Neuro Flashcards
1
Q
what characterizes macrocephaly?
A
> 95th percentile or > 2 percentile lines above height/weight
2
Q
what characterizes microcephaly?
A
< 5th percentile or <2 percentile lines below height/weight
3
Q
what physical exam characteristics should one pay close attention to when looking to see if there may be a neuro defect?
A
- hair, skin teeth, nails
- fontanelles
- ears, eyes
- hands, feet
- midline defects
4
Q
when do the anterior, sphenoidal, and posterior fontanelles close?
A
posterior = 2-3 months sphenoidal = 6 months anterior = 1-3 yrs
5
Q
how to evaluate CN I?
A
smell, can assess based on age
6
Q
how to evaluate CN II?
A
pupillary light reflex, visual acuity
7
Q
how to evaluate CN III, IV, VI?
A
following objects, fixating, oculocephalic reflex, EOMs
8
Q
how to evaluate CN V?
A
sucking/swallowing, light touch
9
Q
how to evaluate CN VII?
A
observe face at rest, crying/blinking
10
Q
how to evaluate CN VIII?
A
hearing
11
Q
how to evaluate CN IX, X?
A
gag reflex, sucking, salivation
12
Q
how to evaluate CN XI?
A
posture, spontaneous movement
13
Q
how to evaluate CN XII?
A
tongue movement
14
Q
signs of upper motor neuron lesion
A
- spastic paralysis
- increased tone
- increased DTRs/+babinski in older children
- minimal muscle atrophy/strength loss
- fasciculations absent
- may have sensory disturbances
15
Q
signs of lower motor neuron lesions
A
- flaccid paralysis
- decreased tone
- absent DTRs
- profound muscle atrophy
- fasciculations present
- may have sensory disturbances
16
Q
reflex - moro
description, age, origin in CNS
A
- sudden head extension, leads to extension then flexion of limbs
- age 0-6 months
- brainstem
17
Q
reflex - grasp
description, age, origin in CNS
A
- finger in palm, flexion of fingers, and elbow and shoulder
- 0-6 months
- brainstem
18
Q
reflex - rooting
description, age, origin in CNS
A
- tactile stimulus near mouth, mouth pursuing stimulus
- age 0-6 months
- brainstem
19
Q
reflex - trunk incurvation
description, age, origin in CNS
A
- stroke skin near vertebrae, curvature of spine opposite
- age 0-6 months
- spinal cord
20
Q
reflex - placing
description, age, origin in CNS
A
- places foot on exam surface when dorsum of foot in contact w/ edge
- age 0-6 months
- cerebral cortex
21
Q
reflex - crossed extension
description, age, origin in CNS
A
- hold one leg in extension and stimulate dorsum of foot, flex/ext/add/toe fanning opposite leg
- age 0-6 months
- spinal cord
22
Q
reflex - tonic neck
description, age, origin in CNS
A
- supine, turn head results in ipsilateral extension of arm and leg
- age 0-6 months
- brainstem
23
Q
reflex - parachute
description, age, origin in CNS
A
- infant sitting, tilt results in extension of ipsilateral arm
- 6months - life
- brainstem
24
Q
reflex - landau
description, age, origin in CNS
A
- infant held about waist and suspended, extension of neck produces extension of limbs
- 6 months - 2 years
- brainstem
25
in a neuro eval, what should be observed in strength of infants/toddlers?
- infants = symmetry of movements when held supine
| - toddlers = reach high, run, walk, hop, climb stairs
26
in a neuro eval, what should be observed in tone of infants? (both passive and active)
- passive = some resistance to stretch normal
| - active = posture adopted when placed in particular position
27
in a neuro eval, what should be observed in gait of infants/toddlers?
- infants = crawling
- toddlers = normally wide-based gait and unsteady, gradually closes until age 6
- ALSO, cerebelar function = finger to nose, RAM, heel to shin, heel to toe walking
28
in a neuro eval, what should be tested for sensory?
- dull vs. sharp
- temperature
- proprioception/vibration
- graphesthesia
- stereognosis
29
in a neuro eval, how should one test mental status?
- developmental milestones
| - age, location, gender, colors, numbers, shapes, hobbies, social interaction
30
define: static (in evolution of symptoms)
seen in first few months and do not change over time (ie: congenital abnormalities or brain injury)
31
define: progressive (in evolution of symptoms)
degenerative disease or neoplasm
32
define: intermittent and brief (in evolution of symptoms)
epileptic or migraine syndromes
33
define: saltatory (in evolution of symptoms)
bursts of symptoms followed by partial recovery (ie: demyelinating and vascular disease)
34
common workup for neurologic impairment in peds? (9 tests)
1) serum enzymes (CK)
2) molecular genetic markers - DNA markers
3) nerve conduction velocities
4) electromyography (EMG)
5) muscle imaging (w/ MRI?)
6) muscle biopsy
7) nerve biopsy
8) EKG channelopathies
9) ECHO - muscular dystrophy
35
```
trisomy 13
(type of disorder & characteristics)
```
- recognized chromosomal disorder
| - cleft lip, midline defect
36
```
trisomy 18
(type of disorder & characteristics)
```
- recognized chromosomal disorder
| - weak cry, early death
37
5P
| type of disorder & characteristics
- recognized chromosomal disorder
| - cri-du-chat syndrome
38
3 genetic diseases associated w/ microcephaly
- microcephaly vera (autosomal rec.)
- microcephaly w/ lissencephaly
- a genesis of corpus collosum
39
infections (congenital) TORCH associated w/ microcephaly
- rubella
- CMV
- toxoplasmosis (usu from the cat)
- syphilis
40
```
angelman syndrome
(type of disorder & characteristics)
```
- MR, ataxia, seizures
| - microcephaly w/ syndrome
41
prader-willi
| type of disorder & characteristics
- hypotonia, cryptorchidism, obesity
| - microcephaly w/ syndrome
42
smith-lemli opitz
| type of disorder & characteristics
- cyptorch, hypospadias, seizures
| - microcephaly w/ syndrome
43
cornelia de lange
| type of disorder & characteristics
- anteverted nostrils, carp mouth, micromelia, synophrys
| - microcephaly w/ syndorme
44
rubinstein-taybi
| type of disorder & characteristics
- broad thumbs and toes, narrow nose, maxillary hypoplasia
| - microcephaly w/ syndrome
45
hallermann-streiff
| type of disorder & characteristics
- micropthalmia, small nose
| - microcephaly w/ syndrome
46
4 toxic microcephalic conditions?
- fetal alcohol syndrome
- PKU
- hypoxic- ischemia
- intrauterine or neonatal injury
47
define: ataxia
| movement disorder
- inability to make voluntary smooth, accurate, and coordinated movements
- can look inebriated
- cerebellum
48
define: bradykinesia
| movement disorder
extreme slowness and stiffness of voluntary movement
49
define: choreo
| movement disorder
- syndrome of continuous involuntary random movements that usu occur at rest and may appear to be fidgety, dancing, or writhing
50
define: dystonia
| movement disorder
- syndrome of abnormal muscle contractions that lead to twisting, jerking, spasms, or stiffening at rest or during attempts at movement
51
define: myoclonus
| movement disorder
condition of very rapid and brief shock-like jerks
52
define: spasticity
| movement disorder
- increase in muscle stiffness that worsens with rapid movement and may be associated w/ increased reflexes and weakness
53
define: tics
| movement disorder
repetitive, stereotyped, and sometimes complex involuntary movements or sounds that may appear similar to purposeful actions
54
define: tremors
| movement disorder
rhythmic involuntary back and forth shaking at rest or with movement
55
what are common causes of headache?
- URI
- fever
- sinusitis
- migraine
- tension
- cluster
56
what are life-threatening causes of headache?
- meningitis, encephalitis, cerebral abscess
- subarachnoid hemorrhage
- trauma
- hydrocephalus, VP shunt malformation
57
what are "other" causes of headache?
- toxin/substance abuse
- postictal phase (after seizure)
- HTN
- psychogenic
- sleep deprivation
58
migraine
| info & presentation
- most common cause of recurrent HA in kids
- common sx = visual aura (classic type) vs no aura (common type)
- throbbing, nausea/vomiting, family history, precipitating factors
- pain bilateral in kids, unilateral in teens
59
migraine
| treatment
- ELIMINATE TRIGGERS
- prophylaxis if >2/wk or >4/month & triggers have been eliminated
- symptomatic management - alkaloids, nasal spray, pill at onset
60
when should one WORRY about headaches?
- most severe on awakening, awaken in middle of night
- severely exacerbated by coughing or bending
- acute onset without prev history
- present daily w/ worsening
- accompanied w/ vomiting
- focal neuro signs
- fever, ill appearing
61
when should one IMAGE w/ a headache?
- abnormal neuro exam
- concern of space occupying lesion
- if your gut tells you to image!
62
define: convulsion
violent involuntary contraction
63
define: tonic (convulsion)
sustained contraction
64
define: clonic (convulsion)
contraction alternating w/ relaxation
65
define: myoclonic (convulsion)
spasm of a muscle or group of muscles
66
define: atonic (convulsion)
sustained relaxation of muscles
67
define: seizure
sudden onset of certain symptoms
68
define: epilepsy
chronic nervous system disorder characterized by repeated convulsions of any type (on a reg basis that person is treated for)
69
define: aura
stereotyped forewarning of an attack, may be sensory/motor/behavioral
70
define: ictus
seizure phase itself
- motor activity involuntary
- if both sides involved, unconscious
- DTRs suppressed
- stereotyped motor activity
- upward deviation of eyes (often)
- amnesia
- bladder/bowel incontinence may be present
71
define: post ictal phase
alteration of consciousness following most seizures (usu stunned state)
- headache, irritable
- sensory and motor impairment temporary
72
name 2 types of partial seizures
- simple partial = conscious
| - complex partial = impaired consciousness
73
name types of generalized seizures (6)
- absence
- tonic-clonic
- atonic
- myoclonic
- tonic
- clonic
74
what perinatal conditions can cause seizures?
- cerebral malformations
- intrauterine infection
- hypoxic-ischemic
- trauma
- hemorrhage
75
what infections can cause seizures?
- encephalitis
- meningitis
- brain abscess
76
what metabolic conditions can cause seizures?
- hypoglycemia
- hypocalcemia
- hypomagnesemia
- hyponatremia
- hypernatremia
- storage diseases
- porphyria
- pyridoxine deficiency
77
what poisonings can cause seizures?
- lead
- cocaine
- drugs
78
what neurocutaneous syndromes can cause seizures?
- tuberous sclerosis
- neurofibromatosis
- sturge-weber syndrome
- klippel-trenaunay-weber syndrome
79
what systemic disorders can cause seizures?
- vasculitis
- SLE
- hypertensive encephalopathy
- renal failure
- hepatic encephalopathy
80
what "other" things can cause seizures?
- trauma
- tumor
- fevers (febrile seizures)
81
febrile seizures
| info & presentation
- 85% simple seizures
- very common
- peak ages 3 months - 5 years old
- usu w/ sudden spike in temp
- usu lasts abt 15 min
- usu w/ family history
- < 5% later acquire epilepsy
82
febrile seizure
| management
- airway
- benzodiazepines if durations > 10-15 min
- figure out cause of fever
- exclude meningitis w/ septic work up
- most children < 12 months need LP (12-18 months grey zone)
83
absence seizures
| info & presentation
- females > males
- onset 4-6 yrs old
- sx = brief loss of environmental awareness w/ simple automatisms (looks like "staring into space"
- can be provoked by flashing lights/hyperventilation
- usu short in duration
- can have dozens/day
- not responsive during episode
- no postictal confusion
- no memory of event
84
status epilepticus
| info & presentation
- seizure lasting > 20-30 min
- concern for irreversible brain injury
- 50% no definitive etiology (25% w/ acute brain injury, 20% w/ hx congenital malformation/brain injury)
85
status epilepticus
| treatment
- ABCs, suction, O2, intubation
- benzodiapepine (valium, ativan)
- if persists, consider phenytoin/fosphenytoin, then phenobarbital, then valproate
- when stable, EEG, LP, imaging
86
infantile spasms
| info & presentation
- typical onset 3-7 months
- brief, symmetric contractions of extremities/trunk/neck muscles (looks like "twitching")
- happens in clusters
- occur before sleep or upon awakening
87
infantile spasms
| diagnostic
- EEG - hypsarrthymia pattern
| - MRI to determine etiology
88
infantile spasms
| treatment
- ACTH (cortisol stimulator)
- vigabatrin
- ketogenic diet
- very poor prognosis (can signal neuro deterioration)
89
what are the developmental milestone categories?
gross motor, fine motor, language, cognitive, social
90
what is considered a developmental delay?
not reaching a milestone 2 standard deviations below the norm
- also, delays in one area may be associated w/ delays in other areas
91
when is a developmental delay considered a disability?
when the delay is permanent
91
what is the Denver II Development Screening Test? (Describe)
- test that provides a broad variety of standardized items to give a quick overview of the child's development
- enables tester to compare a child's development w/ over 2,000 children who were in standardized pop, like a growth curve
- contains behavior rating scale
* ** key = early detection & referral
91
cerebral palsy
| etiology
- motor impairment from brain damage
- 70% caused during prenatal period (majority time cause unknown)
- abt 4-9% directly related to asphyxia at delivery
- incidence rate 2/1000 live births (not rare at all)
91
4 classifications of cerebral palsy
divided based on movement impairments:
- spastic (70-80% cases)
- athetoid/dyskinetic
- ataxic
- atonic
91
describe Arnold-Chiari malformations (spina bifida associated w/ this)
- cerebellum herniation thru foramen magnum
- sx - occipital HA that radiates upward, worsens w/ valsava
- visual/balance problems, CN compressions, muscle weakness may be present
- treatment = pain management & surgical decompression
92
cerebral palsy
| causes
- hypoxia
- birth trauma
- premature birth
- infections
- trauma
- toxins
- structural brain anomalies
* NOT HEREDITARY
92
spina bifida
| info & presentation
- 1/1000 births in US - most frequent/permanently disabling birth defect
- defective closure of neural tube at end of wk 4
- anomalies of L/S vertebrae or spinal cord
- huge range of severity
- common to see dimple or tuft of hair over sacral area of back
- prevented by folic acid administration
- associated w/ Arnold-Chiari malformations
- associated w/ hydrocephalus
92
describe spina bifida w/ MENINGOMYELOCELE
- most severe
- SC or nerve roots exposed on back in sac
- area covered w/ sores & CSF leaks may be present
- leg paralysis/incontinence
- chiari malformations
- needs immediate surgical closure
93
describe spina bifida w/ MENINGOCELE
- rarest form
- only meninges exposed in back in cyst like sac
- underlying SC anatomically and functionally intact
- no hydrocephalus or neuro deficits
- surgically repaired
94
dandy walker syndrome
- enlargement of 4th ventricle
- posterior fossa cyst
- hydrocephalus in 90%
- partial/complete absence of cerebral vermis
- slower motor development
- progressive enlargement of skill allows less HC symptoms early on
- 50% normal IQ
- problems w/ balance, fine motor, stiffness
95
hydrocephalus
| info & presentation
- slowly evolving accum of CSF over weeks to months
- etiology can be obstruction or overproduction of CSF
- sx related to ventricular distention and increased ICP
96
hydrocephalus
| treatment
- temporary = loop diuretic, acetazolamide
| - surgical intervention = remove obstructive lesion, shunt - VP most common (VP = ventriculoperitoneal)
97
cerebral palsy
| management
- OT/PT/ST
- anti-spasmodics (dantrolene, benzos, baclofen)
- botulinum toxin if spasms in one particular area
98
hydrocephalus - intraventricular foramina obstruction
- acute/slow onset
- any age
- unilateral
- etiology = congenital, mass
99
hydrocephalus - aqueduct of sylvius obstruction
- in utero or infancy
- slowly evolving
- etiology = NF, toxo, mass, congenital dysplasia, IVH
100
hydrocephalus - impaired flow form 4th ventrical obstruction
- enlarged occipital shelf
- slow evolving
- etiology = dandy-walker, atresia of foramina of lushka and magendie
101
hydrocephalus - congenital bone lesion obstruction
- slow evolving
- lower cranial nerve dysfunction
- etiology = achondroplasia, rickets, posterior fossa distortion
102
hydrocephalus - extraventricular obstruction (communicating) obstruction
- acute/slowly moving
- etiology = hypoplasia or damage of arachnoid villi
- s/p IVH with blood debris
103
complete spinal cord trauma
nerve damage obstructs every signal coming form brain to body parts below injury
104
incomplete spinal cord trauma
some residual motor and sensory function remains below level of SCI
105
list of neuromuscular diseases w/ proximal muscle weakness
- dystrophy
- duchenne
- limb-girdle
- dermatomyositis, polymyositis
- kugelberg-welander disease (late onset spinal muscular atrophy)
106
acute transverse myelitis
| diagnosis
- MRI (lighter areas show inflammation)
| - CSF analysis
107
list of neuromuscular diseases w/ ophthalmoplegia and limb weakness
- myasthenia gravis
- botulism
- myotonic dystrophy
- miller fisher variant of GB syndrome
108
list of neuromuscular diseases w/ facial and bulbar weakness
- myasthenia gravis
- botulism
- polio
- miller fisher variant of GB syndrome
- myotonic dystrophy
- facioscapulohumeral dystrophy
109
spinal muscular atrophy syndrome
- autosomal rec.
- gen. def. chromosome 5
- progressive weakness of LMN due to degeneration of anterior horn cells
- majority present in infancy
- similar to ALS in adults
- progressive flaccid weakness (symmetric), dec/abs DTR, muscle atrophy
- difficulty sitting, failure to stand
- decreased facial exp, drooling
- death from resp. dysfunction/infection
* *normal cognitive/language/social dev
110
acute transverse myelitis
| info & presentation
- acute inflammation of grey & white matter in one or more spinal cord segments (usu thoracic)
- onset hours to weeks of bilateral motor, sensory, sphincter deficits below level of lesion
- back pain, sudden paresthesias, weakness
111
acute transverse myelitis
| cause
unknown, but related to MS infections, autoimmune or post-infectious inflammation, vasculitis, and certain meds
112
guillain barre syndrome (acute idiopathic polyneuritis
| info & presentation
- inflammatory peripheral neuropathy
- rapid loss of motor strength (ascending)
- symmetric weakness
- may have sensory symptoms w/ few findings
- loss of DTRs early
- may have autonomic nerve dysfunction
113
guillain barre syndrome (acute idiopathic polyneuritis)
| cause
- post-infectious respiratory or GI
| - campylobacter jejuni (majority), cytomegalovirus, EBV, and mycoplasma pneumoniae injections, MCV4/infuenza vaccine
114
what is the tension (endrophonium) test?
1) administer short acting Cholinesterase inhibitor
| 2) ptosis and ophthalmoplegia improve w/in seconds
115
guillain barre syndrome (acute idiopathic polyneuritis)
| treatment
- IVIG
- plasmapheresis
- 1-2% mortality if treated properly, 20% end up on ventilator
116
myasthenia gravis
| info
- chronic disease, autoimmune where antibodies form against Ach nicotinic receptors at NM junctions
- 75% thymus involvement
- begins teen years
- rapid fatigue of striated muscle
- usu immune mediated NM blockade
- post- synaptic muscle membrane less responsive due to circulating receptor binding antibodies
117
myasthenia gravis
| hallmark symptoms
- ptosis, diplopia, ophthalmoplegia, weakness of face/extremities, dysphagia
- symptoms worsen throughout day or w/ exertion
- no primary complains of muscle pain, sleepiness
118
myasthenia gravis
| diagnosis
- CT scan for thymoma
- serology for autoantibodies
- NCS/EMG (repetitive)
- tensilon challenge test (endrophonium)
119
inheritance pattern for hereditary motor sensory MD (charcot-marie tooth)
AD-17p11.2
120
inheritance pattern for duchenne and becker MD?
XR - Xp21.2
121
inheritance pattern for emery-dreyfus MD?
XR - Xq28
122
inheritance pattern for limb girdle MD (two possibilities)
AD - 5q
or
AR - 15q
123
inheritance pattern for congenital MD
AR
124
inheritance pattern for hereditary motor sensory MD (charcot-marie tooth)
AD-17p11.2
125
duchenne + Becker MD
| info & presentation
- most common MD
- onset 3 yrs, fully expressed by 5-6 yrs, wheelchair bound by 10 yrs (Becker has later onset)
- cardiomyopathy in 50-80%
death by 18-20 yrs old (bc resp. problems)
progressive weakness
- shows Gower's sign
- waddling gait
- mild intellectual impairment
- hypertrophy of calves, pelvic weakness
- proliferation of connective tissue in muscle
126
duchenne + Becker MD
| diagnosis
- muscle biopsy - will show degeneration & regeneration w/ connective tissue
- absence of DYSTROPHIN (Ig protein assoc. w/ muscle fiber plasma membrane)
- EMG
- serum CPK levels will be elevated (CPK = creatine phosphokinase)
127
duchenne + Becker MD
| treatment
- symptomatic treatment only
- glucocorticoids
- new therapy = gene therapy, creatine, aminoglycosides
128
emery-dreifuss MD
- group of myopathies that affect muscles of hip/shoulder
- middle to late childhood and early adult presentation
- wheelchair by 30 yrs old
129
limb-girdle MD
- includes other mutations that cause myotubuar myopathy such as adrenoleukocystrophy & block-sulzberger (pigment changes)
- onset 5-15 yrs old
- CM severe (must make decision act heart transplant)
130
symptoms of pediatric brain tumors?
- HA
- nausea, vomiting
- loss of balance
- speech problems
- sleepiness
- personality changes
- seizures
- increased head circumference in infants
131
types of brain tumors?
- gliomas (astrocytoma, brain stem glioma, ependymomas)
| - meduloblastomas (15% brain tumors in kids)
132
two types of neurofibromatosis?
type 1 = more common, AD (chr. 17), peripheral type
type 2 = AD (chr. 22), incurable, multiple intracranial & spinal tumors, central type
133
neurofibromatosis
| presentation
- cafe au lait spots (numerous > .5cm present at birth)
- neurofibromas form late teens (soft or firm pedunculated tumors along a nerve)
- axillary/groin freckling
- greater incidence CNS tumors
- range cognitive impairment
134
neurofibromatosis
| treatment
- surgical excision of tumors
