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185 Cards in this Set

  • Front
  • Back
location of the cell body of hte lower motor neuron
anterior horn of hte spinal cord
embryological origin of muscle
mesenchyme
how is muscle type determined
neuron type (I or II) that innervates it
which fibers are slow twitch
what type of energy do they depend on
type I
oxidative
thin filaments in muscle
actin
causes of peripheral nerve trauma
laceration
avulsion (mechanical force)
crushing
how does peripheral nerve react to blunt or sharp trauma
wallerian degeneration
which fibers are optimally active at acid pH
Type 1
name 2 types of peripheral neuropathies
axonal degeneration
segmental demyelination
wasting away of axon with secondary myelin sheath disintegration
axonal degeneration
axonal degeneration: etiology
toxic (hexane in glue)
metabolic (diabetes, uremia)
nutritional deficiency(thiamine)
infectious (HIV)
hereditary
paraneoplastic
idiopathic
axonal degeneration: where does it begin
distal end of longest and largest-diameter fibers of sensory and motor first
axonal degeneration: reversible?
yes if etiologic condition is reversed
myelin sheath or schwall cell is primary pathologic process
segmental demyelination
segmental demyelination: etiology
immune mediated (B or T cell)
axonal degeneration: clinical presentation
"stocking and glove" distribution
polyneuropathy
poliomyelitis
ALS
segmental demyelination (Guillain-Barre): clinical presentation
rapidly progressive ascending motor weakness
sensory symptoms milder than motor
spontaneous resolution
Guillain-Barre: labs
CSF: elevated protein, w/o incr WBC
EMG/NCV: nerve conduction velocity slowed
diseases associated with denervation atrophy
polio
ALS
peripheral neuropathies
clinical presentation of LMN/UMN lesions
LMN: flaccid areflexic paralysis, decreasing resting tone
UMN: spastic hyperreflexic paralysis, diffuse atrophy
how can you distinguish LMN and UMN lesions on biopsy
UMN demonstrate dissue atrophy (selective type2 atrophy)
LMN denervation atrophy affects Type 1and2 equally
do NMJ disorders produce denervation atrophy in muscle biopsy
NO
inflammatory myopathies
dermatomyosites
polymyositis
inclusion body myositis
non-inflammatory myopathies
muscular dystrophies
congenital myopathies
metabolic myopathies
toxic myopathies
Dermatomyositis: who
adults and chidren
Dermatomyositis: clinical
symmetrical proximal muscle weakness
heliotrope rash
association with visceral malignancy in adult women
Dermatomyositis: lab
elevated CK
Dermatomyositis: etiology
Ab and complement attack on muscle capillaries
Dermatomyositis: biopsy
muscle fiber degeneration
phagocytosis
regen
perivascular B and CD4 T cells
Dermatomyositis: tx
corticosteroids and immunosuppressives
Duchenne's muscular dystrophy: clinical presentation
males around age 5 w/ onset of pelvic girdle weakness
gower's sign
calf pseudohypertrophy
Duchenne's muscular dystrophy: lab
elevated CK
DMD: etiology
lack of distrophin
X-linked recessive
DMD: biopsy
wide range in muscle fiber size
degen-regen-phago
interstitial (endomysial) fibrosis ans fatty infiltration
Becker's muscular dystrophy: clinical
males
later onset than DMD with slower progression and longer survival
Becker's muscular dystrophy: labs
elevated CK
Becker's muscular dystrophy: etiology
truncated dystrophin protein
MC adult muscular dystrophy
myotonic dystrophy
Myotonic Dystrophy: genetics
AD
chromosome 19q
Adult with delayed muscle relaxation after handshake
myotonic dystrophy
frontal baldness, cataracts, muscle weakness, atrophy with characteristic facies, gonadal atrophy, cardiac and smooth muscle involvement, mental retardation
myotonic dystrophy
myotonic dystrophy: onset
childhood to adult
"dive bomber" electromyography
myotonic dystrophy
myotonic dystrophy: etiology
GTG repeats
myotonic dystrophy: 4 histologic features on biopsy
degen-regen-phago
ring fibers
type 1 atrophy
intrafusal fibers show splitting
how are congenital myopathies different from muscular dystrophies
congenital myopathies present at birth and dystrophies present later in development
congenital myopathies: 3 pathologic patterns
central core myopathy
nemaline
centronuclear
floppy baby with joint contractures
congenital myopathies
muscle diseases resulting from a defect in muscle fiber energy production
metabolic myopathies
enlarged mitochondria
ragged red fibers
"parking lot" cristae
mitochondrial myopathies
Glycolytic enzyme deficiency
McArdle's disease
external opthalmoplegia, pigmentary retinal degeneration
heart block
Kearns-Sayre syndrome (mitochondrial myopathy)
T/F carnitine deficiency is an example of glycogenoses
F: Carnitine deficency is a lipid myopathies a type of metabolic myopathy
3 causes of toxic myopathy
endocrine (hyper/hypothyroid)
ethanol
drugs: steroids, chloroquine, statins
MG: who
adults, females>males in pt younger than 40
MG: clinical presentation
muscle fatigues on repeated contraction
extraocular muscle involvement
Tensilon test
temporary improvement with anticholinesterase agents in MG
MG: etiology
IgG autoantibody against post-synaptic Ach receptors
MG: biopsy
no denervation atrophy
endomysiallymphocyte collections
type 2 fiber atrophy
MG: Tx
anticholinesterase
steroids
plasmapheresis
thymectomy
Eaton Lambert syndrome: etiology
paraneoplastic complication (small cell lung carcinoma)
IgG attack on presynaptic membrane
how is Eaton-Lambert different than MG
In Eaton-Lambert the muscle does not fatigue with repeated contraction
no improvement with anticholinesterases
amplitude of muscle AP incr with repetitive stimulation
anerobe that blocks presynaptic ACh release
Clostridium botulinum
head injury directly below the point of impact
coup contusion
man slips on banana peel and hits back of head
contrecoup contusion (damage to back of head and frontal/temporal areas)
major cause of coma in head trauma
diffuse axonal injury
petechial hemorrhages
rotational forces associated with rapid accel-deceleration
diffuse axonal injury
MCC epidural hemorrhage
lac of middle meningeal artery d/t temporal or parietal bone fracture
concussion followed b short lucid interval
epidural hemorrhage
ipsilateral pupil dilation
CNIII palsy
contralateral hemiparesis
deep tendon hyperreflexia
Babinski sign
epidural hematoma
etiology of subdural hemorrhage
rapid change in head velocity causes bridging veins to tear
Shaken baby syndrome
subdural hemorrhage
slowly progressive deterioration
headache, non-specific findings
subdural hemorrhage
where do bridging veins empty into?
superior sagittal sinus
elderly pt presents with headache, apathy, confusion
subdural hematoma
subdural hematoma: labs
CT
CSF: xanthomic (yellow) with low or normal protein
emboli originating in carotid
Pt presents with amorosis fugax, unilateral loss of power
Anterior circulation TIA
amorosis fugax
transient monocular blindness
transient loss of balance, bilateral blindness, bilateral weakness
posterior TIA arising from vertebrobasilar arteries
complication of TIA
stroke within a year
MCC CVA
ischemic infarctions (85%)
hemorrhage (15%)
etiology of ischemic stroke
emboli often of cardiac origin
thrombotic occulsions usually in MCA
how do embolic CVA appear
hemorrhagic
necrosis
ischemic CVA histology:
12-24hrs "red, dead neurons"; cerebral edemal
2-3wks gitter cells (microglia)
2mo gliosis (scarring)
artery that supplies frontoparietal somatosensory cortex
MCA
clinical manifestation of MCA infarction
hemiparesis
artery that supplies frontal lobes and medial aspects of parietal and occipital lobes
ACA
artery that supplies occipital and inferior temporal lobes incld. hippocampus
PCA
vessels that supply putamen
lenticulostriate off MCA
type of brain necrosis
liquefactive
type of brain damage caused by hypovolemic shock
C-shaped infarction (watershed areas)
deep penetrating arterioles (lenticulostriate) of MCA occluded in person with HTN and diabetes
Lacunar stroke
multiple infarcts in basal ganglia, internal capsule, thalamus or brain stem
lacunar stroke
Who gets hemorrhagic stroke?
AA
Asians
HTN
anti-coagulants
cocaine
Chacot-Bouchard microaneurysms case what type of CVA
parenchymal hemorrhage
Chacot-Bouchard microaneurysms: where
putamen
CVA with partial or complete restoration of function over time
parenchymal hemorrhage
MCC subarachnoid hemorrhage
rupture of berry aneurysm
why are berry aneurysms prone to rupture
lack muscular wall and intimal elastic lamina
MC location of berry aneurysm
anterior circle of Willis, usually at bifurcation points of vessels
abrupt onset of severe headache, vomiting, stupor, coma, meningitis-like symptoms, blood in CSF
subarachnoid hemorrhage
subarachnoid hemorrhage associations
ADPKD and collagen disorders (Marfans, Ehlers-Danlos)
4 types of CVAs
atherosclerotic (thrombotic)
embolic
Intracerebal hemorrhage
subarchanoid hemorrhage
2 types of cerebral edema
vasogenic/interstitial
cytotoxic/cellular
cerebral edema caused by disruption of integrity of BBB (increased vessel permeability)
vasogenic
white matter cerebral edema
vasogenic
grey matter cerebral edema
cytotoxic/cellular
cerebral edema d/t hypoxic injury and damage to Na/K pump
cytotoxic/cellular
Causes of cerebral edema
inflammation
mets, trauma, lead poisoning
respiratory acidosis, hypoexemia
Cerebral edema tx
osmotherapy (mannitol)
craniotomy
V-P shunt
Cushing's triad
bradycardia
widened pulse pressure
HTN
3 types of brain herniations
subfalcine
transtentorial/uncinate
tonsilar
cingulate gyrus is pushed under the falx cerebri
subfacine herniation
complication of subfalcine herniation
compression of ACA, but pt don't have many clinical symptoms
uncus of temporal lobe forced into tentorial hiatus
transtentorial herniation
complication of transtentorial herniation
compression of oculomotor nerve, midbrain, cerebellar peduncle, PCA
blown pupil on ipsilateral side
pupil down and out
contralateral paralysis
Cheyne-Stokes
transtentorial herniation
downward displacement of cerebellar tonsils through foramen magnum
tonsilar herniation
complication of tonsilar herniation
compression of midbrain/medullar
MCC brain tumor
mets from breast, lung, renal cancer or melanoma
clinical presentation of CNS tumor
signs of increased intracranial pressure: early am emesis, headache, papilledema
progressive neurological defecits
sudden-onset seizure
MC location of CNS tumors in
a. adults
b. children
a. above tentorium
b. below tentorium, in cerebellum or 4th ventricle
3 types of gliomas
astrocytomas
oligodendrogliomas
ependymomas
MCC primary CNS tumors
fibrillary gliomas
3 types of astrocytomas
fibrillary
pilocytic
glioblastoma multiforme
astrocytoma tumors: grade
pilocytic grade 1
fibrillary grade 2/3
glioblastoma multiforme grade 4
pilocytic astrocytoma:
who
where
a. peds
b. mural of cerebellum, optic nerve
Rosenthal fibers
pilocytic astrocytomas
fibrillary astrocytomas
a. who
b. where
adults
above tentorium (when they do occur in kids they are in pons)
hypercelluar brain tumor
GFAP+
fibrillary astrocytomas
glioblastomas multiforme
a. who
b. gross appearance
c. where
d. prognosis
a. older adults (50-60)
b. hemorrhage/necrosis
c. anywhere includ sp.cd
d. poor
glioblastomas multiforme: molecular markers
de novo: EGFR+
transformation from grade3: p53+
serpentine necrosis
pallisading necrosis
microvascular hyperplasia
glioblastomas multiforme
astrocytomas in white matter
gliomatosis cerebri
ependymoma: where
adults: intra-medullary area of spinal canal; 50% filum terminale
children: 4th ventricle
ependymoma: gross
well-circumscribed, slow growing
perivascular pseudorosettes
ependymoma
drop mets
ependymomas spread through CSF
oligodendroglioma: where
supra-tentoria
fried egg
chicken wire
dystropic calcifications
oligodendroglioma
oligodendroglioma: presentation
middle-age adult presents with seizues
only CNS tumor sensitive to chemo
oligodendroglioma
meningioma: who
middle-aged women
Multiple meningiomas
NF-2
meningioma: where
sagittal sinus
anchors into dura
dura tail on radiography
meningioma
psmammona bodies
meningioma
PR+
meningioma
meningioma: prognosis
benign
only resected if they become large and symptomatic
Vestibular schwannoma aka....
acoustic neuroma
cerebello-pontine angle tumor
vestibular schwannoma: who
middle-age women
bilateral vestibular schwannoma
NF-2
middle aged woman with tinnitus, facial weakness
vestibular schwannoma
Antoni A and B
vestibular schwannoma
A: hypercellular
B: hypocellular
medulloblastoma:
a. who
b. where
a. children<10
b. midline in cerebellum, arising from vermis
AD basal cell nevus syndrome
medulloblastomas and ovarian fibromas, odontogenic keratocysts, pits of palms and soles and generalized overgrowth
Gorlin syndrome
10yo with headache, morning emesis, ataxia, gait disturbance and lethargy
medulloblastoma
Homer-Wright rosettes
medulloblastoma
brain tumor that mets to bone, lymph nodes, lungs and abdomen via CNS
medulloblastoma
Medulloblastoma: tx
surgery
radiation
chemo
medulloblastoma: prognosis
80% 5yr survival
non-hodgkin lymphoma
no lymph node involvement
primary CNS lymphoma
primary CNS lymphoma: who
immunocompromised
primary CNS lymphoma: symptoms
increased intracranial pressure: mental status changes, confusion, memory loss, seizues
EBV+ brain tumor
primary CNS lymphoma in an immunocompromised pt
MCC spinal tumor
mets
types of primary extramedullary spinal cord tumors
neurofibromas
meningiomas
types of primary intramedullary spinal cord tumors
ependymomas
astrocytomas
back pain, weakness, sensory/motor deficits, autonomic dysfunction
spinal cord compression caused by spinal cord tumor
spinal cord tumor: tx
radiation
MCC brain abscess
staph
who is at high risk of brain abscess
congenital heart disease (R to L shunt)
air/fuid level on radiography
abscess (air generated by bacteria)
MCC adult bacterial meningitis
pneumococcus, meningococcus (college), H.influ, L.monocytogenes, staph
MCC children bacterial meningitis
pneumococcus, H.influ
MCC neonate bacterial meningitis
Strep B, E.coli
MCC CNS fungal mycosis
cryptococcal meningitis
AIDS patient exposed to pigeons
cryptococcal meningitis
etiology of progressive multifocal leukoencephalopathy
polyoma JC virus
demylelinating disease that affects nuclei of oligodendrocytes
PML
progressive motor deficits, cognitive decline, visual loss and quick progression to death
PML
mutation in PrP gene
Creutzfeld-Jacob
progressive dementia, central blindness, myoclonus
Creutzfeld-Jacob
micro-vacuolization of cortex
atrophy of cortex
gliosis
no inflammation
Creutzfeld-Jacob
NF-2 chromosome
22q11