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Peds > Neuro > Flashcards

Neuro Flashcards

1
Q

What are the 2 causes of floppy baby syndrome?

A

Infant botulism - canned food, honey
Werdnig-Hoffman syndrome - degeneration of anterior horn cells and CN motor nuclei

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2
Q

Fragile X signs

A

Long face, prominent forehead and chin
Large, protruding ears
Macrocephaly
Joint hypermobility (fingers, wrists)
Hypotonia
Macroorchidism (>age 8)

Speech and motor delays
ADHD
Autistic features

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3
Q

What is genetic inheritance of Fragile X?

A

X-linked dominant, CGG repeat in the FMR1 gene

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4
Q

Rett syndrome signs

A

Progressive brain deterioration
Deceleration of head growth
Developmental regression

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5
Q

What are the classes of breath-holding spell? What are their causes?

A

Cyanotic - Vigorous crying –> breath holding –> LOC –> rapid return to normal

Pallid - Minor trauma (no crying) –> pain/fear –> bradycardia –> pale, diaphoretic –> LOC –> <5 min sleepiness/confusion

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6
Q

What should be screened even in classic breath-holding spell?

A

Iron deficiency anemia

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7
Q

Preterm/low birth weight babies have what imaging finding for cerebral palsy?

A

Periventricular leukomalacia
Intraventricular hemorrhage (friable germinal matrix vessels)

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8
Q

Cerebral palsy signs

A

Motor delay (e.g. rolling over after 4 months age corrected)
Early hand preference (age <1 yr)
Persistent neonatal reflexes (tonic neck reflex >6 months)
Abnormal tone (hypotonic –> hypertonic, hyperreflexia, clonus)

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9
Q

What is the most common defect causing closed spinal dysraphism?

A

Failure of vertebral arch fusion (L5-S1 typically)

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10
Q

Tethered cord syndrome leads to what signs?

A

Lower motor neuron signs: LE weakness/loss of sensation, hypotonia, hyporeflexia

Older children:
Urologic incontinence/retention, recurrent UTI
Back pain, scoliosis
Neuromuscular hip dysplasia
Foot deformities

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11
Q

What is most commonly associated with infantile spasms (seizures in infancy with clusters of brief muscle contractions)

A

Tuberous sclerosis complex

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12
Q

What is the classic CNS lesion in tuberous sclerosis complex? What about others?

A

Subependymal giant cell tumor

Cardiac rhabdomyoma
Renal angiomyolipoma
Retinal hamartoma

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13
Q

What CNS tumor may occur in NF1?

A

Optic nerve glioma

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14
Q

What CNS tumor may occur in von-Hippel-Lindau disease?

A

Cerebellar hemangioblastoma

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15
Q

Sturge-Weber syndrome findings

A

Leptomeningeal angiomatosis - intracerebral vascular lesion
Some infantile spasms (< tuberous sclerosis complex)
Port-wine stain

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16
Q

What is age range of neuroblastoma?

A

<2 yo

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17
Q

How does placental abruption affect the newborn brain?

A

Hypoxic ischemic encephalopathy - cerebral edema or white matter damage

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18
Q

Idiopathic intracranial HTN is most likely to affect children in what way?

A

Vision changes, including affecting CN VI (abducens nerve palsy) causing diplopia and impaired eye abduction

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19
Q

How is idiopathic intracranial HTN diagnosed?

A

Neuroimaging (MRI, MR venography)
If negative, lumbar puncture showing elevated opening pressure is diagnostic

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20
Q

What are the classic findings of retinitis pigmentosa?

A

Retinal vessel attenuation
Optic disc pallor

Night blindness, decreased visual acuity, visual field loss

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21
Q

Infratentorial
Cerebellar hemispheric
Supratentorial

A

Infratentorial - cerebellar signs ipsilateral side + raised ICP
Supratentorial - cerebellar signs on contralateral side + affect sensation
Cerebellar hemispheric - hypotonia and hyporeflexia

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22
Q

What is a major side effect of valproic acid?

A

Hepatotoxicity

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23
Q

Hypoglycemia or hyperglycemia can cause cerebral edema?

A

Hyperglycemia

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24
Q

What is the Cushing reflex to increased ICP? What is the Cushing triad?

A

Hypertension: sympathetic activation to increase systemic BP to maintain cerebral perfusion pressure
Bradycardia: HTN stimulates baroreceptors in carotid artery and aortic arch –> parasympathetic response –> bradycardia

Irregular respirations (part of Cushing triad) - due to impaired brainstem function; indicates cerebral herniation is imminent

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25
Q

What brain part is affected in locked-in syndrome?

A

Isolated pontine infarction - quadriplegia and cannot speak, but retain consciousness, eye opening, and vertical eye movements

26
Q

Huntington disease affects which part of brain?

A

Basal ganglia atrophy - chorea, psychiatric symptoms, dementia

27
Q

Tourette syndrome associated with what brain part?

A

Caudate nucleus volume decrease

28
Q

How are tics different from chorea?

A

Tics can be temporarily suppressed

29
Q

What is milkmaid grip?

A

Intermittently weakened hand grip due to hypotonia and chorea in Sydenham chorea

30
Q

What is pseudobulbar affect?

A

Inappropriate laughter/crying

31
Q

Hydrocephalus pressure on midbrain affects eyes how?

A

Persistent downward gaze due to upward gaze impairment

32
Q

What is Chiari II malformation?

A

Myelomeningocele + herniation of cerebellum through foramen magnum
Obstruction of CSF flow through 4th ventricle + lateral ventricular dilation

33
Q

What are signs of lens dislocation (ectopia lentis)?

A

Painless vision loss
Tremulous iris with eye movement

34
Q

What are two classic causes of lens dislocation?

A

Marfan - want to look for aortic root disease
Homocystinuria - intellectual disability, thrombotic events

35
Q

What is indicated by diffuse leptomeningeal enhancement?

A

Meningitis - increased permeability of meninges

36
Q

What drug is used to control neonatal seizures?

A

Phenobarbital

37
Q

What is Erb-Duchenne palsy? Signs?

A

Most common brachial plexus injury (C5, C6)

Waiter’s tip posture - extended elbow, pronated forearm, flexed wrists and fingers
Moro/biceps reflex decreased, but grasp intact

38
Q

What is Klumpke palsy? Signs?

A

Brachial plexus injury from shoulder dystocia (C8 and T1)

Claw hand - extended wrist, hyperextended MCP joints, flexed ICP joints, absent grasp reflex
Ipsilateral miosis and ptosis (Horner syndrome) - from sympathetic damage

39
Q

Which of the shoulder dystocia palsies has absent grasp reflex?

A

Klumpke palsy - brachial plexus injury of C8 and T1

40
Q

Acute cerebellar ataxia - signs

A

Acute onset after infection - ataxia, nystagmus, dysarthria

41
Q

In strabismus, red reflex is stronger in which eye? Treatment? How long screening?

A

Red reflex more intense in the deviated eye
Light reflexes also asymmetric

Use patch or cycloplegic drops (e.g. atropine) for normal eye

Screen until age 5

42
Q

What is retinitis pigmentosa?

A

Genetic: Progressive retinal degeneration
Night blindness, visual field loss –> decreased visual acuity
Retinal pigment deposition, retinal vessel attenuation (lesser oxygen demand), optic disc pallor

43
Q

What is retinopathy of prematurity?

A

Presents in infants born at <30 wga
Associated retinal detachment and leukocoria

44
Q

Treatment for allergic conjunctivitis

A

Topical antihistamines (eg ketotifen)
Mast cell stabilizers

Allergen avoidance
Cool compresses - induce vasoconstriction
Oral histamines help with concomitant allergic rhinitis

45
Q

Warm or cold compresses for allergic vs viral conjunctivitis?

A

Warm: Viral
Cold: Allergic

46
Q

Acoustic neuroma/vestibular schwannoma are associated with what?

A

Neurofibromatosis type 2

47
Q

What gene is affected in neurofibromatosis types I vs II?

A

I: Neurofibromin
II: Merlin

48
Q

3-Hz spike-wave pattern on EEG indicates?

A

Absence seizures

Treat with ethosuximide

49
Q

Bedwetting is normal until what age?

A

5 yo

50
Q

Chiari malformations
I
II
III

A

Cerebellum inferiorly displaced through foramen magnum
I: Only cerebellar tonsils displaced
II: Cerebellar tonsils + vermis + inferior displacement of medulla; associated with myelomeningocele
III: Severe - herniation into an encephalocele

51
Q

Signs of Chiari I malformation

A

Asymptomatic throughout childhood

Occipital headache and/or neck pain
Dizziness and pain with physical activity or Valsalva due to pressure of cerebellar tonsils on foramen magnum

ICP, neuropathy from brainstem compression (eg dysarthria), cerebellar dysfunction (eg ataxia)

52
Q

What is syringomyelia?

A

Cyst (syrinx) formation, commonly in cervical region
Occurs in 30% of Chiari I malformation
Expansion can cause progressive neurologic dysfunction (eg paresthesia, weakness)

53
Q

What is the greatest risk for intraventricular hemorrhage?

A

Prematurity - hemorrhage may occur from ruptured germinal matrix vessels
Get a head ultrasound

54
Q

What should be used for lead chelation at different lead levels?

A

After confirming venous lead levels:
45-69 - Dimercaptosuccinic acid (succimer)
>=70 - Dimercaprol + EDTA

55
Q

Brain tumor with glial fibrillary acidic protein, bipolar neoplastic cells with hairlike projections, microcysts, and Rosenthal fibers (eosinophilic, corkscrew)

A

Pilocytic astrocytoma - posterior fossa, infratentorial

56
Q

Synaptophysin+, Homer-Wright rosettes, small blue cells

A

Medulloblastoma - posterior fossa, infratentorial

57
Q

Perivascular pseudorosettes, rod-shaped blepharoplasts (basal ciliary bodies) near nucleus

A

Ependymoma - commonly 4th ventricle but also primary spinal cord

58
Q

Craniopharyngioma vs pituitary adenoma

A

Craniopharyngioma has calcifications

59
Q

Craniopharyngioma is derived from…

A

Rathke pouch (ectoderm) - most common suprasellar tumor in children

60
Q

Cancer with Bombesin+ stain on histology, characteristic rosette, small round blue cells

A

Neuroblastoma - stage with metaiodobenzylguanidine (MIBG) scan and bone marrow aspirate

61
Q
A

Peds (25 decks)

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