Mitchell/martin: Neuro Peds 1

Front 1

Largest number congenital anomalies of nervous system

Back 1

Neural Tube Defects(Dysraphism)

Front 2

Failure of complete closure of the neural tube between the 3rd-4th week of intrauterine life

Back 2

Neural Tube Defects(Dysraphism)

Front 3

Spina bifida occulta*
Mylomeningocele (Meningomylocele)*
Encephalocele*
Anencephaly (including Hydranencephaly)*

Back 3

Neural Tube Defects

Front 4

This defect may be caused by autoantibodies against folate receptors.

Back 4

Neural Tube Defect

Front 5

Prevention of Neural Tube defects?

Back 5

--0.4mg folic acid/day all women of childbearing age
--4mg/day starting 1 month before a planned pregnancy in women with a previous neural tube defect pregnancy

Front 6

Midline defect involving failure of closure of the posterior vertebral arches and laminae (typically S5-L1) WITHOUT protrusion of meninges or spinal cord

Back 6

Spina Bifida Occulta

Front 7

--Ordinarily assymptomatic and found incidentally on X-ray…
--May have a hairy patch/nevus, a vascular nevus, or a dermoid sinus on overlying skin…such a sinus may penetrate the dura, leading to recurrent CNS infections

Back 7

Spina Bifida Occulta

Front 8

Midline defect in which the meninges herniate through a defect in the posterior vertebral arches, usually in the lower back

Back 8

Meningocele

Front 9

Midline defect of the posterior vertebral arches with herniation of meninges and neural tissue

Back 9

Myelomeningocele

Front 10

A child with myelomeningocele presents with bladder and bowel incontinence,
perineal anesthesia, no motor involvement. What location is the myelomeningocele?

Back 10

Low sacral region:

Front 11

A child presents with flaccid paralysis of the legs, lack of touch and pain sensation,
clubfeet and subluxed hips, and CHIARI II malformation with hydrocephalus (~80%). Where is the myelomeningocele?

Back 11

Mid-sacral to mid-lumbar region

Front 12

RX of myelocele and myelomeningocele?

Back 12

Immediate surgical intervention as indicated!

Front 13

Child's head is > 95th% but is growting along a NORMAL growth curve. Normal physical and neuro exam. Parents also have large heads.

Back 13

Macrocephaly

Front 14

An infant presents with large bulging fontanel with dilated scalp veins. There is a RAPID increase in head size and abnormal neuro exam.

Back 14

Hydrocephalus.

Abnormal long tract signs: hyperactive deep tendon reflexes, clonus, + Babinskis, spasticity and increased ICP!

Front 15

A child complains of headahce, low academic ability, gradual change in personaility, "cracked pot sound" upon percussion of skull, papilledma, CN6 palsy, and pyramidal tract signs.

Back 15

Hydrocephalus in OLDER children

Front 16

Diverse group of conditions that result from the relative entrapment or (rarely) overproduction of CSF in the ventricular system of the brain

Back 16

Hydrocephalus

Front 17

What type of stimulation increases/decreases CSF production?

Back 17

1. adrenergic stimulation  decreased production
2. cholinergic stimulation  increased production (as much as double

Front 18

Blockage of this flow anywhere within the substance of the brain (i.e. before the CSF passes through the foramina of Luschka and Magendie) results in _____________or non-communicating hydrocephalus.

Back 18

obstructive

Front 19

Overproduction or decreased absorption at the arachnoid villi results in _______________or NON-OBSTRUCTIVE HYDROCEPHALUS

Back 19

communicating

Front 20

Anomaly of the hindbrain, probably due to failure of pontine flexure  elongation of the 4th ventricle, kinking of the brainstem with herniation of the inferior vermis, pons, and medulla into the cervical canal.

Back 20

CHIARI II MALFORMATION(Arnold Chiari Malformation)

Front 21

Displacement of the cerebral tonsils into the cervical canal  usually no sx until ADOLESCENCE or adulthood. (Recurrent headaches, neck pain, urinary frequency, and progressive lower extremity spasticity. Etiology unknown.

Back 21

CHIARI I MALFORMATION

Front 22

What is a a cystic expansion of the 4th ventricle with failure of formation of the roof of the forth ventricle  90% have hydrocephalus?

Back 22

Dandy Walker Malformation

Front 23

What are some causes of Communicating Hydrocephalus?

Back 23

1. Subarachonoid bleed
2. Leukemic infiltrates seeding the subarachnoid space
3. Pneumococcal or tuberculosis meningitis exudates

Front 24

Treatment of hydrocephalus?

Back 24

1. Shunting
2. Acetazolamide & furosemide

Front 25

Bony midline defects (cranium bifidum) in the skull with protrusion of tissue through them. Mostly in occipital, frontal, nasofrontal.

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Cranial Meningocele & Encephalocele

Front 26

What's the difference between cranial meningocele and encephalocele?

Back 26

CM = only a CSF-filled sac

E: sac of CSF + cortex, cerebellum, or brainstem

Front 27

Head circumference < 3 S.D. below the mean for age and sex

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Microcephaly

Front 28

Small head @ birth, not associated with other malformations.

Back 28

familial microcephalies

Front 29

What are secondary causes of microphcephaly?

Back 29

ToRCH
Toxoplasmosis, rubella, cmv, herpes.

Front 30

What two conditions have an absence of cerebral hemispheres?

Back 30

Anencephaly & Hydranencephaly

Front 31

This is d/t failure of rostral neuropore to close, missing cerebellum, and the upper part of skull collapsed or missing.

Back 31

Anencephaly

Front 32

Caused by early embryonic occulusion of both internal carotid aa, +/- cerebellum remnants, skull is normal in shape but enlarges rapidly after birth.

Back 32

Hydranencephaly

Front 33

-Death within days of birth.
-Often have other congenital defects
(anencephaly or hydranencephaly)?

Back 33

Anencephaly

Front 34

Irritable, poor feeders, seizures, spastic quadriplegia, Little or no cognitive development; Life expectancy unpredictable.
Anencephaly or hydranencephaly?

Back 34

Hydranencephaly

Front 35

Results from an insult to the commissural plate (near the anterior neuropore) in early embryologic development.

Back 35

Agenesis of the Corpus Callosum

Front 36

Absence of various of the cranial nerves (or their corresponding central nuclei) produce corresponding clinical deficits

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Agenesis of and Abnormal Innervations by Cranial Nerves

Front 37

weakness of the facial muscles bilaterally due to hyploplasia or agenesis of brainstem nuclei plus decreased muscle fibers (often occurs in association with abducens n. paralysis);

Back 37

Mobius syndrome: Agenesis of CN

Front 38

Dull flaccid facies may suggest MR if the syndrome is not recognized.

Back 38

Mobius syndrome

Front 39

Premature “fusion” of cranial sutures

Back 39

Craniosynostosis