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Clin Med Flashcards

1
Q

describe neurulation

A

thick ectoderm plate (neural plate) –> edges fold to form neural folds –> neural tube –> fuse in cranial and caudal regions, cranial neuropore closes day25 & caudal neuropore day27 b/c it makes open communication b/w neural tube lumen & amniotic cavity

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2
Q

what are NTDs? cause? risk factors?

A

failure of neural tube closing b/w wk3-4 in utero. multifactorial. fhx, maternal sz meds (valproate, folic acid antag: carbamazepine, phenobarbital/toin, trimetho/primidone, tramterene, sulfasalazine), maternal obesity/DM, hispanics > caucasians

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3
Q

how to prevent NTDs?

A

childbearing women take 0.4mg folic acid daily, high risk women take 4mg folic acid daily; supplement > food b/c unstable

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4
Q

how to screen for NTDs? how to interpret AFP lvls? when does it peak?

A

US = screening & diag –> 2nd trimester US/QUAD screen at 18-20wks, maternal serum AFP made by fetal yolk sac/GI tract/liver. if perform at 16-18wks –> >2.5 = abnl. 28-32wks

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5
Q

open spina bifida/spina/cystica. meningocele vs myelomeningocele

A

overlying skin not intact –> meninges and/or spinal herniate thru defect is post vert arches –> meningocele or myelomeningocele. meninges herniate thru defect –> fluid filled sac –> asx or neuro deficits vs meninges, spinal cord, CSF herniate thru defect –> organ dysfxn –> urin/fecal incont; neuro deficits depend on severity

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6
Q

myelomeningocele lvls sxs: high lumbar-thoracic vs low lumbar vs high sacral vs low sacral. any other sxs?

A

flaccid paralysis of LE –> absent DTRs, no touch/pain response, hydrocephalus, walk short distances w/ leg brace then wheelchair vs walk w/ short leg brace and forearm crutches vs urin/fec incont, perianal anesthesia, walk w/ gluteal lurch or foot & ankle brace vs no impaired motor fxn, can walk. other sxs: sacral hair patch, lipoma, hemangioma

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7
Q

myelomeningocele = assoc w/?

A

brainstem dysfxn d/t Arnold Chiari malformation –> dysphagia, vocal cord paresis causing stridor, apneic, strabismus & facial weakness, abnl ventilation response to respiratory gases, weak UE & poor tone (severe). hydrocephalus d/t Chiari II malformation

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8
Q

how to dx myelomeningocele? how to dx spina bifida?

A

PE w/ neuro exam, prenatal US, spine MRI = GOLD STANDARD, head CT to also chk hydrocephalus & chiari malformation, uro/GI eval. spine MRI = GOLD STANDARD, CT for bony abnlities, XR for vertebral defect

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9
Q

how to tx open spina bifida?

A

multidiscip, surgery, periodic ucx & bladder eval, reg bowel cleanouts, GI regimen, fxnal ambulation (wheelchair, cane, brace)

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10
Q

spina bifida occulta/occult spinal dysraphism

A

incomplete osseous fusion posterior elements of spine –> benign midline defect w/o protrusion of meninges or spinal cord b/c intact skin

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11
Q

occult spinal dysraphism = assoc w/?

A

derm sxs (sacral dimple, sacral hair patch, lipoma, skin discoloration, hemangiomas), syringomyelia, tethered cord, neuro sxs in LE, uro sxs, ortho sxs (deformities, scoliosis, kyphosis, dislocation)

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12
Q

sacral dimple vs dermoid sinus

A

<5mm benign skin dimple vs >5mm small skin opening leading to duct –> infxn; can be tethered to spinal cord; may have protruding hair, hairy patch, vascular nevus

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13
Q

anencephaly

A

failure of rostral neuropore to close day 25 –> defect in developing neuroaxis –> developing forebrain & brainstem exposed in utero –> destroyed –> open defect in calvaria, meninges, scalp

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14
Q

causes of anencephaly. how to tx it?

A

multifactorial (genetics, vit defic, folate metab, hyperthermia, low SES). none

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15
Q

encephalocele

A

sac + cerebral cortex, cerebellum, brainstem and/or meninges protruding thru midline defect in skull/cranium bifidum that’s covered in skin

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16
Q

risks for encephalocele. how to dx it?

A

hydrocephalus, visual probs, intellectual disability, sz, limited movement; Meckel-Gruber syndrome: auto rec w/ occipital encephalocele, cleft lip/palate, microcephaly, abnl genitals, polycystic dz, polydactyl. transilluminate, US, brain MRI/CT

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17
Q

sincipital vs basal vs occipital encephalocele. outcomes for each?

A

craniofacial deformities (hypertelorism, telecanthus, orbital dystopia, unilat micro/anophthalmos) vs hypertelorism, broad nose bridge, nasal or epipharyngeal mass, difficult breath, recurrent URI or meningitis, CSF leaks vs CN deficits, poor sucking/feeding, spasticity, blind, sz, developmental delay, Chiari III. craniofacial op vs blank vs depends on size, contents, hydrocephalus, infxn; LOCATION DOESN’T MATTER

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18
Q

hydrocephalus. major sites of CNS block in ventricular system?

A

impaired circ or absoprtion of CSF in lat/3rd/4th ventricles –> excess CSF in those spaces –> dilated. Monro, 3, Sylvius, apertures, subarachnoid space/cisterns

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19
Q

risk factors for hydrocephalus

A

birth wt <1500g, premature <30wks, maternal DM, low SES, X-linked for males, race/ethnic

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20
Q

causes of obstructive/noncommunicating vs nonobstructive/communicating of hydrocephalus

A

aqueduct stenosis/gliosis, vein of Galen malformation, post fossa tumors, Arnold Chiari malformation, Dandy Walker syndrome –> obstruction w/in ventricular system vs subarach hemorrhage, nonaccidental trauma, pneumococcal meningitis, TB menitiis, leukemic infiltrates –> obliteration of subarach cisterns or malfxn of arachnoid villi

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21
Q

sxs vs signs of hydrocephalus

A

irritability, listless/lethargy/fatigue, dec appetite/difficult feeding, emesis, HA, change in personality/behavior/academic performance, fail to meet developmental milestones vs inc head circumference, wide/bulging ant fontanelle, Setting Sun Eye sign, clonus/spasticity/Babinski, brisk tendon reflexes, extreme weakness, Macewen sign: percussion of skull –> “cracked pot”

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22
Q

how to dx vs tx vs prognosis hydrocephalus. can you do LP?

A

transilluminate, head US 0-18mo, brain MRI/CT (peds need to be sedated for MRI) vs mannitol, acetazolamide, furosemide, ventriculoperitoneal shunt, ventricle decompression vs at risk for developmental delays, behavior issues, vision probs, Cushings Triad (HTN, irreg respirations, bradycardia), brain herniation. NO LP not on someone w/ intracranial pressure

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23
Q

Dandy Walker syndrome. assoc w/?

A

congenital cystic expansion of 4th ventricle –> abnl cerebellar development. 3C syndrome, Walker-Warburg, Marden-Walker, Brachio-oculo-facial syndrome, Coffin-Siris, Sotos, XYY syndrome

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24
Q

s/s vs dx vs tx of Dandy Walker

A

hydrocephalus, cerebellar ataxia, cog & motor delay vs brain MRI vs early intervention, neurosurg consult, shunt cystic activity

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25
Arnold Chiari malformations. type of cause?
hindbrain malformation affecting rlatinshp b/w cranial base, cerebell, brainstem, upper cervical cord. multifactorial affecting chrms9-15
26
type I vs II Chiari. sxs?
abnl shaped cerebellar tonsils, assoc w/ hydromyelia or syringomyelia (fluid filled cavity in spinal cord) vs downward displacement of cerebellar vermis, tonsils, med, 4th ventricle --> beaked midbrain --> hydrocephalus or myelomeningocele --> brainstem dysfxn, vocal cord paralysis, apneic. dysphagia, stridor, severe head/neck pain, loss of pain/temp sense, drop attacks, spasticity, bal probs, blurred vision, hypersensitivity to light
27
type III vs IV Chiari
both very rare, incompatible w/ life --> NICU, won't see in office. cerebellar herniation --> congenital malformations vs cerebellar agenesis
28
how to dx vs tx chiari?
brain MRI > CT, brainstem auditory evoked potential, somatosensory evoked potentials vs based on sxs, decompression surgery
29
d/o of neuronal migration. examples?
disrupting migration of neuroblasts during neurogenesis. agenesis corpus callosum, holoprosencephaly, lissencephaly, microcephaly
30
agenesis corpus callosum. heredity? assoc w? tx?
largest white matter interhemispheric tracts connecting cerebral hemis for sensory, motor, visuomotor, & cog processes. can be partially (hypogenesis) or completely (agenesis) absent, X linked or auto dom. Chiari, Dandy Walker, Andermann, Aicardi, schizencephaly (division in brain tissue), holoprosencephaly. supportive
31
holoprosencephaly. cause? assoc w/? dx? tx?
defective cleavage of prosencephalon --> forebrain didn't divide into 2 hemis. maternal DM. chrm d/o, single incisor, cyclopia, maxillary agenesis. fetal US, MRI. multidiscip, supportive
32
3 groups of holoprosencephaly: alobar vs semilobar vs lobar
complete failure to divide into L/R hemis vs partial separation vs distinct L/R ventricles but cerebral continuity across frontal cortex
33
lissencephaly/agyria. assoc w? dx?
failure of nuronal migration during wk12-14 --> lack gyri & sulci --> smooth brain. Miller-Dieker, microcephaly, ventriculomegaly, wide Sylvian fissures, agenesis corpus callosum, failure to thrive, developmental delay, sz. brain MRI
34
2 types of lissencephaly: 1 vs 2
facial dysmorphism, deletion chrm 17q13.3 => Miller-Dieker vs hydrocephaly, dysgenesis of cerebellum
35
microcephaly. assoc w/? tx?
<3 stdev head cirumference for mean age & sex. NTD, holoprosencephaly, lissencephaly. genetic & fam counseling, supportive
36
primary/genetic vs secondary/nongenetic microcephaly
abnl brain development --> dec # of neurons during neurogenesis --> affects forebrain (holoprosencephaly); familial, auto dom vs dec # of dendrites & synaptic connections d/t noxious agents in utero or 1st 2yrs of life (radiation, TORCH infxns, CMV, zika, maternal meds/drugs)
37
grey matter fxn & lesion: ant horn vs post horn vs lat horn/intermediolat gray column)
efferent motor neurons; lesion --> LMN sxs vs afferent sensory neurons; lesion --> sensory deficits vs T1-L2 preganglionic sympathetic autonomic fibers; lesion --> autonomic dysfxn
38
ascending tracts in white matter: ant/lat spinothalamic vs post funiculus vs ant/post spinocerebellar tract
ant spinothalamic --> coarse touch; lat spinothalamic --> pain/temp vs gracilis --> fine touch, vibration, consc proprio of LE; cuneatus --> same w/ gracilis but UE vs unconsc proprio to cerebellum
39
descending tracts in white matter fxn & lesion: pyramidal/corticospinal tract vs extrapyramidal tract vs autonomic pathway
ant/lat for voluntary motor fxn; lesion --> UMN sxs vs voluntary motor fxn vs blank
40
complete spinal cord transection
all ascending tracts below lesion & all descending tracts above lesion = affected --> disturbed sensory (pain/temp, touch, vib, proprio), motor (para/tetraplegia), auto (urin/fec incont). TRANSVERSE MYELOPATHY
41
hemisection of spinal cord
no pain/temp contralat to hemisection, ipsi loss proprio below lesion, ipsi spastic wkness, LMN sxs. BROWN-SEQUARD SYNDROME
42
central cord syndrome
spinal cord dmg starting centrally & move centrifugally --> compromised spinothalamic decussating fibers conveying pain/temp --> ant horn cells involved (neurogenic atrophy, paresis, areflexia), ipsi Horner, dorsal column dysfxn. SYRINGOMYELIA
43
posterolat column syndrome
dorsal column dysfxn, Romberg's sign; bil corticospinal tract dysfxn --> spastic, hyperreflexia, bil Babinski sign. B12 DEFIC, HIV. TABES DORSALIS (TABETIC NEUROSYPHILIS) FOR POST COLUMN SYNDROME
44
ant horn cell syndrome
affects motor neurons; weakness, atrophy, fasciculation, dec muscle tone, dec DTR; sensory tracts not affected. SPINAL MUSCULAR ATROPHY. Lec 7, slide 20
45
combined ant horn & pyramidal tract syndromes
diffuse LMN sxs (muscle atrophy, paresis, fasc), UMN sxs (spastic, paresis, extensor plantar responses)
46
vasc syndrome of spinal cord
ant spinal a infarct --> girdle pain, LMN sxs below lesion, urin/fec incont; proprio, vib, light touch intact b/c not affecting dorsal column
47
LMN vs UMN syndrome. know sxs b/w the 2
dmg to motor neuron, nerve root/plexus, peripheral n vs dmg to descending motor pathway. Lec 7, slide 10
48
sensory vs motor vs reflex features of root dz. cause?
radicular/dermotomal sharp pain inc by movement & Valsalva vs myotomal weakness and/or atrophy, fasc vs dec/absent reflexes. trauma, VZV, Lyme, DM, tumor, herniated disc
49
clinical features of plexopathy
dmg to plexus --> deficit in 1+ spinal or peripheral n
50
clinical features of peripheral neuropathy
motor or sensory, distal weakness, a/sym weakness, denervation changes
51
amyotrophic lat sclerosis
degen of ant horn cell --> of motor nuclei in brainstem --> of corticospinal tract --> LMN sxs & UMN sxs
52
radiculopathies def
d/o affecting nerve roots: myotome --> motor involvement, dermotome --> sensory involvement; DTR involved
53
C5 radiculopathy
myotome: weak lev scap, rhomboids, supra/infraspinatus, delts, biceps, brachioradialis dermotome: lat upper arm DTR: biceps, brachioradialis
54
C6 radiculopathy
myotome: biceps, pron teres, brachioradialis, fl/ex carpi radialis dermatome: lat forarm, lat hand, 1st&2nd digits DTR: biceps, brachioradialis
55
C7 radiculopathy
myotome: triceps, pron teres, fl/ex carpi radialis dermatome: 3rd&4th digits DTR: triceps (even tho it's mostly C8)
56
C8 radiculopathy
myotome: flexor pollcis longus, oppenens pollicis, abductor pollicis brevis, lumbricals, abductor digiti minimi, flexor & extensor carpi ulnaris dermatome: medial forearm, medial hand, 5th digit DTR: triceps, finger flexion
57
L4 radiculopathy
myotome: quads, sartorius, ant tibialis dermatome: knee & medial thigh DTR: knee jerk
58
L5 radiculopathy
myotome: glut min/med, ant tibialis, plantar in/eversion, extensor hallucis longus dermatome: lat thigh, dorsomedial foot, large toe DTR: none
59
S1 radiculopathy
myotome: biceps femoris/knee flexion, gastroc/sol, flexor all toes dermtome: 5th digit, lat foot, sole foot DTR: dec/absent ankle jerk
60
brachial plexopathies causes
trauma, vax, post-rad, (viral) infxn, mass lesion from metastasis from br/lung ca
61
Erb-Duchenne syndrome
lesion at 5/6th cervical roots or upper trunk; trauma separation of head/shoulder at birth or heavy backpacks --> arms adducte & IR, forearm prone, flexed wrist, biceps reflex absent but grasp reflex nml
62
Dejerine-Klumpke syndrome
lesion at 8th cervical & 1st thoracic roots of medial cord; trauma, surgery, mass lesions; paresis in wrist/finger flexors & intrinsic hand muscles, sensory change in medial upper arm/med forearm/ulnar hand, triceps & grasp reflex dec
63
DM neuropathy
T2DM; prone to compressive/entrapment & cranial neuropathies d/t demyelination --> carpal/cubital tunnel, peroneal nerve entrap, rdial nerve injury, facial nerve weakness (Bell's palsy), trigeminal or glossopharyngeal neuralgia
64
small fiber vs large fiber vs autonomic neuropathy
distal, symm, chronic, progressive pain & auto disturbance w/o weakness & sensory loss vs distal, symm, chronic, progressive pain, weakness, areflexia, sensory loss vs distal or generalized; subacute to chronic; auto dysfxn from reddened skin to gastroparesis to postural HTN
65
Guillan Barre syndrome. how to dx? how to tx?
inflmm autoimmune neuropathy d/t demyelination of peripheral nerve at jxn of dorsal/ventral roots; severe = axon degen --> rapid ascending polyneuropathy, areflexia, auto dysfxn, resp distress, Campylobacter jejuni. CSF shows elevated protein & nml cell count; LP, NCV, EMG, sero studies for infectious syndromes like HIV. resp support, plasmapheresis, IV ig
66
chronic inflammatory demyelination polyradiculoneuropathy. how to tx?
chronic progressive demyelinating neuropathy --> axons degen --> sensory disturbance, weakness, atrophy, auto changes. prednisone, plasmapharesis, IV ig, chemo
67
alc neuropathy
poor nutrition --> axon degen --> neuro deficits
68
AIDS. how to tx?
axonal, distal, symm, sensorimotor neuropathy; inflamm demyelinating polyneuropathy d/t autoimmune infxn --> abnl CSF w/ inc protein & WBC. immuomodulators
69
Charcot-Marie-Tooth
progressive sensorimotor forms --> severe pain, cerebellar dysfxn, cranial nerve signs, essential tremor; distal weakness, high arched feet, hammertoes, absent ankle jerks, steppage gait
70
ethics revolve around the 4 principles. surgical ethics involve? what's neuroethics?
character, conduct; justice, fairness requiring critical assessment; relevance, integrity. ethical/legal/social impacts of neurosci --> arising from what we know & what we can do
71
5 issues to consider for neuroethics
neuroenhancement (psychopharm, doping, psychosurg, robotics in paralysis), cog liberty (privacy), stem cells, consciousness (brain death, coma, PVS), neuromarketing/insurance, free will (decoding mental content)
72
informed consent: who can make decision if pt can't?
spouse, adult children, parents, siblings, next of kin; special friend appointed by pt or w/ reg contact; judicial authorization
73
what's one of the biggest problems drs face in medicine?
bias
74
7 ethical issues affecting neurosurgeons
1. accountability of surgeons to pts 2. financial incentives & motivations 3. preservation of pt autonomy 4. matters of leadership 5. info exchange & communication 6. EHR & data security 7. resrc allocation
75
moral distress can lead to?
burnout, staff quiting, avoiding pt interaction, blame, powerless/guilt/self-critic/low self esteem
76
who to talk to if stuck in ethical situation?
peers/nursing/mgmt, ethics board, legal counsel, palliative/hospice care (tx emotional, social, spiritual issues vs providing care post tx)
77
Victor Horsley vs Harvey Cushing vs Fishgold&Mathis
artificial resp --> impossible to kill animal w/ intracranial pressure vs Cushing reflex: HTN, bradycardia, irreg resp vs ID EEG to coma/anoxia
78
1968: Harvard Med School Committee proposed neuro def of brain death/irreversible coma
1. unresponsive 2. no cranial nerve reflexes 3. pos apnea
79
1981: President's commission for legal def of brain death
individual who sustained either: irreversible cessation of circ & resp fxns; or all fxns of entire brain including brainstem
80
who makes the call for brain death? what's brain death in a nutshell?
2 physicians, 1 of them must be neurologist or neurosurgeon. absent cortical brain fxn & brainstem fxn, irreversible; reflexes can still happen (spont limb movement, triple flexion, Lazarus sign)
81
exclusion criteria of brain death
1. time >6h b/w 2 exams (24h for infants 37wks-30d, 12h for 30d-18yo) 2. core temp >35C/95F 3. shock/hypoTN 4. drugs (neuro/musc fx; phenobarbitol
82
what's NOT brain death?
any cortical or brainstem fxn, spont breathing, recovery of brain fxn after cessation of all brain + brainstem fxn for 6h and never documented.
83
how to do exam for brain death
1. motor response to pain 2. cranial n fxn (pupillary light reflex, corneal reflex, occulocephalic reflex/doll's eyes, vestibulo-occular reflex/cold caloric testing, gag&cough reflex) 3. apnea testing 4. ancillary testing (EEG, nuclear, angiography)
84
what's pos apnea test for brain death exam? when is it invalid?
no spont breathing after disconnecting ET, apnea >2min, PaCO2 > 60mmHg or >20mm Hg over baseline, 15min of 100% FiO2 before test, passive O2 at 6L/min. pt breathing, hypoTN, O2 sat < 80%, cardiac arrhythmia
85
how to interpret EEG of ancillary test for brain death exam?. know how to interpret readings
electrocerebral silence = no electrical activity; excludes depressant drugs, hypothermia, shock. Lec 10, slides 43-47
86
nuclear ancillary test for brain death exam
radionucleotide tracer (Tc-99m) injected w/ delayed scans; assess adeq tracer in CCA; lack of tracer in brain tissue for 15-20min & in dural venous sinuses --> brain death
87
angiography vs CT Angio vs transcranial doppler vs SPECT ancillary test for brain death exam
no intracranial flow, no brain fxn vs noninvasive, cheap, avail; not validated, contrast can dmg transplant organs vs shows reverberating signal or systolic spikes. noninvasive, cheap, no pt transport; not validated, eval circ arrest rather than brainstem fxn vs empty skull sign
88
what if ancillary tests don't vs do show brain death?
tx medically till brain death = est on clinical exam/apnea test vs shorten the observation period
89
does brain death = death?
time of death = via neuro exam/apnea test, not when ventilator = remove or heartbeat cessation
90
donation after brain vs cardiac death
pt maintained on ventilator, organs dissected in situ vs pt extubated in OR, surgery starts 5min after cardiac cessation, rapid recovery of organs en bloc

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