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Pathology > Msk > Flashcards

Msk Flashcards

1
Q

2 main responses of peripheral nerve injury determined by target

A

Schwann-segmental demyelination -loss of myelin

Axon-axonal degeneration primary involvement of neuron and its axon, axonal degeneration may be followed by axonal regenerationa nd reinervation of muscle

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2
Q

Enervation atrophy

A

Follows loss of axon

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3
Q

Myopathy

A

Primary abnormality of msucle fiber

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4
Q

Segmental demyelination

A

Schwann cell damage to myelin sheath-Guillain barre

No primary abnormality of axon

Not affect all schwann cells

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5
Q

Denuded axon

A

Stimulus for demyelination

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6
Q

Newly myelinated internodes

A

Shorter than normal

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7
Q

Onion bulbs

A

Concentric layers of schwann cytoplasm and redundant basement membrane surrounding thinly myelinated axon

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8
Q

Traumatic neuroma

A

Failure of the outgrowing axons to find their distal target can make pseudotumor termed traumatic neuroma -a non neoplastic haphazard whirled proliferation of axonal processes and associated schwann cells that results in.a painful nodule-haphazard axons, schwann cells and connective tissue

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9
Q

Axonal degeneration

A

Axonal degeneration-result of primary destruction of axon with secondary disintegration of myelin sheath

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10
Q

Axon damage

A

Focal or generalized affecting whole neuron body (neuronopathy) or its axon (axonopathy)

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11
Q

Wallerian degeneration

A

Focal lesion distal portion

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12
Q

Myelin ovoids

A

Schwann cells catabolize myelin and later engulf axon fragments, producing small oval compartments

Then macrophages recruited

Proximal stump of severed nerve shows degenerative changes in most distal 2-3 internodes, then undergoes regeneration
Slowly evolving neuronopathies or axonopathies, scant evidence of axonal degeneration bc few fibers effecting at ant one time

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13
Q

Enervation atrophy

A

Axonal degeneration->muscle fibers in motor unit lose neural input and undergo denigration atrophy

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14
Q

Angulated fibers

A

Atrophic fibers are smaller and triangular shape when enervation atrophy

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15
Q

Target fibers

A

Rounded zone of disorganized myofibers in center fiber

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16
Q

How do we get growth of regenerating fibers: where does guide come from

A

Schwann cells vacated by degenerating axons

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17
Q

Regenerating cluster

A

Multiple closely aggregated thinly myelinated small caliber axons

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18
Q

Reinnervation fo skeletal muscle changes its composition, altering distribution of

A

The 2 major fiber tubes

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19
Q

__ __ determines fiber type; all muscle fibers of a single unit are the same type

A

Motor neuron

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20
Q

Fibers of a single unit are distributed across the muscle ___ __

A

Checkerboard pattern

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21
Q

Axons of unaffected neighboring motor unit extend sprouts to __ and __ ___ and incorporate them into the _____

A

Reinnervate
Denervated myocytes
Healthy motor unit

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22
Q

Newly adopted reinnervated fibers assume fiber type of their __ __

A

New siblings

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23
Q

Type grouping

A

Patch of contiguous myocytes having same histochemical type

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24
Q

Group atrophy

A

Type group becomes denervated

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25
Q

Type 2 fiber atrophy

A

Inactivity or disues (neurodegenerative dz)

Steroid myopathy-glucocorticoid use

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26
Q

Segmental necrosis

A

Destruction of a portion of myocytes, followed by myophagocytosis; loss of msucle fiber leads to deposition of collagen and fat

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27
Q

Muscle reaction seen in many diseases

A

Vacuolixation, alterations in structural proteins or organelles and accumulation of intracytopalsmic deposits

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28
Q

Reactions of muscle fibers

A

Segmental necrosis
Vacuolization, alterations in structural proteins or organelles and accumulation of intracytopalsmic deposits
Regeneration
Hypertrophy-muscle fiber splitting

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29
Q

Regeneration of muscle fiber

A

Satellite precursor cells proliferate and reconstitute the destroyed portion of fiber; regenerating portion has large internalized )central location) nuclei with prominent nucleoli; cytoplasm laden with RNA is RED

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30
Q

Hypertrophy muscle fiber

A

Response to increase load either through exercise or pathological conditions

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31
Q

Muscle fiber splitting

A

Large fibers may divide longitudinally; cross section=single large fiber with a cell membrane transversing its diameter, often with adjacent nuclei

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32
Q

Peripheral neuropathy pain

A

Tingling, stabbing, burning, or pins and needles

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33
Q

Mononeuropathies

A

Affect single nerve, deficits to nerve distribution

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34
Q

Polyneuropathies

A

Multiple nerves, usually symmetric
Deficits start at the feet and ascend with disease progression

Hand usually deficits same time as knee=stocking and glove distribution

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35
Q

Mononeuritis multiplex

A

Several nerves damaged in haphazard fashion

Vasculitis is a common cause of this pattern (polyarthritis nodosum-PAN)

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36
Q

Polyradiculoneuropathies

A

Nerve roots as well as peripheral nerves

Diffuse symmetric in proximal and distal parts of the body

Asymmetric peripheral neuropathy-multiple mononeuropathy

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37
Q

Bell’s palsy

A

Mononeuropathy
CNVII facial muscle paralysis

Resolves spontaneously

One sided facial droop within 48-72 horus
—URI and DM
-facial tingling , mid severe HA/neck pain , memory problems, balances issues, ipsilateral limb paresthesia, ipsilateral limb weakness

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38
Q

Neurogenic bladder

A

No bladder control from brain and spinal cord or nerve problem

MS, parkinson, diabetes nerve damage

Infection

Spina bifida

Overactive, underactive, Obstructive, flow

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39
Q

Peripheral nerve diseases

A

Inflammatory neuropathies

Infectious polyneuropathies

Hereditary neuropathies

Acquired metabolic and toxic neuropathies

Traumatic neuropathies

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40
Q

Gillian barre

A

Acute inflammatory demyelinating polyneuropathy of PNS
Ascending paralysis start legs and DTR disappear

Inflammation and demyelination of spinal nerve roots and peripheral nerves(radiculoneuropathy)

ACUTE ONSET immune mediated demyelinating neuropathy

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41
Q

Pathogenesis Gillian barre

A

Preceded by acute influenza like illness
Campylobacter jejuni, CMV, epstein Barr, mycopalsma Pneumonias, propria vaccination0

Perivenular and endoneurial inflammation

Segmental demyelination mainly peripheral nerves

Anti myelin antibodies

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42
Q

In Gillian barre cytoplasmic processes of ___ penetrate the BM of __ cells , in the _____ and extend between myelin lamellae stripping myelin sheath away from axon

A

Macrophages
Schwann
Nodes of ranvier

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43
Q

Increased CNS protein production in Gillian barre

A

Due to altered permeability of microcirculation of spinal roots
-inflammatory cells remain confined to the roots, thus little or no CSF pleocytosis

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44
Q

Treat Gillian barre

A

Plasmapheresis IVIg

Many have long term disability

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45
Q

Chronic inflammatory demyelinating polyneuropathy

A

Most common peripheral neuropathy

Symmetrical mixed sensorimotor polyneuropathy >2 months (NEED 2 moths)

Relapses and remits

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46
Q

Diagnose chronic inflammatory demyelinating polyrediculoneuropatthy

A

Time, response to steroids distinguish from Gillian barre

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47
Q

Sign of chronic inflammatory. Demyelinating poly(radiculoneuropathy

A

Onion bulbs

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48
Q

What are onion bulbs

A

Multiple layers of schwann cells wrap around an axon like the layers of an onion

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49
Q

Infectious polyneuropathies

A

Leprosy
Diptheria
Varicella zoster virus

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50
Q

Leprosy: lepromatous

A

Schwann ells invaded by mycobacterium leprae

Segmental demyelination and remyelination and loss of both myelinated and unmyelinated axons

Endoneurial fibrosis and multilayered thickening of perineural sheath

Symmetric polyneuropathy affecting cool extremities

Involves pain fibers, loss of sensation contributes to injury

Large traumatic ulcers

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51
Q

Tuberculoid leprosy

A

Active cell mediated response

Granulomatous nodules in dermis

Localized nerve involvement

Injures cutaneous nerves

Axons, schwann cells and myelin lost

Fibrosis and perineurium and endoneurial

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52
Q

Siptheria

A

Exotoxins affects peripheral nerves and begins with paresthesia and weakness

Early losss of proprioception and vibratory sensation

Sensory ganglia

Selective demyelination of axons that extend into adjacent anterior and posterior roots and into mixed sensorimotor nerves
Periph neuropathy assoc with prominent bulbar and respiratory msucle dysfunction->death or long term disability

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53
Q

Varicella zoster

A

Most common viral infection of PNS

Sensory ganglia
Reactiation of latent -shingles

Neuronal destruction and loss of affected ganglia; abundant mononuclear infiltrate; regional necrosis and hemorrhage may be found

Axonal degeneration of peripheral nerves after death of sensory neurons

Focal destruction of large motor neurons in anterior horns or cranial nerve motor nuclei may be seen at corresponding levels

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54
Q

Diabetes

A

Most common cause of peripheral neuropathy

Ascending distal symmetric sensorimotor polyneuropathy -prevelance of complication depends on duration of DZ. 50% of DM overall; 80% with DM>15 ye

Most common symmetric neuropathy involving distal sensory and motor nerves
-numbness, loss of pain sensation, difficult with balance, paresthesia and dysesthesias-positive sx=painful sensation , diffuse vascular injury major cause of morbidity in diabetes

Foot ankle skin ulcers

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55
Q

Segmental demyelination of DM neuropathy

A

Small myelinated fibers and unmyelinated fibers

Decrease axons ; degeneration myelin sheaths and regeneration of axonal clusters

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56
Q

Dysfunction of autonomic nervous system DM neuropathy

A

20-40% with distal sensorimotor neuropathy-postural hypotension, incomplete emptying of bladder and sexual dysfunction

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57
Q

Hypothyroidism

A

Compression mononeuropathies, distal symm predominantly sensory polyneuropathy

Rarely hyperthyroidism associated with syndrome resembling Gillian barre

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58
Q

Other toxic neuropathies

A

B12, b1, b6, folate, vit E , pb, PB, ethanol

59
Q

Paraneoplastic neuropathies

A

Sensorimotor neuropathy =small cell lung cancer

60
Q

POEMS

A

B celll neoplasm

Polyneuropathy, organometallic, endocrinopathy, monoclonal gammopathy and skin changes

Deposition of paraprotein between noncompacter myelin lamellae

61
Q

Traumatic neuroma

A

Regenerates slowly ; discontinuity complicates

-small bundles of axons randomly oriented, but surrounded by organized layers of schwann cells, fibroblasts and perineural cells

62
Q

Compression/entrapment neuropathy

A

Carpal tunnel

Saturday night palsy

Morton neuroma

63
Q

Carpel tunnel

A

Median nerve

64
Q

Saturday night palsy

A

Radial nerve upper arm

65
Q

Morton neuroma

A

Metatarsalgia, histological lesion=perineural fibrosis

-interdigital nerve and intermetatarsal sites W>M

66
Q

Charcot marie toothe

A

Most common inherited PN

67
Q

Familial polyneuropathies

A

Amyloid deposition within the peripheral nerves

68
Q

Metabolic DOs

A

Leukodystrophies, porphyria, refsum

69
Q

C diff botox

A

Blocks release ach

70
Q

Curare

A

Muscle relaxant blocks archer->flaccid paralysis

71
Q

Myasthenia gravis

A

Circulation autoantibodies to achr

Thymoma thymic hyperplasia

Muscle specific receptor tyrosine kinase

72
Q

Myasthenia gravis

A

Autoantibodies achr

Dismissed response after stimulation

Extraocualr msucles-drooping eyes double vision

More focal involvement if ab to muscle specific tyrosine kinase

73
Q

Treat myasthenia gravis

A

Plasmapheresis and immunosuppressive

Thymectomy

74
Q

Lambert Eaton

A

Ab presynaptic ca channel

Scc

Repetitive stimulation increases msucle response

75
Q

Damage to skeletal muscle

A

Small pool of stem cells=satellite cells

76
Q

Neurogenic disease skeletal muscle

A

Fiber type grouping, group atrophy

77
Q

Dermatomyositis

A

Perifascular atropy

78
Q

Prolonged corticosteroid and disuse

A

Type II fiber atrophy sparing type I fibers

79
Q

Segmental myofiber degeneration and regeneration

A

Release into blood CK-muscle damage marker

Regenerating fibers rich in RNA=basophils staining, enlarged nuclei and prominent nucleoli randomly distributed in cytoplasm

80
Q

Myofiber hypertrophy

A

Adaptation to exercise of chronic Myopathies conditions

81
Q

Cytoplasmic inclusion

A

Vacuoles, aggregates of proteins or clusters of organelles

82
Q

Mitochondrial Myopathies

A

Myopathies associated with inborn errors in metabolism

83
Q

Arthrogryposis floppy baby

A

Congenital myopathies

84
Q

Malignant hyperpyrexia (hypermetabolic state, triggered by anesthetics

A

Ion channel myopathies

85
Q

3 types of inflammatory myopathies

A

Infectious, noninfectious inflammatory, and systemic inflammatory

86
Q

Non infectious inflammatory myopathies

A

Dermatomyositis, polymyositis, inclusion body myositis

87
Q

Dermatomyositis

A

Lilac of heliotrope discoloration of upper eyelids with periorbital edema -telangiectasia, nail fold, eyelids and gums

Grottos lesion

Proximal msucles first dysphagia, interstitial lung disease, cardiac involvement

Anti Mi2, antijo1, anti-155/-140

88
Q

Skin changes dermatomyositis

A

Increase risk visceral cancer
-malignancy
Perifascular atrophy

89
Q

Polymyositis

A

Myalgia and weakness, no cutaneous

Symmetrical proximal muscle involvment

Lacks cutaneous

Endomysial
Random distribution of affected fibers

90
Q

Inclusion body myositis

A

Slowly progressice

Distal msucles esp extensors of knee and flexors of wrist ; asymmetric

Dysphagia

Rimmed vacuoles

91
Q

Treat inflammatory myopathies

A

Corticosteroids

Immunosuppressive drugs

Responds poorly to immunosuppression-inclusion body

92
Q

Statins

A

Toxic myopathies: atorvastatin, simvastatin, pravastatin

93
Q

Chloroquine and hydrocychloroquine

A

Toxic myopathies

94
Q

Original antimalarial

For symmetric autoimmune

Drug induced lysosomal storage myopathy
Slowly progressive muscle weakness TYPE 1 fiber

A

Ok

95
Q

ICU myopathy

A

Aka myosin deficit myopathy

Critical illness with corticosteroid tax profound weakness affects clincila course

96
Q

Thrytoxic myopathy

A

Acute or chronic proximal muscle weakness

Exophthalmos opthamopledia: swelling of eyelids, edema conjunctiva and diplopia

Hypothyroidism: cramping or aching decrease movement slowed reflexes

97
Q

Alcohol

A

Toxic myopathy

Binge drinking may produce rhabdomyolisis, myoglobinuria and renal failrue acute myalgia

98
Q

Congenital myopathies

A

Presents in infancy with muscle defects that tend to by static or to improve over time

99
Q

Muscular dystrophies

A

Progressice msucle damage sx after infancy

Congenital dystrophies end to Present in infancy, often associated with developmental abnormalities of CNS as well as progressive msucle damage-conditions with defects in extracellular matrix surrounding myofibers

Conditions with abnormalities in receipts for extracellular matrix

100
Q

Muscular dystrophies

A

X linked muscular dystrophy

Duchenne
Becker

101
Q

Muscular dystrophy

A

Xp21 dystrophin

Duchess: before 5

Becker late in childhood adolescence; nearly normal lifespan cardiac dz

102
Q

DMD vs BMD

A

No dystrophin

Decrease amount

103
Q

Pseudohypertrophy

A

Enlargement of muscles of lower leg associated with weakness; increase bulk due to increase size of msucle fibers initially; end increase fat and connective tissue

104
Q

DMD histo

A

Ok

105
Q

Myotonic dystrophy

A

Kyoto is : sustained involuntary contractio of a group of msucles; can be elicited by percussion on thenar eminence

Stiff

CTG repeat expansion in DMPK

Gait, atrophy facial muscles-ptosis, hatchet face frontal balding, cataracts, cardiomyopathy

106
Q

Emery Dreyfus muscular dystrophy

A

Mutations in genes that encode nuclear lamina proteins

Triad: slowly progressive humeroperoneal weakness, cardiomyopathy associated with conduction defects, and early contractures of Achilles’ tendon, spine, and elbows

X linked form EMD1 and AD EMD2

107
Q

Carnitine palmitoyltransferase II def

A

Most common disease of lipid of glycogen metabolism ; episodic muscle damage with exercise and fasting

108
Q

McArdle Dz

A

Myophosphorylase def: glycogen storage disease; episodic muscle damage with exercise

109
Q

POMPE dz

A

Generalized glycogenesis of infancy

Acid Maltese defiency: mild in adult with respiratory and trunk muscles

110
Q

Mitochondrial myopathies

A

Weakness, increase serum CK or rhabdomloisis
Extraocular msucle involvement commonly seen

Chronic progressive external ophthalmoplegia common in mitochondria

111
Q

Lever

A

Point mutation mitochondrial myopathy

112
Q

Leigh and barth

A

Mitochondrial myopathies genes encoded by nuclear DNA

Leigh-subacute necrotizing encephalopathy

Barth-infantile x linked cardioskeletal myopathy

113
Q

Kearny’s sayer syndrome

A

Deletions or duplications of mtDNA
Weakness of extraocular muscles ophthalmoplegia

Mitochondrial myopathy

114
Q

Ragged red fibers

A

Mitochondrial

Red trichrome stain

115
Q

Spinal muscular atrophy

A

Spneuropathic disorder , loss of motor neurons, muscle weakness and atrophy

116
Q

Infants with neurologic or neuromuscular disease may present with

A

Generalized hypotonia floppy infant

117
Q

Infant hypotonic

A

Neuropathies: spinal muscular atrophy is a prosthetic example

118
Q

Spinal muscular atrophy (infantile motor neuron disease

A

Destroys anterior horn cells

SMN1 chromosome 5

We Ig hoffman in type 1: onset at birth, floppy Abby death by 3
Muscle weakness of the truncated and extremity muscles initially followed by chewing swallowing and breathing difficulties

119
Q

Histo SMA

A

Round atrophic fibers and innervated hypertrophied fibers

120
Q

RYR1 mutation: malignant hyperthermia

A

Hypermetabolic state(tachycardia, tachypnea, msucle spasm, hyperpyrexia)

Can be triggered by anesthetics; halogenated inhalation agents
Anesthetic triggers increase efflux of ca from SR reticulum->tetany and excessive heat production

121
Q

Central core disease AD

A

RYR1 gene 19q13.1

Malignant hyperthermia

122
Q

NF1 brain tumor syndromes

A

Lesion of optic pathway glioma

Cafe at lair spots

Lisch nodules

17q11 NF1 neurofibromin

123
Q

NF2

A

Bl acoustic schwannomas
NF2 plaque
22q12

Merlin protein

124
Q

Retinoblastoma

A

Pineoblastoma (trilateral retinoblastoma)

Leukokoria

13q14, rb1

125
Q

Von nipple Linda’s

A

Hemangioblastomas of the cerebellum spine

Retinal angiomatosis
3p25

126
Q

Tuberous sclerosis

A

Subependymal giant cell astrocytoma

Ash leaf spots

Retinal astrocytoma

9q34
TSC1

Hamartin

127
Q

Nevoid basal cell carcinoma gorlin syndrome

A

Medulloblastoma
Basal cell carcinoma
9q22.3
APC

128
Q

Lhermitte duclos/crowded

A

Dysplastic ganglioctoma of the cerebellum

Facial trichilemmoma

10q23.3 cowden
TPEN

129
Q

Li fraumeni

A

Malignant glioma

17q between exon5
P53

130
Q

Cowden

A

AD
10q23
PTEN
Lipid phosphatase/benign follicular appendage tumors l internal adenocarcinoma (often breast or endometrial)

131
Q

NF1

A

AD 17q11 NF1/neurofibromin

Negatitively regulates RAS signaling/neurofibromas

132
Q

NF2

A

AD 22q12
NF2/merlin

Integrates cytoskeleton signaling/neurofibromas and acoustic neuromas

133
Q

Tuberous sclerosis

A

AD
9q34 16p13

TSC1/hamartin TSC2/tbuerin

Work together in a complex that negatively regulates mTOR angiofibroma/mental retardation

134
Q

Tuberous sclerosis

A

Hamartomas: cortical tubers, subependymal nodules

Shagreen patches
Ash leaf patches

135
Q

VHL

A

AD polycythemia

Hemangioblastomas of cerebellum and retina
Cysts of pancreas, liver and kidneys, RCC, pheochromocytoma

136
Q

NF1

A

Neurofibromas of peripheral nerves
Optic nerve gliomas
Lisch nodules : pigmented nodules of the iris
Cafe at lair spots: hyperpigmented cutaneous nodules

137
Q

NF2

A

Less common

Bl schwannomas in CNVIII cerebellopontine angle increased meningiomas ependymomas

138
Q

Peripheral nerve sheath tumors

A

Schwannomas, neurofibromas MPNST , neurofibromatosis

139
Q

Schwannoma

A

Cerebellar pontine angle

Acoustic neuroma=CNVIII, tinnitus and hearing loss

NF2: loss of Merlin

Antoni A-spindle cells, verocay bodies-laisading nuclei around nuclear free zones
Antoni B -hypocellualr, myxoid extracellular matrix

140
Q

Neurofibroma

A

NF1
Superficial cutaneous neurofibromas
Diffuse neurofibromas
Bag of worms malignant transformation to MPNST possible -plexiform neurofibroma

141
Q

MPNST

A

85% HG tumors 1/2 arise for NF1 pts

SubtypeL triton tumorL rhabdomyoblastic differentiation

142
Q

VHL

A

Regulate cell cycle control, mRNA stability and activity of HIF

Hemangioblastomas of CNS and retinae
CCRC
Pheochromocytoma

Paraneoplastic

143
Q

NF1

A

Suppressor expression of neurofibromin

Neurofibriomin is homologous of proteins activating GTP are dependent p21 Ras. It’s importance lies int he tumor suppressing by regulating the activation fo ras dependent signal

Cafe at lait, neurofibromas, axillary or inguinal freckling, optic glioma, retinal lisch nodules

Pathology (36 decks)

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