Brainscape's Knowledge GenomeTM

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


See full index

Step 2 Pediatrics > Pediatric Neurologic Disease > Flashcards

Pediatric Neurologic Disease Flashcards

1
Q

True/False?

Cerebral Palsy is a group of non-hereditary and non-progressive neurologic disorders of muscle tone.

A

True.

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

Cerebral palsy is caused by _______.

A

abnormal development or damage to the fetal or infant’s brain;

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

True/False?

The brain insult/injury causing cerebral palsy is thought to occur during birth (perinatal period).

A

False;

the brain insult/injury causing CP can occur in the prenatal, perinatal, or postnatal periods.

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

What are some prenatal causes of cerebral palsy (CP)?

A
  1. Congenital brain malformations
  2. Intrauterine infections
  3. Intrauterine stroke
  4. Chromosomal abnormalities.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are some perinatal causes of cerebral palsy (CP)?

A
  1. Hypoxic-ischemic insults
  2. CNS infections
  3. Stroke
  4. Kernicterus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are some postnatal causes of cerebral palsy (CP)?

A
  1. Accidental/non-accidental trauma
  2. CNS infections
  3. Stroke
  4. Anoxic insults.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Prenatal events account for approx. ____% of cases of cerebral palsy, and postnatal events account for about ____% of cases of the same.

A

Prenatal events cause ~ 80% of CP cases;

Postnatal events cause ~ 10% of CP cases.

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

_____ is the most significant risk factor for cerebral palsy.

A

Prematurity.

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

Complications of prematurity that can cause cerebral palsy include____ (list most).

A
  1. Periventricular leukomalacia
  2. Intraventricular hemorrhage
  3. Periventricular infarcts.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Other than prematurity, what are some other risk factors a/w cerebral palsy?

A

-Low- birth weight.
-multiple gestation,
-IUGR,
-maternal substance abuse,
-preeclampsia,
-chorioamnionitis,
-abnormal placental pathology,
-meconium aspiration,
-perinatal hypoglycemia, and
-genetic susceptibility.

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

True/False?

Cerebral palsy is marked by a delay in motor development and/or developmental regression during infancy or early childhood.

A

False;

Cerebral palsy is marked by ONLY a delay in motor development;
developmental regression in an infant/child is not consistent with cerebral palsy.

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

List the 6 subtypes of cerebral palsy?

A
  1. Spastic diplegic: legs > arms.
  2. Spastic hemiplegic: arms > legs.
  3. Spastic quadriplegic: UE > legs
  4. Dyskinetic/hyperkinetic (choreo-athetoid): involuntary, rapid, dance-like contractions of muscles and slow writhing movements.
  5. Dystonic: involuntary, sustained muscle contractions causing twisting and repetitive movements.
  6. Ataxic: unsteadiness, incoordination; often hypotonic.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

The _____ is a standardized motor assessment used in children under age 5 months corrected age, that observes the quality of spontaneous movements in infants while lying supine.

A

The General Movements (GM) Assessment (98% sensitivity & 89% to 93% inter-rater reliability).

*GMs are spontaneous movements generated by the fetal/neonatal nervous system involving a variable sequence, intensity, speed, and range of motion of the neck, arm, trunk, and leg movements. GMs have a gradual onset and end with an impression of fluency and elegance. GMs mostly have a similar appearance from early fetal life until the end of the second month after term; from term age onward, they are called “writhing movements.” Starting at 6–9 weeks post-term age, writhing movements are gradually replaced by movements of a fidgety character.

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

Abnormal GMs during preterm and term age are classified as ____, ____, and ____ GMs.

A
  1. Poor repertoire GMs: marked by monotonous sequence and lack of variability in the intensity, speed, and range of motion.
  2. Cramped-synchronized GMs: lack smoothness and fluency, and appear rigid as the limb and trunk muscles almost contract simultaneously and relax simultaneously.
  3. Chaotic GMs: abrupt and tremulous with a large amplitude and high speed (rare at/beyond term age except in the moderate preterm age).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Cramped-synchronized general movements and the absence of fidgety movements between 9 to 20 months reliably predict ____.

A

cerebral palsy.

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

List some disorders associated with cerebral palsy.

A

-Seizure disorder
-behavioral disorders
-hearing or vision impairment
-learning disabilities.
-speech deficits
-GI disorders
-Other complications of prematurity.

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

____ before the age of 1 year is a red flag for cerebral palsy.

A

definite hand preference

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

True/False?
Toe walking and scissor gait are commonly seen in young children with cerebral palsy.

A

true

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

Patients with cerebral palsy may p/w ____ type of orthopedic abnormalities.

A

-hip dislocations
-scoliosis

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

List some pharmaceutical agents that can be used to treat spasticity in patients with cerebral palsy.

A

-Baclofen
-Diazepam
-Dantrolene

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

Botulinum toxin may be used to treat ___ in patients with cerebral palsy.

A

localized spasticity.

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

Baclofen pumps and posterior rhizotomy may be used to alleviate ____ in patients with cerebral palsy.

A

severe contractures

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

Febrile seizures typically present in children between the ages of ___ and ____, with a fever greater than ____ that is not associated with a CNS infection, a known seizure-provoking etiology (eg, electrolyte imbalance, hypoglycemia, or substance abuse), or a history of afebrile seizure.

A

generalized seizures;

6 months and 5 years;

fever > 100.4 °F (38 °C) that is not associated with a CNS infection, a known seizure-provoking etiology (eg, electrolyte imbalance, hypoglycemia, or substance abuse), or h/o an afebrile seizure.

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

The primary febrile seizure risk factors appear to include existing neurologic impairment, the presence of a viral infection, a family history of seizure, developmental delay, decreased serum zinc and iron levels, and maternal smoking and stress.[1]

A

-Rapid rise in body temperature.
-close family h/o a febrile seizure.

-existing neurologic impairment,
-viral infection,
-developmental delay,
-decreased sr. zinc and iron levels,
-maternal smoking and stress.

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

True/False?
Febrile seizures may recur in approximately 1 in 3 patients.

A

True.

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

Most febrile seizures are of ____ type.

A

Simple febrile seizure:
defined as a single generalized tonic-clonic seizure, lasting 15 minutes or less, in a 24-hour period and return to the neurologic baseline shortly after the seizure.

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

Complex febrile seizures are defined as ____

A

a long seizure (> 15 minutes), focal seizure, or multiple seizures occurring within 24 hours or no return to the neurologic baseline.

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

Febrile status epilepticus refers to seizures lasting longer than _____ duration.

A

longer than 30 minutes.

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

True/False?

Febrile status epilepticus is associated with more adverse outcomes than simple febrile seizures.

A

True.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

True/False?
80% of viral infections rather than bacterial infections are commonly associated with febrile seizures.

A

True.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

____ is the most common virus a/w febrile seizures in the US and European countries and is observed in up to 1/3rd of patients younger than 2 years.

A

Roseolovirus.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

In Asian countries, ____virus has been frequently associated with febrile seizures.

A

influenza A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Postictal weakness or paralysis on one side of the body following a complex febrile seizure is also known as ____.

A

Todd paralysis.

34
Q

When is LP and CSF study indicated in a patient p/w a febrile seizure?

A

After ruling out raised ICP (papilledema), LP is indicated in patients such as below

-p/w signs of CNS infections (e.g. altered consciousness, meningismus, tense/bulging anterior fontanelle).
-infants < 12 months,
-not adequately immunized against Strep pneumoniae or H influenza type B),
-had a seizure 2 days after fever onset, or
-taking antibiotics (as they may mask the signs of meningitis or other CNS infections).

35
Q

A ____ is not necessary for a patient with simple febrile seizures and a rapid return to baseline, except in cases where s/o ___ are present.

A

lumbar puncture;

except in cases where s/o CNS infections are present.

36
Q

In infants < 6 months of age, ____ work-up must be done regardless of the type of seizure.

A

Sepsis work-up
-CBC
-UA
-Blood, urine, and CSF culture.

37
Q

MRI or CT studies of the head for febrile seizures are not typically considered unless _____ conditions are present (list all).

A

-Increased ICP
-Focal neurologic abnormality
-Suspected structural defect
-Enlarged head
-Severe head injury

38
Q

_____ type of febrile seizures can be managed with only observation and parental counseling.

A

first-time simple febrile seizure.

39
Q

When is treatment with benzodiazepine (IV Lorazepam, rectal diazepam, or intra-nasal midazolam) indicated in the m/m of febrile seizures?

A

In patients with
-febrile status epilepticus, or
-seizures lasting longer than 5 minutes.

40
Q

Studies suggest that around 30% of children with a history of febrile seizures are at an increased risk of having recurrent episodes. Children younger than 12 months at the time of their first febrile seizure have a 50% chance of experiencing a second seizure within the first year. However, this risk decreased to 30% in the following year. Factors such as young age during the initial seizure, a family history of febrile seizures, low fever intensity during the seizure, and a short interval between fever onset and the seizure may indicate a higher likelihood of recurrent febrile seizures. On the other hand, features associated with complex febrile seizures do not necessarily increase the risk of recurrence.

A
41
Q

Studies suggest that around ___% of children with a history of febrile seizures are at an increased risk of having recurrent episodes.

A

30%

Data Source: https://www.ncbi.nlm.nih.gov/books/NBK448123/

42
Q

Children younger than 12 months at the time of their first febrile seizure have a____ % chance of experiencing a second seizure within the first year, and ___ % in the following year.

A

50%;

30% in the following year.

Data Source: https://www.ncbi.nlm.nih.gov/books/NBK448123/

43
Q

children with a history of ____ type of febrile seizure have a higher risk (estimated 2-10%) of developing epilepsy as compared to ___ groups.

A

complex febrile seizure;

as compared to children with simple febrile seizures, and the general population.

44
Q

Central causes of hypotonia in infants include ____ conditions/disorders.

A

-hypoxic encephalopathy,
-brain anomalies/insults,
-genetic/chromosomal syndromes,
-Infections (congenital/acquired), and
-metabolic disorders.

45
Q

Peripheral causes of hypotonia in infants include ____ conditions/disorders.

A

-spinal muscular atrophy,
-myasthenia gravis,
-drug/toxin exposure,
-hereditary neuropathies,
-muscular dystrophies,
-congenital/metabolic myopathies, and
-congenital myotonic dystrophies.

46
Q

Central hypotonia accounts for the majority of cases of hypotonia in infants, and is seen in up to ___% of cases.

A

60%-80% of cases.

47
Q

Hypotonia at birth with a poor Apgar score may indicate _____ as the cause of hypotonia, whereas poor muscle tone developing 12 - 24 hours after birth may indicate ____.

A

hypoxic-ischemic encephalopathy;

metabolic disorder

48
Q

Muscle weakness in an infant may manifest with ____ s/s.

A

-decreased spontaneous movements (evaluate the cry, suck reflex, spontaneous movements, facial expressions, antigravity movements, and respiratory effort).

49
Q

What is the mechanism of hypotonia in botulism?

A

Clostridium botulinum toxin prevents the pre-synaptic release of Ach.

*infantile disease acquired from spores in soil or unpasteurized honey.

50
Q

____ may be the first presenting sign of infantile botulism.

A

constipation

51
Q

_____ is the most common suprasellar brain tumor in children, leading to bi-temporal hemianopsia d/t compression of the ____.

A

Cranio-pharyngioma;

compression of the optic chiasm.

52
Q

____ distinguishes a craniopharyngioma from a pituitary adenoma on neuroimaging.

A

calcification.

53
Q

Cholesterol crystals in the fluid within a ____ brain tumor give the appearance of “motor-oil”-like fluid within the tumor.

A

craniopharyngioma

54
Q

Craniopharyngioma is a ___ (? benign, malignant) tumor of childhood.

A

benign;
surgical resection is the mainstay t/t.

55
Q

______ is the most common CNS tumor in children.

A

Pilocytic astrocytoma.

*GFAP +ve

56
Q

_____ is a highly malignant (but radiosensitive) CNS tumor in children, that is of primitive neuroectodermal origin (PNET).

A

Medulloblastoma

*highly malignant hence m/m with surgical resection coupled with RT and CT.

57
Q

Medulloblastoma, a PNET usually arises from ___ posterior fossa/infratentorial locations.

A

-4th ventricle (obstructive hydrocephalus, headache, papilledema, vomiting)

or
-cerebellar vermis (truncal ataxia)

58
Q

Seed metastasis into the subarachnoid space or spinal cord may be seen in ____ CNS tumor.

A

Medulloblastoma.

59
Q

The most common type of spinal dysraphism is ____.

A

spina bifida occulta (closed spinal dysraphism).

60
Q

Examples of open spinal dysraphism include ____ (list all).

A

-Meningocele
-Meningomyelocele

61
Q

Open spinal dysraphisms such as Meningocele or Meningomyelocele are often detected by high levels of _____ in a ____ test.

A

high levels of AFP in a quad screen conducted during pregnancy.

62
Q

AFP is synthesized in the ____ during pregnancy; it is also re-expressed in multiple adult tumors of ______ origin.

A

yolk sac, fetal liver, and fetal GI tract during pregnancy;

also re-expressed in multiple adult tumors of mixed mesodermal and endodermal origin.

63
Q

Elevated maternal Serum AFP Levels are associated with ____ conditions (list all).

A

-NTDs (spina bifida, anencephaly)

-Fetal GI Tract anomalies (Omphalocele, Gastroschisis)

-Sacrococcygeal teratoma
-Placental abnormalities
-Cystic Hygroma
-Renal abnormalities (polycystic kidney or absent kidney, urinary obstruction, congenital nephrosis).

-Osteogenesis imperfecta
-Threatened abortion
-IUGR

64
Q

Low maternal Serum AFP Levels are a/w ____ conditions (list all).

A

-Down syndrome
-Increased maternal weight
-Fetal demise
-Hydatidiform mole
-Trisomy 18 (Edward Syndrome)
-Incorrect gestational age (older than calculated).

65
Q

Elevated serum AFP level in a male or non-pregnant female is a/w ____ malignancies (list all).

A

Hepatocellular cancer
Metastatic liver cancer
Liver cirrhosis
Hepatitis
Germ cell tumors
Yolk sac tumor
Ataxia-telangiectasia

66
Q

____ type of spinal dysraphism can be managed with monitoring only as patients are mostly asymptomatic.

A

closed spinal dysraphism (e.g. spina bifida occulta).

67
Q

Open spinal dysraphisms must be treated with ____ within ____ duration after birth to prevent ___ complications.

A

treated with surgery within 3 days of birth to prevent infection of the exposed meninges, and spinal cord.

68
Q

Breath-holding spells typically occur in children between the ages of ____ and ____.

A

6 months to 6 years of age.

69
Q

Breath-holding spells may be a/w _____ anemia.

A

iron-def. anemia

70
Q

____ is the most common variant of the breath-holding spells.

A

Cyanotic

71
Q

True/False?
Pallid variant of breath-holding spell often develops in response to a mild trauma to the head or upper body, and is marked by pallor and diaphoresis followed by loss of muscle tone and consciousness.

A

true.

72
Q

The vast majority of children experience complete resolution of breath-holding spells by ___ age.

A

8 years of age

73
Q

Rett syndrome is a neurodevelopmental disorder due to a mutation in the _____ gene.

A

MECP2 gene on x-chromosome.

74
Q

Why does Rett syndrome commonly manifest only in girls?

A

because male fetuses with the mutated gene mostly die in utero or immediately after birth.

75
Q

Clinical manifestation of Rett syndrome begins around ___ age, and is marked by characteristic c/p that includes ___ s/s.

A

onset ~ age 6-18 months in girls;
Characteristic C/P:
-“Regression” after normal growth (deceleration of head growth, progressive loss of spoken language, fine & gross motor skills, and intellectual abilities.

-DISTINCTIVE FEATURE: stereotypical hand movements such as grasping of hair or clothing, and episodes of inconsolable irritability.

ADDITIONAL manifestations:
-Scoliosis
-growth failure
-Epilepsy
-loss of bone mineral density
-cardiac defects
-autonomic dysfunction
-sleep dysfunction
-abnormal breathing while awake

76
Q

The diagnosis of Rett syndrome is confirmed by ____.

A

DNA analysis showing the mutation in the MECP2 gene.

77
Q

Chiari I malformation marked by herniation of the _____ through the foramen magnum typically presents during childhood with ___ s/s.

A

herniation of the cerebellar tonsils through the foramen magnum;

c/p:
-occipital headaches (worse with valsalva maneuvers e.g. cough)
-ataxia and dizziness (cerebellar s/s).

78
Q

Patients with Chiari II malformation present at birth with apnea, stridor, dysphagia, and non-communicating hydrocephalus due to herniation of ______ through the foramen magnum.

A

herniation of the cerebellar tonsils, vermis, and medulla

79
Q

____ type of Chiari malformation is usually a/w Syringomyelia, whereas ___ type is a/w lumbosacral myelomeningocele?

A

Chiari I with Syringomyelia;

Chiari II with lumbosacral myelomeningocele (lower limb weakness and sensory loss).

80
Q

Microcephaly is defined as head circumference (HC) of less than ____, and macrocephaly is defined as HC of more than ____.

A

Microcephaly: HC < 2 SD below the mean;

Macrocephaly: HC > 2 SD above the mean.

81
Q

True/False?
Benign familial micro-/macro-cephaly is marked by normal growth and development in a child with a normal parent with a similar head size.

A

true.

Step 2 Pediatrics (15 decks)

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