Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

NMSP Exam 1 > Demyelinating/Degenerative/Genetic/Toxic/Eye > Flashcards

Demyelinating/Degenerative/Genetic/Toxic/Eye Flashcards

1
Q

Generally describe the characteristics of demyelinating diseases

A

Acquired
Damage myelin by preserve axon
–Immunological vs inherited

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Describe multiple sclerosis

A

Autoimmune demyelinating DO with neuro deficits separated by time and region of the body

Relapses decrease frequency but increase in intensity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are some common neurologic symptoms and signs of multiple sclerosis

A

Unilateral vision impairment: frequent initial symptom
10-50% Pts with optic neuritis develop MS
Ataxia and nystagmus
Motor and sensory impairment with spasticity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Describe the epidemiology of MS

A

Women 2X to men

There is a genetic component DR2 gene and IL-2 and IL-7 receptors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What would you see on gross pathology of someone with MS

A

multiple well circumscribed, slightly depressed lesions, irregularly shaped plaques around ventricles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

You see a histo slide of a patient with ongoing MS. On the slide you see an abundance of macrophages, perivascular inflammatory infiltrate at outer edge of plaque. Overall there is relative preservation of the axons. What type of plaque is observes

A

Active Plaque

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

You see a histo slide of a patient with ongoing MS. On the slide you see no inflammation. There is an absence of myelin, oligodendrocyte nuclei, and decreased axons. However astrocytosis and gliosis are prominent. What type of plaque is observed.

A

Inactive Plaque

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Describe a shadow plaque in MS

A

Thinned out myelin sheaths with partial & incomplete remyelination by surviving oligos. Not well circumscribed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the CSF findings in MS

A

Elevated protein
Pleocytosis
IgG increase
Oligoclonal IgG bands in immunoelectrophoresis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Describe Neuromyelitis optica

A

Synchronous BL optic neuritis with SC demyelination

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How does a spinal tap from a person with neuromyelitis optica compare to that of one from someone with bacterial meningitis

A

Elevated neutrophils with opening pressure but NO change in glucose.

*** could be good compare question.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Describe Acute Disseminated Encephalomyelitis (ADEM)

A

Perivenous Encephalomyelitis - diffuse monophasic demyelinating disease that follows either viral infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Describe the morphology of Acute Disseminated Encephalomyelitis (ADEM)

A

Grayish discoloration around white matter vessels. Myelin loss, axon preservation
Accumulation of lipid laden macrophages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Describe the key features of Acute Necrotizing Hemorrhagic Encephalomyelitis (ANHE)

A

Children and young adults
Fulminant syndrome of CNS demyelination
Recent URI
Fatal in most cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Describe Central Pontine Myelosis

A

Loss of myelin in symmetric pattern involving basis pontis and portions of the pontine tegmentum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Describe the symptomology of Central Pontine Myelosis

A 
Acute paralysis
Dysphagia
Dysarthria 
Diplopia 
Coma

From Hyponatremia over correction!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Can you make a true Alzheimer’s Dx while they patient is alive

A

nope need the brain

**seems like a putoff distinction but who knows

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Describe neuritic (senile) plaques in alzheimer’s disease

A

focal spherical collections of dilated tortuous neuritic processes around amyloid core

(congo red)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Describe diffuse plaques in Alzheimer’s disease

A

No amyloid core!

Early stage of plaque development
Down syndrome: early onset of AD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

To remember neurofibrillary tangles of specific neural cells use this mnemonic, or dont

A

Pyramidal cells - Flame (pyramid schemes go down in flames)
Round - Globus (globes are round)
Basophilic fibrillary structures - Bilschowsky stain (B with B)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Do # of tangles or # of plaques correlate better to degree of dementia

A

of tangles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is granulovacuolar degeneration

A

small clear intraneuronal cytoplasmic vacuoles which contain granules. Normal in aging but hella in AD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are hirano bodies

A

Elongated glassy eosinophilic bodies in hippocampal pyramidal cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Describe Pick Disease

A

Rare, early onset behavioral changes due to frontal lobe deterioration and language disturbances (temporal lobe)

Knife edge thin gyri of only the front 1/3 of superior temporal gyrus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Describe progressive supranuclear palsy
Truncal rigidity with dysequilibrium & nuchal dystonia, abnormal speech, ocular disturbances Males >females 5-7 decade Fatal within 5-7 years of onset
26
Describe the histology of Progressive supranuclear palsy
Globose neurofibrillary tangles: 4R tau straight filaments
27
Describe vascular dementia
if due to vasculitis, improves with treatment progressive cognitive disorders associated with vascular injury -> widespread areas of infarction
28
What areas are usually affected by strategic infarcts
Hippocampus Dorsomedial Thalamus Cingulate gyrus of frontal cortex
29
Describe the clinical syndrome of parkinsonism
``` Diminished facial expression Stooped posture Slowness of voluntary posture Festinating gait Rigidity Pill rolling tremor ```
30
Describe parkinson disease
progressive L-dopa-responsive signs of parkinsonism Autosomal Dominant a-synuclein 4q21 juvenile is AR parkin
31
describe gross morphology and histology of parkinson disease
Pallor of substantia nigra Lewy bodies - cytoplasmic, eosinophilic inclusions with a dense core
32
Describe Multiple system atrophy
sporadic disorder characterized by cytoplasmic inclusions of a-synuclein
33
Describe the three distinct neuroanatomic systems involved in Multiple System Atrophy
``` Striatonigral circuit (parkinsonism) Olivopontocerebellar circuit ( ataxia) ANS (autonomic dysfunction) ```
34
Describe huntington disease
Autosomal Dominant Movement disorder with dementia caused by polyglutamine trinucleotide repeats 4p16.3 CAG repeats (10-35 copies normal); age of onset determined by number of repeats
35
What is anticipation and what is its relationship with HD
Repeat expansions during spermatogenesis leads to earlier presentation for offspring
36
What are the main areas of atrophy in HD
Caudate Nucleus; Putamen later Globus Pallidus Frontal lobes
37
What are the two spinocerebellar ataxias
Friedreich ataxia Ataxia-telangiectasia
38
Describe Friedreich ataxia
Autosomal recessive GAA trinucleotide repeat 9q13 Frataxin protein Onset 1st decade with gait ataxia then hand clumsiness
39
What are associated comorbidities and causes of death in patients with friedreich ataxia
Cardiac arrhythmias and CHF DM in 10% COD: Intercurrent pulmonary infection & heart disease
40
Describe Ataxia-Telangiectasia
Autosomal recessive childhood onset with CNS, conjunctival and skin telangiectasias. Death in 2nd decade from Lymphoid neoplasms, gliomas, and carcinomas Immunodeficiency Failure to remove cells with DNA damage
41
What gene is mutated in Ataxia Telangiectasia
ATM 11q22-q23 Very susceptible to X-ray
42
Describe Amyotrophic lateral sclerosis
loss of lower motor neurons in SC and Brain stem and upper motor neurons that project into corticospinal tracts Presents in 5th decade or later SOD1 mutation chr 21
43
What is the specific pathology of ALS
Anterior roots of SC are thin with decrease anterior horn neurons Precentral gyrus may be atrophic Neurons contain bunina bodies (PAS+) Skeletal muscles undergo neurogenic atrophy
44
What are the symptoms of ALS
Early: Asymmetric weakness of hands, dropping objects Fasciculations Progressive atrophy Progressive bulbar palsy (difficulty speaking and swallowing 50% alive at 2 years
45
Which degenerative diseases have Tau inclusions
Alzheimer's Frontotemporal lobar degeneration Progressive Supranuclear palsy Corticobasilar degeneration
46
Which degenerative diseases have a-synuclein inclusions
Parkinson disease | Multiple system atrophy
47
What are the protein inclusions in ALS
TDP-43 | SOD-1(familial disease)
48
Describle leukodystrophies
autosomal recessive (adrenoleukodystrophy is X-linked) Involvement of white matter -> deterioration of motor skills, spasticity, hypotonia and ataxia
49
Describe mitochondrial encephalomyopathies
Oxphos disorders | involve gray matter and skeletal muscle
50
Describe tay-sachs
HEXA gene (hexosaminidase A) chr 15 GM2 Gangliosides never go away cherry red spots on maculae presents at 1 y/o dead 2-3 y/o
51
Describe MELAS
Mitochondrial Encephalopathy Lactic Acidosis Stroke tRNA mutations Most common mitochondrial neuro syndrome
52
what is the symptomatology of MELAS
Muscle involvement with lactic acidosis | Stroke episodes produce REVERSIBLE deficits that do not correspond to vascular territories
53
Describe MERRF
Myoclonic Epilepsy Ragged Red Fibers tRNA mutations
54
What is the symptomatology of MERRF
Myoclonus seizure disorder, w/ evidence of myopathy | Ataxia
55
What is Kearn-Sayre Syndrome
Ophthalmoplegia Plus Sporadic disorder often associated with a large mitochondrial DNA deletion/rearrangement
56
What is the symptomatology of Kearn-Sayre syndrome
Cerebellar ataxia Progressive external ophthalmoplegia Pigmentary Retinopathy Cardiac Conduction Defects
57
Describe leigh syndrome (subacute necrotizing encephalopathy)
Presents in early childhood with ``` Lactic acidemia Arrest of psychomotor development Feeding problems seizures Hypotonia ```
58
What are the neurologic effects of B12 deficiency
Numbness, tingling, slight ataxia | progress to spastic weakness
59
describe histology of b12 deficiency
swelling of myelin layers -> vacuoles form at midthoracic level both ascending and descending tracts degenerate
60
What is the main difference between Wernicke and Korsakoff syndrome
Wernicke may reverse with thiamine while Korsakoff is largely irreversible with confabulation and memory disturbances
61
What layers of the cortex are most effected with CO poisoning
Layers III and V of cerebral cortex, sommer's sector, purkinje cells Also have BL necrosis of globus pallidi Brain will be nice and red opposed to grey/tan
62
What is the main area affected by methanol and what is the toxic byproduct
Retina Selective BL putaminal necrosis Formate is toxic byproduct
63
How does Graves Orbitopathy affect the eye
hyperthyroidism most common cause of uni/bilateral exophthalmos Enlargement of the extraocular muscles; increase of glycosaminoglycans
64
how does sarcoid affect the eye
systematic disease -> granulomatous UVeitis and sympathetic ophthalmia - -mutton fat (keratic ppt in anterior segment) - -Candle wax drippings
65
What is the most common tumor of periocular skin
Basal cell carcinoma - younger patients - nodulo-ulcerative
66
Who is most affected by squamous cell carcinoma
>40 y/o asian women 2/3 upper eyelid, meibomian gland
67
What is masquerade syndrome
unilateral keratoconjunctivitis unresponsive to therapy
68
What is a pinguecula
thin epithelium (hyperplastic or dysplastic collagen) basophilic degeneration appears on sclera of the eye
69
What is a pterygium
encroaches onto cornea in winglike fashion
70
What is the most common primary intraocular tumor in adults
uveal melanoma GNAQ & GNA11 in 85% of uveal melanomas
71
Describe open-angle glaucoma
complete open access to trabecular meshwork, resistance to aqueous outflow --primary most common form
72
What are the risk factors for glaucoma
Age Race (AA and hispanics) Family Hx DM
73
What is the leading cause of blindness in the us
age related macular degeneration
74
What is the most common symptom of retinoblastoma
Leukocoria (white pupillary reflex)

NMSP Exam 1 (17 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions