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