Lecture 13 Pediatrics Flashcards by Alexander Lee

When does the nervous system first appear in gestation

On average when does the neural tube form

21 days

25 days

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What are the 3 primary brain vesicles

Prosencephalon, mesencephalon, rhombencephalon

Note: by week 5 there’s 5 vesicles

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What part of the fetus brain becomes the cerebral hemispheres

What part becomes the midbrain

Telencephalon

Mesencephalon

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What nutrient is essential for preventing neural tube defects

Folate

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What is dysraphism?

What is anencephaly

Disorder of neural tube closure

Fatal disorder w/ absence of brain in development.

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What are the risk factors of dysraphism

Low socioeconomic status, maternal nutritional status, teratogens (alcohol and medications)

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What are the 3 types of spina bfidia

Occulta

Meningocele

Myelomeningocele

Occulta- vertebral arch defect

Meningocele- dura and arachnoid herniation

Myelomeningocele- herniation of spinal cord

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Where is spina bfida occulta most common?

L5 to s1

Note: usually asymptomatic

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What are the potential complications of Myelomeningocele

Hydrocephalus
Risk of tethered cord syndrome

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What is tethered cord syndrome?

What category of motor neuron disorders does it cause?

Traction of spinal cord (stuck at one level and unable to move normally)

Causes lower motor neuron disorder

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What is Arnold-chiari malformation

Hindbrain is sticking out of foramen magnum

Note: sometimes asymptomatic, if it’s over 5mm we are concerned

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What are the 3 types of Arnold Chiari malformation

Type 1: cerebellar tonsils displaced > 6mm

Type 2: associated with Myelomeningocele

Type 3: associated with encephalocele

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What are the symptoms of Arnold chiari malformation

Hydrocephalus, headache, cerebellar/medullary/cranial nerve signs

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What is dandy walker malformation?

Cerebellar vermis developmental anomoly w/ large cyst in posterior fossa (theyre misising their cerebellum)

50% associated w/ mental retardation

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How do doctors treat hydrocephalus in children?

Ventriculo-peritoneal shunt

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Microcephaly

Macrocephaly

Craniostenosis

Microcephaly - small head (Causes: idiopathic, chromosomal, toxic)

Macrocephaly - Large head (hydrocephalus, genetic, gigantism)

Craniostenosis- Skull sutures fuse early and alter the shape of the head

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How do you classify a global delay?

If there is a delay in 2+ of the domains.

-Gross motor
-fine motor
-expressive language
-receptive language
-social/adaptive behavior

There is a delay if a child is less than 70% of the norm

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Sitting unsupported should happen at what age?

6 months

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walking should happen at what age?

12 months

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tricycle riding should happen at what age?

3 years

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first words should happen at what age?

12 months

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putting words together should happen at what age?

2 years

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What is an important biological measurement that pediatricians measure on kids?

head size

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child headsize that suddenly increases or decreases ____ percentiles is concerning

2 percentiles

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What are the 5 normal primitive reflexes

Moro reflex tonic neck reflex grasp reflex step reflex crawl reflex

Red flag: head size ____________ percentiles

crossing 2

red flag: __________ to respond to sounds

failure

red flag: __________ beyond 3 months

fisting

red flag: rolling _______ 3 months

before (indicates one side is weak and other is favored)

Red flag: persistent __________ reflex after 6 months

MORO reflex (where they abduct their arms when theyre leaned back or when theyre startled)

red flag: persistent ____________________ reflex and not rolling beyond 7 months

asymmetric tonic neck reflex

red flag: Not sitting after ____________

9 months

red flag: Delayed smiling ____________ laughing ______________ cooing _____________ first words ____________

>1 month >2-3 month >3-4 months >12 months

red flag: handedness ___________________

before 12 months (indicates one side is weak and other is favored)

red flag: persistent echolalia after _______ red flag: noticable __________ after 4 years

3 years stutter At any age: decline in speech ability or vocab

How does a delay differ from a regression

**Regression**: acheived milestones are lost *more serious*

What are predominant causes of speech delays?

* Multilingual family * Hearing loss * Infantile autism

What conditions can mimic regression in a child

* New onset of seizures * Misperception of milestones * Increased spasticity * Movement disorders * Hydrocephalus

Down syndrome (trisomy 21) And Fragile X are both examples of what kind of disorder? Which is the most common cause of intellectual disability of this disorder class?

Chromosomal disorders Fragile X is the most common chromosomal cause of intellectual disability

Who does Down syndrome affect more? Risk increases with advanced ______ 2% risk of reoccurrence in next child

Men Advanced maternal age (35+)

What are the facial features associated with down syndrome

Broad flat face Slanted eyes Epicanthic eye fold Short nose

What are the facial features of fragile X syndrome? What is the average IQ

Long face, enlarged ears, high arched palate IQ 40-55 Note: should be considered in all cases with intellectual disability or autism spectrum disorders

What does TORCHS stand for?

Infectious causes of developmental delay * **T**oxoplasmosis * **O**ther * **R**ubella * **C**ytomegalovirus * **H**erpes or **H**IV * **S**yphilis

What is the most important intervention for developmental delays

Refer to specialist if under 3

Is cerebral palsy progressive or non progressive

Non progressive CP is damage to the brain one time and then they’re left with permanent deficits but those deficits don’t get worse

What can cause CP?

Any disturbance to the developing brain in utero or around the time of the delivery

What is the most common time CP is caused? Anenatal (before birth) Perinatal (during birth) Post natal

Anenatal

What are protective factors against CP

Magnesium sulfate Antibiotics Corticosteroids

What are the 4 CP classifications? Which is most common?

Spastic (more common) Dyskinetic Ataxic Mixed

What are the classifications of spastic CP?

Spastic Hemiparesis Spastic diplegia Spastic quadriplegia Athetoid/extrapyramidal

How does spastic Hemiparesis present

Arm > leg Child develops handedness early 18-50% intellectual disability

What is damaged in spastic Hemiparesis and what is the common cause

Lesion of one corticospinal system in cerebral hemisphere Stroke in MCA

How does spastic diplegia present

Weakness in legs more then arms Scissoring gait (adductor spasm) Usually normal intelligence

What causes spastic diplegia

Damage to ACA and PCA

What is the most severe form of CP

Spastic quadriplegia

What causes dyskinetic CP

Damage to basal ganglia Causes chorea, dystonia, athetosis

How does dyskinetic CP present

Hypotonia in infancy, abnormal movement starting at 1 Unable to walk

What is the rarest form of CP What structure is involved

Ataxic CP Cerebellum Truncal and gait ataxia that doesn’t respond well to to drugs or PT

What is baclofen for

Spasticity

What is commonly injected to treat CP

Botulinum (Botox)

What is a selective dorsal rhizotomy

Surgery for CP They cut the nerve causing spasticity

Neurofibromatosis and tuberous sclerosis are what

Neurocutaneous disorder

What are the two forms of neurofibromatosis Which is more severe? Which chromosomes are affected? Peripheral or central?

NF1 - chromosome 17 Primarily affects skin and peripheral nerves NF2- chromosome 22, primarily affects CNS, **more severe**

Neurofibromatosis has what kind of inheritance

Autosomal dominant

Tuberous sclerosis has what kind of inheritance

Autosomal dominant

How does tuberous sclerosis present

**Lesions** in nervous system, skin, bones, retina, kidneys **White hard nodules** on surface of brain and into ventricals **Calcifications** 50% **mental retardation seizures** Skin has **depigmented macules** (look like bad acne).

What kind of genetic metabolic disorders are demyelinating

Leukodystrophies

What kind of inheritance does adrenoleukodystrophy have?

recessive X linked (affects only boys)

How does adrenoleukodystrophy usually present

Normal early development At about **8 - progressive dementia, seizures, spasticity**

What is metachromatic leukodystrophy

Demyelination of PNS and CNS can affect any age Gait failure, mental deterioration, seizures

In infants UMN damage does what to tone?

Decreases it Unlike in adults where it increases tone

What is duchennes muscular dystrophy? What muscles is it evident in

Muscular dystrophy at 3-5 Hyperlordotic posture, toe walking, ankle dorsiflexor/ hip weakness **Calf pseudohypertrophy**

What is the prognosis of Duchens muscular dystrophy

More severe > BMD boys will be in **wheelchair by 9-12** Life expectancy about **20** 1/3 with mental retardation

How does Becker muscular dystrophy differ from duchens

Ambulatory for longer (30) IQ normal

How are dystrophies diagnosed

Phenotype (observation) -> test for high CK -> DNA analysis

How does Becker muscular dystrophy present?

Myalgia Isolated quad weakness Rhabdo Cardiomyopathy

How is spinal muscular atrophy inherited? How does it present

Autosomal recessive Progressive loss of motor neurons in spinal cord

Spinal muscular dystrophy Type 0 Type 1

Type 0- prenatal - death Type 1- age of onset 6 months. Never Sit. Dies by 2

Spinal muscular dystrophy 2 3 4

2- Never **Stand** (age 6-12 months) 70% survive to **25**) 3- never **run**, age 12 months+ (nearly **normal life expectancy**) 4- adult onset. **Proximal leg weakness**

What is the cool new 2 million dollar treatment for Spinal muscle atrophy

Zolgensma One time treatment

Where does infantile botulism come from What does it cause

Honey and corn syrup Prevents release of acetylcholine Acute generalized weakness

What nerve roots r most commonly hurt in kids due to brachial plexus injuries

C5-C6 Good prognosis

Head pushed down away from shoulder causes what palsy

Erbs Palsy C5-c6 Weakness of shoulder abduction, elbow flexion, supination

Injury caused by arm pulled upward

Klumpke Palsy C8-T1 damaged Claw hand