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(3) MS: Pediatric Conditions > S3_L1: Neuromuscular Diseases part 1 > Flashcards

S3_L1: Neuromuscular Diseases part 1 Flashcards

1
Q

What is the most commonly encountered genetic
pediatric neuromuscular condition?

A

Duchenne muscular dystrophy (DMD)

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2
Q

Second commonest form of CMT

A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

E. Charcot Marie Tooth Type X

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3
Q

Inheritance: autosomal recessive
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

D. Charcot Marie Tooth Type 4

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4
Q

Inheritance: autosomal dominant; referred to as the axonal CMT.
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

B. Charcot Marie Tooth Type 2

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5
Q

Also known as Dejerine-Sottas disease. Inheritance is either dominant or recessive.
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

C. Charcot Marie Tooth Type 3

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6
Q

More severe demyelinating neuropathy than CMT 1 (Most severe than type 1).
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

C. Charcot Marie Tooth Type 3

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7
Q

Most common form of CMT; Inherited autosomal dominant
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

A. Charcot Marie Tooth Type 1

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8
Q

Begins in infancy and infants have severe muscle atrophy, weakness
delayed motor skills development, sensory problems, and scoliosis (a side effect upon growth).
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

C. Charcot Marie Tooth Type 3

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9
Q

A rare type of CMT with a pathology of demyelinating and axonal degeneration and motor neuropathies.
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

D. Charcot Marie Tooth Type 4

Note: type 4 is the combination of type 1 & 2

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10
Q

Type of CMT that develop symptoms of leg weakness in childhood and by adolescence they may not be able to
walk.
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

D. Charcot Marie Tooth Type 4

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11
Q

Affects men more severely than
women. In males, it is present in the first decade of life, though their lifespan is usually normal.
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

E. Charcot Marie Tooth Type X

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12
Q

Accounts for 10 to 15% of all CMT cases. The mother is the carrier which affects males.
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

E. Charcot Marie Tooth Type X

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13
Q

X-linked, signs and symptoms are muscle weakness mostly in the feet, atrophy, and change in sensation
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

E. Charcot Marie Tooth Type X

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14
Q

Directly damages the peripheral nerve axon, resulting in axonal degeneration.
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

B. Charcot Marie Tooth Type 2

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15
Q

Onset of this type is between 10 to 20 years old
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

B. Charcot Marie Tooth Type 2

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16
Q

Characterized by a slow progression with manifestations of muscle weakness starting in the feet, atrophy/wasting, hammer toe, pes cavus, and a decrease in sensation. Individuals may have vocal cord or phrenic nerve involvement causing speech or breathing problems.
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

B. Charcot Marie Tooth Type 2

Note: vocal cord or phrenic nerve is not affected in type 1

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16
Q

Normal life expectancy and remain ambulatory with AD
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

A. Charcot Marie Tooth Type 1

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17
Q

Onset is the first or second decade of life and usually more severe form of CMT. Symptoms start in childhood starting with distal areas (i.e., most commonly in the feet; lower part of legs, hands, and forearm).
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

A. Charcot Marie Tooth Type 1

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18
Q

Pathology is damage of the myelin sheath, so it is also known as demyelinating CMT
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

A. Charcot Marie Tooth Type 1

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19
Q

Require orthotics for ankle support
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

A. Charcot Marie Tooth Type 1

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20
Q

Characterized by slow progression with manifestations of muscle weakness and atrophy, a loss of coordination, balance and proprioception, hammer toes, pes cavus, and paresthesia (change in sensation.) Individuals are slow runners in childhood.
A. Charcot Marie Tooth Type 1
B. Charcot Marie Tooth Type 2
C. Charcot Marie Tooth Type 3
D. Charcot Marie Tooth Type 4
E. Charcot Marie Tooth Type X

A

A. Charcot Marie Tooth Type 1

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21
Q

Charcot Marie Tooth Type 1 disease mostly affects which chromosome?

A

Chromosome 17

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22
Q

Individuals may present with involvement of facial and bulbar muscles, resulting in (B) facial weakness. Other signs and symptoms include the inability to swallow or speak, a loss of DTRs, and respiratory complications.
A. Regional GBS Syndrome
B. Acute Motor Axonal Neuropathy (AMAN)
C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)
D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

A

A. Regional GBS Syndrome

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23
Q

Miller Fisher variant of GBS; one body part is affected and this type is associated with problems in the
head/face.
A. Regional GBS Syndrome
B. Acute Motor Axonal Neuropathy (AMAN)
C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)
D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

A

A. Regional GBS Syndrome

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24
Q

Historically known as the Chinese paralytic syndrome
A. Regional GBS Syndrome
B. Acute Motor Axonal Neuropathy (AMAN)
C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)
D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

A

B. Acute Motor Axonal Neuropathy (AMAN)

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25
Q

Aggressive type with rapid symptoms. It causes total paralysis of all 4 limbs in less than 1 week and severe muscle wasting.
A. Regional GBS Syndrome
B. Acute Motor Axonal Neuropathy (AMAN)
C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)
D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

A

C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)

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26
Q

Damages axons directly; accounts for 4.7% of cases.
A. Regional GBS Syndrome
B. Acute Motor Axonal Neuropathy (AMAN)
C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)
D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

A

C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)

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27
Q

Manifests with weakness of lower arms and LE with no
numbness and sensory problems. Recovery is fast and complete, presenting with a good prognosis.
A. Regional GBS Syndrome
B. Acute Motor Axonal Neuropathy (AMAN)
C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)
D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

A

B. Acute Motor Axonal Neuropathy (AMAN)

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28
Q

Attacks glial cells (producing myelin) surrounding the axons
A. Regional GBS Syndrome
B. Acute Motor Axonal Neuropathy (AMAN)
C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)
D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

A

D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

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29
Q

Most common form of GBS
A. Regional GBS Syndrome
B. Acute Motor Axonal Neuropathy (AMAN)
C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)
D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

A

D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

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30
Q

Symptoms start as sensory changes then weakness starts from the toes and fingertips spreading
upwards. Symptoms are symmetrical and individuals also present with areflexia.
A. Regional GBS Syndrome
B. Acute Motor Axonal Neuropathy (AMAN)
C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)
D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

A

D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

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31
Q

Causes a high protein level in the CSF
A. Regional GBS Syndrome
B. Acute Motor Axonal Neuropathy (AMAN)
C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)
D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

A

D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

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32
Q

Triad of Miller Fisher Syndrome

A

ophthalmoplegia, ataxia, and areflexia

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33
Q

Conditions affecting the peripheral nerve

  1. S/sx: high-stepped gait with frequent tripping or
    falling
  2. Most common cause of progressive flaccid
    paralysis (symmetrical weakness)
  3. Hereditary motor and sensory neuropathy
    (HMSN)
  4. S/sx: finger dysesthesia

A. Charcot Marie Tooth Disease
B. Guillain Barre Syndrome

A
  1. A
  2. B
  3. A
  4. B
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34
Q

Most common inherited progressive peripheral neuropathy

A

Charcot Marie Tooth Disease

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35
Q

Neuromuscular diseases comprises a spectrum of diseases with onset in childhood and where the primary area of pathology or lesion is in the peripheral nervous system or _____.

A

motor unit

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36
Q

Most common symptom in neuromuscular diseases

A

Weakness

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37
Q

TRUE OR FALSE: Symptoms of diseases affecting the peripheral nervous system / motor unit are hypotonia, flaccidity, fasciculations, atrophy, hypo-/areflexia, & (-) Babinski.

A

True

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38
Q

TRUE OR FALSE: Duchenne muscular dystrophy (DMD) is a primary muscle disease that affects males and is an x-linked condition, with an estimated prevalence of approximately 1:5,000 boys.

A

True

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39
Q
  1. multi‐systematic disorder with a significant muscle component
  2. neuronopathy affecting the anterior horn cell
  3. disease of the peripheral nerve

A. Spinal muscular atrophy
B. Myotonic dystrophy
C. Charcot Marie Tooth disease

A
  1. B
  2. A
  3. C
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40
Q

TRUE OR FALSE: It is important to always take Prenatal and Perinatal History as symptoms of neuromuscular diseases start during pregnancy and mothers often feels
these.

A

True

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41
Q

Neuromuscular Diseases

  1. ↓ Apgar score
  2. Fetal distress, respiratory difficulty, need for
    resuscitation or ventilation
  3. ↓ Quality of fetal movement or pregnancy
    complications
  4. Difficulty sucking
  5. Hypotonia

A. In prenatal history
B. In perinatal history

A
  1. B
  2. B
  3. A
  4. B
  5. B
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42
Q

Acquired group of autoimmune
polyneuropathy, involving sensory, motor, and
autonomic nerves

A

Guillain Barre Syndrome

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43
Q

Acquired disorder that usually follows an infection or a vaccination (from injection, 2-3 wks after, sx
occurs). It is usually seen in Johnson & Johnson vaccine

A

Guillain Barre Syndrome

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43
Q

The cause of this syndrome is due to temporary inflammation and demyelination of peripheral nerves myelin sheaths resulting in axonal degeneration

A

Guillain Barre Syndrome

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44
Q

It is preceded/triggered by upper respiratory or gastrointestinal tract infections or diarrhea one to three weeks prior to their
onset. Other predisposing factors include recent
surgery, lymphoma, and systemic lupus erythematosus (SLE).

A

Guillain Barre Syndrome

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45
Q

TRUE OR FALSE: There is no cure for Charcot Marie Tooth disease, only supportive treatments are available.

A

True

46
Q

Bacteria in the gastrointestinal tract that causes diarrhea and can trigger Guillain Barre Syndrome

A

Campylobacter jejuni

47
Q

Enumerate the viral and bacterial infections in the respiratory tract that can trigger Guillain Barre Syndrome

A
  1. Cytomegalovirus
  2. Epstein-Barr virus
  3. Mycoplasma pneumoniae (causes respiratory symptoms)
48
Q

In GBS, Schwann cells that produce myelin the peripheral nervous system are destroyed but the ____ are left intact.

A

axons

49
Q

In GBS, after ___ weeks of demyelination, the Schwann cells begin to proliferate (grow again),
inflammation subsides, and re-myelination begins.

A

2-3

Note: There is a good chance the pt will recover.

50
Q

In GBS, an immune response causes across-reaction with the neural tissue causing ____
and destruction of the myelin & cause weakness.

A

inflammation

51
Q

In CSF fluid analysis for GBS, results are usually normal during first (1)__ days. When taking a CSF fluid analysis after this period, results show an elevated protein level due to (2)___.

A
  1. 10
  2. lymphocytosis (high lymphocyte count)
52
Q

Electrophysiological studies (EMG-NCV) for GBS are normal in early stages. In later stages, it shows a delayed F wave and slowing of _____.

A

Nerve conduction

53
Q

There is no known cure for GBS, although ____ is used to prevent severity of
condition if given early; plasmapheresis is also a treatment for GBS.

A

IV immunoglobulin

54
Q
  1. prominent features are fever, malaise, anorexia, and headache
  2. soreness and stiffness of the posterior muscles of the neck, trunk, and limbs

A. Mild / Inapparent Poliomyelitis
B. Abortive Poliomyelitis
C. Nonparalytic Poliomyelitis
D. Paralytic Poliomyelitis

A
  1. B
  2. C
55
Q
  1. fleeting paralysis of the bladder and constipation are frequent
  2. nonspecific influenza-like syndrome occurs 1–2 wk after infection

A. Mild / Inapparent Poliomyelitis
B. Abortive Poliomyelitis
C. Nonparalytic Poliomyelitis
D. Paralytic Poliomyelitis

A
  1. C
  2. B
56
Q

3 clinically recognizable syndromes that represent a continuum of infection differentiated only by the portions of the CNS most severely affected: spinal paralytic poliomyelitis, bulbar poliomyelitis, and polioencephalitis.

A. Mild / Inapparent Poliomyelitis
B. Abortive Poliomyelitis
C. Nonparalytic Poliomyelitis
D. Paralytic Poliomyelitis

A

D. Paralytic Poliomyelitis

57
Q

Presents with non specific signs and symptoms or (some may not have symptoms at all) are observed and usually resolve within a few days (i.e., fever, headache, nausea, vomiting, abdominal pain, and oropharyngeal hyperemia).

A. Mild / Inapparent Poliomyelitis
B. Abortive Poliomyelitis
C. Nonparalytic Poliomyelitis
D. Paralytic Poliomyelitis

A

A. Mild / Inapparent Poliomyelitis

58
Q
  1. Recovery is complete, and no neurologic signs or sequelae develop
  2. Presents with signs of abortive poliomyelitis & more intense headache, nausea, and vomiting

A. Mild / Inapparent Poliomyelitis
B. Abortive Poliomyelitis
C. Nonparalytic Poliomyelitis
D. Paralytic Poliomyelitis

A
  1. B
  2. C
59
Q
  1. Nuchal and spinal rigidity during the 2nd phase.
  2. Vomiting occurs irregularly. Individuals may experience a sore throat and abdominal or muscular pain.

A. Mild / Inapparent Poliomyelitis
B. Abortive Poliomyelitis
C. Nonparalytic Poliomyelitis
D. Paralytic Poliomyelitis

A
  1. C
  2. B
60
Q

Physical examination may be normal or may reveal nonspecific pharyngitis, abdominal or muscular tenderness, and weakness

A. Mild / Inapparent Poliomyelitis
B. Abortive Poliomyelitis
C. Nonparalytic Poliomyelitis
D. Paralytic Poliomyelitis

A

B. Abortive Poliomyelitis

61
Q

basis for the diagnosis of nonparalytic poliomyelitis during the 2nd phase

A

Nuchal and spinal rigidity

62
Q

Determine the most severely affected CNS structure in the ff. forms of Polio

  1. Brainstem, medulla
  2. Brain
  3. Spinal cord

A. spinal paralytic poliomyelitis
B. bulbar poliomyelitis
C. polioencephalitis

A
  1. B
  2. C
  3. A
63
Q

A rare form of the disease in which higher centers of the brain are severely involved. Seizures, coma, and spastic paralysis with increased reflexes may be observed.
A. Spinal paralytic poliomyelitis
B. Bulbar poliomyelitis
C. Polioencephalitis

A

C. Polioencephalitis

64
Q
  1. The only type of polio presenting with hyperreflexia
  2. deviation of the palate, uvula, or tongue

A. Spinal paralytic poliomyelitis
B. Bulbar poliomyelitis
C. Polioencephalitis

A
  1. C
  2. B
65
Q

On Physical Examination: Asymmetric distribution of paralysis is characteristically spotty. Single muscles, multiple muscles, or groups of muscles may be involved in any pattern. Within 1–2 days, asymmetric flaccid paralysis or paresis occurs. Involvement of 1 leg is most common, followed by involvement of 1 arm.

A. Spinal paralytic poliomyelitis
B. Bulbar poliomyelitis
C. Polioencephalitis

A

A. Spinal paralytic poliomyelitis

66
Q
  1. Absence of effective coughing, shown by constant fatiguing efforts to clear the throat
  2. Irritability, disorientation, drowsiness, and coarse tremors peripheral or cranial nerve paralysis that coexists or ensues
  3. Affects proximal more than distal areas (E.g., Shoulders more affected than hands)

A. Spinal paralytic poliomyelitis
B. Bulbar poliomyelitis
C. Polioencephalitis

A
  1. B
  2. C
  3. A
67
Q

Severe muscle pain and sensory and motor phenomena (e.g., paresthesia, hyperesthesia, fasciculations, and spasms), nuchal stiffness or rigidity, muscle tenderness, weakness of some of the muscles of the neck, abdomen, trunk, diaphragm, thorax, or extremities

A. Spinal paralytic poliomyelitis
B. Bulbar poliomyelitis
C. Polioencephalitis

A

A. Spinal paralytic poliomyelitis

68
Q

Initially hyperactive deep tendon reflexes (for a short period) followed by absent or diminished reflexes, and paresis or flaccid paralysis. Sensation is intact.
A. Spinal paralytic poliomyelitis
B. Bulbar poliomyelitis
C. Polioencephalitis

A

A. Spinal paralytic poliomyelitis

68
Q

A condition that affects Polio survivors after years of recovery from initial Poliovirus infection. It is a result of the deterioration of motor neurons which leads to loss of muscle strength and dysfunction.

A

Post-polio syndrome

Note: affects the same/different muscle, but that doesn’t mean pt. got polio again, the fibers just degenerated

69
Q

Most common symptom of post-polio syndrome

A

Fatigue

69
Q

Poliomyelitis presents with _____ and muscle atrophy which are the hallmarks of its clinical manifestations

A

flaccid (focal) asymmetric weakness

70
Q

Enumerate the ff in relation to Poliomyelitis
1. Natural host
2. Mode of transmission
3. Sex predilection
4. Common age of host
5. Sources

A
  1. Natural host: Human
  2. Mode of transmission: Nasopharyngeal
  3. Sex predilection: Male
  4. Common age of host: 6 mos to 3 yrs
  5. Sources: Poor sanitation, contaminated food & water
71
Q

The incubation period for poliovirus is ___ days

A

5-35

72
Q

Optimum position of limb in bed rest for polio patients

A

hip: slight flexion
knee: 5º flexion
foot: neutral

72
Q

In pregnancy, a history of ____ can result in infants with these manifestations: central core myopathy, Duchenne Muscular Dystrophy, Becker’s Dystrophy, Fukuyama congenital muscular dystrophy, limb girdle dystrophy, facioscapulohumeral dystrophy, period paralysis, and myotonia congenita

A

malignant hyperthermia: complication during gas anesthesia, temperature shooting up (fever), tachycardia, difficulty in breathing

72
Q

In CMT, the lower legs may take on an _____ or stork leg appearance shape due to the loss of muscle bulk

A

“inverted champagne bottle”

73
Q

Epidemiology of GBS for age and gender

A

Age: young adulthood: 15 to 35 y/o and middle aged to elderly: 50 – 75 y/o
Gender: more common in men

74
Q

Poliovirus is resistant in feces at (1)___ Celsius for months and -20° Celsius for years. It is inactivated by heat, (2)___, UV light, and chlorine.

A
  1. 4°
  2. formaldehyde
75
Q

In GBS, s/sx begin 1-3 weeks after GI or respiratory illness and peak of symptoms happen (1)___ days from 1st neurologic symptom reaching peak by 4 weeks. Recovery occurs (2)___ weeks after the progression stops

A
  1. 12
  2. 2 – 4

Additional: Mean time to clinical recovery is 200 days

76
Q

Convalescent:
The lower limb muscles are more often involved, flexion contractures of hip and knee and equinus deformity of the ankle are common; also presents with atrophy
A. Spinal type of polio
B. Bulbar type of polio
C. Spinobulbar type of polio
D. Post encephalic type of polio

A

A. Spinal type of polio

76
Q

Convalescent:
The most important is inability to swallow due to pharyngeal paralysis
A. Spinal type of polio
B. Bulbar type of polio
C. Spinobulbar type of polio
D. Post encephalic type of polio

A

B. Bulbar type of polio

77
Q

Convalescent:
Require ventilatory support for respiration and tube for feeding for swallowing
A. Spinal type of polio
B. Bulbar type of polio
C. Spinobulbar type of polio
D. Post encephalic type of polio

A

B. Bulbar type of polio

78
Q

Convalescent:
Difficulty breathing, feeling of suffocation, slight cyanosis, use of sternomastoids, alae nasae and other accessory muscles of respiration.
A. Spinal type of polio
B. Bulbar type of polio
C. Spinobulbar type of polio
D. Post encephalic type of polio

A

B. Bulbar type of polio

79
Q

Convalescent:
Combination of spinal and bulbar type
A. Spinal type of polio
B. Bulbar type of polio
C. Spinobulbar type of polio
D. Post encephalic type of polio

A

C. Spinobulbar type of polio

80
Q
  1. Travels via the bloodstream to the anterior horn cells, spinal cord or brain
  2. Disorder that is manifested by a loss of anterior horn cells
  3. Inherited as an autosomal recessive disorder with defect located on chromosome 5q13
  4. Disease of the anterior horn motor neurons of the spinal cord and brainstem caused by poliovirus
  5. Infantile paralysis

A. Poliomyelitis
B. Spinal muscular atrophy

A
  1. A
  2. B
  3. B
  4. A
  5. A
81
Q
  1. Most common form
  2. Weakness initially in pelvic girdle associated with difficulty ambulating or running
  3. Diminished DTRs, CN usually spared

A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A
  1. A
  2. C
  3. B
82
Q

Characteristics: weakness and wasting of extremities and trunk musculature
A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A

B. SMA II: Dubowitz Disease

83
Q

Autosomal recessive disorder that is usually seen at time of birth, noted within the first 3 months of life
A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A

A. SMA I: Acute Werdnig Hoffman Disease

83
Q

On physical examination: presence of fasciculations in tongue and fine tremors (mini polymyoclonus)
A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A

B. SMA II: Dubowitz Disease

84
Q

Autosomal recessive disease with slowly progressive weakness and atrophy of proximal muscles and girdle muscles
A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A

C. SMA III: Kugelberg Welander Disease

85
Q

Occurs more in males, onset is from childhood to seventh decade of life, usually at age 2 to 17 years old. Symptoms are usually present by age 3 years old.
A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A

C. SMA III: Kugelberg Welander Disease

86
Q
  1. Defect seen in chromosome 5q
  2. Average of survival from time of diagnosis is 6 to 9 months but does not exceed 3 years

A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A
  1. C
  2. A
87
Q
  1. Waddling gait
  2. Cause of death is usually respiratory failure
  3. Normal neonatal period during the 1st 3-24 mos, can achieve unaided sitting
  4. (+) Gower’s sign

A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A
  1. C
  2. A
  3. B
  4. C
88
Q
  1. Sits independently but never stands/walks without aids; skeletal deformities
  2. Stands/walks without support
  3. Never sits/stands without support; can’t walk

A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A
  1. B
  2. C
  3. A
89
Q

Survival

  1. Usually > 2 yrs.; Often to adulthood
  2. Usually < 2 yrs., limited life expectancy, and respiratory failure
  3. Adulthood

A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A
  1. B
  2. A
  3. C
89
Q

TRUE OR FALSE: The polio vaccine is recommended to be given at these ages:
1. 2 months
2. 4 months
3. Between 6 and 18 months
4. Between 4 and 6 years

A

True

90
Q

Vaccine that has been known to cause vaccine-linked paralytic poliomyelitis. It should not be given to people with a weak immune system.

A

Oral poliovirus vaccine (OPV)

91
Q

Vaccine given through injection and can’t cause polio because the virus has been killed, thus it is safe for people with a weak immune system.

A

Inactivated Poliovirus Vaccine (IPV)

92
Q

_____ has been associated with the highest rate of complications and a mortality rate as high as 60%; it has the worst prognosis among all types of polio, followed by spinal poliomyelitis.

A

Bulbar paralytic poliomyelitis

93
Q

TRUE OR FALSE: Patients with inapparent or abortive poliomyelitis recover without significant sequelae/residuals.

A

True

94
Q

Convalescent:
Mental disturbance, coma, paralysis of facial muscles, and meningitis symptoms (i.e., headache, vomiting, neck stiffness)
A. Spinal type of polio
B. Bulbar type of polio
C. Spinobulbar type of polio
D. Post encephalic type of polio

A

D. Post encephalic type of polio

95
Q

Most prevalent type of spinal muscular atrophy

A

SMA I (Acute Werdnig Hoffman Disease)

96
Q

Onset

  1. ≥ 18 months
  2. ≤ 18 months
  3. ≤ 6 months

A. SMA I: Acute Werdnig Hoffman Disease
B. SMA II: Dubowitz Disease
C. SMA III: Kugelberg Welander Disease

A
  1. C
  2. B
  3. A
97
Q

Poliovirus is an ____ infection

A

enteroviral (enters mouth, nasopharynx & exit to feces)

98
Q

Management for scoliosis is recommended to Cobb’s angle more than (1)___ degrees. Scoliosis is prevalent in SMA 1 and 2, usually thoracic scoliosis with (2)___. A thoracolumbar orthosis is used but with adaptation for (3)___ support.

A
  1. 20
  2. kyphosis
  3. respiratory
99
Q

For SMA 2 non ambulators, lung function declines usually after ___ years old

A

13

100
Q

Stretching exercises for SMA are done (1)__ times a week to maximum of 5-6x a week for those who cannot walk. For walkers, these are done (2)__ times a week to maximum of 3-5x a week.

A
  1. 3-5
  2. 2-3
101
Q

61% of patients with SMA 1 will require intubation within 1 year after birth and 100% of children with SMA 1 older than ___ months will need ventilatory support. Pulmonary rehabilitation includes manual or mechanical chest physiotherapy, postural drainage, and breathing exercises

A

12

102
Q

Swallowing was noted to deteriorate in SMA 1 at ___ months with initiation of tube feeding

A

6

103
Q

Diagnostic Evaluation

  1. To provide information whether the disease process is primarily neurogenic or myopathic
  2. Evaluate the ultrastructural changes of muscle fiber organelles (i.e., mitochondria, cytoplasm)
  3. Provides information about amount muscle protein (e.g. dystrophin, etc)

A. Molecular Genetic Studies
B. Histology / Histochemistry
C. Immunoblotting and Immunostaining
D. Electron microscopy

A
  1. B
  2. D
  3. C
104
Q

Diagnostic Evaluation

  1. In this test, 1st choice is to use proximal muscles (biceps/triceps, vastus lateralis)
  2. Elevation in transaminases, aldolases, creatine kinase
  3. Uses sural nerve; median/femoral nerve not used since it’ll have motor problems

A. Nerve biopsy
B. Muscle biopsy
C. Electrodiagnostic studies
D. Serum laboratory examinations

A
  1. B
  2. D
  3. A
105
Q

most important sign seen in the acute stage of polio

A

Muscle tenderness

106
Q

For the diagnosis of CMT, in which specific nerves in the UE and LE do enlargement of the nerve (onion bulb) occur?

A

UE: ulnar nerve
LE: peroneal / fibular nerve

(3) MS: Pediatric Conditions (11 decks)

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