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AP LQ2 4th bi > PNS > Flashcards

PNS Flashcards

1
Q

The primary target of demyelinating neuropathies is

a. Axon
b. Neuron
c. Schwann cells with their myelin sheaths
d. Node of ranvier

A

C. Schwann cells with their myelin sheats

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2
Q

Infections associated with Guillain-Barre

a. Campylobacter jejuni
b. CMV
c. EBV
d. All of the above

A

D. All of the above

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3
Q

It is the most common chronic acquired inflammatory peripheral neuropathy, characterized by symmetrical mixed sensorimotor polyneuropathy that persists for 2 months or more.

A

Answer: Chronic Inflammatory Demyelinating Polyneuropathy

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4
Q

Most common cause of peripheral neuropathy, wherein there is ascending distal symmetric sensorimotor polyneuropathy

a. Diabetes
b. Varicella zoster virus
c. Lung disease

A

A. Diabetes

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5
Q

Hypercellular portion of schwannoma

a. Antoni A
b. Antoni B - hypocellular
c. Verocay bodies

A

A. Antoni A

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6
Q

Conditions of complete loss of neurofibromin

a. Neurofibromas
b. Schwannoma
c. MPNST
d. Neuroma

A

A. Neurofibromas

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7
Q

Neurofibroma which has the highest risk for MPNST transformation

Choices not recorded.

A

Plexiform Neurofibroma

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8
Q

These are small, well- delineated but unencapsulated nodular lesions that arise in the dermis and subcutaneous fat.

a. schwannoma
b. localized cutaneous neurofibroma
c. plexiform neurofibroma
d. diffuse neurofibroma

A

b. localized cutaneous neurofibroma

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9
Q

This is an autosomal dominant disorder resulting in a range of tumors, most commonly bilateral eighth nerve schwannomas, multiple meningiomas, and ependymomas of the spinal cord.

A

Neurofibromatosis Type 2

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10
Q

These tumors grow within and expand nerve fascicles, entrapping associated axons. The expanded, ropy thickening of multiple nerve fascicles results in what is sometimes referred to as a “bag of worms” appearance.

a. plexiform neurofibroma
b. schwannoma
c. diffuse neurofibroma
d. localized cutaneuos neurofibroma

A

a. plexiform neurofibroma

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11
Q

biopsy of muscle in patient with dermatomyositis will show

a. deposition of Ig
b. deposition of MAC
c. deposition of IgA
d. mononuclear infiltrates

A

d. mononuclear infiltrates

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12
Q

Antibodies showing a strong association with prominent Gottron papules and heliotrope rash in Dermatomyositis

a. Anti-P155/P140 antibodies
b. Anti-Mi2 antibodies
c. Anti-basement membrane antibody
d. Anti-Jo1 antibodies

A

b. Anti-Mi2 antibodies

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13
Q

Antibodies associated with interstitial lung disease, nonerosive arthritis, and a skin rash
described as “mechanic’s hands.”

a. Anti-Mi2 antibodies
b. Anti-Jo1 antibodies
c. Anti-basement membrane antibody
d. Anti-P155/P140 antibodies

A

b. Anti-Jo1 antibodies

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14
Q

most common inflammatory myopathy in children

a. dermatomyositis
b. polymyositis
c. inclusion body myositis
d. toxic myopathies

A

a. dermatomyositis

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15
Q

autoimmune Diseases often associated with distinct antibodies … several acute muscle
weakness .. significantly increased creatinine levels

a. dermatomyositis
b. Immune-Mediated Necrotizing Myopathy
c. Inclusion Body Myositis
d. Toxic myopathies

A

d. Toxic myopathies

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16
Q

Immune-Mediated Necrotizing Myopathy associated with antibodies against

a. Anti MI2 antibodies
b. HMG-CoA reductase
c. Anti Jo antibodies

A

b. HMG-CoA reductase

17
Q

In polymyositis, these cells are a prominent part of the inflammatory infiltrate in affected muscle, and it is hypothesized that these cells are mediators of tissue damage.

A

CD8+ cytotoxic T cells

18
Q

This is a disease of late adulthood that typically affects patients older than 50 years and is the most common inflammatory myopathy in patients older than age 65 years

a. Polymyositis
b. Immune-Mediated Necrotizing Myopathy
c. Inclusion Body Myositis
d. Toxic myopathies

A

c. Inclusion Body Myositis

19
Q

Most Common Etiology of Toxic Myopathies:

a. Chloroquine
b. Statin
c. Antibiotic
d. Steroids

A

b. Statin

20
Q

Drugs interfere with normal lysosomal function and can cause a drug-induced lysosomal storage myopathy that presents with slowly progressive muscle weakness. The muscle tissue shows myopathic changes including vacuolation that predominantly affects type I fibers.

a. Chloroquine
b. Statin
c. Antibiotic
d. Steroids

A

a. Chloroquine

21
Q

Drugs associated in ICU myopathy

a. Chloroquine
b. Statin
c. Antibiotic
d. Steroids

A

d. Steroids

22
Q

This is an inherited muscular disorder that tends to present in infancy and are often associated with developmental abnormalities of the CNS as well as progressive muscle damage.

a. Congenital myopathies
b. Muscular dystrophies
c. Congenital muscular dystrophies
d. Congenital myositis

A

c. Congenital muscular dystrophies

23
Q

This is a cause of loss-of-function mutations in the dystrophin gene on the X chromosome

A. Duchenne and Becker muscular dystrophy
B. Limb-Girdle Muscular Dystrophy
C. Emery-Dreifuss Muscular Dystrophy (EMD)
D. Myotonic Dystrophy

A

A. Duchenne and Becker muscular dystrophy

24
Q

Characteristic of Duchenne and Becker muscular dystrophy
A. Girls appear normal
B. Walking is not delayed
C. …..
D. clumsiness and inability to keep up with peers

A

D. clumsiness and inability to keep up with peers

25
Q

This is caused by mutations in genes that encode nuclear lamin proteins

A. Duchenne and Becker muscular dystrophy
B. Limb-Girdle Muscular Dystrophy
C. Emery-Dreifuss Muscular Dystrophy (EMD)
D. Myotonic Dystrophy

A

C. Emery-Dreifuss Muscular Dystrophy (EMD)

26
Q

Triad of Emery-Dreifuss Muscular Dystrophy (EMD) except:

a. slowly progressive humeroperoneal weakness
b. cardiomyopathy associated with conduction defects
c. early contractures of the Achilles tendon, spine, and elbows
d. clumsiness and inability to keep up with peers

A

d. clumsiness and inability to keep up with peers

27
Q

This is an autosomal dominant disorder associated with a characteristic pattern of muscle involvement that includes prominent weakness of facial muscles and muscles of the shoulder girdle.

a. Duchenne and Becker muscular dystrophy
b. Limb-Girdle Muscular Dystrophy
c. Emery-Dreifuss Muscular Dystrophy (EMD)
d. Fascioscapulohumeral Dystrophy

A

d. Fascioscapulohumeral Dystrophy

28
Q

The pathogenesis of facioscapulohumeral dystrophy is complex mechanism that culminate to the overall expression of gene called

a. DAX4
b. DUX4
c. BOX
d. TATA

A

b. DUX4

29
Q

This is the most common disorder of lipid metabolism to cause episodic muscle damage with exercise or fasting

a. Carnitine
b. Myophosphoryl deficiency
c. Acid maltase deficiency

A

a. Carnitine

30
Q

Mutations affecting this channel cause Andersen-Twail syndrome, an autosomal disorder associated with periodic paralysis, heart arrhythmias, and skeletal abnormalities.

A. SCN4A
B. CACNA1S
C. KCNJ2
D. RYR 1

A

C. KCNJ2

31
Q

Mutations that lead to congenital myopathy central core disease and to malignant hyperthermia

A. SCN4A
B. CACNA1S
C. KCNJ2
D. RYR 1

A

D. RYR 1

32
Q

Automimmue disease associated with autoantibodies directed against ACh receptors

A. MG
B. Thymoma

A

A. MG

33
Q

Organism associated with botulism

a. C. perfringens
b. C. tetani
c. C. Botulinum
d. E. maslinii

A

C. C. Botulinum

34
Q

This disorder most common autosomal recessive mode of inheritance are marked by varying degree of muscle weakness identified in genes and increased presynaptic, synaptic, post synaptic proteins. The most common of these are loss-of-function mutations in the gene encoding the ε-subunit of the ACh receptor.

a. MG
b. Congenital Myasthenic Syndrome
c. Lambert-Eaton Myasthenic Syndrome
d. Botulism

A

B. Congenital Myasthenic Syndrome

35
Q

Autoimmune disorder caused by antibodies that block ACh release by inhibiting presynaptic calcium channel

A. MG
B. Congenital Myasthenic Syndrome
C. Lambert-Eaton Myasthenic Syndrome
D. Botulism

A

C. Lambert-Eaton Myasthenic Syndrome

36
Q

True of type II muscle fiber

A. Low resistance to fatigue
B. High lipid content
C. High mitochondrial density
D. Aerobic respiration

A

A. Low resistance to fatigue

AP LQ2 4th bi (4 decks)

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