Neuro_Exam 1_Neurodegenerative Disease

Question 1

neurodegenerative diseases are a result of ___ and this could be from ____

Answer 1

protein alterations, genetic factors, or environmental factors

Question 2

neurodegenerative diseases are distinguishable because the proteins from ______

Answer 2

inclusion bodies in cells

Question 3

when determining motor neuron diseases, ____ has both umn and lmn lesions, lmn alone_____ umn alone ____

Answer 3

ALS, spinal muscular atrophie (sma), primary lateral sclerosis

Question 4

umn lesion signs include:

Answer 4

spasticity, weakness, incresed reflex, babinski

Question 5

lmn lesion signs include:

Answer 5

hypotonia, weakness and atrophy

Question 6

als is degeneration of ____, progression to death is due to______

Answer 6

umn and lmn, respiratory failure

Question 7

als is called:

Answer 7

amyotrophic (atrophy, of skeletal muscle) lateral sclerosis=gliosis of lateral corticospinal tract, inclusion bodies in motor neurons

Question 8

in als, the picture includes myelin stain, and only the _____ is stained dark (showing a ____). There is also a smaller ____ than ____

Answer 8

dorsal column, decrease in myelin in the anterior and lateral column), ventral nerve root compared to the dorsal nerve root

Question 9

3 microscopic pathologies in als:

Answer 9

hyaline (pale circle), bunina bodies (in a tear drop shape), skein-like inclusion (brown stick)

Question 10

a similar degenerative disease to als is ______, that results from ____

Answer 10

cervical spondylosis, compression of the spinal nerve roots and spinal cord

Question 11

difference between cervical spondylosis is ____

Answer 11

it can be treated surgically and als can’t

Question 12

characteristics of cervical spondylosis that differentiate it from als ______

Answer 12

fasciculations in legs or tongue are more for ALS, neck pain favor spondylosis

Question 13

____ is an xlinked degenerative disease which is different than als bc it has _____ and is ____

Answer 13

no umn signs an x-linked spinobulbar muscular atrophy

Question 14

kennedy’s disease is characterised by ______

Answer 14

cag repeats in first exon of androgen receptor gene, no UMN signs!

Question 15

the _____ are a group of diseases that include lmn only, and have an autosomal recessive defect on ______

Answer 15

spinal muscular atrophies, chromosome 5

Question 16

_____ is a spinal muscular atrophie known as ____ and occurs in children _____, and includes ______, require ___ to be diagnosed

Answer 16

werdnig-Hoffman, less than 6 months, swallowing feeding, breathing, etc, most die before 2 years, muscle biopsy

Question 17

only _______ has both umn and lmn signs

Answer 17

ALS (amyotrophic lateral sclerosis)

Question 18

synucleinopathies involve _____ which form ______ which are _______

Answer 18

alpha-synculein, lewy bodies, inclusions containing alpha-synuclein

Question 19

2 most common synucleinopathies include___ and ____

Answer 19

pd and dementia with lewy bodies

Question 20

parkinsonism includes______ and include the syndrome consisting of:

Answer 20

not just parkinson’s, but the characteristics of pd, bradykinesia, rigidity, resting tremor, postural instability

Question 21

pd can be caused by _____, _____, _____ and _____

Answer 21

drugs, toxins, infectionns, neurodegenerative disease

Question 22

pd is a disease effecting the ____ and is characterized by a loss of ____ in the ____

Answer 22

midbrain, pigmentation, substantia nigra

Question 23

the substantia nigra in pd patients will include_____

Answer 23

lewy bodies (halo) positive for a-synuclein, also seen in dementia with lewy bodies

Question 24

_____ includes clinical features including parkinsonism, cerebellar dysfunction, autonoomic and wrinary dysfunction, corticospinal tract dysfunction (hyperflexia)

Answer 24

multiple system atrophy (msa)

Question 25

corticospinal dysfunction results in ______ and is caused by _____

Answer 25

hyperflexia,

Question 26

msa includes gross findings_

Answer 26

pallor of sub nigra, and locus ceruleus, discolored putamen, mild cortical atrophy, normal spinal cord

Question 27

msa microscope findings include:

Answer 27

neron and oligodendrogical loss, and gliosis; loss of myelination, glial cytoplasmic inclusions in oligodendroglia, neuronal cytoplasmic and nuclear inclusions

Question 28

_____ includes ____ protein and lewy bodies

Answer 28

pd, alpha syncuclein

Question 29

tauopathies are associated with ______ and are the ______ most common cause of pd with _____ more rapid than pd

Answer 29

psp or progressive supranuclear palsy, 2nd, cognitive decline

Question 30

psp shows ______ while pd shows _____

Answer 30

neuronal loss and gliosis, lewy bodies

Question 31

psp also shows _____ when staining (similar to ad) as well as ____

Answer 31

neurofibrillary tangles of tao proteins globose tangles

Question 32

______ includes asymmetrical rigidity, clumsiness “alien limb” phenomenon, aphasia and dementia

Answer 32

corticobasal degeneration

Question 33

______parkinsonism occurs _____, includes _____ and

Answer 33

post-encephalitic parkinsonism, after infection, reduced pigment in sn and lc,

Question 34

tau protein tangles occur in ______

Answer 34

corticobasal degeneration, post-encephalitic parkinsonism, progressive supranuclear palsy

Question 35

wilson’s disease is _____ and includes ______

Answer 35

aurosomal recessive, accumulation of copper in tissues (brain, eye, liver)

Question 36

the test that will show wilson’s disease is ____

Answer 36

decreased copper and ceruloplasmin (bc it is accumulating in the tissues)

Question 37

wilson’s disease can be detected via _____

Answer 37

copper accumulation in the eye, liver cirrhosis, basal gang necrosis and discoloration

Question 38

______ diseases include HD, spinocerebellar ataxia

Answer 38

triplet repeats

Question 39

HD includes ______ repeats, and results in _______

Answer 39

cag repeats, atrophy of caudate and putamen (striaum), frontal cortical atrophy, loss of dendrites

Question 40

____ is the most common form of progressive spinocerebellar ataxia

Answer 40

friedreich ataxia

Question 41

____ causes iron to

accumulate in the mitochondria, and includes _____

Answer 41

friedreich ataxia, atrophy of spinal cord

Question 42

Friedrich ataxia is due to ____ repeats (involved with the protein _____), ___, ____ and _____ have cag repeats

Answer 42

gaa, frotaxin, HD, spinocerebellar ataxia (sca) autosomal dominant form, kennedys disease