Neuro Flashcards
Which neuro abnormalities are seen in pts w/ static sxs?
congenital abnormalities or brain injury (think cerebral palsy)
Which neuro abnormalities are seen in pts w/ progressive sxs?
degenerative disease or neoplasn
Which neuro abnormalities are seen in pts with intermittent sxs?
epileptic or migraine syndromes
Which neuro abnormalities are seen in pts with saltatory sxs?
bursts of sxs followed by partial recovery
-vascular, demyelinating d/o
What PE findings may indicate spine bifida?
Midline defects:
tufts of hair, lipomas, dimpling
Accelerating pattern of head circumference may indicate? decelerating pattern?
hydrocephalus
degenerative neurologic disorder
How can you assess CN II?
pupillary light reflex, visual acuity
How can you assess CN III, IV, VI?
following objects, fixating, oculocephalic reflex, EOMs
How can you assess CN V?
sucking/swallowing, light touch
How can you assess CN VII?
observe fast at rest, crying/blinking
How can you assess CN VIII?
hearing
How can you assess CN IX, X?
gag reflex, sucking, salivation
How can you assess CN XI?
posture, spontaneous movement
How can you asses CN XII?
tongue movement
What are some primitive reflexes? When do most of them disappear?
Moro, grasp, rooting, foot placing, tonic neck
4-6 months
asymmetry indicates focal brain or PNS lesions
s/s seen w/ upper motor neurons
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
s/s seen w/ lower motor neurons?
Flaccid paralysis
Decreased tone
Absent DTRs
Profound muscle Atrophy
Fasciculations present
May have sensory
disturbances
WEAKNESS, DECREASED PASSIVE TONE
What are some red flags for children w/ headaches?
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
What is the most concerning headache pattern in children?
chronic progressive
>4 months or >15x/month
usually increased ICP
possible psych factors
When should you order imaging in child presenting w/ HA?
Abnormal neurologic exam
Concern for space occupying lesion
Typical pediatric sxs for migraines?
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
Tx for migraines?
eliminate triggers: HA diary
acute: NSAIDs, APAP, triptans, antiemetics
Prophylaxis:
<6: Cyproheptadine
>6: Propranolol, Amitriptyline, Topiramate
Non pharm: B12
What is Pseudotumor Cerebri - Idiopathic intracranial hypertension (IIH)
elevated ICP w/ norm cerebrospinal fluid composition, and no other cause of intracranial hypertension
s/s: HA, papilledema, vision loss, intracranial noises, photopsia
Epidemiology of pseudotumor cerebri?
MC in females of childbearing age; does occur in peds- usually adolescents (11 years +)
MC risk factor for pseudotumor cerebri?
obesity
assoc. meds: tetracycline, steroids, retinol
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
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
What is the main complication of pseudotumor cerebri?
vision loss
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
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)
Prenatal causes of cerebral palsy?
Prematurity
IU growth restriction
IU infection
Antepartum hemorrhage
Severe placental pathology
Multiple pregnancy
Hypoxic brain injury
Stroke
Cerebral dysgenesis
Postnatal causes of cerebral palsy?
Stroke Kernicterus Trauma Near-drowning Toxins Hypoxic brain injury
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
What are the 4 major classification of CP?
spastic (MC)
athetoid/dyskinetic
Ataxic (extremely rare)
Atonic
Describe spastic CP
features of an upper motor neuron syndrome
Describe athetoid/dyskinetic CP
Athetoid: slow, smooth, writhing movements that involve distal muscles
Dyskinetic: decreased spontaneous movement, hypotonia, and suppressed primitive reflexes
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
Describe Atonic CP
Severe hypotonia,
never stand or walk.
may have cerebral
Dysgenesis,
microcephaly,
profound intellectual disability
CP tx goals?
Social and emotional development
Communication
Education
Nutrition
Mobility
Maximal independence in ADLs
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
What are the subtypes of spina bifida?
Meningomyelocele-most serious!
Meningocele
Spina bifida occulta
Describe a meningomyelocele
Meninges and spinal cord exposed
total paralysis, loss of B/B control
accompanying Chiari II malformation*
Describe a meningocele
Spinal canal and meninges are exposed, underlying spinal cord is intact
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
Prevention of spina bifida?
FOLIC ACID supplementation- pre-preg & during preg
Dx of spina bifida
Routine screening w/ alpha-fetoprotein (AFP) level (16-18 w) in maternal serum & US (12-14 w & 18-20 w)
management of spina bifida?
Surgical closure
VP shunt
Adjunct supportive therapies etc.
Cognitive disabilities, self-catheterization, etc.
px varies
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
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
Chiari malformations cause…
malformation obstructs the outflow of cerebral spinal fluid (CSF) through the posterior fossa –>
HYDROCEPHALUS
Almost all pts w/ a myelomeningocele have…
chiari II malformation
and most have assoc. hydrocephalus
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
What is communicating hydrocephalus?
CSF circulates through the ventricular system & into the subarachnoid space w/o obstruction
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
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.
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.
Tx for hydrocephalus?
Temporary, symptomatic relief: loop diuretic, acetazolamide
Surg intervention: remove obstructive lesion, VP shunt
Inheritance pattern for the common forms of SMA?
autosomal recessive
caused by biallelic deletion or mutations in the SMN1 gene on chromosome 5q13
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
When do most cases of SMA present?
in infancy
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
When should SMA be suspected?
for any infant with unexplained weakness or hypotonia
Presents with progressive proximal weakness , decreased spontaneous mvmt, “floppiness”
Dx of SMA?
EMG, muscle Bx, DNA testing
homozygous deletion of exon 7 of SMN1 (confirms diagnosis)
Tx of SMA?
No tx
Sx therapy to improve flexibility, prevent infections, maintain social, language and intellectual stimulation
What is Guillain barre syndrome (acute idiopathic polyneuritis)
Acute immune-mediated polyneuropathy
Heterogenous syndrome, variant forms
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
Etiology of GBS?
Causes: Post-infectious
- Resp or GI infection
- Campylobacter jejuni MC
Dx of GBS?
LP- Elevated CSF protein without increased WBC
Electromyography (EMG)
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
Tx of GBS?
IVIG: combat invading organisms- lessen attack on NS
Plasmapheresis:
decrease severity and duration, removes Ab from circulation
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
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
PE findings seen in DMD?
Calf hypertrophy and proximal leg/pelvic weakness
Waddling gait and inability to rise from ground easily
Gower’s sign
Dx of DMD?
Muscle biopsy
Myopathic EMG
Serum CK levels
Genetic testing available for prenatal dx
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
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
What is neurofibromatosis type 1?
Multisystem disorder
Genetic disorder in which nerve tissue grows tumors that cause nerve/brain damage
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
What are neurofibromas?
soft or firm pedunculated tumors that develop along a nerve
Tx of NF type 1?
Surgical excision of tumors
Genetic counseling & screening (risk to sibs is 50%)
Complications of NF type 1?
seizures, deafness, short stature, early puberty & HTN (<25%)
optic glioma ~15%
What is NF type 2?
Dominantly inherited neoplasia syndrome- rare autosommal dominant disease
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
Will see cafe au lait sports in NF type 2?
NO
