Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pediatrics > Neurology > Flashcards

Neurology Flashcards

1
Q

Spinal muscular Atrophy

A

AR; Most common genetic cause of death in the first year. poor tone, fasciculations, poor feeding, usually starts with LE then UE then respiratory muscles. Diagnose with gene mutation screening

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

Febrile seizures occur in what ages

A

6mos to 5 years

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

Definition of febrile seizure

A

generalized seizure that lasts <15min, occuring only once in 24 hour period, in a febrile child without an intracranial infection

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

Treatment of absence seizures

A

Ethosuximide

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

Treatment of complex partial seizures

A

Carbamazepine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Juvenile myoclonic epilepsy

A

adolescent onset, generalized tonic-clonic seizures, and fast involuntary muscle jerks termed “myoclonus.” both the first generalized tonic-clonic seizures and the myoclonic jerking commonly occur in the morning. worse with sleep deprivation. normal intelligence. lifelong condition. Tx with valproate.

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

Most common comorbidity in Tourette Syndrome

A

ADHD

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

Diagnosis of comorbidity

A

clinical

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

SMA

A

AR, defect in SMN gene, anterior horn nerve degeneration. Wornig Hoffman is the worst type. Facial muscles not involved so face looks “bright”, diffuse hypotonia, tongue fasciculations, areflexia. No cure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Asymmetric crying facies

A

At rest there is symm, with crying there is drooping of the lower lip on one side. The side that droops is normal. Absence of specific muscle development 2/2 nerve compression. . It may improve if 2/2 compression, but if dont have the muscle, dont improve. Can have other congenital issues (heart defect).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Rett syndrome

A

X linked disorder, more in females, MEPC2 gene. normal development for the first 6 mos, the development plateaus, head growth slows down, loss of speech or social skills., hand-wringing sterotyped movements. Seizures.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Erb’s palsy

A

Brachial plexus injury. . Inability to abduct,.

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

Guillain-Barre syndrome

A

Acute flaccid paralysis, areflexia, can causes cardiac arrythmias, can have autonomic involvement. Increased protein with no increase in cells in CSF. treated with IVIG or plasmapheresis

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

When does handedness develop?

A

not before 18mos

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

Causes of tethered cord

A

spinal lipoma, shortened filum terminale, dermal sinus tract

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

hypotonia and dysmorphism in infancy

A

Praderwilli, Downs, Zellweger.

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

Who needs 4g folic acid before and during pregnancy?

A

Women who have had kids with NTD or on anticonvulsants.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the Dandy-Walker malformation

A

posterior fossa cyst that is continuous with the 4th ventricle, partial or complete absence of the cerebellar vermis and hydrocephalus.

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

Type 1 Arnold Chiari Malformation

A

The cerebellar tonsils or vermis are down below the foramen magnum. Symptoms occur due to dysfunction of lower cranial nerves, brainstem or spinal cord. Dysphagia, vertigo, sleep apnea, ataxia, headache and neck pain. STRONG association with syringomyelia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Type 2 Arnold Chiari Malformation

A

Most common forma. When 4th ventricle and lower medulla are pushed down below level of foramen magnum. Associate with myelomeningocele and hydrocephalus. More severe than type 1.

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

Type III Arnold Chiari malformation

A

Herniation of cerebellum through a cervical spina bifida defect

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

Klippel-Feil syndrome

A

Short neck, limited neck motion, low occpital hairline - cervical spine is fused. Associated congenital abnormalities are common - deafness, macrocephaly, hydrocephaly.

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

Lissencephaly

A

Associated with Miller Dieker syndrome. No cerebral convolutions - poorly formed sylvian fissure - smooth cerebral surface with thickened cortical mantle.

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

Miller Dieker Syndrome

A

deletion of Chromosome 17. Prominent forehead, bitemporal hollowing, anteverted nostrils, micrognathia, lissencephaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Schizencephaly

A

Unilateral or bilateral clefts within the cerebral hemispheres. Seizure d/o, microcephaly, Intellectual disability, spastic quadrileplgia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Spastic diplegia

A

legs have more spasticity than arms. Most commonly associated with IVH and periventricular leukomalacia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Moya moya disease

A

chronic, occlusive cerebrovascular disease that is associated with sickle cell diease, NF1, trisomy 21, and cranial irradiation. There are extensive collateral vessels surrounding circle of Willis formed from previous occlusions.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Erb palsy

A

From birth trauma - upper brachial plexus damage. Arm hangs limp in internal rotation with pronated wrist. Normal sensory exam. Treat by putting arm in external rotation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Klumpke palsy

A

Lower plexus root injury - more sensory effects and vosmotor involvement. Paralysis of flexors and extensors of forearm

30
Q

What vaccines need to be avoided with egg alelrgY?

A

Can give MMR. If have had anaphylactic reaction, refer to allergy before giving flu shot. If have mild reaction to egg, give inactivated and watch in office for 30 minutes after.

31
Q

Landau Kleffner Syndrome

A

aquired epileptic aphasia - healthy kids lose previously acquired language skills

32
Q

Side effects of valproate

A

Hepatotox - weight gain, thrombocytopenia. Very teratogenic.

33
Q

Carbamazepine side effects

A

hepatotox, leukopenia, SIADH

34
Q

Phenytoin side effects

A

gum hypertrophy, SJS, blood dyscrasia, hirsuitism, ataxia, nystagmus

35
Q

Lamotrigine side effect

A

SJS 8/1000 patients

36
Q

What is most common age for febrile seizures?

A

6-60 months

37
Q

What defines simple febrile seizure?

A

<15 minutes, nonfocal, no recurrance

38
Q

What defines complex febrile seizure?

A

lasts >15 minutes, focal, recurs in 24 hours

39
Q

How often do febrile seizures recur?

A

35% have recurrance, of those, 50% have a third

40
Q

What % of kids with febrile seizure go on to develop epilepsy?

A

1-2%.

41
Q

Duchenne Muscular dystrophy inheritance

A

1/3000 male births. X linked recessive

42
Q

Duchenne MD presentation

A

waddling gait, toe walking, ages 2-6, calf muscle pseudohypertropy, gowers sign, CPK elevated

43
Q

Complications of Duchenne MD

A

respiratory failure, cardiomyopathy

44
Q

Diagnosis of duchenne MD

A

muscle biopsy or gene test

45
Q

Treatment of GBS

A

IVIG or plasmapheresis

46
Q

Treatment of transverse myelitis

A

steroids

47
Q

What is spasmus nutans?

A

Spasmus nutans is an acquired form of nystagmus that occurs in children typically within the first 2 years of life. It presents as a clinical triad of 1) nystagmus 2) head bobbing 3) torticollis. The current theory holds among researchers that head bobbing and torticollis are compensatory mechanisms that improve vision by reducing the frequency and asymmetry of the nystagmus. Nystagmus usually persists until age 5-12

48
Q

What kind of nystagmus does phenytoin cause?

A

horizontal

49
Q

What is a common associated disease in patients with myelomeningocele?

A

80-85% develop hydrocephalus in association with Type II Chiari defect

50
Q

What is Klippel-Fiel syndrome?

A

Short neck, limited neck motion, low occipital hairline. Fused cervical vertebrae

51
Q

Which drug is associated with agenesis of coprus collosum

A

maternal use of cocaine

52
Q

What diseases is Moya Moya associated with?

A

Sickle cell? , NF1, trisomy 21, cranial radiation

53
Q

What conditions are saccular aneurysms associated with?

A

Coarctation, polycystic kidney disease, Marfan or Ehlers danlos

54
Q

What part of the brachial plexis is ERbs palsy associated with?

A

Upper plexus root. Absent biceps tendon, triceps is present

55
Q

What part of the brachial plexus is Klumpke’s palsy associated with?

A

Lower C8-T1. Have more motor and sensory involvement. Can also have Horners syndrome is T1 nerve root involved. Poorer prognosis than with ERbs

56
Q

Risk factors for CP in full term infants

A

Risk factors for CP in full-term infants include advanced maternal age, black race, and intrauterine growth retardation

57
Q

Risk factors for repeated febrile seizure

A

younger age at the first febrile seizure, family history of febrile seizures, lower degree of fever at the time of seizure, and shorter time interval between the onset of fever and the first seizure.

58
Q

Describe generalized seizure

A

involve both hemispheres of the brain, aprupt onset without aura, loss of consciousness bilateral motor activity with change in tone

59
Q

Simple partial seizure

A

last 10-20sec. often have aura, focal, simple means they can still interact with environment, no LOC

60
Q

Complex partial seizures

A

LOC, focal, automatisms and aura common. Usually due to limbic structures

61
Q

What is black box warning on vigabatrin?

A

permanent vision loss

62
Q

Lennox Gestualt syndrome

A

severe seizures, intellectual disability, and characteristic EEG pattern - bilateral, synchronous, sharp-wave and slow wave complexes. Many have history of infantile spasms

63
Q

Rasmussen syndrome

A

immunologic process involving one side of the head, start with generalized seziures, but soon become focal, unremitting, limited to one side of the body. Deteriorates to a stable neurologic deficit

64
Q

What are pathologic laughter seizures associated with?

A

hypothalamic hamartoma

65
Q

which kind of seizures can you use valproate for?

A

generalized tonic-clonic, atypical absence, myotonic

66
Q

ethosuximide side effects

A

skin rash, liver dysfunction, leukopenia

67
Q

How long should kidney with idiopathic epilepsy who have been stable be on meds?

A

2 years seizure-free

68
Q

What neuro deficits are noted in SMA

A

motor deficits, prox>distal, lower>upper. No sensory, facial or intellectual issues

69
Q

type 1 SMA

A

Most severe, presents <6 months, most die by 2 from resp failure

70
Q

Type 2 SMA

A

chronic; lost milestones at age 2, slowing progressive weakness

Pediatrics (25 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions