pediatrics Flashcards
when does the nervous system first appear
about 21 days of gestation
when does neural tube closer occurs
at 23-25 days from anterior to posteior
what are the 3 primary brain vesicles from
-prosencephalon
-mesencephalon
- phrobencephalon
by week ___ there are 5 vesicles
5
what is another word for neural tube defects
dysraphism
what is the diagnosis and prevention for dysraphism (neural tube deficits)
Diagnosis: ultrasound, alpha-fetal protein
* Prevention: Folate
is the dysraphism
disorders of neural tube closure
anterior neurpore open > cranial dysraphism
what is fatal , absent brain with associated skull defects like dysraphism
anencephaly
what is spinal dysraphism
spina bifida
what are the 3 different spina bifida
-occulta
-meningocele
- myelomeningocele
what type of spina bifida is
- vertebral arch defect only, up to 10% of population
-Dura and arachnoid herniation through vertebral defect
-herniation of spinal cord and meninges through defect
occulta
meingocele
myelomeningocele
if a pateint has a turf of hair on their low back what can we maybe suspect
spina bifida occulta
what are the symptoms for spina bifida occulta and where is it most common
usually asymptomatic
common at L5-S1
skin abnormality may be present over defect
what kind of deficits foes myelomeningocele have
myelopathy and/or radiculopathic
what is usually associated with myelomeningocele and what is there a later risk for
associated with hydrocephalus and later risk fro tethered cord syndrome
what is a prevention of normal cephalad movement of conus medullaris , child or adult develops progressive neurologic dysfunction due to traction on cord and/or nerve roots and is most common is unilateral lower motor neuron dysfucntion in one leg
tethered cord
what symptoms is most common in tethered cord
unilateral lower motor neuron dysfucntion in one leg
what can you see in the arnold chiari malformation on the CT/MRI scan
you can see the cerebellum going below the skull
what is a Congenital anomaly of hindbrain, downward elongation of brainstem and cerebellum into cervical portion of spinal cord
arnold chiari malformation
what is type 1 , 2 and 3 arnold chiari malformation
o Type I: cerebellar tonsils displaced > 6mm
o Type II: associated myelomeningocele.
o Type III: associated encephalocele
what are the symptoms for arnold chiari malformation
- early hydrocephalus
-later with cerebellar , medullary or CN signs
what is the treatment of arnold chiari malformation
closure of myelomeningocele and placement of shunt to relieve hydrocephalus
– Cerebellar vermis developmental anomaly with** large cyst** in posterior fossa
– 50% with associated mental retardation
– Hydrocephalus common
– Spastic diplegia from associated malformations
what is this
dandy walker malformation
dandy walker malformation .. there is 50% associate with what
associated mental retardation
what is common with dandy walker malformation
hydrocephalus
Ventriculo-peritoneal shunt for Treatment of _____
Hydrocephalus
what is microcephaly
small size of head (idiopathic, chromosomal, toxic)
what is Macrocephaly
Large head (hyrdrocephalus, genetic, gigantism)
what is Craniostenosis
skull sutures fuse early, altering the shape of the head
what are domains for the developmental domains
– Gross Motor
– Fine Motor
– Expressive Language
– Receptive Language
– Socialization/Adaptive Behaviors
what is the developmental quotient
developmental age/chronological age
what is global developmental delay
Delay in 2 or more domains
by 12 months waht shoudl a baby be doing
walking and first words
when should a baby be able to sit unsupported
6 month
when shoudl a baby be able to put words together and ride a tricycle
2 years and 3 years
what is the denver 2 scale used for
check milestone in kids
what is considered a vital sign for babies and is very important for measure of brain growth
head growth
what are the 5 normal primitive reflexes for a bay
- moro reflex (baby puts hands out if pretend like dropping them)
-tonic neck reflex (baby will turn head to side of stretched out arm) - step reflex (baby will act like stepping when held up)
-crawl reflex (baby will try to crawl when places on tummy - grasp reflex (baby will grasp ur finger if placed in palm)
what are first 7 red flags for a baby
- Head size crossing two percentiles
- Failure to respond to sounds
- Fisting beyond 3 months
- Rolling before 3 months
- Persistent MORO** >6months**
- Persistent asymmetric tonic neck reflex, not rolling > 7 months
- Not sitting>9months
6, red flags continued ….
- Delayed smiling (>1 mo.), laughing (>2-3 mo.), cooing (>3-4 mo.), first words (>12 mo.)
- Handedness below 12 months
- No words by 2years
- Persistent echolalia >3yrs.
- Noticeable stutter after 4 years
- At any age: decline in speech ability or vocabulary
what does IDEA define this as “significantly sub-average general intellectual functioning, existing concurrently with deficits in adaptive behavior and manifested during the developmental period, that adversely affects a child’s educational performance.”
intellectual disability
is delay or regression worse and why
regression bc that is saying u learned something and then regressed backwards and loss the milestone
what are causes of predominant speech delay
– Multilingual family
– Hearing loss
– Infantile Autism
what are causes of Predominant Motor Delay:
– Ataxia
– Hemiplegia
– Hypotonia
– Paraplegia
if a kid has 3 copes of trisomy 21 what syndrome do they have
down syndrome
- 1:700 live births; Male to Female ratio 3:2
- Risk increases sharply with advanced maternal age (>age 35)
- 2% risk of recurrence
- Associated with:
– Typical facial features
– skeletal anomalies
– infantile hypotonia
– Intellectual disability
– Seizures
– congenital cardiac defects – GI structural defects
what is this
down syndrome : trisomy 21
what is down syndrome associated with
- typical facial features
- skeletal anomalies
-hypotonia - intellectual disability
-seizures
-cardiac defects
-GI defects
if a kid presents with a shirt stature , stubby finger and toes , increased space between 1st adn 2nd toes, small pelvis , inward curvature of 5th finger and antlatoaxial instability (AAI) of cervical spine what can we seuspect
down syndrome
what is the most common chromosomal cause of intellectual disability and is MOST common in MALES
fragile x syndrome
what is Caused by tri-nucleotide repeat expansion in the FMR1 gene and Clinical:
– IQ 40-55, Attention deficit and hyperactivity, seizures
– Long face, enlarged ears, macro-orchidism, hyperextensible joints, low muscle town, high arched palate
fragile x syndrome
what is Toxic Cause Developmental Delay:
fetal alcohol syndrome
what are infectious causes of developmental delay : intrauterine infections
TORCH(S)
– Toxoplasmosis
– Other (Zika)
– Rubella
– Cytomegalovirus
– Herpes Simplex, HIV – Syphilis
what does Congenital Toxoplasmosis causes
calcification in the brain adn eye
what is the most important intervention from developmental delay
referral to specialist if under 3
– MRI
– Karyotype (± FISH)
– AST, ALT, NH3, lactate, CBC, Chem 20 – Serum amino acids, urine organic acids
what are these
usualt test for developmental delay
• A group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to NON PROGRESSIVE disturbances occurring in the developing fetal or infant brain.
• Often accompanied by disturbances of sensation, perception, cognition, communication and behavior, by epilepsy and by secondary musculoskeletal problems
cerebral palsy
is CP a progressive or non progressive disturbances occurring in the developing fetal or infant brain
non
those who are born 28 was gestation have higher risk of what
CP
what are the causes of CP
any disturbance of developing a brain in utero or around the time of delivery
hypoxia is 10%
what are risk factors of antenatal for development of CP
- pre mature
-infections
-multiple gestation
-complications
what are risk factors of perinatal for development of CP
-birth asphyxia
- complication
what are risk factors of postnatal for development of CP
- non accidental injury (child abuse)
-head trauma
-meningitis/ encephalitis - cardio arrest
what are the 4 classifications of CP
-spastic (most common)
-dyskinetic
-ataxic
mixed
how would u classify spastic CP
-unilateral or bilateral
-velocity dependent increased tone
- hyperreflexia
- UMN signs
how would u classify dyskinetic CP
-recurrent uncontrolled/incoluntary movement
-tone may be abnormal
-dystonic
- choreoathletotic
how would u classify ataxic CP
– Hypotonic
– Loss of coordination
what is the most common mixed CP
Most commonly spastic with dyskinesias
where is the lesion for spastic hemiparesis
lesionin the CORTICOSPINAL system of ONE cerebral hemisphere
what is the most common causes of spastic hemiparesis
intrauterine stroke affecting middle cerebral artery
does spastic hemiparesis affect the arm or leg more an when may it be apparent
arm and when child developed handedness before 18 months
if a pt presents with a lesion in the corticopsinal system from an intrauterine stroke affecting the middle cerebral artery and affecting the arms more then the legs so the pt can work and is apparent in a 12 month old what do we think
spastic hemiparesis
how will a patient with spastic diplegia present
spasticity and weakness in their legs
what is spastic diplegia casued by
-prematurity with bilateral hemorrhages
or
- peri ventricular white matter disease
- perinatal ischemia in border zone of ACA and PCA
what kind of gait will a pt with spastic diplegia presents with
scissoring gait bc of adductor spams
if a patient presents with spasticity and weakness in the left and presents with a SCISSORING gait w normal intelligence what so we suspect
spastic diplegia
what is the most severe spastic variant of CP
spastic quadriplegia
what is wrong with teh brain in spastic quadriplegia , what is it associated with and what is frequent
diffuse malformation
associated with mod-serve intellectual disability
frequent seizures
what are casues of spastic quadriplegia
hypoxia -ischemia , infection , CNS malformation
where is the lesion for dyskinetic CP and whar does it present with
BG lesions leading to chorea , dystonia and athetosis
what kind of CP does neonatal hyperbilirubinemia
(kernicterus) and severe anoxia cause
dyskinetic CP
can children walk with dyskinetic CP n
naur
kids with dyskinetic CP will present with ____ in infancy , abnormal movements will begin at 1 year old
hypotonia
what is the rarest form of CP and is the abnormal development of cerebellum or its pathways
ataxia CP
what kind of ataxia does ataxic CP present with
truncal and gait
do drugs help ataxic CP
NAURRR may improve with age tho
what are the oral medication for CP spasticity
• baclofen
• Diazepma
• tizanidine
Others: trihexyphenidyl, gabapentin, tetrabenazine)
• baclofen
• Diazepma
• tizanidine,
• Others: trihexyphenidyl, gabapentin, tetrabenazine)
what are these medications for
CP spasticity
what is the injection for CP spasticity
botulinum A
what is the sx for CP spasticity
• Intrathecal baclofen
• selective dorsal rhizotomy
• DBS
what is the prognosis for mild to moderate CP
normal life
