(7) Paediatric Neurology Flashcards
When is the CNS most vulnerable to damage?
during periods of rapid changes such as when the areas of neural network are being formed
What does congenital mean?
present from birth
What are teratogens?
- substances that cause congenital disorders in developing embryo
- anything female exposed to or ingests during pregnancy that known cause foetal abnormalities
What is a cytomegalovirus?
- infectious agent
- if passed onto unborn may cause hearing loss, developmental & learning problems
What are some factors influencing development?
- genetics & metabolic
- chromosomal
- CNS malformations
- dietary
- drugs & toxins
- environment and social factors
- utero infections
- neonatal seizures or hypoxia
What causes genetic abnormalities?
- caused by mutation in a single gene (monogenic), multiple genes (polygenic) or by chromosomal abnormality
When are symptoms of genetic abnormalities usually seen?
- may give rise to neurological disorders from birth but others may not show symptoms for months or years
- normal development followed by regression
What are examples of chromosomal disorders?
- Down’s syndrome (additional chromosome 21)
- Edward’s syndrome (additional chromosome 18)
What is involved in the maturation of the CNS?
- myelination & regional brain maturation
- changes in synaptic density
- changes that occur postnatally including development learning & proprioception
What is the main cause of neurological disorders in childhood?
maldevelopment of the nervous system or as a result of adverse factors during pregnancy & birth
What are the 3 categories of neurological disorders most common in children?
- CP
- Epilepsy
- Complex learning disability
What are commonly encountered neurological conditions?
- CP
- epilepsy
- traumatic brain injury
- spinal injuries
- genetic conditions
- hydrocephalus
What are developmental coordination disorders?
- children w/o TBI or CP who lack motor coordination to perform tasks that most children can
- dyspraxia
- difficulty attending more than “one thing” at a time
- no genetic defect found
- associated with premature birth
What are some neurological disorders of children?
- CNS infection
- Brain tumours
- Serious head injury
- TBI
- ABI (acquired brain injuries)
What are common CNS malformations?
- neural tube defect
- microcephaly (smaller than normal head)
- Chiari malformation
- Dandy Walker Syndrome
What is Dandy Walker Syndrome?
congenital brain malformation involving the cerebellum with enlargement of the 4th ventricle and partial or complete absence of the vermis of cerebellum
What is microcephaly?
- Abnormally small brain
- motor deficits
What is hydrocephalus?
- enlargement of cerebral ventricles due to impaired CSF drainage
- learning & language disorders
What is Lissencephaly?
- smooth brain without normal central gyri
- learning difficulties, hypotonia, epilepsy
What is Porencephaly?
- destructive changes in one cerebral hemisphere
- hemiplegia, epilepsy, learning difficulties
What is cerebellar hypoplasia?
- maldevelopment of cerebellar hemispheres or vermis
- ataxia, hypotonia, disordered eye mvmts
What is chiari malformation?
- cerebellar tonsils protrude through the foramen magnum
- Type 1 asymptomatic, Type 2 occurs with spina bifida
What is spina bifida occulta?
- maldevelopment of lower spinal cord & vertebrae covered by skin
- progressive unilateral foot deformity & weakness, neuropathic bladder
What is Schizencephaly?
- characterized by abnormal slits or clefts in the cerebral hemispheres of the brain
