Term
anatomist : leg
lay person : __________ |
|
Definition
|
|
Term
| all 4 muscles of front leg are innervated by the _____________ nerve |
|
Definition
|
|
Term
| large nerve that travels down the back of the thigh |
|
Definition
|
|
Term
| Behind the knee, the sciatic splits into the _________ nerve (which travels down the back of the knee & top of the leg) & the ___________ nerve (which travels down the lateral side of the knee & top of the leg) |
|
Definition
tibial
common fibular (peroneal) |
|
|
Term
| nerve that travels down the back of the knee before splitting near the top of the back of the leg |
|
Definition
|
|
Term
| What nerve arises behind the knee & travels down the middle of the leg, lateral to the tibia? |
|
Definition
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|
Term
| All 4 muscles in the front of the leg contribute to what action? |
|
Definition
|
|
Term
| What 2 muscles on the front of the leg invert the foot (turn the sole inward)? |
|
Definition
tibialis anterior
extensor hallucis longus |
|
|
Term
| What 2 muscles on the front of the leg evert the foot (turn the sole of the foot outward)? |
|
Definition
extensor digitorum longus
fibularis tertius |
|
|
Term
| What muscle of the front of the leg extends all toes but the big toe? |
|
Definition
| extensor digitorum longus |
|
|
Term
| What muscle in the front of the leg extends the big toe? |
|
Definition
|
|
Term
| What muscle originates on the upper two-thirds of the lateral surface of the tibia and inserts on the ankle & top of the medial foot (big toe side)? |
|
Definition
|
|
Term
| What nerve innervates the tibialis anterior? |
|
Definition
|
|
Term
| What does the tibialis anterior do? |
|
Definition
| dorsiflexes and inverts foot (turn sole inward) |
|
|
Term
| What muscle originates on the top of anterior of tibia and inserts on the top of the toes? |
|
Definition
| extensor digitorum longus |
|
|
Term
| What nerve innervates the extensor digitorum longus? |
|
Definition
|
|
Term
| What 3 things does the extensor digitorum longus do? |
|
Definition
extends toes
dorsiflexes foot
everts foot (turns sole outward) |
|
|
Term
| What originates on the lower third of anterior of tibia and inserts on the top of foot on little-toe side? |
|
Definition
|
|
Term
| What nerve innervates the fibularis tertius? |
|
Definition
|
|
Term
| What does the fibularis tertius do? |
|
Definition
| dorsiflexes & everts foot (turns sole outward) |
|
|
Term
| What muscle originates on the medial of anterior of fibula and inserts on the top of big toe |
|
Definition
|
|
Term
|
Definition
|
|
Term
| What nerve innervates the extensor hallucis longus? |
|
Definition
|
|
Term
| The bodies of upper motor neurons (aka primary motor neurons) are in ______________ & the axons end in __________________ |
|
Definition
the primary motor cortex
the spinal cord |
|
|
Term
| Lower motor neurons synapse on upper motor neurons in the ________________ of the spinal cord, and the axons end at _______________ |
|
Definition
ventral (anterior) horn
the neuromuscular junction |
|
|
Term
| An upper motor neuron lesion has abrupt onset, symptoms contralateral to the lesion, “pure motor” losses (no other pathways affected). This problems most likely occured in the __________ or _________ (large divisions of the brain). |
|
Definition
cerebrum
pons
(lower would have neighborhood signs or ipsilateral lesion with other pathways affected b/c they're all bundled so close together) |
|
|
Term
| A person shows ipsilateral “neighborhood” and contralateral “long tract” signs. Where is the lesion? |
|
Definition
|
|
Term
| loss of the ability to execute or carry out learned purposeful movements, despite having the desire and the physical ability to perform the movements |
|
Definition
|
|
Term
| Does Primary Lateral Sclerosis show upper motor neuron signs, or lower motor neuron? |
|
Definition
upper motor neuron
(remember: PRIMARY Lateral Sclerosis affects PRIMARY motor neurons--not spinal or peripheral motor neurons) |
|
|
Term
| Does Amyotrophic Lateral Sclerosis (ALS, “Lou Gehrig’s Disease”) show upper motor neuron signs, or lower motor? |
|
Definition
|
|
Term
| Do Upper Motor Neuron lesions show increase or decrease of extereoreceptive reflexes? |
|
Definition
decrease or loss
(exteroreceptive reflexes are reflexes dependent on stimulation of sensory receptors of skin, such as abdominal reflex or cremaster reflex) |
|
|
Term
| How do you stimulate the abdominal reflex, & what does it do? |
|
Definition
| if you scrape the skin tangential to or toward the belly button, the abdominal muscles in that quadrant of the abdomen will contract, causing belly button to jerk toward the stimulated quadrant |
|
|
Term
| How do you stimulate the cremaster reflex, & what does it do? |
|
Definition
| if you gently stroke the inner part of the upper thigh, the cremaster muscle on that side pulls the scrotum up |
|
|
Term
| The plantar reflex is induced by gently stroking the bottom of a foot that has been _________ |
|
Definition
| everted (soles turned out) |
|
|
Term
| Do upper motor neuron lesions or lower motor neuron lesions directly cause muscle atrophy? |
|
Definition
|
|
Term
| In the upper extremity, hypertonia due to upper motor neuron lesion causes _________ (extensors /flexors) to act more strongly than _____________ , while in lower limbs ______________ (extensors /flexors ) are stronger than _____________ |
|
Definition
upper limbs: flexors stronger than extensors
lower limbs: extensors stronger than flexors |
|
|
Term
| lack of sensory information from a joint (neuropathy) leads to misuse, which leads to injury |
|
Definition
|
|
Term
| In the medullary pyramids, most of the corticospinal tract decussates and forms the __________, while a small portion does not decussate & forms the ______________ |
|
Definition
lateral corticospinal tract
anterior corticospinal tract |
|
|
Term
What travels:
from motor cortices
thru internal capsule
thru cerebral peduncles in the pons
thru anterior of medulla into medullary pyramids, where most of it decussates
and down through the spine? |
|
Definition
|
|
Term
| a collection of axons that travel between the cerebral cortex of the brain and the spinal cord; mostly contains motor axons |
|
Definition
|
|
Term
| a white matter pathway whose CNS portion that starts in the cerebral cortex and ends in the brainstem |
|
Definition
| Corticobulbar tract (the “bulb” is an old word for medulla oblongata) |
|
|
Term
| muscles of the face, head, neck, & throat (including tongue, pharynx and larynx) are innervated by the ________________ tract |
|
Definition
|
|
Term
| What 2 tracts are the pyramidal tracts and why are they called that? |
|
Definition
corticospinal tract
corticobulbar tract
they're called pyramidal tracts because MOST nerves in those tracts decussate in the pyramid in the inferior medulla oblongata |
|
|
Term
|
Definition
| anterior inferior medulla oblongata |
|
|
Term
| A lesion to the corticospinal tract in the superior medulla oblongata will cause _____________ (contralateral/ ipsilateral/ both contralateral & ipsilateral) losses |
|
Definition
| contralateral b/c the corticospinal tract decussates in the pyramids in the inferior medulla (mostly; a small party, which will become the anterior corticospinal tract, decussates in the spine just b/4 it exits at each vertebrae; but it's still below the superior medulla) |
|
|
Term
| majority of corticospinal tract; decussates at pyramidal decussation |
|
Definition
| lateral corticospinal tract |
|
|
Term
| What is clasp knife spasticity and what sort of lesion produces it? |
|
Definition
when a joint is moved briskly, resistance occurs initially and then fades (like the opening of a pocketknife blade)
seen in upper motor neuron lesions |
|
|
Term
| What is clonus & what sort of lesion produces it? |
|
Definition
rhythmic contractions of muscles in response to sudden, passive movements (seen in wrist, knee, & ankle)
produced by upper motor neuron lesions |
|
|
Term
| A lesion of the lateral corticospinal tract below pyramidal decussation causes _______ (ipsilateral/ contralateral) deficits of what type? |
|
Definition
|
|
Term
| A lesion of the anterior corticospinal tract below pyramidal decussation causes _______ (ipsilateral/ contralateral) deficits of what type? |
|
Definition
contralateral motor deficits
anterior/ventral corticospinal tract fibers decussate at spinal levels in the ventral white commissure
(lateral corticospinal tract, the largest part of the corticospinal tract, decussates in the pyramids of the medulla oblongata) |
|
|
Term
| Where does the ventral/anterior corticospinal tract decussate? |
|
Definition
| in the ventral white commissure of the spine, at the level of the spinal root in which that portion of the tract leaves the spine |
|
|
Term
| Why are the contralateral motor deficits produced by a lesion of the anterior/ventral corticospinal tract relatively mild? |
|
Definition
| becuase the Ventral/Anterior corticospinal tract carries a small number of nerves (most motor nerves are in the lateral corticospinal tract) |
|
|
Term
Hereditary disease caused by bilateral degeneration of corticospinal tracts
Results in gradual development of spastic weakness of the legs with increased difficulty in walking |
|
Definition
| Hereditary spastic paraplegia or diplegia |
|
|
Term
| Is Poliomyelitis a UMN or LMN disease? |
|
Definition
|
|
Term
| Is Progressive Infantile Muscular Atrophy (Werdnig-Hoffman disease)a UMN or LMN disease? |
|
Definition
|
|
Term
| Is Kugelberge-Welander Disease a UMN or LMN disease? |
|
Definition
| LMN (it's aka Juvenile Hereditary LMN Disease) |
|
|
Term
| Kugelberge-Welander Disease (Juvenile Hereditary LMN Disease) appears at what age,and affects what muscles first? |
|
Definition
age 3-20
Affects large girdle muscles first; then distal muscles |
|
|
Term
| What age group & gender are most likely to get Amyotrpohic Lateral Sclerosis (ALS)? |
|
Definition
Usually occurs 50-70 years
Twice as many men as women |
|
|
Term
| Is Amyotrophic Lateral Sclerosis (ALS) a UMN or LMN disease? |
|
Definition
| both (either may dominate the clinical picture) |
|
|
Term
| paralysis of muscles innervated by cotricobulbar pathway due to neuropathy of lower motor neurons (CN IX, X, XII) |
|
Definition
|
|
Term
| paralysis of muscles innervated by corticobulbar pathway due to lesions of corticobulbar pathway in the pyramidal tract (that is, UMNs) |
|
Definition
|
|
Term
| progressive muscle weakness in the voluntary muscles due to loss of UMN innervations |
|
Definition
| Primary lateral sclerosis |
|
|
Term
| UMN and LMN lesion that often presents with difficulty chewing, swallowing, and speaking (speech may be slurred initially), as well as inappropriate emotional outbursts |
|
Definition
| Amyotrpohic Lateral Sclerosis (ALS, “Lou Gehrig Desease”) |
|
|
Term
| a small tract from red nucleus in the midbrain to the spine that facilitates the flexion and inhibits the extension in the upper extremities |
|
Definition
|
|
Term
| What portions of the spine are not affected by ALS? |
|
Definition
| dorsal horns & dorsal columns (where sensory neurons are) |
|
|
Term
| Sensory neurons are in the ________ portion of the spine |
|
Definition
|
|
Term
| ALS initially affects the ______________ of the spinal cord, then affects the _____________ columns |
|
Definition
ventral gray horns
lateral
(that is, the lateral corticospinal tract and lateral rubrospinal tract) |
|
|
Term
| Name the 2 lateral columns of the spinal cord? |
|
Definition
lateral corticospinal tract
lateral rubrospinal tract |
|
|
Term
What illness does the following describe?
Infection -->immune response -->cross-reacts with certain gangliosides in peripheral nerve -->inflammation -->peripheral demyelination -->impairment of action |
|
Definition
|
|
Term
| Guillain-Barre Syndrome is often preceded by an infection by _____________ or ___________________ |
|
Definition
Campylobacter jejuni
cytomegalovirus |
|
|
Term
| a component of the cell plasma membrane that modulates cell signal transduction events and serves as distinguishing surface markers for cell recognition & cell-to-cell communicaiton |
|
Definition
ganglioside
(gangliosides on neurons are the targets for auto-immune attacks during Guillain-Barre Syndrome) |
|
|
Term
| What would nerve conduction studies show in Guillain-Barre Syndrome? |
|
Definition
| slowing of nerve conduction as a consequence of demyelination |
|
|
Term
| Guillain-Barre syndrome is characterized by gradually increasing __________ (upper motor neuron/lower motor neuron) symptoms & sensory loss over a period of weeks. |
|
Definition
|
|
Term
| Occlusion of the ______________ artery could cause Medial Medullary Syndrome (of Dejerine). |
|
Definition
|
|
Term
| Medial Medullary Syndrome (of Dejerine) includes lesions to the ______________ tract, ___________ tract, and ________________ nerve roots. |
|
Definition
corticospinal tract
medial leminiscus tract
hypoglossal nerve roots |
|
|
Term
| Describe the 3 main symptoms of Medial Medullary Syndrome (of Dejerine). |
|
Definition
contralateral upper motor neuron hemiparesis in trunk & extremities
contralateral loss of proprioception, fine touch, and vibratory sense in trunk & extremities
Ipsilateral lower motor neuron paralysis of the tongue (flaccid paralysis with atrophy) |
|
|
Term
| a rare type of stroke of the posterior cerebral artery, with a range of neurological symptoms affecting the midbrain, cerebellum and other related structures. May involve the oculomotor nerve (CN III), red nucleus, superior cerebellar peduncle, & sometimes the medial leminiscus |
|
Definition
|
|
Term
| one of two or more forms of the DNA sequence of a particular gene |
|
Definition
|
|
Term
| the specific location of a gene or DNA sequence on a chromosome |
|
Definition
|
|
Term
|
Definition
| when one gene affects multiple traits |
|
|
Term
|
Definition
| when one locus has multiple alleles in a population |
|
|
Term
Which of the following are heterozygous, homozygous, and hemizygous?
AA
aa
Aa
aA
A
a |
|
Definition
AA--homozygous
aa--homozygous
Aa--heterozygous
aA--heterozygous
A--hemizygous
a--hemizygous |
|
|
Term
|
Definition
only have one allele (A or a, not Aa or AA or any other 2-allele combo)
in humans, males are hemizygous for most X-linked genes (not hemizygous for the small subset of X-linked genes that are also on Y chromosome) |
|
|
Term
|
Definition
one locus can be mutated in more than one ways in order to cause the same disorder
(different from polymorphism, when one locus has multiple alleles in a population--but those different alleles may have different phenotypes) |
|
|
Term
|
Definition
| mutations at more than one locus can cause the same disorder |
|
|
Term
What kind of inheritance has the following traits?
about equal in males & females
common in family
never skips a generation |
|
Definition
|
|
Term
What kind of inheritance has the following traits?
about equal males & females
can skip a generation (unaffected gives birth to affected)
rare unless inbreeding |
|
Definition
|
|
Term
What kind of inheritance has the following traits?
Common in family
Daughters of affected Dad all have
No sons of affected Dad have
Never skips a generation |
|
Definition
|
|
Term
What kind of inheritance has the following traits?
Much more common in males than females
No sons of affected Dad have
Grandsons of affected grandpa (via daughter) may have, or great-grandsons etc. through the female line
Affected sons can be somewhat common in family |
|
Definition
| X-linked recessive inheritance |
|
|
Term
What kind of inheritance has the following traits?
Passed from father to son
Never skips a generation (if dad is affected, all sons are affected) |
|
Definition
|
|
Term
What kind of inheritance has the following traits?
If mom is affected, all of her kids are affected
If dad is affected, none of his kids are affected |
|
Definition
| Mitochondrial inheritance |
|
|
Term
| a person in a pedigree who isn’t affected, but who must be a carrier based on analysis of their family tree |
|
Definition
|
|
Term
proportion of individuals carrying a particular variation of a gene (allele or genotype) that also express an associated trait (phenotype)
(ex., 25% of people with genotype A have disease B) |
|
Definition
|
|
Term
variations of a phenotype in individuals carrying a particular genotype
(ex., This person has more of the disease, and this person has less) |
|
Definition
|
|
Term
| when a gene is expressed differently depending on whether it’s inherited from the mom or the dad |
|
Definition
|
|
Term
| If a child inherits the Prader-Willi/Angelman's genotype from his mom, he will get ___________ |
|
Definition
|
|
Term
| If a child inherits the Prader-Willi/Angelman's genotype from his dad, he will get ___________ |
|
Definition
|
|
Term
| If a girl with Prader-Willi syndrome has a baby, the baby is at increased risk for ___________ |
|
Definition
|
|
Term
|
Definition
| the presence of two populations of cells with different genotypes in one individual who has developed from a single fertilized egg. May result from mutation during development of embryo or chimerism |
|
|
Term
|
Definition
when some gametes carry a mutation, but others don’t; usually caused by mutation that occurred in an early stem cell that gave rise to all or part of the gonadal tissue.
Can cause only some children to be affected, even for a dominant disease |
|
|
Term
|
Definition
| a new mutation--could cause a new appearance of an inherited disorder in a family with no history of the disorder |
|
|
Term
| A man's brother has an autosomal recessive disorder. The man is unaffected. What are the odds that the man is a carrier? |
|
Definition
2/3
Mom is Aa & Dad is Aa, so the possible phenotypes for kids are AA, Aa, aA, or aa
But we know that the man not affected (aa), so there are 2 ways he could be a carrier (Aa, aA), and one that he could not (AA). |
|
|
Term
| Inheritence pattern of achondroplasia |
|
Definition
|
|
Term
Achondroplasia is autosomal dominant. It shows up in a family with no history of it.
What are the odds that another child will have achondroplasia? |
|
Definition
Very low (~0%). (Because the first time was due to a de novo mutation)
Could be >0 if it’s due to germline mosaicism, but that is much less likely than de novo mutation, so it’s still ~0%. |
|
|
Term
| What are intrinsic muscles of hand? |
|
Definition
| muscles that have proximal and distal attachments in hands |
|
|
Term
| The superficial muscles on the thumb side (lateral side) of the palm causes the bump called the _________________ |
|
Definition
|
|
Term
| The superficial muscles on the pinkie side (medial side) of the palm causes the _________________ |
|
Definition
|
|
Term
| The thenar group of muscles is chiefly responsible for: |
|
Definition
|
|
Term
| The abductor pollicis and flexor pollicis brevis is part of the ___________ group of muscles, which is responsible for opposition of the thumb. |
|
Definition
|
|
Term
| The abductor digiti minimi & flexor digiti minimi brevis are part of the ____________ group of muscles. |
|
Definition
|
|
Term
| What muscle attaches to the trapezoid & scaphoid bones of wrist (toward the pinkie side of wrist) & inserts on the lateral side of first metacarpal (outside of thumb hand bone) |
|
Definition
|
|
Term
|
Definition
| hand bone (the finger bones that are within the palm area) |
|
|
Term
| What does the opponens pollicis do? |
|
Definition
| opposes the thumb (pulls it medially & rotates it medially) |
|
|
Term
| What nerve innervates the opponens pollicis? |
|
Definition
| recurrent branch of median nerve |
|
|
Term
| What intrinsic muscle of the hand abducts the thumb? |
|
Definition
|
|
Term
| What do the 2 heads of the flexor pollicis brevis do? |
|
Definition
one head flexes thumb
other abducts thumb to lateral side (straight out) |
|
|
Term
| What does the adductor pollicis do? |
|
Definition
|
|
Term
| What does the abductor digiti minimi do? |
|
Definition
|
|
Term
| What does the flexor digiti minimi brevis do? |
|
Definition
|
|
Term
| What does the opponens digiti minimus do? |
|
Definition
| opposes pinkie (touches it to thumb) |
|
|
Term
| small muscles that lie between the metacarpals within the palm |
|
Definition
|
|
Term
| What do the palmar interosseus muscles do? |
|
Definition
|
|
Term
| What does the adductor pollicis muscle do? |
|
Definition
|
|
Term
| All interosseus muscles are innervated by a deep branch of the _______ nerve |
|
Definition
|
|
Term
| What are extrinsic muscles of hand? |
|
Definition
| muscles that act on hand, but have their bellies in the forearm |
|
|
Term
| What muscle originates on medial epicondyle of humerus (medial elbow) and inserts on the aponeurosis of the palm? |
|
Definition
|
|
Term
| What does the palmaris longus do? |
|
Definition
|
|
Term
| What nerve innervates the palmaris longus? |
|
Definition
|
|
Term
|
Definition
| (apo “to become” + neuron “sinew”)——a thickening of the palmar fascia in the middle of the palm; palmar longus tendon attaches on one side |
|
|
Term
| What does the abductor hallucis do? |
|
Definition
| abducts & flexes the big toe |
|
|
Term
| What nerve innervates most of the muscles on the sole of the foot? |
|
Definition
medial plantar nerve
(the larger of the two terminal divisions of the tibial nerve)
|
|
|
Term
| What does the abductor digiti minimi do? |
|
Definition
|
|
Term
| Behind the knee, _________ nerve branches into ______ & _________ |
|
Definition
sciatic
tibial
peroneal (common fibular) |
|
|
Term
| Behind the ankle, the ______ nerve branches into the ______________(larger) and ____________ (smaller), both of which travel down the sole of the foot |
|
Definition
tibial
medial plantar nerve
lateral plantar nerve |
|
|
Term
| innervates abductor hallucis, flexor digitorum brevis, medial lumbricals, and flexor hallucis brevis |
|
Definition
medial plantar nerve
(the larger of the two terminal divisions of the tibial nerve) |
|
|
Term
| innervates the following muscles in the foot: abductor digiti minimi, lateral 3 lumbricals, the 3 plantar interossei, the 4 dorsal interossei |
|
Definition
|
|
Term
| halfway down the foot (on sole side of foot), the lateral plantar nerve branches into ______________ and ______________ |
|
Definition
deep branch of lateral plantar nerve
superficial branch of lateral plantar nerve |
|
|
Term
| What muscle does the deep branch of the lateral plantar nerve innervate? |
|
Definition
|
|
Term
| What muscle does the superficial branch of the lateral plantar nerve innervate? |
|
Definition
| flexor digiti minimi brevis |
|
|
Term
| thickened fascia over middle of foot (in a triangle from heel to toes) |
|
Definition
| plantar aponeurosis (apo “to become” + neuron “sinew”) |
|
|
Term
Explain this mnemonic:
Old Opie Occasionally Tries Trigonometry And Feels Very Gloomy, Vague, And Hypoactive |
|
Definition
I--Olfactor
II--Optic
III--Oculomotor
IV--Trochelar
V--Trigeminal
VI--Abducens
VII--Facial
VIII--Vestibulocochlear
IX--Glossopharyngeal
X--Vagus
XI--Accessory
XII--hypoglossal |
|
|
Term
| What does the olfactory nerve (CN I) do? |
|
Definition
|
|
Term
| What does the Optic nerve (CN II) do? |
|
Definition
|
|
Term
| What 3 cranial nerves move the eye? |
|
Definition
III--oculomotor
IV--trochlear
VI--abducens |
|
|
Term
| What cranial nerve contributes to head sensation & jaw movement? |
|
Definition
|
|
Term
| What cranial nerve contributes to facial muscles & taste? |
|
Definition
|
|
Term
| What cranial nerve contributes to hearing & balance? |
|
Definition
|
|
Term
| What nerve contributes to Cutaneous sensation (including pain) to the posterior 1/3 of the tongue; taste; sensation in pharynx? |
|
Definition
|
|
Term
| What cranial nerve contributes to sensory and motor innervation pharynx; massive output to glands and smooth muscles of body? |
|
Definition
|
|
Term
| What cranial nerve innervates the trapezius and sternocleidomastoid? |
|
Definition
|
|
Term
| What cranial nerve innervates the tongue muscles? |
|
Definition
|
|
Term
|
Definition
|
|
Term
| Anterior cranial fossa fractures can damage CN I, which can lead to: |
|
Definition
| loss of ability to smell (anosmia) |
|
|
Term
|
Definition
|
|
Term
|
Definition
| defect in visual field (patch of blindness or complete blindness) |
|
|
Term
| What are the 4 quadrants of the visual field of an eye? |
|
Definition
upper nasal (in)
lower nasal
upper temporal (out)
lower temporal |
|
|
Term
| How is the image projected onto the retina different than the real world being seen? |
|
Definition
| it upside down & backwards |
|
|
Term
| The nasal fibers transmit visual information from the ___________ field. |
|
Definition
|
|
Term
| The temporal fibers transmit visual information from the ___________ field. |
|
Definition
|
|
Term
| Where do the nasal fibers decussate? |
|
Definition
|
|
Term
| Where do the temporal fibers of the eye decussate? |
|
Definition
|
|
Term
| a defect in visual field that affects ~1/2 of the visual field (either nasal half or temporal half) |
|
Definition
|
|
Term
|
Definition
affects the nasal half of both eyes’ field of vision, or the temporal half of both eyes’ field of vision
(so you either can see in the middle, or you can't see on the outside) |
|
|
Term
|
Definition
| affecting the left half of both eyes’ field of vision, or the right half of both eyes’ |
|
|
Term
| If the optic nerve is completely destroyed what do you lose? |
|
Definition
all vision in that eye (ipsilateral blindness)
(optic nerve is what leaves the eye b/4 it encounters the optic chiasm) |
|
|
Term
| located at the bottom of the brain immediately below the hypothalamus |
|
Definition
|
|
Term
| A lesion in the optic chiasm causes: |
|
Definition
loss of temporal fields of vision for both eyes (a type of heteronymous hemianopsia)
b/c nasal fibers of both eyes (which carry temporal field information) decussate in the optic chiasm) |
|
|
Term
| What kind of vision loss is caused by damage to the optic tract after the optic chiasm |
|
Definition
Contralateral homonymous hemianopsia (so if lesion is on left, you lose right-field vision of both eyes)
The decussated nasal fibers (carry temporal field info) and non-decussated temporal fibers (carry nasal field info) have joined back up, so lose ipsilateral nasal field & contralateral temporal field |
|
|
Term
| After the optic chiasm, the optic tract carries what info? |
|
Definition
| contralateral temporal field (nasal fibers) & ipsilateral nasal field (temporal fibers) |
|
|
Term
| After passing through the ______________ in the thalamus, the optic tract splits into 2 radiations. |
|
Definition
| lateral geniculate nucleus |
|
|
Term
| the optic radiation carries information from the UPPER part of the ipsilateral nasal field & contralateral temporal field |
|
Definition
|
|
Term
| the optic radiation that carries information from the LOWER part of the ipsilateral nasal & contralateral termporal field |
|
Definition
|
|
Term
| Bilateral macular cortex damage causes |
|
Definition
| biateral macular scotomas |
|
|
Term
| What type of hypersensitivity is an allergic reaction provoked by re-exposure to a specific type of antigen? |
|
Definition
|
|
Term
| What type of hypersensitivity consists of antibodies produced by the immune response bind to antigens on the patient's own cell surfaces? |
|
Definition
|
|
Term
| What type of hypersensitivity is caused by incompletely cleared immune complexes? |
|
Definition
|
|
Term
| What type of hypersensitivity is delayed, cell-mediated, and primarily against intracellular antigens? |
|
Definition
|
|
Term
| What is immune privileged tissue? |
|
Definition
| tissue in which the immune response is much less than in most tissues (but not non-existant) |
|
|
Term
| Why are the brain, eye, fetus, and testes immune privileged? |
|
Definition
brain & eye because inflammation in those could be very bad
fetus is foreign & needs to be
sperm are very immunogenic |
|
|
Term
| In the brain, astrocytes produce _________, an anti-inflammatory cytokine |
|
Definition
TGF-beta
(transforming growth factor beta) |
|
|
Term
| In the brain, astrocytes produce TGF-beta, an anti-inflammatory cytokine, and __________ are also anti-inflammatory |
|
Definition
|
|
Term
| What kind of lymphocytes can pass the Blood-Brain Barrier? |
|
Definition
| effector T cells (that is, activated helper T cells & cytotoxic T cells) |
|
|
Term
| macrophages of the CNS, which present antigen to T cells |
|
Definition
|
|
Term
| multifactorial inheritance |
|
Definition
| when more than one gene is involved with or without the presence of environmental triggers |
|
|
Term
| HLA-DR2 alleles of class II MHC molecule, are a risk factor for _______________ |
|
Definition
|
|
Term
| Some polymorphisms of _________, which is in the alpha chain of _________, are risk factors for multiple sclerosis |
|
Definition
CD25
IL-2R (IL-2 Receptor) |
|
|
Term
| IL-2 is especially important for the development and maintenance of ______ T cells! |
|
Definition
|
|
Term
| Multiple sclerosis is mostly caused by type __________ hypersensitivity against antigens in ___________, but there may also be some type _______ Hypersensitivity mediated by antibodies from B cells |
|
Definition
IV (cell-mediated)
myelin
II
(no one is sure why) |
|
|
Term
|
Definition
| pathogens present an epitope that is similar to self-epitope; cross-activates autoreactive T cells |
|
|
Term
|
Definition
| activation of T cells specific for an antigen X during an immune response against antigen Y |
|
|
Term
| CD4+ TH1 produce _________, the main activator of macrophages. |
|
Definition
|
|
Term
Activated CD4+ TH1 cells produce the following cytokines. What do those cytokines do?
IFN-gamma
IL-2
Tumor Necrosis Factor alpha (TNF-alpha) |
|
Definition
IFN-gamma—activates macrophages
IL-2—promotes T cell proliferation
Tumor Necrosis Factor alpha (TNF-alpha)—stimulates acute phase inflammation (a local increase in concentration of TNF will cause the cardinal signs of Inflammation to occur: heat, swelling, redness, and pain.)
Macrophages produce more TNF-alpha than T cells |
|
|
Term
| Activated CD4+ TH17 cells produce IL-17, which: |
|
Definition
| promotes inflammation (along with TNF-alpha) |
|
|
Term
| All activated effector T cells express _________ that allow them to cross the BBB |
|
Definition
|
|
Term
| What 2 integrins do T cells produce, and what do they bind to? |
|
Definition
LFA-1 (binds ICAM-1 on endothelium)
VLA-4 (binds VCAM-1 on endothelium) |
|
|
Term
Explain this mnemonic with regard to integrins:
V to V, L to I—L & I are both tall & thin) |
|
Definition
VLA-4 (binds VCAM-1 on endothelium)
LFA-1 (binds ICAM-1 on endothelium) |
|
|
Term
| In multiple sclerosis, activated leukocytes & their injurious products cause: |
|
Definition
demyelination
(we're not sure exactly how) |
|
|
Term
| What happens to the IgG/albumin ratio in the CSF in multiple sclerosis? |
|
Definition
| goes up (IgG goes up; albumin stays the same) |
|
|
Term
| If you do protein electrophoresis of CSF of a patient with multiple sclerosis, 90% will show ___________________ |
|
Definition
oligoclonal bands
(• Each of the two to five oligoclonal bands seen by protein electrophoresis represent proteins (or protein fragments) secreted by plasma cells, although why exactly these bands are present, and which proteins these bands represent is not yet clear) |
|
|
Term
| What do white spots on a gadolinium-enhanced MRI indicate? |
|
Definition
inflammation in the brain (often seen in Multiple Sclerosis, MS)
gadolinium is an MRI contrast agent that cannot cross the BBB; spots of accumulated gadolinium indicate a breakdown in the BBB due to active inflammation |
|
|
Term
| If you take a gadolinium-enhanced MRI of someone with multiple sclerosis, what will you see and why? |
|
Definition
white spots
spots of accumulated gadolinium indicate a breakdown in the BBB due to active inflammation; symptoms of multiple sclerosis is caused by demyelination of nerves by active inflammatory response against myelin |
|
|
Term
| What is glatiramir acetate, and why do you give to someone with MS? |
|
Definition
A synthetic mixture of random combinations of the 4 most common amino acids in myelin basic protein (MBP)
Decreases immune response against myelin. No one is sure why it works, but it may incompletely bind to anti-meylin T cells and send them into anergy. |
|
|
Term
| Why wouldn't you give glatiramir acetate to someone who didn't have MS? |
|
Definition
Glatiramir acetate is a synthetic mixture of random combinations of the 4 most common amino acids in myelin basic protein (MBP)
Wouldn’t want to give to people without MS, because it could cause bystander activation (similar to self antigen; activates against self) or molecular mimicry (identical to self antigen; activates against self) |
|
|
Term
| Why would you give IFN-beta to someone with MS? |
|
Definition
| Promotes shedding of adhesion molecules (so T cells have a tougher time getting into the CNS) |
|
|
Term
| Why would you give fingolimond (causes retention of lymphocytes in lymph nodes) to someone with Multiple Sclerosis? |
|
Definition
| Causes retention of lymphocytes in lymph nodes so they can’t go to CNS & cause damage |
|
|
Term
| Why would you give mitoxantrone, which prevents DNA unwinding, to someone with MS? |
|
Definition
| Prohibits proliferation of immune cells |
|
|
Term
| Why would you give someone with Multiple Sclerosis Alemtuzumab (a monoclonal antibody against VLA-4)? |
|
Definition
VLA-4 is a T cell integrin
Reduces migration of lymphocytes across BBB |
|
|
Term
| Where do viruses typically enter nerves? |
|
Definition
At synaptic boutons (esp. at neuromuscular junctions)
Myelinated parts aren’t very susceptible (tough to get thru myelin)
Synaptic boutons (esp. at neuromuscular junctions) are much more susceptible; have proteins that viruses can use to enter |
|
|
Term
| an immunoglobulin-like receptor that’s present on many cells, including immune cells & Peyer's patches (lymphoid tissue in intestine), which polio virus uses to enter those cells |
|
Definition
|
|
Term
| What do Polio, West Nile, Human lymphoTropic Leukemia Virus (HTLV-1) have in common? |
|
Definition
| they're all viruses that cause weakness or paralysis |
|
|
Term
| What are polio virus's family & genus? |
|
Definition
Family: Picornaviridae (small RNA viruses)
Genus: Enterovirus |
|
|
Term
| genomic structure of polio virus |
|
Definition
|
|
Term
| Is polio virus enveloped or non-enveloped? |
|
Definition
|
|
Term
| Where does poliovirus replicate? |
|
Definition
| in the gut (oropharynx & intestine) |
|
|
Term
| What virulence feature does polio have that helps it survive in the gut? |
|
Definition
|
|
Term
| Does polio cause UMN or LMN lesions? |
|
Definition
|
|
Term
| Polio mostly kills nerves in __________________ |
|
Definition
| anterior (ventral) horns of the spinal cord |
|
|
Term
| How does the polio virus kill motor neurons? |
|
Definition
| virus produces protease, which cleaves cell’s elF-4G protein, which hijacks the ribosome & prevents it from making neuron proteins |
|
|
Term
| Polio presents as _________ (asymmetrical/symmetrical) __________ (flaccid, spastic) paralysis ______ (with/without) pain. |
|
Definition
asymmetrical
flaccid
with pain |
|
|
Term
| Describe abortive poliomyelitis |
|
Definition
2-3 days of fever, headache, sore throat, vomiting, and/or abdominal pain
No sequelae
Could be contagious for months after symptoms (still shedding virus in poo) |
|
|
Term
| Where does an infected person shed polio virus? |
|
Definition
|
|
Term
| Describe non-paralytic poliomyelitis. |
|
Definition
similar to abortive poliomyelitis (2-3 days of fever, headache, sore throat, vomiting, and/or abdominal pain)
plus 2-10 days of signs of meningeal irritation (stiff neck, aches & pains, headache) |
|
|
Term
| Spinal paralytic poliomyelitis shows symptoms like abortive poliomyelitis for 1 to 3 days (fever, headache, sore throat, vomiting, and/or abdominal pain), then no symptoms for 2-5 days, then signs of meningitis & inreased WBC in CSF. What happened during those 2-5 days? |
|
Definition
the virus entered the axons of motor nerve cells at the neuromuscular junction and has been traveling up the cell
(meanwhile, the immune system mopped up the viruses that were causing prodromal illness) |
|
|
Term
|
Definition
| increased white blood cell count |
|
|
Term
| What is different between the CSF of someone with poliomyelitis and the CSF of someone with Guillain-Barre syndrome? |
|
Definition
poliomyelitis-->slight to moderate protein elevation in CSF
Guillain-Barre (gee-YAHN, bah-RAY)-->lots of protein in CSF |
|
|
Term
| How are symptoms different in poliomyelitis and Guillain-Barre syndrome? |
|
Definition
polio is strictly motor deficits
Guillain-Barre also has sensory involvement |
|
|
Term
| A person has motor and sensory deficits. Could this be polio? Why or why not? |
|
Definition
| Probably not. Polio's cell-killing affects are very specific to MOTOR neurons; rarely cause sensory deficits. |
|
|
Term
| 2-5 days after prodromal symptoms disappear, what symptoms are initially seen in spinal paralytic poliomyelitis? |
|
Definition
muscle fasciculations
pain
weakness
(later, paralysis) |
|
|
Term
| Bulbar Paralytic Poliomyelitis is like poliomyelitis, but affects some additional parts of the CNS. What additional problems does that cause? |
|
Definition
paralysis extends to muscles of the pharynx, vocal cords and respiration
(additional parts are brain stem, particularly medulla, cranial nerves IX, glossopharyngeal, & X, Vagus, etc.) |
|
|
Term
| What is the route of transmission of polio? |
|
Definition
|
|
Term
| Where is polio still endemic? |
|
Definition
still endemic to parts of India & Africa (esp. Nigeria)
virtually unseen in the Americas, Europe, and the Western Pacific |
|
|
Term
| What are the advantages & disadvantages of the Salk Vaccine (Inactivated Polio Vaccine)? |
|
Definition
advantages: never causes polio
disadvantages: less effective immunization
(Induces humoral immunity, but no intestinal immunity in the gut--IgG B cells, but no IgA) |
|
|
Term
| What are the advantages & disadvntages of the Sabin vaccine (Oral Polio Vaccine)? |
|
Definition
Good: Induces humoral immunity and intestinal immunity
Given orally so it actually infects mucosa
IgG memory B cells and IgA in gut
Much better resistance
Bad: Rarely, the virus evolves back to being pathogenic (1/500k)
These pathogenic strains can cause outbreaks if mass immunization campaign doesn’t reach a large portion of the population |
|
|
Term
| The _________ vaccine contains formalin-killed polio virus, while the ____________ vaccine contains live, attenuated virus (each virus was derived from a wild virus, but carries up to 10 distinct mutations that make them non-pathogenic) |
|
Definition
Salk Vaccine, aka Inactivated Polio Vaccine (IPV)
Sabin Vaccine, aka Oral Polio Vaccine (OPV) |
|
|
Term
| What virus causes an illness that looks just like polio, but rarely causes permanent damage. |
|
Definition
|
|
Term
| a group of genetic disorders of muscle membrane proteins or support proteins |
|
Definition
|
|
Term
|
Definition
| pathological weakness of muscles |
|
|
Term
| What are the 2 types of dystrophinopathies (illnesses caused by problems with dystrophin protein)? |
|
Definition
Duchenne Muscular Dystrophy
Becker Muscular Dystrophy |
|
|
Term
| Most common type of muscular dystrophy. |
|
Definition
| Duchenne Muscular Dystrophy |
|
|
Term
| What is the most common cause of Duchenne's Muscular Dystrophy? How is this different than the cause Becker's Muscular Dystrophy? |
|
Definition
Duchenne's: large deletions of DMD gene-->almost no production of dystrophin protein
Becker's: Various mutations of DMD gene-->decrease in dystrophin amount and/or a shorter protein with only partial function |
|
|
Term
| Duchenne's Muscular Dystrophy appears in the age range _________ and has the following clinical features: |
|
Definition
0-5 years old
Progressive symmetrical muscular weakness
Proximal (pelvic/shoulder girdles) atrophy is greater than distal--> Gower's sign
Calf hypertrophy due to fat infiltration (muscle atrophies)
Usually don’t live beyond 25 years |
|
|
Term
| Becker's Muscular Dystrophy appears in the age range _________ and has the following clinical features: |
|
Definition
after age 5
Progressive symmetrical muscular weakness
Proximal (pelvic/shoulder girdles) atrophy is greater than distal--> Gower's sign
Calf hypertrophy due to fat infiltration (muscle atrophies)
Lifespan may be normal or moderately decreased
(same symptoms as Duchenne's, but slower progression) |
|
|
Term
Due to defect in genes encoding sarcolemma proteins
Most commonly due to defect in the sarcoglycan in the Dystrophin-glycoprotein complex (sarcoglycans)
Inheritance is Autosomal Dominant or Autosomal Recessive
Clinical features:
Weakness & wasting restricted to limb musculature (proximal greater than distal)
Can have various causes, age of onset, progression, & weakness distribution |
|
Definition
| Limb-girdle Muscular Dystrophy |
|
|
Term
| What causes Emery-Dreyfuss muscular dystrophy? |
|
Definition
Defect in nuclear proteins
(Emerin—a protein in the inner nuclear membrane
Or Lamin—a component of the nuclear lamina meshwork underlying the nuclear membrane)
both proteins maintain nuclear shape and regulate chromatin organization & gene expression
defect in nuclear proteins-->defects in stability & expression of muscle genes |
|
|
Term
| Clincal features of Emery-Dreyfuss Muscular Dystrophy |
|
Definition
early contractures of elbow flaxors, Achilles tendons, & limitation of neck flexion
slow progressive wasting & weakness
cardiac disease with conduction defects and arrhythmias
variable onset |
|
|
Term
| A terminally differentiated myoblast is a __________, or muscle cell |
|
Definition
|
|
Term
| a type of embryonic progenitor cell that gives rise to myocytes (aka a muscle cells). |
|
Definition
|
|
Term
What is the relationship between the following words?
muscle cell
myocyte
muscle fiber
myotube |
|
Definition
| they all mean the same thing |
|
|
Term
| Myocytes fuse together to form a ______________, a large cell-like structure filled with cytoplasm containing many nuclei |
|
Definition
|
|
Term
| delicate layer of reticular fibers & extracellular matrix (ECM) surrounding each muscle fiber (myocyte) |
|
Definition
|
|
Term
| Muscle fibers (myocytes) are bundled into ___________ surrounded by perimysium |
|
Definition
|
|
Term
| layer of reticular fibers & extracellular matrix (ECM) that surrounds muscle fascicles |
|
Definition
|
|
Term
| Muscle fascicles are bundled into ________ surrounded by epimysium |
|
Definition
|
|
Term
| dense connective tissue surrounding entire muscle |
|
Definition
|
|
Term
| What are the purposes of endomysium, perimysium, and epimysium |
|
Definition
The connective tissue coverings furnish support and protection for the delicate cells and allow them to withstand the forces of contraction.
The coverings also provide pathways for the passage of blood vessels and nerves |
|
|
Term
|
Definition
| the cell membrane of a muscle cell |
|
|
Term
|
Definition
| the cell membrane of a muscle cell |
|
|
Term
|
Definition
| cylindrical organelle within a myocyte consisting of long chains of sarcomeres |
|
|
Term
|
Definition
| a special type of smooth ER found in smooth & striated muscle; stores & pumps Ca2+ |
|
|
Term
| The sarcoplasmic reticulum stores & pumps what ion? |
|
Definition
|
|
Term
|
Definition
the basic unit of a muscle's cross-striated myofibril (cylindrical organelles found within muscle cells), with dark A bands and light I bands.
Sarcomeres are multi-protein complexes composed of:
thick filament primarily of myosin
thin filaments of primarily of actin, which also involves tropomyosin and troponin. Thin filaments & thick filaments are aka myofilaments |
|
|
Term
|
Definition
| Thin filaments & thick filaments in a sarcomere |
|
|
Term
| Sarcomeres are multi-protein complexes composed of a thick filament primarily of ________, and a thin filament primarily of _____, which also involves __________ and __________. |
|
Definition
myosin
actin
tropomyosin
troponin |
|
|
Term
Explain the following mnemonic:
acTIN is THIN |
|
Definition
| thin filaments are primarily composed of actin (thick filametns are primarily composed of myosin) |
|
|
Term
Explain the following mnemonic:
A bands are dArk
I bands are lIght
H bands are Heavily Dark |
|
Definition
A bands occurs where thick filament is; appear dark in light microscope images of sarcomere
I bands occur where thin filament doesn't overlap with thick filament; appear light in light microscopy
H bands occur where thin and thick filament overlaps with thin filament; appear very dark 7 thick in light microscopy |
|
|
Term
| What is a Z disc in a sarcomere? |
|
Definition
| a z disc is a disc in the sarcomere to which thin filaments are attached |
|
|
Term
What happens to the following when the a sarcomere contracts:
A bands
I bands
H zones |
|
Definition
A bands (thick filament) stay the same length (b/c thick filament doesn't get shorter)
I bands (only thin filament) get shorter (because overlap between thick and thin filaments increases)
H zone (only thick filament) gets shorter (b/c overlap increases) |
|
|
Term
| Where are the nuclei of myocytes? |
|
Definition
| they tend to be peripheral (stuck up next to the edge of the cell) |
|
|
Term
| How is the strength of a muscle contraction adjusted? |
|
Definition
by recruiting more or fewer muscle fibers
single muscle fiber contractions are “all or none” |
|
|
Term
| Explain the relationship between actin, myoson, tropomyosin, and troponin when the muscle is in a resting state. |
|
Definition
Thin filament—tropomyosin cock-blocks myosin from interacting with actin; troponin locks tropomyosin in place
Thick filament—myosin heads are energized by ATP; ready to stroke actin as soon as tropomyosin gets out of the way |
|
|
Term
| If you described the parts of a sarcomere as a horny but poor young man, horny young maiden, her greedy guardian, his generous uncle, a chastity belt, and money, which part would be which? |
|
Definition
Myosin is the lecherous bachelor
Actin is the young maiden
tropomyosin is a chastity belt
troponin is a greedy guardian
Ca2+ is money
DHP is the generous uncle
In resting state, troponin keeps myosin & actin from interacting by keeping tropomyosin in place, but when DHP releases Ca2+, it causes troponin to move the tropomyosin out of the way for a bit |
|
|
Term
| How is Ca2+ involved in neurotransmitter release? |
|
Definition
action potential travels from cell body in ventral horn of spinal cord to synaptic bouton
local depolarization in synaptic bouton opens voltage-gated Ca2+ channels
more cytosolic Ca2+ causes synaptic vesicles filled with neurotransmitter (such as acetylcholine) to fuse with pre-synaptic membrane and release neurotransmitter into synapse |
|
|
Term
| deep invaginations of sarcolemma (myocyte cell membrane) that allow action potentials to quickly penetrate interior of muscle cell |
|
Definition
|
|
Term
| When an action potential hits the sarcolemma, ______ (voltage-gated Ca2+ channels in sarcoplasmic reticulum) open and release Ca2+ |
|
Definition
|
|
Term
| In the sarcomere, Ca2+ binds to _________, which moves ___________ out of the way. |
|
Definition
| troponin moves tropomyosin |
|
|
Term
| What happens after a stroke is achieved in the sarcomere? |
|
Definition
Myosin releases actin. ATP cocks myosin for action again, but…
Ca2+ is pumped back into the sarcoplasmic reticulum
Troponin puts tropomyosin back
No more action for myosin unless the whole cascade happens again. |
|
|
Term
| How do curare & curariform drugs affect muscle contraction? |
|
Definition
paralyzes muscles
it is a competitive antagonist of AcetylCholine at nicotinic AcetylCholine receptors
Not enough stimulus to initiate an Action Potential in muscle-->no action potential-->no contraction
(curare itself is used by South American tribes to hunt monkeys; curariform drugs are used to paralyze patients during surgery) |
|
|
Term
| How does botulinum toxin affect muscle contraction? |
|
Definition
Decrease release of Acetylcholine from nerve terminals
Not enough stimulus to initiate an Action Potential in muscle
Flaccid paralysis |
|
|
Term
| Explain the relationship between the length of the sarcomere and the development of tension. |
|
Definition
Up to a certain point, the shorter the muscle before stimulation (pre-load), the more tension the muscle creates after stimulation b/c more overlap mean more myosin heads can interact with actin
Once the thin & thick filaments overlap as much as possible, tension remains the same for a a little bit.
As thick filaments begin to overlap, tension decreases precipitously
Normally, ligaments & bones keep muscles close to ideal range |
|
|
Term
|
Definition
| (“same tension”)—constant tension, variable shortening |
|
|
Term
|
Definition
| (“same length”)—variable tension, constant muscle length |
|
|
Term
Only use oxidative metabolism
High myoglobin content (so, dark red)
High capillary density (b/c need a lot of O2)
Many mitochondria (for lots of oxidative metabolism)
Low glyoclytic enzyme content (don’t need as much glycolysis b/c many more ATP per glucose)
Fatigue-resistant |
|
Definition
| Slow twitch muscle fibers |
|
|
Term
Use mostly anaerobic (glycolytic) metabolism
Large diameter
Low myoglobin content (so pale)
Low capillary density (don’t need as much oxygen)
Few mitochondria (not as much oxidative metabolism)
High glycolytic enzyme content (lots of anaerobic metabolism
Easily fatigued |
|
Definition
| Fast twitch muscle fibers |
|
|
Term
| An autosomal recessive group of 3 LMN disorders that begin in childhood |
|
Definition
| spinal muscular atrophy (SMA) |
|
|
Term
| What causes spinal muscular atrophy type 1 (SMA1), aka Werdnig Hoffman syndrome? |
|
Definition
deletion of Survival of Motor Neuron 1 gene (SMN1 gene)-->no SMN protein
It is thought that the SMN protein is important for
axonal transport
neuromuscular junction integrity |
|
|
Term
Most common form of spinal muscular atrophy
Onset is at birth or within first 4 months of life
Severe hypotonia (floppy baby)
Usually die within first 3 years of life |
|
Definition
|
|
Term
Autosomal recessive disorder in which many copies of SMN2 gene-->messed up SMN2 protein (more copies-->more severe)
Onset: 3-15 months
Usually die in childhood |
|
Definition
| Spinal Muscular Atrophy type 2 (SMA2) |
|
|
Term
| What type of Spinal Muscular Atrophy presents after 2 years of age, and sometimes allows its victims to live into adulthood |
|
Definition
|
|
Term
|
Definition
| progressive wasting of muscles |
|
|
Term
| What do the three words in Amyotrophic Lateral Sclerosis mean? |
|
Definition
Amyotrophy—atrophy of muscles
Lateral identifies the areas in a person's spinal cord where portions of the nerve cells that are affected are located
Sclerosis—hardening
as the lateral area of the spine degenerates in ALS, it leads to scarring or hardening n the region. |
|
|
Term
| There are 2 types of Amyotrophic Lateral Sclerosis (ALS). 5-10% are ____________, which is usually autosomal dominant. 90% are _______________, with unknown cause. |
|
Definition
|
|
Term
| What disease has lower motor neuron loss in spinal cord & brainstem and upper motor neuron loss in neurons that project to the corticospinal tracts? |
|
Definition
Amyotrophic Lateral Sclerosis (ALS)
(either kind of lesion may be more prominent) |
|
|
Term
| When does ALS usually manifest? |
|
Definition
|
|
Term
| Do patients with familial or sporadic ALS develop symptoms earlier? |
|
Definition
|
|
Term
|
Definition
asymmetric weakness of hands-->difficulty with fine motor tasks & dropping things
spasticity & cramping of arms & legs |
|
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Term
| As ALS progresses, what signs & symptoms manifest? |
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Definition
decreased muscle bulk & strength
fasciculations
progressive bulbar palsy (difficulty speaking & swallowing) |
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Term
|
Definition
|
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Term
|
Definition
| production of sound by vibrating vocal cords |
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Term
| ALS patients may get recurrent pulmonary infections if: |
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Definition
| respiratory muscles are involved |
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Term
| Which spinal roots have an abnormal appearance in ALS, and how are they strange? |
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Definition
Thin anterior spinal roots
(b/c anterior horn cells are dying) |
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Term
| A person stains the neurons of a person with ALS with Periodic Acid Schiff stain (PAS) and little dark-colored dots show up. What are these called, and what are they? |
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Definition
Bunina bodies
balled-up neuron proteins that appear to be remnants of autophagic vacuoles |
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Term
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Definition
an excess of astroglia in damaged areas of the CNS
(gliosis is common in Multiple Sclerosis & Multiple System Atrophy; may also be seen in Parkinson's, ALS, & Huntingtons) |
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Term
| Why do people with ALS have gliosis in the anterior horn of the spinal cord? |
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Definition
Gliosis is an excess of astroglia in damaged areas of the CNS
ALS kills cells whose bodies are in the anterior horn of the spinal cord (UMNs; also kills LMNs in brainstem & spinal cord) |
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Term
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Definition
| nuclear or cytoplasmic aggregates of stainable substances, usually proteins. |
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Term
|
Definition
| (“eating onself”)— degradation of a cell's own components by the cell, usually via lysosomes |
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Term
| ALS causes neurogenic atrophy. What's that? |
|
Definition
| atrophy of muscles due to loss of LMN innervation |
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Term
| In ALS, loss of UMNs leads to degeneration of ___________ tracts |
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Definition
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Term
| An x-linked disease in which a CAG (cystine adenine guanine) / polyglutamine repeat expansion in the androgen receptor causes loss of sensitivity to androgen and a toxic gain of function in lower motor neurons |
|
Definition
bulbospinal muscular atrophy (Kennedy’s syndrome)
(Note: CAG expansions cause Kennedy's syndrome & Huntington's chorea) |
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|
Term
adult onset
lower motor neuron degeneration involving the brainstem & spinal cord
distal limb amyotrophy (progressive muscle wasting)
bulbar signs: dysphagia (difficulty swallowing), tongue fasciculations, etc.
other findings
androgen insensitivity
gynecomastia
testicular atrophy
oligospermia (low concentration of sperm) |
|
Definition
| bulbospinal muscular atrophy (Kennedy’s syndrome) |
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Term
What does the spinothalamic tract do?
What does the corticospinal tract do? |
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Definition
spinothalamic--senses crude touch, pain & temp for most of body
corticospinal--provides motor innervation to most of body |
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Term
| ptosis, miosis, & anhydrosis |
|
Definition
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Term
Distinguish
Medial Medullary Syndrome of Dejerine
Lateral Medullary Syndrome of Wallenberg |
|
Definition
Medial Medullary Syndrome of Dejerine
Tongue deviates ipsilateral to lesion
Hemiparesis contralateral to lesion
Loss of discriminative touch, conscious proprioception, & vibration sense contralateral to lesion
Spinothalamic tracts (pain, temp, crude touch) and facial feeling are spared (trigeminal)
Lateral Medullary Syndrome of Wallenberg
Contralateral loss of pain & temp in body
Ipsilateral loss of pain & temp in face
ipsilateral Horner's syndrome (ptosis, miosis, & anhydrosis) |
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Term
Explain the following mnemonic:
Lateral Jack Horner
Runs through the town
Can’t feel pain or cold now
In his nightgown
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|
Definition
LATERAL Medullary Syndrome of Wallenberg
spares corticospinal (can run)
causes ipsilateral Horner syndrome
causes loss of pain & temp ipsilaterally in face, contralaterally in body |
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Term
Explain the following mnemonic
MEDea licks her children’s blood
She can’t feel their fine touch
She cannot move, she is so grieved
Her cold heart hurts so much |
|
Definition
Medial Medullary Syndrome of Dejerine
Tongue deviates ipsilateral to lesion
Hemiparesis contralateral to lesion
Loss of discriminative touch, conscious proprioception, & vibration sense contralateral to lesion
Spinothalamic tracts (pain, temp, crude touch) and facial feeling are spared (trigeminal) |
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Term
| Bunina bodies most likely indicate __________ |
|
Definition
amyotrophic lateral sclerosis (ALS)
Bunina bodies are balled-up neuron proteins that appear to be remnants of autophagic vacuoles |
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Term
| You do protein electrophoresis of a patient's CSF and see 2-5 oligoclonal bands. What might the patient have? |
|
Definition
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|
Term
| What 3 cranial nerves carry taste information? |
|
Definition
CN VII facial
CN IX glossopharyngeal
CN X vagus |
|
|
Term
Explain this mnemonic:
Baum sees below
Meyer sees the moon. |
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Definition
Baum's loop is the optic radiation of the optic tract that carries information from the LOWER part of the ipsilateral nasal & contralateral temporal field
Meyer's loop is the optic radiation of the optic tract that carries information from the UPPER part of the ipsilateral nasal & contralateral temporal field
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Term
| After passing through the lateral geniculate nucleus in the _________, the optic tract splits into 2 radiations. |
|
Definition
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|
Term
| What cytokine is important for maintenance of all T cells, but particularly for Tregs? |
|
Definition
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|
Term
| What is the function of the vagus nerve (CN X) above the shoulders? |
|
Definition
| provides sensory and motor innervation of the pharynx |
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