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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

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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

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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

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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

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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

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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

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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

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8
Q

Describe a shadow plaque in MS

A

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

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9
Q

What are the CSF findings in MS

A

Elevated protein
Pleocytosis
IgG increase
Oligoclonal IgG bands in immunoelectrophoresis

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10
Q

Describe Neuromyelitis optica

A

Synchronous BL optic neuritis with SC demyelination

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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.

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12
Q

Describe Acute Disseminated Encephalomyelitis (ADEM)

A

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

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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

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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

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15
Q

Describe Central Pontine Myelosis

A

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

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16
Q

Describe the symptomology of Central Pontine Myelosis

A 
Acute paralysis
Dysphagia
Dysarthria 
Diplopia 
Coma

From Hyponatremia over correction!

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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

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18
Q

Describe neuritic (senile) plaques in alzheimer’s disease

A

focal spherical collections of dilated tortuous neuritic processes around amyloid core

(congo red)

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19
Q

Describe diffuse plaques in Alzheimer’s disease

A

No amyloid core!

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

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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)

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21
Q

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

A

of tangles

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22
Q

What is granulovacuolar degeneration

A

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

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23
Q

What are hirano bodies

A

Elongated glassy eosinophilic bodies in hippocampal pyramidal cells.

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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

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25
Q

Describe progressive supranuclear palsy

A

Truncal rigidity with dysequilibrium & nuchal dystonia, abnormal speech, ocular disturbances

Males >females 5-7 decade

Fatal within 5-7 years of onset

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26
Q

Describe the histology of Progressive supranuclear palsy

A

Globose neurofibrillary tangles: 4R tau straight filaments

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27
Q

Describe vascular dementia

A

if due to vasculitis, improves with treatment

progressive cognitive disorders associated with vascular injury -> widespread areas of infarction

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28
Q

What areas are usually affected by strategic infarcts

A

Hippocampus
Dorsomedial Thalamus
Cingulate gyrus of frontal cortex

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29
Q

Describe the clinical syndrome of parkinsonism

A 
Diminished facial expression
Stooped posture 
Slowness of voluntary posture
Festinating gait 
Rigidity 
Pill rolling tremor
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30
Q

Describe parkinson disease

A

progressive L-dopa-responsive signs of parkinsonism
Autosomal Dominant a-synuclein 4q21

juvenile is AR parkin

31
Q

describe gross morphology and histology of parkinson disease

A

Pallor of substantia nigra

Lewy bodies - cytoplasmic, eosinophilic inclusions with a dense core

32
Q

Describe Multiple system atrophy

A

sporadic disorder characterized by cytoplasmic inclusions of a-synuclein

33
Q

Describe the three distinct neuroanatomic systems involved in Multiple System Atrophy

A 
Striatonigral circuit (parkinsonism)
Olivopontocerebellar circuit ( ataxia) 
ANS (autonomic dysfunction)
34
Q

Describe huntington disease

A

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
Q

What is anticipation and what is its relationship with HD

A

Repeat expansions during spermatogenesis leads to earlier presentation for offspring

36
Q

What are the main areas of atrophy in HD

A

Caudate Nucleus; Putamen later
Globus Pallidus
Frontal lobes

37
Q

What are the two spinocerebellar ataxias

A

Friedreich ataxia

Ataxia-telangiectasia

38
Q

Describe Friedreich ataxia

A

Autosomal recessive
GAA trinucleotide repeat 9q13 Frataxin protein

Onset 1st decade with gait ataxia then hand clumsiness

39
Q

What are associated comorbidities and causes of death in patients with friedreich ataxia

A

Cardiac arrhythmias and CHF
DM in 10%
COD: Intercurrent pulmonary infection & heart disease

40
Q

Describe Ataxia-Telangiectasia

A

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
Q

What gene is mutated in Ataxia Telangiectasia

A

ATM 11q22-q23

Very susceptible to X-ray

42
Q

Describe Amyotrophic lateral sclerosis

A

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
Q

What is the specific pathology of ALS

A

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
Q

What are the symptoms of ALS

A

Early: Asymmetric weakness of hands, dropping objects
Fasciculations
Progressive atrophy
Progressive bulbar palsy (difficulty speaking and swallowing

50% alive at 2 years

45
Q

Which degenerative diseases have Tau inclusions

A

Alzheimer’s
Frontotemporal lobar degeneration
Progressive Supranuclear palsy
Corticobasilar degeneration

46
Q

Which degenerative diseases have a-synuclein inclusions

A

Parkinson disease

Multiple system atrophy

47
Q

What are the protein inclusions in ALS

A

TDP-43

SOD-1(familial disease)

48
Q

Describle leukodystrophies

A

autosomal recessive (adrenoleukodystrophy is X-linked)

Involvement of white matter -> deterioration of motor skills, spasticity, hypotonia and ataxia

49
Q

Describe mitochondrial encephalomyopathies

A

Oxphos disorders

involve gray matter and skeletal muscle

50
Q

Describe tay-sachs

A

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
Q

Describe MELAS

A

Mitochondrial Encephalopathy
Lactic Acidosis
Stroke

tRNA mutations
Most common mitochondrial neuro syndrome

52
Q

what is the symptomatology of MELAS

A

Muscle involvement with lactic acidosis

Stroke episodes produce REVERSIBLE deficits that do not correspond to vascular territories

53
Q

Describe MERRF

A

Myoclonic Epilepsy
Ragged Red Fibers

tRNA mutations

54
Q

What is the symptomatology of MERRF

A

Myoclonus seizure disorder, w/ evidence of myopathy

Ataxia

55
Q

What is Kearn-Sayre Syndrome

A

Ophthalmoplegia Plus

Sporadic disorder often associated with a large mitochondrial DNA deletion/rearrangement

56
Q

What is the symptomatology of Kearn-Sayre syndrome

A

Cerebellar ataxia
Progressive external ophthalmoplegia
Pigmentary Retinopathy
Cardiac Conduction Defects

57
Q

Describe leigh syndrome (subacute necrotizing encephalopathy)

A

Presents in early childhood with

Lactic acidemia 
Arrest of psychomotor development 
Feeding problems 
seizures 
Hypotonia
58
Q

What are the neurologic effects of B12 deficiency

A

Numbness, tingling, slight ataxia

progress to spastic weakness

59
Q

describe histology of b12 deficiency

A

swelling of myelin layers -> vacuoles form at midthoracic level
both ascending and descending tracts degenerate

60
Q

What is the main difference between Wernicke and Korsakoff syndrome

A

Wernicke may reverse with thiamine while Korsakoff is largely irreversible with confabulation and memory disturbances

61
Q

What layers of the cortex are most effected with CO poisoning

A

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
Q

What is the main area affected by methanol and what is the toxic byproduct

A

Retina
Selective BL putaminal necrosis

Formate is toxic byproduct

63
Q

How does Graves Orbitopathy affect the eye

A

hyperthyroidism most common cause of uni/bilateral exophthalmos

Enlargement of the extraocular muscles; increase of glycosaminoglycans

64
Q

how does sarcoid affect the eye

A

systematic disease -> granulomatous UVeitis and sympathetic ophthalmia

  • -mutton fat (keratic ppt in anterior segment)
  • -Candle wax drippings
65
Q

What is the most common tumor of periocular skin

A

Basal cell carcinoma

  • younger patients
  • nodulo-ulcerative
66
Q

Who is most affected by squamous cell carcinoma

A

> 40 y/o asian women

2/3 upper eyelid, meibomian gland

67
Q

What is masquerade syndrome

A

unilateral keratoconjunctivitis unresponsive to therapy

68
Q

What is a pinguecula

A

thin epithelium (hyperplastic or dysplastic collagen)
basophilic degeneration
appears on sclera of the eye

69
Q

What is a pterygium

A

encroaches onto cornea in winglike fashion

70
Q

What is the most common primary intraocular tumor in adults

A

uveal melanoma

GNAQ & GNA11 in 85% of uveal melanomas

71
Q

Describe open-angle glaucoma

A

complete open access to trabecular meshwork, resistance to aqueous outflow

–primary most common form

72
Q

What are the risk factors for glaucoma

A

Age
Race (AA and hispanics)
Family Hx
DM

73
Q

What is the leading cause of blindness in the us

A

age related macular degeneration

74
Q

What is the most common symptom of retinoblastoma

A

Leukocoria (white pupillary reflex)

NMSP Exam 1 (17 decks)

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