Neurology

Question 1

In what Sarnat stage are seizures most common?

Answer 1

Stage 2,
Most common in sarnat stage 2. Stage 3 there is severe neuronal damage that prevents progression of seizures.

Question 2

A glycine cerebrospinal fluid to plasma ratio >1 is consistent with ?

Answer 2

non-ketotic hyperglycinemia

Question 3

Are Chiari I or Chiari II malformations associated with myelomenigocele?

Answer 3

Chiari Malformation II
Chiari 2, involve both cerebellum and brainstem extending into foramen magnum

Question 4

Difference between OAE and ABR hearing

Answer 4

OAE tests auditory pathway to cochlear hair response
ABR evaluates complete auditory and neural pathways

Question 5

Intermittent, Limited, Extensive, Pervasive help classify what?

Answer 5

level of support needed for Mental deficiency
(Intermittent being least, constant need for support not needed
Pervasive being most need, almost all activities)

Question 6

Is gross motor affected in spastic CP?

Answer 6

It is affected, but fine motor is not
increase in tone
cognitive function is normal

Question 7

MCAD deficiency predisposes to what kind of seizures?

Answer 7

Hypoglycemia

Question 8

Myelomeningocele is almost associated with?

Answer 8

Chiari II malformation and tethered cord

Question 9

Until what gestational age is an aEEG usually discontinuous?

Answer 9

28 weeks, after that it it’s mainly confined to sleep

Question 10

Whare the 5 neurocutaneous syndromes?

Answer 10

Sturge-Weber
Tuberous Sclerosis
Neurofibromatosis
McCune Albright
Von Hippel-Lindau

Question 11

What are common radiologic signs of Chiari II malformation?

Answer 11

Frontal calavarial scalloping (lemon sign)
anteriorly curved cerebellum (banana sign)
cereberal tonsilar herniation

Question 12

What are some features of infantile spinal muscular atrophy?

Answer 12

infants show tongue fasciculations, decreased tendon reflexes, and hypotonia.

Question 13

What do you see in imaging in Sturge Weber Syndrome?

Answer 13

Gyriform calcifications can be seen on skull x-rays and are classically described as a “tram-track” sign. Computed tomography is the best modality to detect calcifications and to show other changes, such as cortical atrophy and leptomeningeal enhancement in post-contrast studies

Question 14

What does alobar holoprosencephayl look like radiology?

Answer 14

single ventricle with fused thalami

Question 15

What does EEG show in non-ketotic hyperglycinemia?

Answer 15

myoclonic seizures and burst supression

Question 16

what does hipsarrythmia and infantile spasms show on eeg?

Answer 16

“very high voltage, random, slow waves and spikes in all cortical areas.”

Question 17

What is dermatologic finding in McCune Albright?

Answer 17

irregular brown pigmentations
(coast of maine apperance)

Question 18

What is dermatologic finding in Neurofibromatosis Syndrome?

Answer 18

Cafe au lait spots, do not cross midline (sharp borders), not present at birth, freckling in axilla, inguinal folds and perineum

Question 19

What is dermatologic finding in Sturge-Weber Syndrome?

Answer 19

Pink-Purple, flat, hemangioma (Port Wine) distrubtion of trigeminal nerve

Question 20

What is dermatologic finding in Von Hippel Lindau?

Answer 20

Multiple systemic hemangiomata

Question 20

What is dermatologic finding in Tuberous Sclerosis?

Answer 20

50% hypopigmented lesions, ashe leaf macules, variable in number, most on back and butt

Question 21

What is treatment for non-ketotic hyperglycinemia?

Answer 21

Sodium Benzoate and dextromethoprhan

Question 22

what suture is closed prematurely in bracnycephaly?

Answer 22

bicoronal suture

Question 23

what suture is closed prematurely in palgeocephaly?

Answer 23

lamboid

Question 24

what suture is closed prematurely in scalphocephaly?

Answer 24

saggital Scaphocephaly and dolichocephaly are both types of flat head syndrome that involve an elongated head shape. Scaphocephaly is a more specific term for a type of dolichocephaly with a distinct skull shape.

Question 25

what suture is closed prematurely in trigoncephaly?

Answer 25

metopic suture leads to triangle shape head and hypertelorism

Question 26

what type of weakness do you see in Werdnig Hoffman?

Answer 26

weakness, proximal muscle weakness predominates. affected children may never sit or stand.

Question 27

When do most newborns recover from facial nerve palsy?

Answer 27

1-3 weeks

Question 28

When does myelomengocele occur?

Answer 28

Myelomeningocele occurs during a defect in primary neurulation when the caudal neuropore fails to close approximately during day 27.

Question 29

Why are infants more susceptible to IVH?

Answer 29

Blood vessel morphology in the germinal matrix of the neonate differs from that in other cortical areas, mainly due to the increased metabolic demand required by the rapid turnover of precursor cells in this region. Blood vessels supplying the germinal matrix have a higher density and area than other cortical regions.

Question 30

How could you treat hipsarrythmia?

Answer 30

ACTH (induces steroid release)

Question 31

Where is Erb's Palsy located?

Answer 31

C5-C6

Question 32

what happens if the forebrain vesicle DOES NOT differentiates into a diencephalon and a telencephalon

Answer 32

holoprosencephaly.

Question 33

When does palmar grasp disappear?

Answer 33

5-6 months

Question 34

Brain structures most commonly affected by HIE

Answer 34

white matter, basal ganglia, thalamus, globus pallidus

Question 35

What medicine should you give to infant who is suffering from medically refractory seizures

Answer 35

trial of pyroxidine, might have pyridoxine-dependent epilepsy

Question 36

When does neural tube close?

Answer 36

5-6 week of gestation (ventral induction)

Question 37

7th to 20th week of gestation marks which stage of neurodevelopment in fetus?

Answer 37

Neuroepthileal cell proliferation and migration

Question 38

Age of dissapearance of primitive reflex in order Step, Moro, Grasp, Palmar, rooting

Answer 38

Step (2 months) < Rooting (3 months) < Moro & Tonic Neck (5-6 months) < Palmar (6 months) < Plantar grasp (9-10 months)

Question 39

How does phenobarbital work?

Answer 39

hyperpolarize by keeping CL channels open via GABA-A receptors

Question 40

IVH outcomes depend on what?

Answer 40