Neuro Flashcards

1
Q

Which neuro abnormalities are seen in pts w/ static sxs?

A

congenital abnormalities or brain injury (think cerebral palsy)

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2
Q

Which neuro abnormalities are seen in pts w/ progressive sxs?

A

degenerative disease or neoplasn

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3
Q

Which neuro abnormalities are seen in pts with intermittent sxs?

A

epileptic or migraine syndromes

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4
Q

Which neuro abnormalities are seen in pts with saltatory sxs?

A

bursts of sxs followed by partial recovery

-vascular, demyelinating d/o

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5
Q

What PE findings may indicate spine bifida?

A

Midline defects:

tufts of hair, lipomas, dimpling

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6
Q

Accelerating pattern of head circumference may indicate? decelerating pattern?

A

hydrocephalus

degenerative neurologic disorder

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7
Q

How can you assess CN II?

A

pupillary light reflex, visual acuity

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8
Q

How can you assess CN III, IV, VI?

A

following objects, fixating, oculocephalic reflex, EOMs

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9
Q

How can you assess CN V?

A

sucking/swallowing, light touch

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10
Q

How can you assess CN VII?

A

observe fast at rest, crying/blinking

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11
Q

How can you assess CN VIII?

A

hearing

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12
Q

How can you assess CN IX, X?

A

gag reflex, sucking, salivation

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13
Q

How can you assess CN XI?

A

posture, spontaneous movement

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14
Q

How can you asses CN XII?

A

tongue movement

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15
Q

What are some primitive reflexes? When do most of them disappear?

A

Moro, grasp, rooting, foot placing, tonic neck

4-6 months

asymmetry indicates focal brain or PNS lesions

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16
Q

s/s seen w/ upper motor neurons

A

Spastic paralysis, Increased tone

Increased DTRs/+Babinski (in older children, normal in infants); usually with clonus

Minimal muscle atrophy/strength loss

Fasciculations absent

May have sensory disturbances

STIFFNESS, INCREASED PASSIVE TONE

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17
Q

s/s seen w/ lower motor neurons?

A

Flaccid paralysis

Decreased tone

Absent DTRs

Profound muscle Atrophy

Fasciculations present

May have sensory
disturbances

WEAKNESS, DECREASED PASSIVE TONE

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18
Q

What are some red flags for children w/ headaches?

A

headache in child <5y/o

new (“explosive onset”) & worsening HA in a previously healthy child

worst HA of life

unexplained fever

night time or early morning awakening HA

HA w/ vomiting

HA worse w/ straining

postural HA

neurocutaneous stigmata

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19
Q

What is the most concerning headache pattern in children?

A

chronic progressive
>4 months or >15x/month

usually increased ICP

possible psych factors

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20
Q

When should you order imaging in child presenting w/ HA?

A

Abnormal neurologic exam

Concern for space occupying lesion

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21
Q

Typical pediatric sxs for migraines?

A

Frontal, bitemporal or unilateral throbbing for 2-72 hrs (Unilateral sxs usually after puberty)

sxs relived by sleep

+/- visual aura

N/V, abdominal pain, phono/photophobia

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22
Q

Tx for migraines?

A

eliminate triggers: HA diary

acute: NSAIDs, APAP, triptans, antiemetics

Prophylaxis:
<6: Cyproheptadine
>6: Propranolol, Amitriptyline, Topiramate

Non pharm: B12

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23
Q

What is Pseudotumor Cerebri - Idiopathic intracranial hypertension (IIH)

A

elevated ICP w/ norm cerebrospinal fluid composition, and no other cause of intracranial hypertension

s/s: HA, papilledema, vision loss, intracranial noises, photopsia

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24
Q

Epidemiology of pseudotumor cerebri?

A

MC in females of childbearing age; does occur in peds- usually adolescents (11 years +)

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25
MC risk factor for pseudotumor cerebri?
obesity assoc. meds: tetracycline, steroids, retinol
26
Dx criteria for pseudotumor cerebri?
each of the following: Papilledema OR VI (abducens) nerve palsy (unilateral or bilateral) Normal neuro exam, except for papilledema and CN abn Neuroimaging: norm brain parenchyma w/o evidence of hydrocephalus, mass, structural lesion, or meningeal enhancement Normal CSF composition Elevated LP opening pressure
27
When should you check imaging in pt with concern for pseudotumor cerebri?
Imaging BEFORE lumbar puncture Increased ICP may cause cerebral herniation when LP is performed if obstructive hydrocephalus or mass
28
What is the main complication of pseudotumor cerebri?
vision loss
29
Tx for pseudotumor cerebri?
sometimes sxs resolve after diagnostic LP wt loss for obese pts Meds: (decreases the volume & pressure of CSF w/in the CNS): Acetazolamide Topiramate Surgery - optho eval critical - CSF shunt when all else fails decrease salt intake
30
What is cerebral palsy?
heterogeneous group of conditions involving permanent nonprogressive central motor dysfunction that affect muscle tone, posture, and movement results from brain injury or malformation (before birth, during or after delivery)
31
Prenatal causes of cerebral palsy?
Prematurity IU growth restriction IU infection Antepartum hemorrhage Severe placental pathology Multiple pregnancy Hypoxic brain injury Stroke Cerebral dysgenesis
32
Postnatal causes of cerebral palsy?
``` Stroke Kernicterus Trauma Near-drowning Toxins Hypoxic brain injury ```
33
What are the MC delayed motor milestones in children w/ CP?
Not sitting by 8 mo Not walking by 18mo Early asymmetry of hand function (hand preference) before 1yr
34
What are the 4 major classification of CP?
spastic (MC) athetoid/dyskinetic Ataxic (extremely rare) Atonic
35
Describe spastic CP
features of an upper motor neuron syndrome
36
Describe athetoid/dyskinetic CP
Athetoid: slow, smooth, writhing movements that involve distal muscles Dyskinetic: decreased spontaneous movement, hypotonia, and suppressed primitive reflexes
37
Describe ataxic CP
wide-based gait, intention tremor, slow, jerking movements affects fine coordinated movements of the UE motor milestones & language skills are usually delayed ataxia may improve w/ time
38
Describe Atonic CP
Severe hypotonia, never stand or walk. may have cerebral Dysgenesis, microcephaly, profound intellectual disability
39
CP tx goals?
Social and emotional development Communication Education Nutrition Mobility Maximal independence in ADLs
40
What is spina bifida?
Neural tube disorder (NTD)= defective closure of the caudal neural tube early in gestation (about week 4) -spina bifida is MC
41
What are the subtypes of spina bifida?
Meningomyelocele-most serious! Meningocele Spina bifida occulta
42
Describe a meningomyelocele
Meninges and spinal cord exposed total paralysis, loss of B/B control accompanying Chiari II malformation*
43
Describe a meningocele
Spinal canal and meninges are exposed, underlying spinal cord is intact
44
Describe spina bifida occulta
Skin intact but underlying defects in bone and spinal canal present May see sinus tract, dimple or tuft of hair. May have neuro deficits
45
Prevention of spina bifida?
FOLIC ACID supplementation- pre-preg & during preg
46
Dx of spina bifida
Routine screening w/ alpha-fetoprotein (AFP) level (16-18 w) in maternal serum & US (12-14 w & 18-20 w)
47
management of spina bifida?
Surgical closure VP shunt Adjunct supportive therapies etc. Cognitive disabilities, self-catheterization, etc. px varies
48
What is a chiari malformation?
heterogeneous group of disorders: anatomic anomalies of the cerebellum, brainstem, and craniocervical junction, w/ downward displacement of the cerebellum, either alone or together with the lower medulla, into the spinal canal 
49
What are the types of chiari malformations?
Chiari 1: downward displacement of medulla and cerebral tonsils, syringomyelia Chiari 2 (Arnold-chiari malformation) Chiari 3 Chiari 4: hypoplasia/aplasia of the cerebellum w/ spina bifida NOT COMPATIBLE W/ LIFE
50
Chiari malformations cause...
malformation obstructs the outflow of cerebral spinal fluid (CSF) through the posterior fossa --> HYDROCEPHALUS
51
Almost all pts w/ a myelomeningocele have...
chiari II malformation and most have assoc. hydrocephalus
52
What is hydrocephalus?
Increased volume of CSF w/ progressive ventricular dilation Slowly evolving accumulation of CSF over weeks to months can be congenital or acquired
53
What is communicating hydrocephalus?
CSF circulates through the ventricular system & into the subarachnoid space w/o obstruction
54
What is non-communicating hydrocephalus?
an obstruction blocks the flow of CSF w/in the ventricular system or blocks CSF from going from the ventricular system into the subarachnoid space
55
Presentation of hydrocephalus?
Sx related to ventricular distention and increased ICP obstruction of CSF flow HA, vomiting, AMS, visual changes, ocular nerve palsies, and focal neuro findings Infants: nonspecific --> vomiting, lethargy, irritability, bulging fontanelle, poor feeding, etc.
56
PE findings seen in pt with hydrocephalus?
VS- bradycardia, HTN, altered respiration (think brainstem) Increased head circumference, bulging anterior fontanelle, abn skull contour, CN dysfunction, papilledema.
57
Tx for hydrocephalus?
Temporary, symptomatic relief: loop diuretic, acetazolamide Surg intervention: remove obstructive lesion, VP shunt
58
Inheritance pattern for the common forms of SMA?
autosomal recessive caused by biallelic deletion or mutations in the SMN1 gene on chromosome 5q13
59
What are SMA disorders?
characterized by degeneration of the anterior horn cells in the spinal cord and motor nuclei in the lower brainstem, which results in progressive muscle weakness and atrophy
60
When do most cases of SMA present?
in infancy
61
What are the 5 subtypes of SMA?
SMA 0= prenatal onset, severe weakness, hypotonia and often reflexia at birth SMA1: most devastating, present at birth, progress to respiratory failure by 1 SMA2: sxs begin later in life, weakness and decreased DTRs by 2 SMA3: weakness near adolescence SMA4
62
When should SMA be suspected?
for any infant with unexplained weakness or hypotonia Presents with progressive proximal weakness , decreased spontaneous mvmt, “floppiness”
63
Dx of SMA?
EMG, muscle Bx, DNA testing homozygous deletion of exon 7 of SMN1 (confirms diagnosis)
64
Tx of SMA?
No tx Sx therapy to improve flexibility, prevent infections, maintain social, language and intellectual stimulation
65
What is Guillain barre syndrome (acute idiopathic polyneuritis)
Acute immune-mediated polyneuropathy Heterogenous syndrome, variant forms
66
Presentation of GBS?
Usually presents as an acute paralyzing illness provoked by a preceding infection Ascending weakness (symmetric) Many have sensory sxs Loss of DTRs early May have autonomic nerve dysfunction Children- refusal to walk and leg pain are MC presenting sxs
67
Etiology of GBS?
Causes: Post-infectious - Resp or GI infection - Campylobacter jejuni MC
68
Dx of GBS?
LP- Elevated CSF protein without increased WBC Electromyography (EMG)
69
Px of GBS?
85% of children have excellent recovery May require ventilator support Death from autonomic dysfunction, respiratory failure, complications (3-4%) -PNA, PE, CV collapse
70
Tx of GBS?
IVIG: combat invading organisms- lessen attack on NS Plasmapheresis: decrease severity and duration, removes Ab from circulation
71
Transmission of duchenne muscular dystrophy (DMD)?
X-linked recessive- absence of dystrophin protein Boys exhibit trait around age 3- rapidly worsens usually 2-6 yrs
72
Presentation of DMD?
Slower to develop motor milestones- walking/climbing stairs, walking on toes Proximal muscles affected before distal; lower before upper May have mild cognitive development, global developmental delay
73
PE findings seen in DMD?
Calf hypertrophy and proximal leg/pelvic weakness Waddling gait and inability to rise from ground easily Gower’s sign
74
Dx of DMD?
Muscle biopsy Myopathic EMG Serum CK levels Genetic testing available for prenatal dx
75
Tx of DMD?
sxs: - Glucocorticoids - new tx: gene therapy, creatine, aminoglycosides close pulmonary & cardiac FU due to risk of cardiopulmonary failure in 2nd & 3rd decade of life
76
Px of DMD?
Usually Wheel chair bound by 12 yo Loss of UE mvmt by 16 Death due to PNA or CHF (in early 20s) Average lifespan ~25
77
What is neurofibromatosis type 1?
Multisystem disorder Genetic disorder in which nerve tissue grows tumors that cause nerve/brain damage
78
s/s of neurofibromatosis type 1?
criteria: need 2 or more - cognitive or psychomotor sxs MC - learning disabilities (40%) - cafe au lait spots >6 - 2 or more neurofibromas - freckling or the axilla or inguinal regions - optic alimoa - 2 or more lisch nodes - distinctive bony lesions - 1st deg relative w/ NF type 1
79
What are neurofibromas?
soft or firm pedunculated tumors that develop along a nerve
80
Tx of NF type 1?
Surgical excision of tumors Genetic counseling & screening (risk to sibs is 50%)
81
Complications of NF type 1?
seizures, deafness, short stature, early puberty & HTN (<25%) optic glioma ~15%
82
What is NF type 2?
Dominantly inherited neoplasia syndrome- rare autosommal dominant disease
83
Presentation of NF type 2?
Manifests as bilateral vestibular schwannomas (VIII nerve tumors) vestibular schwannomas virtually never occur in NF type 1 other tumors of brain/spinal cord: meningiomas, other CN schwannomas, ependymomas
84
Will see cafe au lait sports in NF type 2?
NO