Peds neuro Flashcards
1
Q
What is static
A
Sx seen in the first few months, does not change over time
congenital abnormality or brain injury ex: cerebral palsy
2
Q
What is progressive
A
degenerative disease or neoplasm
3
Q
What is intermittent
A
epileptic or migraine syndromes
4
Q
What is saltatory
A
bursts of Sx followed by partial recovery
5
Q
On neuro PE note
A
- hair, skin, teeth, nails (brain and skin have same embryonic origin)
- head circumference
- fontanelles (some bulging with crying or vomiting is normal. but stay bulged with high ICP or infection)
- eyes, ears
- hands, feet: single simian crease in down’s syndrome, polydactyly abnormal
- midline defect (hair tuft, lipoma, dimpling): can indicate spina bifida
6
Q
What is the cut off point for micro and macrocephaly
A
2-3 SD above or below normal
7
Q
What patterns can mean different things in head circumference
A
accelerated growth: hydrocephalus
decelerated growth: degenerative neurologic disorder
abnormal shape: craniosynostosis (premature closure)
8
Q
How do you test the cranial nerves
A
I: smell II: pupil light reflex, visual acuity III, IV, VI: EOM V: suck, swallow, light touch VII: observe face at rest, crying, blinking VIII: hearing IX, X: gag reflex, sucking, salivation XI: posture, spontaneous movement XII: tongue movement
9
Q
When do fontanelles close
A
Posterior: 2 months
Anterolateral: 3 months
Posterior lateral: 1 year
Anterior: 2 years
10
Q
What are the primitive reflexes
A
Moro Grasp Rooting Foot placing Tonic neck (fencing ninja) -Most disappear by 4-6 months old Asymmetry= focal brain or PNS lesions
11
Q
Where do upper motor neurons originate
A
in motor region of cerebral cortex or brainstem, and they terminate at brainstem and spinal cord
12
Q
Upper motor neurons cause
A
spastic paralysis increased tone increased DTR (babinski) minimal muscle atrophy/strength loss No fasciculations sensory disturbance
13
Q
Lower motor neurons originate
A
in brainstem and spinal cord and terminate at skeletal muscle fibers
14
Q
Lower motor neurons cause
A
flaccid paralysis decreased tone absent DTR profound muscle atrophy fasciculations present sensory disturbances
15
Q
How do you assess strength in infants vs Toddlers
A
infant: symmetry of movement when held supine
toddler: reach high, run, walk, hop, climb
16
Q
How do UMN and LMN lesions present with strength
A
UMN: stiffness
LMN: weakness
17
Q
How do you assess tone
A
infant: passive (resistance to stretch) and active (posture adopted when put in a specific position) movement
18
Q
How do UMN and LMN lesions present with tone
A
UMN: (slide 12)
LMN: decreased passive tone
19
Q
How does gait evolve
A
it narrows until age 6 then normalizes
20
Q
How can you check cerebellar function
A
finger to nose
heel to shin
heel toe walking
exchange objects
21
Q
What is normal babinski in kids
A
neonate: variable
older kids: toes down is normal after 18 months
22
Q
How do you check sensory function
A
infant (harder): light touch vs pinprick. simulate by withdrawing limb
Older: proprioception, vibration, graphesthesia, stereognosis, 2 point discrimination
23
Q
Red flags for kid headache are
A
pt < 5 y/o
new explosive onset and worsening HS in prev. healthy kid
worst HA of life
unexplained fever
nighttime or warly morning awakening
HA w/ vomiting or worse w/ straining
postural HA (worse w/ lying or standing)
neurocutaneous stigma (cafe au lait spots, hypopigmented macules, etc)
24
Q
Explain acute vs acute recurrent HA
A
acute: single episode, no Hx. MC 2/2 febrile illness
AR: pattern of episodes separated by pain free intervals. typical migraine and tension HA pattern
25
What is the most concerning peds HA pattern
chronic progressive- may be increased ICP
| DDx think pseudotumor, brain tumor, hydrocephalus, chronic meningitis, abscess. etc. (BAD shit)
26
What is a chronic non-progressive HA pattern
>4 months or >15x month
27
What are worsening HA signs and symptoms
most severe on awakening, wake in middle of night
Severely exacerbated by coughing or bending
acute onset
present daily w/ progression
associated vomiting
focal neuro Sx
aggravated by valsalva
28
What imaging should you get for a headache and when
MRI! but you have to sedate peds..
| Get if abnormal neuro exam, or concern for space occupying lesion
29
Typical peds migraine Sx are
frontal, bitemporal, or unilateral throbbing for 2-72 hours (usually unilateral after puberty)
Sx relieved by sleep
+/- aura 15-30 min prior
Sensory and motor Sx rare
30
Some migraine triggers include
```
diet
menses
stress
increased exercise, sleep, routines
-Eliminate triggers by keeping HA diary!
```
31
Acute migraine Tx is
NSAIDs, APAP, triptans (>12 y/o), antiemetics (compazine-benadryl-toradol)
32
migraine prophylaxis is
<6: cyproheptadine
>6: propranolol, amitryptaline, topiramate
non-pharm: B12 25-400mcg
33
prognosis of migraines
good- can improve with time
34
What is pseudotumor cerebri (idiopathic, intracranial HTN)
d/o w/ Sx of high ICP (HA, papilledema, vision loss)
Elevated ICP with normal CSF and no other cause of IC HTN on neuroimaging
MC in females of childbearing age, but can happen <11 y/o
Obesity is a RF
Tetracycline, steroids, and retinol also linked
35
What are Sx of pseudotumor
```
HA
transient visual obscurations
IC noises )pulsatile)
Photopsia
back pain
retrobulbar pain
diplopia
sustained visual loss
```
36
In order to diagnose pseudotumor, the following must be present
```
Papilledema OR CN6 palsy
Normal neuro exam other than papilledema and CN abn
Normal neuroimaging
Normal CSF composition
Elevated LP opening pressure
```
37
What MUST you do before an LP
imaging! increased ICP may cause cerebral herniation when LP is preformed if it is obstructed by a mass
38
Major morbidity with pseudotumor is
loss of vision!
| but persistent HA are a major source of disability
39
How do you treat pseudotumor
Sometimes LP resolves Sx
weight loss if obese
Acetazolamide, Topiramate
Surgery (optic nerve sheath fenestration, optho eval, CSF shunt when all else fails)
40
What is cerebral palsy
heterogenous group of conditions involving permanent, nonprogressive central motor dysfunction
Muscle tone, posture, and movement ffected
2/2 abnormalities in developing fetal or infantile brain, brain injury or malformation before birth or during/after delivery
(affects person's ability to move and maintain posture)
41
Prenatal causes of cerebeal palsy are
```
prematurity
IUGR
IU infection
anteartum hemorrhage
severe placental pathology
multiple pregnancy
hypoxic brain injury
stroke
cerebral dysgenesis
```
42
Postnatal causes of cerebral palsy are
```
stroke
kernicterus (high bilirubin)
trauma
near drowning
toxins
hypoxic brain injury
```
43
MC developmental delays are
not sitting by 8 months
not walking by 18 months
early asymmetry of hand function (hand preference) before 1 y/o
44
4 major classifications of cerebral palsy are
Spastic (MC): UMN syndrome
Dyskinetic (decreased spontaneous movement) / Athetoid (slow, smooth writhing of distal muscles)
Ataxic (rare): wide gait, slow jerking movements, affects fine coordination, language and motor skills delayed, ataxia improves with time
Atonic: severe hypotonia, cerebral dysgenesis, microcephaly, intellectual disability
45
Treatment goals of cerebral palsy are
```
improve social and emotional development
communication
education
nutrition
mobility
maximal independence in ADL
```
46
What is spina bifida
neural tube disorder where caudal tube closes defectively in early gestation (by week 4)
Can be just a defect of L5-S1 vertebral arch, or more serious like exposure of meninges and spinal cord
47
What are the subtypes of spina bifida
- meningomyelocele: meninges and spinal cord are exposed, total paralysis, loss of bowel/bladder control, chiari II malformation
- meningocele: spinal canal and meninges exposed but underlying spinal cord intact
- occulta: skin intact w/ underlying bone and spinal canal defects. +/- sinus tract, dimple, or tuft of hair, +/- neuro deficits
48
How can you prevent spina bifida
Folic Acid supplementation in pre-pregnancy and during pregnancy
49
How can you diagnose spina bifida
routine screen AFP at 16-18 weeks in mom, and US at 12-14 and 18-20 wks
50
How do you manage spina bifida
surgical closure
VP shunt
adjust support (self cath, dealing w/ cognitive disabilities, atc.)
51
What are chiari malformations
heterogenous group of disorders w/ anatomic anomalies of cerebellum, brainstem, and craniocervical junction w/ DOWNward displacement of cerebellum into spinal canal
52
What are the types of chiari malformations
1: down displacement of medulla
2: hydrocephalus, kink in medulla, myelomeningocele
3: herniation of cerebellum below foramen magnum
4: not compatable w/ life- cerebellar hypoplasia
53
How do chiari malformations create hydrocephalus
the malformation obstructs the outflow of CSF
54
What is hydrocephalus
increased volume of CSF w/ progressive ventricular dilation
| congenital or acquired (tumor, meningitis, infx, hemorrhage, cyst, TBI, idiopathic)
55
Difference between communicating and non-communicating hydrocephalus
C: CSF circulates through ventricular system and into subarachnoid space w/o obstruction
NC: obstruction blocks flow of CSF w/in the ventricular system or from ventricular system into subarachnoid space
56
What Sx indicate obstruction of CSF (increased ICP)
HA, vomiting, AMS, visual changes, Ocular nerve palsy (CN6)*, FND
infant: vomiting, lethargy, irritable, bulging fontanelle, poor feeding, macrocephaly, excessive head growth (more non-specific)
57
How do you treat hydrocephalus
Temp: loop diuretic, acetazolamide
Long: remove obstructive lesion, VP shunt*
58
What are spinal muscular atrophy disorders
Degeneration of anterior horn cells in the spinal cord and motor nuclei in the lower brainstem= progressive muscle weakness and atrophy
Autosomal recessive dz 2/2 mutation in SMN gene on chromosome 5q13
59
What are the subtypes of SMA
0: prenatal onset, hypotonia and areflexia at birth
1 (Hoffman disease): worst, mild weak at birth, respiratory failure by 1 y/o
2: weakness and decreased DTR by 2 y/o
3: weakness in adolescence
4
60
Always suspect SMA in infants
with unexplained weakness or hypotonia
progressive proximal weakness
decreased spontaneous movement
floppiness (loss of head and leg control)
decreased facial expression and drooling
-usually by 6 mo - 6 y/o
61
SMA kids have normal
mental status
social and language skills
sensation
62
How do you diagnose SMA
EMG, muscle biopsy, DNA testing
| homozygous deletion of exon 7 of SMN1 confirms Dx
63
How do you treat SMA
No Tx
| Sx therapy improves flexibility, prevents infection, maintains social language and intellectual stimulation
64
What is Guillain Barre syndrome
acute immune mediated polyneuropathy; Acute paralyzing illness provoked by preceding infection
MC: Campylobacter jejuni
85% of kids have great recovery
65
Sx of Guillain barre are
*Ascending weakness (symmetric)- feet dorsiflexed
Sensory Sx
loss of DTR
ANS dysfunction
Problems with respiration, talking, swallowing, B/B fxn
Kids refuse to walk and w/ leg pain
66
How do you diagnose Guillain Barre
LP: elevated protein W/O high WBC
| EMG
67
Worrisome outcomes of Guillain barre are
death from ANS dysfunction, respiratory failure, and PNA/PE/CV collapse
Long term paresthesias, fatigue, and limb weakness
68
How do you treat Guillain Barre
IVIG (combat invading organisms)
| Plasmapheresis: decrease severity and duration, remove Ab from circulation
69
What is DMD
X linked recessive d/o with absence of dystrophin protein
Sx by age 3 and rapidly worsen
slower to develop motor milestones (walking, climbing stairs)
70
How does DMD present
```
Proximal muscles affected first** LE before UE**
Calf hypertrophy
pelvic weakness
waddling gait
can't stand from ground easily
**Gower's sign
```
71
Early DMD manifestations (2-6 y/o) are
```
Clumsy
walking on toes
waddling gait w/ excessive lumbar lordosis
Gower's sign
difficult to climb stairs
```
72
How do you diagnose DMD
**Muscle biopsy: shows degeneration and regeneration, fiber size varies, inflammation, connective tissue proliferation,
**Myopathic EMG
Serum CK levels (high before Sx set in, peak at 2 y/o)
can do genetic testing
73
How do you treat DMD
Glucocorticoids prolong independent ambulation by 2.5 years if started between 4-8 y/o
Gene therapy, creatine, aminoglycosides
Mainly supportive care
Close cardiac and pulm follow up 2/2 risk of failure in 20-30's
74
What is prognosis of DMD
Wheel chair by 12 y/o
Loss of UE movement by 16
Death from PNA or CHF in 20's
Lifespan: 25 years
75
What is neurofibromatosis
genetic d/o where nerve tissue grows tumors that cause nerve/brain __?
mutation in NF1 gene on chromosome 17q11.2
76
To diagnose neurofibromatosis you need 2+ of the following
- *Cognitive or psychomotor probles
- learning disabilities
- cafe au lait spots by 1 y/o
- >6 cafe au lait spots (5mm diameter prepubertal, 15mm in pubertal)
- 2+ neurofibromas
- Freckling of axillary or inguinal regions
- Optic glioma
- 2+ lisch nodules*
- Distinct bony lesions
- 1st degree relative with NF type 1
77
Physical findings in neurofibromatosis are
care au lait spots
Neurofibromas in late teens (along a nerve)
Greater incidence of CNS tumors (astrocytoma, meningioma, Schwannoma)
78
How can cognitive impairment present in neurofibromatosis
benign learning disability - mental retardation Seizures, macrocephaly, neuropathy
79
How do you treat neurofibromatosis
Surgical excision of tumors
| genetic counseling and screening (esp in siblings)
80
Complications of neurofibromatosis are
```
seizure
deafness
short stature
early puberty
HTN
optic glioma
```
81
What is NF type 2
dominantly inherited neoplasia syndrome (AD)
| can appear in kids as loss of hearing
82
How are T1 and T2 NF different
T1: has cafe au lait spots, no vestibular schwannomas
T2: Has bilateral vestibular schwannomas, no cafe au lait spots
83
What other brain and spinal cord tumors are common
meningiomas
CN schwannomas
Ependymomas
