3 Peds Neurology Flashcards

(86 cards)

1
Q

____% of kids 18 or younger experience some sort of developmental delay

A

18%

Common concern presenting to primary care, with many etiologies

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2
Q

What is important to ascertain when investigating a potential developmental delay?

A

You want to see a TREND over time, not a single out-of-range measurement/milestone

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3
Q

Non-progressive central motor impairment secondary to fetal or infantile brain injury

A

Cerebral Palsy

Incidence: 2 per 1,000 births

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4
Q

Possible etiologies of cerebral palsy

A
Hypoxia***
Trauma
Premature birth
Infections
Toxins
Structural abnormalities
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5
Q

What are the different subtypes of cerebral palsy?

A

SPASTIC - most common (70-80%)
Muscles appear stiff and tight, arises from MOTOR CORTEX damage

ATAXIC (6%)
Characterized by the shaky movements; affects balance and sense of positioning in space; arises from CEREBELLUM damage

DYSKINETIC (6%)
Involuntary movements, arises from BASAL GANGLIA damage

Can have a combo, sometimes not noticed until 18-24 months

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6
Q

SSx of Cerebral Palsy

A
Abnormal tone and/or posture
RETAINED PRIMITIVE REFLEXES
Not reaching milestones 
Excessive irritability
Poor feeding, drooling
Poor visual attention
Difficult to hold, cuddle
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7
Q

What are some retained primitive reflexes common in cerebral palsy

A

ATNR (Asymmetric tonic neck reflex - the fencer’s pose) - baby turns head to extended arm; usually gone by 4-6 months

Moro Reflex - baby stretching out his arms with a startle on suddenly being released for an instant

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8
Q

Management of Cerebral Palsy

A

EARLY RECOGNITION, REFERRAL, AND INTERVENTION

Parental support and counseling

Symptomatic management
• OT, PT, bracing
• Anti-spasmodic
• Botulism toxin

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9
Q

Nervous system malformations are present in ____% of infants who die <1 year

A

40%

Type of malformation dependent on what gestational period the insult occurs

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10
Q

Etiologies of congenital malformations of CNS

A
Infections 
Toxins
Genetic
Metabolic
Vascular
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11
Q

Cerebellum tonsils displaced causally below the forsaken magnum

A

Chiari Type I

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12
Q

Can be associated with syringomyelia (fluid filled cyst within spinal cord)

A

Chiari Type I

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13
Q

Symptoms of this CNS malformation often don’t present until teen or adult years

A

Chiari Type I

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14
Q

Loss of abdominal reflex

Neurological Sx assoc with syringomyelia

Cape-like numbness

A

Chiari Type I

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15
Q

What’s the other name for Chiari Type II?

A

Arnold-Chiari malformation

It’s a Type I + myelomeningocele

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16
Q

CNS malformation usually detected prenatally or at birth

A

Chiari Type II

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17
Q

Hydrocephalus

Dysphagia

UE weakness

Apneic spells and aspiration

A

Chiari Type II

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18
Q

What are the three types of Spinal Dysraphism?

A

Spina Bifida Occulta (most mild)

Meningocele

Myelomeningocele (most severe)

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19
Q

Incomplete closure of the spinal canal, usually on the lower back but can be anywhere

A

Spina Bifida Occulta

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20
Q

Hairy patch, lumbar dimple, dark spot, swelling on the back at the site of the gap in the spine

A

Spina Bifida Occulta

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21
Q

Outpouching of the spinal fluid and meninges through a vertebral cleft

A

Meningocele

Mild problems associated with the sac protrusion

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22
Q

Spinal cord and/or nerves protrude from the vertebral cleft

A

Myelomeningocele

Most severe form of spina bifida

Weakness, loss of bladder and/or bowel control, hydrocephalus, and inability to walk result

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23
Q

Spinal Dysraphisms can occur anywhere along the spine but _________ is most common

A

Lumbar spine

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24
Q

What are some common etiologies of spina bifida

A

Genetics

LOW FOLATE***

Medications during pregnancy (esp anti seizure meds)

Poorly managed diabetes

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25
Early recognition of spina bifida is possible by...
U/S Alpha-feto protein markers
26
Best way to prevent Spina Bifida
Prenatal vitamins including folic acid 3 months prior to conception
27
Increased volume of CSF causing ventricular dilation and increased intracranial pressure
Hydrocephalus
28
What are the two types of hydrocephalus?
Obstructive - due to blockage Non-obstructive - impaired absorption or (rarely) overproduction
29
Etiologies of Hydrocephalus
``` CNS malformations Infection Intraventricular hemorrhage Genetic defects Trauma CNS tumors ```
30
SSx of hydrocephalus
``` (Can be asymptomatic) Bradycardia, HTN, altered respiratory rate HA, N/V, behavior changes Papilledema Macrocephaly Spasticity Diplopia Spinal abnormalities ```
31
How is hydrocephalus diagnosed?
Newborns/infants: U/S Older infants/children - MRI or CT
32
Treatment for hydrocephalus
Refer to neurosurgeon Shunt
33
What is the definition of Microcephaly
Head circumference ≥2 standard deviations below average or <5th percentile Can be primary (congenital) or secondary (postnatal)
34
Lack of brain development or abnormal development due to timing of insult
Primary microcephaly
35
Injury or insult to a previously normal brain
Secondary (postnatal) microcephaly
36
If microcephaly is symptomatic, what might you see?
Delayed milestones Seizures or spasticity Fontanelle may close early and sutures may be prominent
37
What is the definition of Macrocephaly?
Head circumference ≥2 standard deviation above average or >95th percentile Can be due to increase in size of any components of cranium (brain, CSF, blood, or bone)
38
Rapid growth —> macrocephaly is suggestive of...
Increased intracranial pressure
39
Catch-up growth macrocephaly is typically seen in...
Premature infants (neurologically intact)
40
Normal growth rate macrocephaly is usually the result of ...
Familial macrocephaly or megaloencephaly
41
How are micro- and macrocephaly managed?
Neurology referral Labs/imaging Treat underlying cause
42
Inheritance pattern for Neurofibromatosis (NF1)
Autosomal dominant
43
What neurological abnormalities are associated with neurofibromatosis?
Macrocephaly, seizures, cognitive defects
44
Clinical manifestations of NF1
Cafe-au-lait macules (appear 1st year) Axillary and/or inguinal freckling (3-5 years) Lisch nodules Optic gloom a Neurofibromas
45
Most common types of primary headache
Migraine and tension
46
Most common secondary headache
Acute Febrile Illness
47
Migraine or Tension H/A: Focal, unilateral or bilateral
Migraine
48
Migraine or Tension H/A: Diffuse, frontal or temporal
Tension
49
Migraine or Tension H/A: Duration = 2-72 hours
Migraine
50
Migraine or Tension H/A: Duration = 30 min - 7 days
Tension
51
Migraine or Tension H/A: Moderate to severe intensity, pulsatile/throbbing
Migraine
52
Migraine or Tension H/A: Mild to moderate intensity, constant pressure/non-throbbing
Tension
53
Migraine or Tension H/A: Aggravated with activity or reduced activity
Migraine
54
Migraine or Tension H/A: Not aggravated with activity
Tension
55
When to worry about a headache
Abnormal neurologic or visual exam Severe upon awakening or awaken in the middle of the night Daily symptoms with progressive worsening Accompanied with vomiting Acute onset with previous history Increased with coughing or bending
56
Idiopathic intracranial hypertension without mass or hydrocephalus, typically seen in obese teenage girls
Pseudotumor cerebri
57
SSx of Pseudotumor Cerebri
``` HA*** Papilledema*** Visual symptoms Visual field loss, visual acuity loss Pulsatile tinnitus ```
58
How is pseudotumor cerebri diagnosed?
Neuro eval —> MRI to exclude secondary causes, LP Ophthalmologic eval
59
What will an LP show in pseudotumor cerebri?
Elevated opening pressure
60
Treatment of pseudotumor cerebri is focused on ...
Managing symptoms and preserving vision DOC - ACETAZOLAMIDE to reduce rate of CSF production Topiramate to help control HA and reduce weight Furosemide to reduce fluid and pressure Weight loss Shunting of fluid Optic nerve fenestrations
61
Sudden, transient disturbance of brain function manifested by involuntary sensory, motor, autonomic symptoms with or without loss of consciousness
Seizure
62
What is the definition of epilepsy?
≥ 2 seizures occurring more than 24 hours apart ``` Types: Focal (partial) - with or without impaired awareness Generalized Unknown Unclassified ```
63
Sudden impairment of consciousness w/o loss of tone, provoked by hyperventilation and presumed to be genetic
Absence Seizures Presents between age 4-10 and most often spontaneously remits by puberty Arrest in activity generally lasts 9-10 sec and may occur 10x/day
64
1st line medication for absence seizures
Ethosuximide
65
Convulsion w/ temp > 38˚C (100.4˚F) in a child age 6 months to 5 years
Febrile seizure Often associated with viral infection, genetic predisposition
66
Most common type of febrile seizure, lasting <15 min
Simple febrile seizure
67
How are complex febrile seizures defined?
Focal, last >15 min or occur >1/24 hours
68
How do you manage a febrile seizure
Generally self-limiting If >5 min, give IV benzodiazepines
69
Acute immune-mediated polyneuropathy preceded by illness (most commonly campylobacter)
Guillian-Barre Syndrome
70
Most common cause of acute flaccid paralysis in health infants/children
Guillian-Barre Syndrome
71
ASCENDING symmetric weakness, neuropathic pain, gait instability/refusal to walk, and absent reflexes
Guillian-Barre Syndrome
72
What is the primary diagnostic tool for Guillian-Barre syndrome?
Electrodiagnostic studies (EMG) Can also do CSF analysis or Spinal MRI
73
Treatment of Guillian-Barre Syndrome
Hospitalization and close monitoring Treatment with IVIG or plasma exchange
74
Honey and home canning are no-nos because of ...
Risk of ingesting clostridium botulinum spores —> botulism
75
90% of infants with botulism are _______
<6 months old
76
DESCENDING weakness Constipation, poor feeding Hypotonia, loss of DTRs Irritable and lethargic
Botulism
77
How is botulism diagnosed?
Stool sample EMG
78
How is botulism treated?
Hospitalization and close monitoring Botulism immune globulin (BIG-IV or BabyBIG)
79
Inheritance pattern for Duchenne Muscular Dystrophy
X-linked recessive Defect is in genes responsible for muscle function —> elevated muscle enzymes
80
What is the outlook for kids with DMD?
Wheelchair bound by age 13 and mortality by 18-20 years
81
Progressive weakness, proximally before distally, LE before UE
Duchenne Muscular Dystrophy
82
What is Gower’s sign?
Using hands to push up from floor (DMD)
83
Conditions associated with DMD?
DCM Orthopedic complications (ie scoliosis) Cognitive impairment
84
Diagnosis of Duchenne Muscular DystrophY
Elevated muscle enzymes - CK ≥10-20x normal Genetic testing
85
Treatment for DMD
GLUCOCORTICOIDS
86
How is Becker Muscular Dystrophy different from DMD?
Symptom onset later Muscle involvement not as severe CK elevated ≥5x Cardiomyopathy may be more predominant