIAN (PALEOCEREBELLUM)
* Joubert syndrome
Holoprosencephaly
Encephalocele
AGENESIS OF CORPUS CALLOSUM
MICROENCEPHALY
MEGALENCEPHALY
CORTICAL DYSPLASIA
Polymicrogyria
TUBEROUS SCLEROSIS
Sturge-Weber Disease
Port-wine stain or nevus
Flammeus tuypical of Sturge-Weber, occurring in V1 distribution.

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Sexual Development

Sexual Development

UROGENITAL SINUS & TUBERCLE
VULVA
UTERUS
OVARY
VAGINA
UTERINE TUBE
MULLERIAN DUCT
SEXUAL DEVELOPMENT
PROSTATE
PENIS
SEMINAL VESICLE
RETE TESTIS
TUBULUS RECTUS
EFFERENT DUCT
EPIDIDYMIS
DUCTUS DEFERNS
BULBOURETHRAL GLAND
urethra
INTERSTITIAL CELLS
SEMINIFEROUS TUBULE
TESTIS
WOLFFIAN DUCT
MESONEPHRIC DUCT
PARAMESONEPHRIC DUCT
GONAD on hold
OVARY
TESTIS
INTERSTITIAL CELLS
SEMINIFEROUS TUBULE
TUBULUS RECTUS
OVARY
GONAD on hold
Sex-determining Factor
Default pathways
Testosterone
Mullerian-inhibiting Factor
MULLERIAN DUCT
WOLFFIAN DUCT
UROGENITAL SINUS & TUBERCLE
Driven pathways
SERTOLI CELL
OVARY
GONAD on hold
Default pathway
and much more topics are covered

Sexual Development.ppt

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