S3_L1: Neuromuscular Diseases part 1

Question 1

What is the most commonly encountered genetic
pediatric neuromuscular condition?

Answer 1

Duchenne muscular dystrophy (DMD)

Question 2

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

Answer 2

E. Charcot Marie Tooth Type X

Question 3

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

Answer 3

D. Charcot Marie Tooth Type 4

Question 4

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

Answer 4

B. Charcot Marie Tooth Type 2

Question 5

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

Answer 5

C. Charcot Marie Tooth Type 3

Question 6

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

Answer 6

C. Charcot Marie Tooth Type 3

Question 7

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

Answer 7

A. Charcot Marie Tooth Type 1

Question 8

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

Answer 8

C. Charcot Marie Tooth Type 3

Question 9

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

Answer 9

D. Charcot Marie Tooth Type 4

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

Question 10

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

Answer 10

D. Charcot Marie Tooth Type 4

Question 11

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

Answer 11

E. Charcot Marie Tooth Type X

Question 12

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

Answer 12

E. Charcot Marie Tooth Type X

Question 13

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

Answer 13

E. Charcot Marie Tooth Type X

Question 14

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

Answer 14

B. Charcot Marie Tooth Type 2

Question 15

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

Answer 15

B. Charcot Marie Tooth Type 2

Question 16

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

Answer 16

B. Charcot Marie Tooth Type 2

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

Question 16

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

Answer 16

A. Charcot Marie Tooth Type 1

Question 17

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

Answer 17

A. Charcot Marie Tooth Type 1

Question 18

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

Answer 18

A. Charcot Marie Tooth Type 1

Question 19

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

Answer 19

A. Charcot Marie Tooth Type 1

Question 20

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

Answer 20

A. Charcot Marie Tooth Type 1

Question 21

Charcot Marie Tooth Type 1 disease mostly affects which chromosome?

Answer 21

Chromosome 17

Question 22

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)

Answer 22

A. Regional GBS Syndrome

Question 23

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)

Answer 23

A. Regional GBS Syndrome

Question 24

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)

Answer 24

B. Acute Motor Axonal Neuropathy (AMAN)

Question 25

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)

Answer 25

C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)

Question 26

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)

Answer 26

C. Acute Motor-Sensory Axonal Neuropathy (AMSAN)

Question 27

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)

Answer 27

B. Acute Motor Axonal Neuropathy (AMAN)

Question 28

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)

Answer 28

D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

Question 29

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)

Answer 29

D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

Question 30

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)

Answer 30

D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

Question 31

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)

Answer 31

D. Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

Question 32

Triad of Miller Fisher Syndrome

Answer 32

ophthalmoplegia, ataxia, and areflexia

Question 33

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

Answer 33

1. A 2. B 3. A 4. B

Question 34

Most common inherited progressive peripheral neuropathy

Answer 34

Charcot Marie Tooth Disease

Question 35

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 _____.

Answer 35

motor unit

Question 36

Most common symptom in neuromuscular diseases

Answer 36

Weakness

Question 37

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

Answer 37

True

Question 38

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.

Answer 38

True

Question 39

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

Answer 39

1. B 2. A 3. C

Question 40

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.

Answer 40

True

Question 41

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

Answer 41

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

Question 42

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

Answer 42

Guillain Barre Syndrome

Question 43

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

Answer 43

Guillain Barre Syndrome

Question 43

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

Answer 43

Guillain Barre Syndrome

Question 44

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).

Answer 44

Guillain Barre Syndrome

Question 45

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

Answer 45

True

Question 46

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

Answer 46

Campylobacter jejuni

Question 47

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

Answer 47

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

Question 48

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

Answer 48

axons

Question 49

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

Answer 49

2-3 Note: There is a good chance the pt will recover.

Question 50

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

Answer 50

inflammation

Question 51

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)___.

Answer 51

1. 10 2. lymphocytosis (high lymphocyte count)

Question 52

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

Answer 52

Nerve conduction

Question 53

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.

Answer 53

IV immunoglobulin

Question 54

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

Answer 54

1. B 2. C

Question 55

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

Answer 55

1. C 2. B

Question 56

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

Answer 56

D. Paralytic Poliomyelitis

Question 57

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

Answer 57

A. Mild / Inapparent Poliomyelitis

Question 58

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

Answer 58

1. B 2. C

Question 59

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

Answer 59

1. C 2. B

Question 60

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

Answer 60

B. Abortive Poliomyelitis

Question 61

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

Answer 61

Nuchal and spinal rigidity

Question 62

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

Answer 62

1. B 2. C 3. A

Question 63

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

Answer 63

C. Polioencephalitis

Question 64

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

Answer 64

1. C 2. B

Question 65

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

Answer 65

A. Spinal paralytic poliomyelitis

Question 66

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

Answer 66

1. B 2. C 3. A

Question 67

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

Answer 67

A. Spinal paralytic poliomyelitis

Question 68

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

Answer 68

A. Spinal paralytic poliomyelitis

Question 68

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.

Answer 68

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

Question 69

Most common symptom of post-polio syndrome

Answer 69

Fatigue

Question 69

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

Answer 69

flaccid (focal) asymmetric weakness

Question 70

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

Answer 70

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

Question 71

The incubation period for poliovirus is ___ days

Answer 71

5-35

Question 72

Optimum position of limb in bed rest for polio patients

Answer 72

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

Question 72

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

Answer 72

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

Question 72

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

Answer 72

"inverted champagne bottle"

Question 73

Epidemiology of GBS for age and gender

Answer 73

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

Question 74

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.

Answer 74

1. 4° 2. formaldehyde

Question 75

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

Answer 75

1. 12 2. 2 – 4 Additional: Mean time to clinical recovery is 200 days

Question 76

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

Answer 76

A. Spinal type of polio

Question 76

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

Answer 76

B. Bulbar type of polio

Question 77

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

Answer 77

B. Bulbar type of polio

Question 78

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

Answer 78

B. Bulbar type of polio

Question 79

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

Answer 79

C. Spinobulbar type of polio

Question 80

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

Answer 80

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

Question 81

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

Answer 81

1. A 2. C 3. B

Question 82

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

Answer 82

B. SMA II: Dubowitz Disease

Question 83

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

Answer 83

A. SMA I: Acute Werdnig Hoffman Disease

Question 83

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

Answer 83

B. SMA II: Dubowitz Disease

Question 84

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

Answer 84

C. SMA III: Kugelberg Welander Disease

Question 85

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

Answer 85

C. SMA III: Kugelberg Welander Disease

Question 86

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

Answer 86

1. C 2. A

Question 87

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

Answer 87

1. C 2. A 3. B 4. C

Question 88

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

Answer 88

1. B 2. C 3. A

Question 89

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

Answer 89

1. B 2. A 3. C

Question 89

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

Answer 89

True

Question 90

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

Answer 90

Oral poliovirus vaccine (OPV)

Question 91

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.

Answer 91

Inactivated Poliovirus Vaccine (IPV)

Question 92

_____ 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.

Answer 92

Bulbar paralytic poliomyelitis

Question 93

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

Answer 93

True

Question 94

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

Answer 94

D. Post encephalic type of polio

Question 95

Most prevalent type of spinal muscular atrophy

Answer 95

SMA I (Acute Werdnig Hoffman Disease)

Question 96

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

Answer 96

1. C 2. B 3. A

Question 97

Poliovirus is an ____ infection

Answer 97

enteroviral (enters mouth, nasopharynx & exit to feces)

Question 98

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.

Answer 98

1. 20 2. kyphosis 3. respiratory

Question 99

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

Answer 99

13

Question 100

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.

Answer 100

1. 3-5 2. 2-3

Question 101

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

Answer 101

12

Question 102

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

Answer 102

6

Question 103

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

Answer 103

1. B 2. D 3. C

Question 104

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

Answer 104

1. B 2. D 3. A

Question 105

most important sign seen in the acute stage of polio

Answer 105

Muscle tenderness

Question 106

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

Answer 106

UE: ulnar nerve LE: peroneal / fibular nerve