Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Pediatrics > Neurology > Flashcards

Neurology Flashcards

(79 cards)

Start Studying
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Inheritance of Spinal Muscular Atrophy

A

AR

-Mutation is on SMN1 gene on Cr. 5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Management of SMA

A

Supportive

-Gastrostomy tube feeding, respiratory management, physical therapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

First sign of botulism

A

Constipation

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

EMG in botulism

A

Incremental increase in response during high frequency stimulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Tx of botulism

A

Anti-toxin

-Antibiotics are CI’d

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Dandy-Walker Malformation

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Congenital aqueduct stenosis

A

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

-May have severe MR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Most common ACQUIRED cause of hydrocephalus in preterm infants

A

Intraventricular hemorrhage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Teratogens causing SB

A

Valproate

Phenytoin

Cancer drugs (colchicine, vincristine, vincristine, MTX)

***MOST COMMONLY DECREASED FOLIC ACID INTAKE THO

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Meningocele

A

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

-Requires surgical repair

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

MCC of coma

A

Nonaccidental trauma

Near-drowning

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

MCC of coma in >5yrs old

A

Accidental head injury

Drug OD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Decerbrate posturing

A

Extension of arms and legs

-Indicates subcortical injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

DeCORTICATE posturing

A

Flexion of arms and extension of legs

-Indicative of bilateral CORTICAL injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Asymmetric motor response during a coma
Indicative of hemispheric (localized) injury
26
Hypoventilation during coma
Suggests opiate or sedative OD
27
Hyperventilation during coma
Suggests metabolic acidosis, neurogenic pulmonary edema, or MIDBRAIN injury
28
Cheynes-Stokes breathing during coma
Suggests bilateral cortical injury -alternating apneas and hyperpnea
29
Apneustic breathing during coma
Suggests pontine injury -Pauses at full inspiration
30
Ataxic breathing during coma
Indicates medullary injury -BAD SIGN; probs about to die tho
31
Pupillary size during coma
Unilateral dilated pupil = UNCAL HERNIATION Bilateral dilated pupils= Brainstem injury, postictal state Bilateral constricted pupils= PONTINE injury, opiate ingestion
32
Doll's eyes
Injured brainstem leading to movement of the head causing no eye movement
33
Epilepsy
The occurrence of 2 or more spontaneous seizures without and obvious precipitating cause -Some bullshit Loudin would ask
34
Status epilepticus
Seizure that lasts greater than or equal to 30 minutes during which the patient does not regain consciousness
35
Epidemiology of seizure in children
4-6% before 6 years old -However, very few actually get epilepsy
36
MCC of seizure in children
Unknown (60-70% of seizures)
37
Neurologic studies w/ seizure
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
Status epilepticus tx.
Short-acting benzos followed by Phenobarbital
39
Generalized epilepsy tx.
Valproic acid or Phenobarbital
40
Absence seizure tx.
Ethosuximide
41
Partial epilepsy tx.
Carbamazepine or Phenytoin
42
Medically intractable epilepsy tx.
Surgery to remove affected tissue -Best outcome w/ temporal lobe lesiosn
43
Alternative epilepsy treatments
1. Vagal nerve stimulator; can cause HOARSENESS | 2. Ketogenic diet
44
No epileptic episodes after 2 years?
Wean off medication as long as EEG is normal
45
Frequent, recurrent febrile seizure tx.
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
West Syndrome
"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
Tx of West Syndrome
ACTH intramuscular Valproic acid= 2nd line *****VIGABATRIN W/ TUBEROUS SCLEROSIS -Pts. often still develop severe MR
48
Absence epilepsy inheritance
AD inherited; begins b/w ages 5-9 yrs
49
Absence epilepsy EEG
Generalized 3-Hz spike and wave discharge from both hemispheres
50
West Syndrome EEG
Hypsarrhythmia pattern with highly disorganized, high amplitude spikes and waves all around the brain
51
Benign rolandic epilepsy
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
MCC of common partial epilepsy in childhood
Benign rolandic epilepsy AD inherited
53
EEG in Benign rolandic epilepsy
Biphasic spike and sharp wave disturbance in the mid-temporal and central regions
54
Tx. of Benign rolandic epilepsy
Valproic Acid Carbamazepine -Excellent prognosis
55
MCC of headaches in children and adolescents
Migraine without aura; usually begins at
56
Migraine equivalent
Prolonged period of cyclic vomiting, abdominal pain, or vertigo, however, no headache ever develops
57
Ophthlmoplegic migraine
Unilateral ptosis or CN III palsy accompanying migraine
58
Basilar artery migraine
Vertigo, tinnitus, ataxia, or dysarthria (cerebellar signs/symptoms) preceding the onset of a migraine headache
59
Migraine pathophysiology
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
Tx of migraines
Sumatriptan (5-HT agonist) Propanolol (prophylaxis)
61
Tension headace
Rare during childhood, often accompanied by contraction of the temporalis and/or masseter
62
Acute Cerebellar Ataxia of Childhood
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
MCC of ataxia in children
Acute Cerebellar Ataxia of Childhood
64
Guillain-Barre Syndrome
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
Miller-Fisher Syndrome
A variant of Guillain-Barre syndrome characterized by ophthalmoplegia, ataxia, and areflexia
66
Albuminocytologic dissociation
CSF finding in Guillain-Barre where there is increased CSF protein and normal cell count
67
EMG in Guillain-Barre Syndrome
Decreased nerve conduction velocity/block
68
Guillain-Barre tx.
IVIG, plasmapheresis
69
Sydenham's Chorea
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
Neuroimaging in Sydenham's Chorea
MRI: Increased signal intensity in the caudate and putamen on T2 sequences SPECT: Increased perfusion to thalamus and striatum (caudate and putamen)
71
Tx of Sydenham's Chorea
Haloperidol Valproic Acid Phenobarbital
72
Tourette's Syndrome
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
Tx of Tourette's Syndrome
Pimozide = DOC Clonidine (ADR=sedation) Haloperidol (ADR= tardive dyskinesia)
74
DMD
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
BMD
Same as DMD but less severe; patients lose ability to walk after 20 years old and have no cognitive impairment
76
Juvenile Myasthenia Gravis
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
Neonatal myasthenia
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
Diagnosis of MG
Edrophonium chloride (AchE inhibitor) =>> Improvement of ptosis Decremental response to repetitive nerve stimulation (as opposed to what condition) Presence of AchR antibody titers
79
Tx of Juvenile Myasthenia Gravis
Pyridostigmine bromide (AchE inhibitor) -Sometimes use corticosteroids, plasmapheresis, or IVIG ***OFTEN USE THYMECTOMY as well

Pediatrics (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions