S3_L2: Lower Motor Neuron Diseases

Question 1

Refer to the spinal and cranial nerve cells that will eventually innervate the skeletal muscles

Answer 1

Lower motor neuron

Question 2

1. Presents most commonly with progressive spasticity, weakness of the lower limbs,
   hypertonic urinary bladder, and impaired vibration sense
2. Progressive and begins more often in the legs versus arms or bulbar muscles
3. Asymmetric limb onset

A. Progressive Lateral Sclerosis (PLS)
B. Hereditary Spastic Paraplegia (HSP)

Answer 2

1. B
2. A
3. A

Question 3

Most common presenting symptom of Progressive Lateral Sclerosis (PLS)

Answer 3

spasticity

Question 4

Most severely affected neurons in Hereditary Spastic Paraplegia (HSP) are those of the (1)____, specifically caudal to rostral degeneration of the (2)____ tract with mild involvement of the DCML

Answer 4

1. spinal cord
2. corticospinal

Question 5

Hereditary Spastic Paraplegia (HSP)

1. spasticity, urinary disturbance
2. combination of pure plus neurologic disorders such as seizures, impaired cognition, dementia, extrapyramidal disturbance, peripheral neuropathy in the absence of coexisting disorders
3. vibration sense impairment

A. Pure/classic presentation
B. Complex presentation

Answer 5

1. A
2. B
3. A

Question 6

1. Lack of familial inheritance
2. Sporadic; Etiology is unknown

A. Progressive Lateral Sclerosis (PLS)
B. Hereditary Spastic Paraplegia (HSP)

Answer 6

1. A
2. A

Question 7

Rare disorders of progressive spasticity with mostly spinal and occasional bulbar region
onset beginning at the 5th decade of life

Answer 7

Progressive Lateral Sclerosis (PLS)

Question 8

45% of those diagnosed with Progressive Lateral Sclerosis (PLS) will develop LMN symptoms and progress to ____, but this is less likely if the patient does not develop LMN symptoms after (2)____ years from diagnosis

Answer 8

1. Amyotrophic Lateral Sclerosis (ALS)
2. 4

Question 9

1. Feeling of stiffness in the limbs
2. Muscle atrophy
3. Fasciculations
4. Loss of dexterity
5. Bulbar muscle weakness

A. Upper motor neuron (UMN) presentation
B. Lower motor neuron (LMN) presentation
C. Can be both
D. Neither

Answer 9

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

Question 10

1. Hyporeflexia
2. Spasticity
3. Flaccid dysarthria
4. Muscle cramping
5. Pathologic reflexes

A. Upper motor neuron (UMN) presentation
B. Lower motor neuron (LMN) presentation
C. Can be both
D. Neither

Answer 10

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

Question 11

1. Hypotonia
2. Pseudobulbar affect
3. Spastic dysarthria
4. Hyperreflexia
5. Retained reflex in atrophic limb
6. Muscle weakness

A. Upper motor neuron (UMN) presentation
B. Lower motor neuron (LMN) presentation
C. Can be both
D. Neither

Answer 11

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

Question 11

1. More common in males (4:1)
2. Associated with thymoma 75% of the
   time
3. Proximal weakness
4. Decreased ACh receptors
5. Ptosis and diplopia - initial manifestation

A. Myasthenia Gravis
B. Lambert-Eaton Myasthenic Syndrome
C. Both

Answer 11

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

Question 12

1. Occasional bulbar sign
2. Decreasing ACh released
3. More common in females (2-3/2:1)
4. Pre-synaptic
5. Often associated with bronchogenic
   carcinoma

A. Myasthenia Gravis
B. Lambert-Eaton Myasthenic Syndrome
C. Both

Answer 12

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

Question 13

1. Post-synaptic
2. Dry mouth
3. Initially with strength, then decreases throughout the day or with repeated use
4. Slurring speech
5. 2nd wind phenomenon

A. Myasthenia Gravis
B. Lambert-Eaton Myasthenic Syndrome
C. Both

Answer 13

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

Question 13

1. Sexual dysfunction
2. Fluctuating bulbar paralysis
3. Does not affect the smooth and cardiac muscles
4. Tendon reflexes are often diminished but not completely extinct
5. Hyporeflexia

A. Myasthenia Gravis
B. Lambert-Eaton Myasthenic Syndrome
C. Both

Answer 13

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

Question 14

Juvenile Amyotrophic Lateral Sclerosis Types

1. defect on chromosome 15q
2. defect on the senataxin gene of chromosome 9q34
3. defect on chromosome 2q33

A. ALS2
B. ALS4
C. ALS5
D. All of the above

Answer 14

1. C
2. B
3. A

Question 14

Juvenile Amyotrophic Lateral Sclerosis Types

1. presenting with progressive limb spasticity, distal limb weakness and muscle atrophy
2. presenting with facial and limb spasticity, pseudobulbar affect
3. presenting with several distal muscle weakness and pyramidal signs in the absence of bulbar and sensory abnormalities

A. ALS2
B. ALS4
C. ALS5
D. All of the above

Answer 14

1. C
2. A
3. B

Question 15

Spinal Muscular Atrophy

1. Unable to sit independently
2. Mean onset in mid-30s
3. Sits independently, but with no independent ambulation
4. Hand tremor, tongue fasciculations, and areflexia are common

A. SMA I: Werdnig Hoffman disease
B. SMA II: Dubowitz disease
C. SMA III: Kugelberg-Welander disease
D. SMA IV: Adult Onset

Answer 15

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

Question 16

Spinal Muscular Atrophy

1. Onset before 6 months of age
2. Joint contractures, severe progressive scoliosis and restrictive lung disease are present in most cases
3. Poor survival
4. Onset after 18 months of age

A. SMA I: Werdnig Hoffman disease
B. SMA II: Dubowitz disease
C. SMA III: Kugelberg-Welander disease
D. SMA IV: Adult Onset

Answer 16

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

Question 17

Type of Spinal Muscular Atrophy that is also known as Chronic Infantile SMA?

Answer 17

SMA 2

Question 18

Hereditary Sensory Motor Neuropathy

1. present with weakness of the diaphragm, vocal cord, and intercostal muscles
2. Refsum’s disease
3. Its B subtype affects chromosome 1
4. Dejerine Sottas Disease

A. HSMN I
B. HSMN II
C. HSMN III
D. HSMN IV

Answer 18

1. B (CMT 2)
2. D
3. A (CMT 1b)
4. C

Question 19

Hereditary Sensory Motor Neuropathy

1. with optic atrophy
2. with retinitis pigmentosa
3. presents with altered mitochondria within the Schwann cell
4. presents with spinocerebellar degeneration

A. HSMN IV
B. HSMN V
C. HSMN VI
D. HSMN VII

Answer 19

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

Question 20

Hereditary Sensory Motor Neuropathy

1. varying degrees of neuropraxia are common
2. Its A subtype affects chromosome 17
3. present with prominent demyelination and remyelination
4. lesser hypertrophic change in myelin with more neuronal or axonal involvement

A. HSMN I
B. HSMN II
C. HSMN III
D. HSMN IV

Answer 20

1. C
2. A (CMT 1a)
3. C
4. B (as compared to HSMN 1)

Question 21

Among the 7 types of Hereditary Sensory Motor Neuropathy, which is/are the most common type/s?

Answer 21

HSMN I and II (prevalence at 10 to 20 per 100,000)

Question 22

Among the 4 types of Spinal Muscular Atrophy (SMA), which is/are the most common type/s?

Answer 22

SMA I, II, and III

Question 23

TRUE OR FALSE: SMA II and III have proximal weakness greater than the distal.

Answer 23

True

Question 24

Which type/s of Spinal Muscular Atrophy can live normal life spans?

Answer 24

SMA III and IV Additional: many SMA II patients are now living into adulthood

Question 25

Refers to twitching of the upper eyelid that appears a moment after the patient moves the eyes from a downward to a primary position

Answer 25

"Lid-twitch" sign

Question 26

Refers to 1 central and 2 lateral longitudinal furrows in the tongue (fasciculations in the tongue).

Answer 26

"Trident tongue" (Triple furrow tongue)

Question 26

Motor neurons are divided into 4 body areas (pertained to as regions). Identify these areas.

Answer 26

1. Bulbar 2. Cervical 3. Thoracic 4. Lumbosacral (Lumbar)

Question 27

Nerve cell for processing motor information

Answer 27

Motor neuron

Question 28

Hallmark of motor neuron diseases

Answer 28

Weakness

Question 29

Cardinal sign of Amyotrophic Lateral Sclerosis (ALS)

Answer 29

Muscle weakness

Question 30

1. Originates from anterior horn cells 2. If affected, manifestations are excitatory or "more" because nothing inhibits them 3. Originates from primary motor cortex 4. Doer of function, facilitates movement A. Lower motor neuron B. Upper motor neuron C. Both D. Neither

Answer 30

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

Question 31

Betz cells are located within which layer of the primary motor cortex?

Answer 31

Fifth layer

Question 32

1. Post-polio syndrome 2. Amyotrophic lateral sclerosis 3. Spinal muscular atrophy 4. Progressive Lateral Sclerosis 5. Progressive Bulbar Palsy A. UMN and LMN involvement B. UMN involvement C. LMN involvement D. None of the above

Answer 32

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

Question 33

1. Familial Amyotrophic Lateral Sclerosis 2. Progressive Muscular Atrophy 3. Atypical Upper & Lower Motor Neuron Disorders 4. Juvenile Amyotrophic Lateral Sclerosis 5. Fazio-Londe Disease A. UMN and LMN involvement B. UMN involvement C. LMN involvement D. None of the above

Answer 33

1. A 2. C 3. A 4. A 5. C

Question 34

1. Hereditary Spastic Paraplegia 2. Spinal and Bulbar Muscular Atrophy 3. Poliomyelitis 4. Sporadic Amyotrophic Lateral Sclerosis A. UMN and LMN involvement B. UMN involvement C. LMN involvement D. None of the above

Answer 34

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

Question 35

Autosomal Dominant vs Autosomal Recessive 1. Both parents have the disease 2. Only one of the parents has the disease A. Autosomal Dominant B. Autosomal Recessive

Answer 35

1. B 2. A

Question 36

Juvenile Amyotrophic Lateral Sclerosis Types 1. Onset: after age 10 2. Most common type 3. Onset: teenage years A. ALS2 B. ALS4 C. ALS5 D. All of the above

Answer 36

1. A 2. C 3. C

Question 37

Juvenile Amyotrophic Lateral Sclerosis Types 1. ALS2 2. ALS4 3. ALS5 A. Autosomal dominant B. Autosomal recessive C. Both D. Neither

Answer 37

1. B 2. A 3. B

Question 38

Type of neuropathy that is a tick-borne illness caused by Borrelia burgdorferi. People with this neuropathy may get a “Bull’s eye rash”.

Answer 38

Lyme disease

Question 39

If Lyme disease is left untreated, it can cause _______ neuropathy leading to progressive muscle weakness.

Answer 39

patchy axonal

Question 40

TRUE OR FALSE: In neuropathies, the Schwann cell is affected.

Answer 40

True

Question 41

TRUE OR FALSE: Neuropathies can preferentially affect nerves of specific characteristics (e.g., large or small fibers).

Answer 41

True

Question 42

Chromosome affectation in Spinal Muscular Atrophy (SMA)

Answer 42

Chromosome 5q11.2-13.3

Question 43

The pre-paralytic stage of poliomyelitis is characterized by fever for ___ days with meningeal irritation, headache, muscle soreness (felt in neck and back), altered sensorium, and seizures.

Answer 43

4-7 Note: The presence of seizures implies CNS involvement (similar to meningitis).

Question 43

The prodromal stage of poliomyelitis is characterized by flu-like symptoms for ____ hrs, followed by 2-3 days of wellness

Answer 43

38-47

Question 44

New, slowly progressive muscle weakness occurring in individuals with a confirmed history of acute poliomyelitis.

Answer 44

Post-polio Syndrome

Question 45

One popular theory in post-polio syndrome (PPS) that infers acute poliovirus infections kill specific spinal anterior horn cells and brainstem motor nuclei. Functional recovery is mediated by the neuritic sprouting of surviving motor neurons with attendant re-innervation of denervated skeletal muscle. However, re-innervation sprouting leads to the enlargement of motor units, adding metabolic stress to remaining motor neurons. Chronically increased metabolic stress could eventually trigger a second wave of neurodegeneration and disability, corresponding with the emergence of PPS.

Answer 45

Neural fatigue theory

Question 45

Pharmacological test done to see clinical improvement in patients affected by myasthenia gravis

Answer 45

Neostigmine test

Question 46

Pharmacologic test used to help the MD diagnose myasthenia gravis

Answer 46

Edrophonium test (Tensilon test)

Question 47

The paralytic stage of poliomyelitis is characterized by paralysis occurring ___ days after the pre-paralytic stage, or may be delayed for 2-3 weeks. Additionally, motor loss lasts 3-5 days.

Answer 47

2-5

Question 47

TRUE OR FALSE: In poliomyelitis, weakness can happen randomly depending on which motor neuron was affected by the virus.

Answer 47

True

Question 48

In poliomyelitis, the ____ muscles are usually involved. In severe cases, respiratory and cardiac muscles are affected; acute cerebellar ataxia, isolated facial nerve palsies, and transverse myelitis may also be seen.

Answer 48

Limb

Question 48

Also known as Lou Gehrig’s disease

Answer 48

Amyotrophic Lateral Sclerosis (ALS)

Question 49

1. X-linked recessive inheritance 2. Sporadic degenerative disease selectively affecting the anterior horn cells without signs of UMN involvement 3. Autosomal recessive A. Fazio-Londe Disease B. Progressive Muscular Atrophy C. Spinal and Bulbar Muscular Atrophy (SBMA)

Answer 49

1. C 2. B 3. A

Question 49

1. Onset: 2-13 y/o d/t degeneration of the AHC 2. Usually occurs after 30 y/o 3. Median age of onset: 57 y/o A. Fazio-Londe Disease B. Progressive Muscular Atrophy C. Spinal and Bulbar Muscular Atrophy (SBMA)

Answer 49

1. A 2. C 3. B

Question 50

1. Degeneration of sensory neurons in the dorsal root ganglia preceding the onset of motor dysfunction 2. All bulbar neurons are affected 3. Bulbar symptoms not typically evident at diagnosis, but develops over the course A. Fazio-Londe Disease B. Progressive Muscular Atrophy C. Spinal and Bulbar Muscular Atrophy (SBMA)

Answer 50

1. C 2. A 3. B

Question 51

1. Presents with stridor, ptosis, dysarthria, and facial paralysis 2. Slowly progressive; ability to ambulate lost later in life 3. Presents with weakness of the face, tongue, and pharynx A. Fazio-Londe Disease B. Progressive Muscular Atrophy C. Spinal and Bulbar Muscular Atrophy (SBMA)

Answer 51

1. A 2. C 3. A

Question 52

1. Selective destruction of the anterior horn cells 2. Disease of the anterior horn neurons of the spinal cord and brainstem 3. Bulbar onset, CN affectations, vital organs affectation A. Spinal Muscular Atrophy (SMA) B. Progressive Bulbar Palsy C. Poliomyelitis

Answer 52

1. A 2. C 3. B

Question 53

1. Leads to development of acute flaccid paralysis that can be bulbar or spinal in distribution 2. Degeneration of motor neurons of CN IX, X, XI, and XII 3. Usually autosomal recessive A. Spinal Muscular Atrophy (SMA) B. Progressive Bulbar Palsy C. Poliomyelitis

Answer 53

1. C 2. B 3. A

Question 54

1. Manifests with fever, malaise, myalgia 2. Manifests with sore throat, GI tract upset 3. Disorders with childhood onset, hereditary A. Spinal Muscular Atrophy (SMA) B. Progressive Bulbar Palsy C. Poliomyelitis

Answer 54

1. C 2. C 3. A

Question 55

Enumerate the genes affected in Spinal Muscular Atrophy (SMA)

Answer 55

survival motor neuron genes 1 and 2 (SMN1 and SMN2)

Question 56

TRUE OR FALSE: The onset of bulbar symptoms in Progressive Muscular Atrophy demonstrated more rapid decline with early death.

Answer 56

True

Question 57

Also known as Kennedy Disease

Answer 57

Spinal and Bulbar Muscular Atrophy (SBMA)

Question 57

Structure that degenerates in Spinal and Bulbar Muscular Atrophy (SBMA), but is spared in Amyotrophic Lateral Sclerosis (ALS).

Answer 57

Onuf nucleus

Question 58

Poliomyelitis is caused by poliovirus, an RNA virus of the ___ groups of the picornavirus family

Answer 58

enterovirus

Question 59

Transmission of poliomyelitis

Answer 59

Fecal-oral route

Question 60

type of poliomyelitis most often associated with paralytic disease

Answer 60

Type 1 (Brunhilde virus)

Question 61

Clinical manifestation of poliomyelitis that is caused by the severe atrophy of the quadriceps. It results in the inability to keep the knee extended in heel strike, thus the patient needs to use their hand to push the knee back to avoid knee buckling.

Answer 61

Polio gait

Question 62

TRUE OR FALSE: In polio, 90% of muscles that are completely paralyzed at 6 mos. will remain paralyzed.

Answer 62

True

Question 63

Criteria for diagnosis of post-polio syndrome 1. Previous paralytic poliomyelitis with evidence of (1)___ loss 2. Period of partial or complete functional recovery followed by a functionally stable period of at least (2)___ yrs 3. Onset of progressive and persistent new muscle weakness or decreased endurance with or without fatigue, muscle atrophy, or muscle and joint pain 4. Persistent symptoms for at least ___ months 5. Exclusion of other causative neurologic, medical, and orthopedic problems

Answer 63

1. motor neuron 2. 15 3. 12 (1 year)

Question 64

Most common cause of acute neuromuscular paralysis in the Western world (1 to 2 per 100,000)

Answer 64

Guillain-Barre Syndrome

Question 65

Guillain-Barre Syndrome presents with ____ weakness of the limbs with hyporeflexia or areflexia, with or without sensory abnormalities

Answer 65

progressive, symmetrical Note: GBS is unlike ALS and polio that presents with asymmetrical weakness

Question 66

Most common presenting symptom of Progressive Muscular Atrophy

Answer 66

distal limb weakness with muscle atrophy

Question 67

TRUE OR FALSE: In Progressive Muscular Atrophy, the legs are slightly more affected than the arms with either symmetric or asymmetric presentation of distal limb weakness.

Answer 67

False Arms > Legs

Question 68

Progressive Muscular Atrophy is a rare disease with unknown etiology. It has a median survival rate of ____ months and a 5 year survival rate of 45%.

Answer 68

56

Question 69

Median Survival from onset of symptoms (in years) in bulbar and limb dominant Sporadic Amyotrophic Lateral Sclerosis

Answer 69

Bulbar dominant: 2-3 years Limb dominant: 3-5 years

Question 70

TRUE OR FALSE: The survival rate of Sporadic Amyotrophic Lateral Sclerosis is 50% at 2.5 years after diagnosis. Only 4% will survive longer than 10 years.

Answer 70

True

Question 71

Poor or Good prognosis: Sporadic Amyotrophic Lateral Sclerosis 1. Older age at time of onset 2. Long period from symptom onset to diagnosis 3. Bulbar and/or pulmonary dysfunction early in the clinical course of the disease 4. Predominance of LMN findings at diagnosis A. Poor B. Good

Answer 71

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

Question 72

Risk factors: Sporadic Amyotrophic Lateral Sclerosis 1. Cigarette smoking 2. Increased ___ consumption

Answer 72

glutamate

Question 73

Patients maintain the ability to ____ into midlife and have a normal lifespan in Juvenile Amyotrophic Lateral Sclerosis

Answer 73

ambulate

Question 74

Longest surviving patient with ALS

Answer 74

Stephen Hawking

Question 75

Enumerate the CN not commonly affected in ALS

Answer 75

CN 3, 4, & 6 (oculomotor muscles)

Question 76

Enumerate the CN affected in ALS

Answer 76

CN V, VII, IX, X, and XII

Question 77

Cranial nerves can be affected with ___ and ___ weakness in Guillain Barre Syndrome

Answer 77

bulbar and facial

Question 78

In GBS, this is a common complaint even after recovery

Answer 78

Fatigue

Question 78

Immune-mediated disorder of the peripheral nervous system

Answer 78

Chronic Inflammatory Demyelinating Polyneuropathy

Question 79

Hallmark presentations of hereditary sensory and motor neuropathy are ___ and distal leg atrophy, weakness, sensory loss and areflexia. Weakness is common to all types of HSMN.

Answer 79

peroneal

Question 79

Deformities in the foot are equinovarus, calcaneovarus, calcaneovalgus, and pes cavus due to contracture A. Lyme Disease B. Hereditary Sensory Motor Neuropathy C. Chronic inflammatory demyelinating polyneuropathy (CIDP) D. Guillain-Barre Syndrome

Answer 79

B. Hereditary Sensory Motor Neuropathy

Question 80

Clinical Criteria of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) 1. Central nervous system involvement 2. Symmetric proximal and distal weakness and sensory abnormalities in all extremities 3. Reduced or absent reflexes 4. Pure motor or sensory presentation A. Typical B. Atypical

Answer 80

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

Question 80

Clinical Criteria of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) 1. Progressive over 2 months, stepwise or recurrent 2. Predominantly distal findings 3. Cranial nerves can be affected 4. Asymmetric or focal presentations A. Typical B. Atypical

Answer 80

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

Question 80

Clinical Grading (MG foundation): Any ocular muscles weakness; may have weakness of eye closure; all other muscle strength is normal A. Class I B. Class II C. Class IIa D. Class IIb E. Class III

Answer 80

A. Class I

Question 81

Clinical Grading (MG foundation): Mild weakness predominantly affecting limb, axial muscles, or both; may have lesser involvement of oropharyngeal muscles A. Class I B. Class II C. Class IIa D. Class IIb E. Class III

Answer 81

C. Class IIa

Question 82

Clinical Grading (MG foundation): Mild weakness predominantly affecting oropharyngeal muscles, respiratory muscles, or both; may also have lesser or equal involvement of limb, axial muscles, or both A. Class I B. Class II C. Class IIa D. Class IIb E. Class III

Answer 82

D. Class IIb

Question 83

Clinical Grading (MG foundation): Moderate weakness predominantly affecting limb, axial muscles, or both; may have lesser involvement of oropharyngeal muscles A. Class IIIa B. Class IIIb C. Class IV D. Class IVa E. Class IVb

Answer 83

A. Class IIIa

Question 84

Clinical Grading (MG foundation): Moderate weakness predominantly affecting oropharyngeal muscles, respiratory muscles, or both; may also have lesser or equal involvement of limb, axial muscles, or both A. Class IIIa B. Class IIIb C. Class IV D. Class IVa E. Class IVb

Answer 84

B. Class IIIb

Question 85

Clinical Grading (MG foundation): Severe weakness predominantly affecting oropharyngeal muscles, respiratory muscles, or both; may also have lesser or equal involvement of limb, axial muscles, or both A. Class IIIa B. Class IIIb C. Class IV D. Class IVa E. Class IVb

Answer 85

E. Class IVb

Question 86

Clinical Grading (MG foundation): Severe weakness predominantly affecting limb, axial muscles, or both; may have lesser involvement of oropharyngeal muscles A. Class IIIa B. Class IIIb C. Class IV D. Class IVa E. Class IVb

Answer 86

D. Class IVa

Question 87

Dropped head syndrome is common in what 3 conditions?

Answer 87

Amyotrophic lateral sclerosis, poliomyelitis, & myasthenia gravis

Question 88

most frequently affected trunk muscles in myasthenia gravis

Answer 88

erector spinae

Question 88

Initial manifestation of myasthenia gravis

Answer 88

weakness of the levator palpebrae or extraocular muscles (ocular palsies, ptosis, weakness of eye closure, diplopia)

Question 89

asymmetrical weakness of several muscles in both eyes

Answer 89

diplopia

Question 90

TRUE OR FALSE: Myasthenic weakness increases throughout the day or with repeated use. Patients are stronger in the morning.

Answer 90

True

Question 91

Cardinal feature of myasthenia gravis

Answer 91

Myasthenia: fluctuating weakness of voluntary (skeletal) muscles

Question 91

Neuromuscular transmission is impaired in several ways in Myasthenia Gravis: 1. (1)___ block the binding of ACh to ACh receptors 2. (2)___ from myasthenic patients has been shown to induce an increase in the degradation rate of ACh receptors 3. Antibodies cause a complement-mediated destruction of the (3)___

Answer 91

1. Antibodies 2. Serum IgG 3. post synaptic folds

Question 92

TRUE OR FALSE: In myasthenia gravis, women are 2 to 3 times more affected under the age of 40 but later in life male incidence is higher.

Answer 92

True

Question 93

Peak age of onset in myasthenia gravis is between ___ to ___ years for women and between 50 to 60 years in men.

Answer 93

20 to 30

Question 94

Atypical Upper & Lower Motor Neuron Disorders 1. Hexosaminodase A deficiency 2. Lymphoma, Breast, Small cell, and Ovarian origin 3. Western Pacific ALS with Frontotemporal Dementia 4. Motor Neuron Disease associated with Electrical Injury A. Paraneoplastic B. Hereditary C. Trauma D. Sporadic

Answer 94

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

Question 95

A decline in forced vital capacity within the first ___ mos. after diagnosis of progressive muscular atrophy indicates a poor prognosis.

Answer 95

6

Question 96

Classic example of motor neuron disease because it has both manifestations of upper and lower motor neuron lesion

Answer 96

Amyotrophic Lateral Sclerosis (ALS)

Question 97

Initial Presentation of Amyotrophic Lateral Sclerosis (ALS)

Answer 97

Painless, asymmetric limb weakness

Question 98

Amyotrophic Lateral Sclerosis is a rapidly fatal progressive neurodegenerative disease leading to (1)___, (2)___, (3)___, and (4)___, eventually leading to death.

Answer 98

weakness, spasticity, muscular atrophy, and respiratory compromise

Question 99

Amyotrophic Lateral Sclerosis (ALS) is caused by the destruction of motor neurons in what 3 neurologic structures?

Answer 99

primary motor cortex, brain stem, and spinal cord

Question 100

Onset of Amyotrophic Lateral Sclerosis

Answer 100

Insidious

Question 101

Familial Amyotrophic Lateral Sclerosis results from the _____ gene defect.

Answer 101

copper-zinc superoxide dismutase (SOD1)

Question 102

TRUE OR FALSE: Familial Amyotrophic Lateral Sclerosis is autosomal dominant disease with some autosomal recessive, X-linked, and mitochondrial patterns reported.

Answer 102

True

Question 103

Anterior horn cell disease present with either UMN or LMN side during its slow, initial onset, then progresses to affectation of both UMN and LMN.

Answer 103

Juvenile Amyotrophic Lateral Sclerosis

Question 104

GBS Disability Scale: Minor symptoms and capable of running A. 0 B. 1 C. 2 D. 3 E. 4 F. 5 G. 6

Answer 104

B. 1

Question 105

Refers to vibration of the chest wall that results from sound vibrations created by speech or other vocal sounds. When a person speaks, airflow from the lungs causes the vocal cords in the larynx to vibrate.

Answer 105

Tactile fremitus / tactile vocal fremitus

Question 106

GBS Disability Scale: Healthy state A. 0 B. 1 C. 2 D. 3 E. 4 F. 5 G. 6

Answer 106

A. 0

Question 107

GBS Disability Scale: Bedridden or chairbound A. 0 B. 1 C. 2 D. 3 E. 4 F. 5 G. 6

Answer 107

E. 4

Question 108

GBS Disability Scale: Requiring assisted ventilation for at least part of the day A. 0 B. 1 C. 2 D. 3 E. 4 F. 5 G. 6

Answer 108

F. 5

Question 109

GBS Disability Scale: Able to walk 10 m across an open space with help A. 0 B. 1 C. 2 D. 3 E. 4 F. 5 G. 6

Answer 109

D. 3

Question 110

GBS Disability Scale: Able to walk 10 m or more without assistance but unable to run A. 0 B. 1 C. 2 D. 3 E. 4 F. 5 G. 6

Answer 110

C. 2

Question 111

Age range where Familial Amyotrophic Lateral Sclerosis is prevalent

Answer 111

30-40 y/o Additional: Progression of FALS is more rapid than SALS

Question 112

Age range where Sporadic Amyotrophic Lateral Sclerosis is prevalent

Answer 112

40-50 y/o

Question 113

Amyotrophic lateral sclerosis is more common in men than women. The incidence rate for Familial ALS is equal between men and women. A. Only the 1st statement is true B. Only the 2nd statement is true C. Both statements are true D. Both statements are false

Answer 113

C. Both statements are true

Question 114

Age onset of Juvenile Amyotrophic Lateral Sclerosis is before ___ y/o

Answer 114

25

Question 115

GBS Disability Scale: Dead A. 0 B. 1 C. 2 D. 3 E. 4 F. 5 G. 6

Answer 115

G. 6

Question 116

Age range where Amyotrophic Lateral Sclerosis is prevalent

Answer 116

40-60 y/o

Question 117

Determine if the clinical feature is true for GBS 1. Sensory loss is variable 2. Marked asymmetry 3. Reflexes are lost early in the disease 4. Bowel or bladder involvement at onset 5. Fever A. True B. False

Answer 117

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

Question 118

Determine if the clinical feature is true for GBS 1. Areflexia 2. Severe pulmonary involvement early, without significant weakness 3. Progressive ascending flaccid paralysis 4. Well-delineated sensory level A. True B. False

Answer 118

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

Question 119

Determine if the clinical feature is true for GBS 1. Progressive onset of limb weakness both proximally and distally that is typically symmetrical 2. Maximal weakness occurs within 4 weeks 3. Mild sensory symptoms A. True B. False

Answer 119

1. A 2. A 3. A

Question 120

Enumerate the clinical features that are required to be present for diagnosis of GBS

Answer 120

1. Progressive weakness in both arms and legs 2. Areflexia

Question 121

Used by the MD to measure the severity of polio

Answer 121

Sharrad's Index

Question 122

In HSMN, ambulation is impaired with higher incidences for falls. Effective support for balance and footwear should be noted during assessment of HSMN. A. Only the 1st statement is true B. Only the 2nd statement is true C. Both statements are true D. Both statements are false

Answer 122

C. Both statements are true

Question 123

TRUE OR FALSE: Diabetes mellitus, Systemic lupus erythematosus, HIV infection, and Thyroid disease are some of the diseases commonly associated with Chronic Inflammatory Demyelinating Polyneuropathy.

Answer 123

True

Question 124

Neuromuscular junction disorder where the eyelids and the muscles of eye movement are first to be affected. It also affects the face, jaw, throat and neck muscles.

Answer 124

Myasthenia gravis

Question 125

Isolated muscle groups may occasionally remain permanently weak even when the ocular and generalized weakness has resolved in myasthenia gravis. Identify these muscles.

Answer 125

anterior tibialis, triceps, and portions of the face

Question 126

In myasthenia gravis, the danger of (1)___ is greatest in the first year after onset due to respiratory complications of pneumonia and aspiration. The 2nd period of danger is (2)___ years after onset.

Answer 126

1. death 2. 4 to 7

Question 127

Lambert-Eaton myasthenic syndrome is observed most often in patients with ____ carcinoma of the lung.

Answer 127

Oat cell Additional: Small numbers have also occurred in carcinoma of the breast, prostate, stomach, rectum, and lymph nodes.

Question 128

TRUE OR FALSE: Muscles of the trunk, shoulder girdle, pelvic girdle, and lower extremities are the ones that become weak and fatigable in Lambert-Eaton myasthenic syndrome.

Answer 128

True

Question 129

1. First symptoms: difficulty in arising from a chair, climbing stairs, and walking; the shoulder muscles are usually affected later 2. Fasciculations are not seen 3. “Lid-twitch” sign 4. “Trident tongue” A. Myasthenia gravis B. Lambert-Eaton myasthenic syndrome

Answer 129

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

Question 130

Determine if the clinical feature is true for GBS 1. Increased mononuclear cell in CSF (>50 x 10^6/L) 2. Cranial nerve involvement 3. High protein concentration in the CSF 4. Polymorphonuclear cells in CSF 5. Muscles of respiration are frequently affected ± decline in vital capacity A. True B. False

Answer 130

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

Question 131

Determine if the clinical feature is true for GBS 1. Symmetry of symptoms 2. Autonomic system can be impaired ± tachycardia, hypertension, cardiac arrhythmia 3. Slow progression, no respiratory involvement 4. Pain (deep, aching, affecting the back, buttocks, and posterior thighs) 5. Severe sensory symptoms with minimal weakness A. True B. False

Answer 131

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

Question 132

1. Muscle atrophy is not a significant feature 2. Ancillary procedures: lumbar puncture, electrodiagnostic testing 3. Progression occurs over at least 2 months A. Guillain-Barre Syndrome B. Chronic inflammatory demyelinating polyneuropathy

Answer 132

1. B 2. A 3. B

Question 133

Lambert-Eaton Myasthenic syndrome is caused by a defect in the release of the Ach from the presynaptic nerve terminals with increased surface area of the (1)___; loss of voltage gated (2)___ channels on the presynaptic motor nerve terminal.

Answer 133

1. postsynaptic receptor membrane 2. calcium

Question 134

Hereditary adult-onset disease that causes preferential degeneration of LMNs, leading to weakness and atrophy of bulbar, facial, and limb muscles.

Answer 134

Spinal and Bulbar Muscular Atrophy Note: SBMA also presents with sensory impairment, endocrinologic disturbances, and decreased reflexes.

Question 135

Most common infectious agent causing the antecedent infection 1-3 weeks before onset of weakness in GBS

Answer 135

campylobacter jejuni

Question 136

Spinal and Bulbar Muscular Atrophy is not associated with abnormalities in the SMN gene. It is caused by a novel mutation -> the expansion of trinucleotide, cytosine-adenine-guanine (CTG) repeat in the first exon of the androgen receptor gene. A. Only the 1st statement is true B. Only the 2nd statement is true C. Both statements are true D. Both statements are false

Answer 136

C. Both statements are true

Question 137

El Escorial Criteria: LMN + UMN signs in 3 anatomical regions A. Possible ALS B. Probable ALS laboratory suspected C. Probable ALS D. Definite ALS E. Definite Familial ALS laboratory suspected

Answer 137

D. Definite ALS

Question 138

El Escorial Criteria: LMN + UMN signs in 2 anatomical regions A. Possible ALS B. Probable ALS laboratory suspected C. Probable ALS D. Definite ALS E. Definite Familial ALS laboratory suspected

Answer 138

C. Probable ALS

Question 139

El Escorial Criteria: LMN + UMN signs in 1 anatomical region or UMN signs in ≥ 1 region. EMG shows acute denervation ≥ 2 limbs. A. Possible ALS B. Probable ALS laboratory suspected C. Probable ALS D. Definite ALS E. Definite Familial ALS laboratory suspected

Answer 139

B. Probable ALS laboratory suspected

Question 140

El Escorial Criteria: LMN + UMN signs in 1 anatomical region. A. Possible ALS B. Probable ALS laboratory suspected C. Probable ALS D. Definite ALS E. Definite Familial ALS laboratory suspected

Answer 140

A. Possible ALS

Question 141

El Escorial Criteria: LMN + UMN signs in 1 anatomical region with identified DNA gene. A. Possible ALS B. Probable ALS laboratory suspected C. Probable ALS D. Definite ALS E. Definite Familial ALS laboratory suspected

Answer 141

E. Definite Familial ALS laboratory suspected

Question 142

TRUE OR FALSE: Poliomyelitis presents with dysautonomia and asymmetric weakness and atrophy in legs more than the arms or bulbar muscles. The sensory system is not affected.

Answer 142

True Note: Dysautonomia may present as having labile blood pressure, cardiac arrhythmia, and GI and urinary dysfunction.

Question 143

Disease spreads insidiously from the cranial to the limb and axial muscles in this neuromuscular junction disorder.

Answer 143

Myasthenia gravis

Question 144

Sensory involvement typically affects large fiber nerves (vibration and proprioception) in chronic inflammatory demyelinating polyneuropathy. The sensory symptoms generally progress from distal to proximal, although hand involvement is often perceived as early as the in the feet. A. Only the 1st statement is true B. Only the 2nd statement is true C. Both statements are true D. Both statements are false

Answer 144

C. Both statements are true

Question 145

Characterized as a symmetric neuropathy that affects motor function predominantly and both proximal and distal muscles are affected.

Answer 145

Chronic inflammatory demyelinating polyneuropathy