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Pediatrics > Neurology > Flashcards

Neurology Flashcards

1
Q

Central hypotonia

A

Dysfunction of UMNs (cortical pyramidal neurons and descending corticospinal pathways)

*Assoc. w/ seizures during the neonatal period

-Other signs:
Altered level of consciousness
Increased DTRs
Ankle clonus

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2
Q

Peripheral hypotonia

A

Dysfnxn of LMNs (spinal motor neurons)

  • Presents as decreased resistance to passive stretch of muscles
  • Assoc. w/ prenatal history of decreased fetal movements and breech presentation

Other signs:
Decreased muscle size
Decreased DTRs

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3
Q

Type I Spinal Muscular Atrophy

A

“Werdnig-Hoffman Disease”

Anterior horn cell degeneration that presents w/ hypotonia, weakness, and tongue fasciculations; presents at

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4
Q

Inheritance of Spinal Muscular Atrophy

A

AR

-Mutation is on SMN1 gene on Cr. 5

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5
Q

Management of SMA

A

Supportive

-Gastrostomy tube feeding, respiratory management, physical therapy

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6
Q

SMA Types II and III

A

II: Presents at age 6-12 months; survival until adolescent

III: Presents at >3 yrs; survival until adolescent

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7
Q

First sign of botulism

A

Constipation

-Progresses to weak cry and suck, loss of milestones, hyporeflexia, “descending, symmetric paralysis”

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8
Q

EMG in botulism

A

Incremental increase in response during high frequency stimulation

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9
Q

Tx of botulism

A

Anti-toxin

-Antibiotics are CI’d

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10
Q

Congenital myotonic dystrophy

A

AD inherited disorder presenting with weakness and hypotonia progressing to the inability to relax contracted muscles

-Gene is on Cr. 19 and inherited from MOM

Antenatal: Polyhydramnios (decreased swallowing)

Neonatal: Feeding/respiratory probs

***PE: Facial diplegia (bilateral weakness), hypotonia, areflexia, arthrogryposis (multiple joint contractures)

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11
Q

Age at which myotonia develops in Congenital myotonic dystrophy

A

5yrs

  • Once they reach adulthood, pts. have stiff smile, ptosis, MR (average IQ is 50-65) and inability to release grip after masturbating
  • Suspect this in ALL INFANTS W/ HYPOTONIA

Dx: DNA test

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12
Q

Chiari Type II malformatin

A

Downward placement of the cerebellum and medulla thru the foramen magnum

  • ALWAYS assoc. w/ lumbrosacral myelomeningocele
  • Pts. may end up w/ decreased intelligence and language probs
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13
Q

Dandy-Walker Malformation

A

Congenital malformation of the cerebellar vermis and cystic enlargement of the 4th ventricle producing HYDROCEPHALUS

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14
Q

Congenital aqueduct stenosis

A

X-LINKED cause of hydrocephals asoc. w thumb abnormalities and spina bifida

-May have severe MR

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15
Q

Most common ACQUIRED cause of hydrocephalus in preterm infants

A

Intraventricular hemorrhage

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16
Q

Sunset sign

A

Downward deviation of both eyes caused by pressure from the enlarged 3rd ventricles on the upward gaze center of the midbrain

Other signs of hydrocephalus:
   Headache
   N/V
   Unilateral Abducen's palsy 
   Papilledema
   Brik DTRs (Deep Tendon Reflexes)

***If these signs are present, GET A CT SCAN

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17
Q

Teratogens causing SB

A

Valproate

Phenytoin

Cancer drugs (colchicine, vincristine, vincristine, MTX)

***MOST COMMONLY DECREASED FOLIC ACID INTAKE THO

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18
Q

SB occulta

A

Vertebral cleft w/ no herniation of tissue

-Assoc. w/ small patch of hair on back; sometimes w/ congenital aqueductal stenosis

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19
Q

Meningocele

A

Herniation of meninges thru bony cleft on spine; not assoc. w/ any neural defects

-Requires surgical repair

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20
Q

Myelomeningocele

A

Herniation of meninges and spinal tissue thru bony defects; assoc. w/ paraplegia (above L3) or bowel defects (below S3)

*20x more common than meningocele

Associations:
Chiari Type II malformations
Cervical hydrosyringomyelia (fluid in the spinal cord and canal)
Defects in neuronal migration (agenesis of corpus callosum)
Orthopedic issues (rib and lower extremity malformations)
GU defects

  • *Requires urgent surgery within 24 hrs after identification from PE after birth
  • Pts. may have future filled w/ seizure, MR, paralysis, bladder probs
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21
Q

MCC of coma

A

Nonaccidental trauma

Near-drowning

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22
Q

MCC of coma in >5yrs old

A

Accidental head injury

Drug OD

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23
Q

Decerbrate posturing

A

Extension of arms and legs

-Indicates subcortical injury

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24
Q

DeCORTICATE posturing

A

Flexion of arms and extension of legs

-Indicative of bilateral CORTICAL injury

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25
Q

Asymmetric motor response during a coma

A

Indicative of hemispheric (localized) injury

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26
Q

Hypoventilation during coma

A

Suggests opiate or sedative OD

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27
Q

Hyperventilation during coma

A

Suggests metabolic acidosis, neurogenic pulmonary edema, or MIDBRAIN injury

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28
Q

Cheynes-Stokes breathing during coma

A

Suggests bilateral cortical injury

-alternating apneas and hyperpnea

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29
Q

Apneustic breathing during coma

A

Suggests pontine injury

-Pauses at full inspiration

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30
Q

Ataxic breathing during coma

A

Indicates medullary injury

-BAD SIGN; probs about to die tho

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31
Q

Pupillary size during coma

A

Unilateral dilated pupil = UNCAL HERNIATION

Bilateral dilated pupils= Brainstem injury, postictal state

Bilateral constricted pupils= PONTINE injury, opiate ingestion

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32
Q

Doll’s eyes

A

Injured brainstem leading to movement of the head causing no eye movement

33
Q

Epilepsy

A

The occurrence of 2 or more spontaneous seizures without and obvious precipitating cause

-Some bullshit Loudin would ask

34
Q

Status epilepticus

A

Seizure that lasts greater than or equal to 30 minutes during which the patient does not regain consciousness

35
Q

Epidemiology of seizure in children

A

4-6% before 6 years old

-However, very few actually get epilepsy

36
Q

MCC of seizure in children

A

Unknown (60-70% of seizures)

37
Q

Neurologic studies w/ seizure

A

Abnormal EEG is not required for diagnosis

-Neuroimaging should be done on all children w/ EPILEPSY except if its absence or benign rolandic epilepsy

38
Q

Status epilepticus tx.

A

Short-acting benzos

followed by

Phenobarbital

39
Q

Generalized epilepsy tx.

A

Valproic acid

or

Phenobarbital

40
Q

Absence seizure tx.

A

Ethosuximide

41
Q

Partial epilepsy tx.

A

Carbamazepine

or

Phenytoin

42
Q

Medically intractable epilepsy tx.

A

Surgery to remove affected tissue

-Best outcome w/ temporal lobe lesiosn

43
Q

Alternative epilepsy treatments

A
  1. Vagal nerve stimulator; can cause HOARSENESS

2. Ketogenic diet

44
Q

No epileptic episodes after 2 years?

A

Wean off medication as long as EEG is normal

45
Q

Frequent, recurrent febrile seizure tx.

A

Daily valproic acid or phenobarbital tx. alongside ABORTIVE TX. OF RECTAL DIAZEPAM

-If it’s the first seizure, don’t worry about it dude, don’t even get a spinal tap if you dont think meningitis is there just fucking chill

46
Q

West Syndrome

A

“Infantile spasms”

Brief, myoclonic jerks lasting 1-2 seconds occurring in clusters over 3-5 minutes; starts at 3-8 months of age

  • Also called “jackknife seizure due to sudden arm extensions
  • MCC=TUBEROUS SCLEROSIS
  • Other causes include PKU, hypoxic-ischemic injury, meningitis blehh
47
Q

Tx of West Syndrome

A

ACTH intramuscular

Valproic acid= 2nd line

*****VIGABATRIN W/ TUBEROUS SCLEROSIS

-Pts. often still develop severe MR

48
Q

Absence epilepsy inheritance

A

AD inherited; begins b/w ages 5-9 yrs

49
Q

Absence epilepsy EEG

A

Generalized 3-Hz spike and wave discharge from both hemispheres

50
Q

West Syndrome EEG

A

Hypsarrhythmia pattern with highly disorganized, high amplitude spikes and waves all around the brain

51
Q

Benign rolandic epilepsy

A

Nocturnal partial seizures that begin w/ oral-buccal movements (groaning, drooling) and spread to the body ending as a tonic-clonic seizure

-Excellent outcome

52
Q

MCC of common partial epilepsy in childhood

A

Benign rolandic epilepsy

AD inherited

53
Q

EEG in Benign rolandic epilepsy

A

Biphasic spike and sharp wave disturbance in the mid-temporal and central regions

54
Q

Tx. of Benign rolandic epilepsy

A

Valproic Acid

Carbamazepine

-Excellent prognosis

55
Q

MCC of headaches in children and adolescents

A

Migraine without aura; usually begins at

56
Q

Migraine equivalent

A

Prolonged period of cyclic vomiting, abdominal pain, or vertigo, however, no headache ever develops

57
Q

Ophthlmoplegic migraine

A

Unilateral ptosis or CN III palsy accompanying migraine

58
Q

Basilar artery migraine

A

Vertigo, tinnitus, ataxia, or dysarthria (cerebellar signs/symptoms) preceding the onset of a migraine headache

59
Q

Migraine pathophysiology

A

Change in cerebral flow secondary to release of 5-HT, substance , and VIP altering neuronal activity

Signs/Symptoms:
Headache starts in periorbital area and heads posteriorly
N/V
Blurred vision, scotoma, fortifications (streaks of light outlining objects)
Photo/Phonophobia

-Symptoms can be improved by sleep

60
Q

Tx of migraines

A

Sumatriptan (5-HT agonist)

Propanolol (prophylaxis)

61
Q

Tension headace

A

Rare during childhood, often accompanied by contraction of the temporalis and/or masseter

62
Q

Acute Cerebellar Ataxia of Childhood

A

Unsteady gait secondary to autoimmune or postinfectious (2-3 weeks later) cause

Common causes: EBV, VZV, influenza, mycoplasma

Signs/Symptosm: Truncal ataxia, slurred speech, nystagmus, AFEBRILE

-May need head CT to rule out serious conditions

Tx: Supportive (will resolve on own)

63
Q

MCC of ataxia in children

A

Acute Cerebellar Ataxia of Childhood

64
Q

Guillain-Barre Syndrome

A

Demyelinating polyneuritis characterized by ascending weakness, areflexia, and normal sensation following a C. jejuni, EBV, HZV, or CMV infxn

*Neurons are demyelinated at the ventral spinal roots of myelinated nerves due to cross reactivity of antibody w/ Schwann cell membrane

Signs/Symptoms:
Ascending, symmetric paralysis
CN involvement (facial weakness)
No sensory loss

65
Q

Miller-Fisher Syndrome

A

A variant of Guillain-Barre syndrome characterized by ophthalmoplegia, ataxia, and areflexia

66
Q

Albuminocytologic dissociation

A

CSF finding in Guillain-Barre where there is increased CSF protein and normal cell count

67
Q

EMG in Guillain-Barre Syndrome

A

Decreased nerve conduction velocity/block

68
Q

Guillain-Barre tx.

A

IVIG, plasmapheresis

69
Q

Sydenham’s Chorea

A

AI disorder assoc. w/ Rheumatic fever presenting w/ uncontrolled, proximal limb movements and emotional lability

-Occurs due to cross-reactivity of anti-GAS abs cross reacting w/ BASAL GANGLIA cells

Signs/Symptoms:
Restlessness
Altered speech
****Chameleon tongue (unable to sustain protrusion of tongue)
****Choreic hand (hand is flexed and hyperextended at metacarpals)
**Milkmaid’s grip (unable to maintain strong grip)

70
Q

Neuroimaging in Sydenham’s Chorea

A

MRI: Increased signal intensity in the caudate and putamen on T2 sequences

SPECT: Increased perfusion to thalamus and striatum (caudate and putamen)

71
Q

Tx of Sydenham’s Chorea

A

Haloperidol

Valproic Acid

Phenobarbital

72
Q

Tourette’s Syndrome

A

Presence of motor tics (blinking, shaking) and phonic tics (coughin, barking, echolalia) that are present for at least one year

-May also have coprolalia; also assoc. w/ ADHD, learning disorders, OCD

73
Q

Tx of Tourette’s Syndrome

A

Pimozide = DOC

Clonidine (ADR=sedation)

Haloperidol (ADR= tardive dyskinesia)

74
Q

DMD

A

X-linked deletion of the dystrophin gene that associates with actin and other structural membrane elements

-Appears as degenerating muscle fibers on EM w/ infiltration of lymphocytes as well as replacement of fibers w/ lipid deposits

Signs/Symptoms:
Slow, progressive weakness affecting legs first
-usually around 2-5 years of age
Pseudohypertrophy of calves
Gower’s Sign
Cardiac involvemnt (tachycardia, cardiomegaly)
-MILD COGNITIVE IMPAIRMENT ONLY IN DMD

-DMD pts. are wheelchair dependent by 10 years old and die before 20

75
Q

BMD

A

Same as DMD but less severe; patients lose ability to walk after 20 years old and have no cognitive impairment

76
Q

Juvenile Myasthenia Gravis

A

Antibodies against AchR at NMJs presenting as progressive weakness or diplopia

Signs/Symptoms:
Bilateral ptosis (****MC presenting sign)
Increasing weakness as day goes on
Diplopia
Other AI disorders coexisting (DM I, thyroid disease)

77
Q

Neonatal myasthenia

A

Transfer or maternal AChR abs from mother w/ myasthenia gravis to newborn thru placenta; weakness is only transient

Signs/Symptoms:
Hypotonia
Weakness
Feeding problems

-Management is symptomatic

78
Q

Diagnosis of MG

A

Edrophonium chloride (AchE inhibitor) =» Improvement of ptosis

Decremental response to repetitive nerve stimulation (as opposed to what condition)

Presence of AchR antibody titers

79
Q

Tx of Juvenile Myasthenia Gravis

A

Pyridostigmine bromide (AchE inhibitor)

-Sometimes use corticosteroids, plasmapheresis, or IVIG

***OFTEN USE THYMECTOMY as well

Pediatrics (9 decks)

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