Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

(3) Neurological Examination > S3_L2: Lower Motor Neuron Diseases > Flashcards

S3_L2: Lower Motor Neuron Diseases Flashcards

1
Q

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

A

Lower motor neuron

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q
  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)

A
  1. B
  2. A
  3. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Most common presenting symptom of Progressive Lateral Sclerosis (PLS)

A

spasticity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

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

A
  1. spinal cord
  2. corticospinal
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

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

A
  1. A
  2. B
  3. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q
  1. Lack of familial inheritance
  2. Sporadic; Etiology is unknown

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

A
  1. A
  2. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

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

A

Progressive Lateral Sclerosis (PLS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

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

A
  1. Amyotrophic Lateral Sclerosis (ALS)
  2. 4
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q
  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

A
  1. A
  2. B
  3. B
  4. A
  5. C
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q
  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

A
  1. B
  2. A
  3. B
  4. B
  5. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q
  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

A
  1. B
  2. A
  3. A
  4. A
  5. A
  6. C
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q
  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

A
  1. B
  2. A
  3. C
  4. A
  5. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q
  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

A
  1. B
  2. B
  3. A
  4. B
  5. B
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q
  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

A
  1. A
  2. B
  3. A
  4. A
  5. B
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q
  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

A
  1. B
  2. A
  3. A
  4. B
  5. B
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

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

A
  1. C
  2. B
  3. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

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

A
  1. C
  2. A
  3. B
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

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

A
  1. A
  2. D
  3. B
  4. C
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

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

A
  1. A
  2. B
  3. A
  4. C
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

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

A

SMA 2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

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

A
  1. B (CMT 2)
  2. D
  3. A (CMT 1b)
  4. C
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

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

A
  1. C
  2. D
  3. A
  4. B
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

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

A
  1. C
  2. A (CMT 1a)
  3. C
  4. B (as compared to HSMN 1)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

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

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

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

A

SMA I, II, and III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

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

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

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

A

SMA III and IV

Additional: many SMA II patients are now living into adulthood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

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

A

“Lid-twitch” sign

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

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

A

“Trident tongue” (Triple furrow tongue)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

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

A
  1. Bulbar
  2. Cervical
  3. Thoracic
  4. Lumbosacral (Lumbar)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Nerve cell for processing motor information

A

Motor neuron

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Hallmark of motor neuron diseases

A

Weakness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Cardinal sign of Amyotrophic Lateral Sclerosis (ALS)

A

Muscle weakness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q
  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

A
  1. A
  2. B
  3. B
  4. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

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

A

Fifth layer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q
  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

A
  1. C
  2. A
  3. C
  4. B
  5. C
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q
  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

A
  1. A
  2. C
  3. A
  4. A
  5. C
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q
  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

A
  1. B
  2. C
  3. C
  4. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

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

A
  1. B
  2. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

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

A
  1. A
  2. C
  3. C
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Juvenile Amyotrophic Lateral Sclerosis Types

  1. ALS2
  2. ALS4
  3. ALS5

A. Autosomal dominant
B. Autosomal recessive
C. Both
D. Neither

A
  1. B
  2. A
  3. B
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

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

A

Lyme disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

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

A

patchy axonal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

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

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

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

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Chromosome affectation in Spinal Muscular Atrophy (SMA)

A

Chromosome 5q11.2-13.3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

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.

A

4-7

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

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

A

38-47

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

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

A

Post-polio Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

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.

A

Neural fatigue theory

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

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

A

Neostigmine test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Pharmacologic test used to help the MD diagnose myasthenia gravis

A

Edrophonium test (Tensilon test)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

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.

A

2-5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

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

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

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.

A

Limb

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Also known as Lou Gehrig’s disease

A

Amyotrophic Lateral Sclerosis (ALS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q
  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)

A
  1. C
  2. B
  3. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q
  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)

A
  1. A
  2. C
  3. B
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q
  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)

A
  1. C
  2. A
  3. B
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q
  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)

A
  1. A
  2. C
  3. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q
  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

A
  1. A
  2. C
  3. B
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q
  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

A
  1. C
  2. B
  3. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q
  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

A
  1. C
  2. C
  3. A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Enumerate the genes affected in Spinal Muscular Atrophy (SMA)

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

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

A

True

57
Q

Also known as Kennedy Disease

A

Spinal and Bulbar Muscular Atrophy (SBMA)

57
Q

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

A

Onuf nucleus

58
Q

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

A

enterovirus

59
Q

Transmission of poliomyelitis

A

Fecal-oral route

60
Q

type of poliomyelitis most often associated with paralytic disease

A

Type 1 (Brunhilde virus)

61
Q

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.

A

Polio gait

62
Q

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

A

True

63
Q

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
A
  1. motor neuron
  2. 15
  3. 12 (1 year)
64
Q

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

A

Guillain-Barre Syndrome

65
Q

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

A

progressive, symmetrical

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

66
Q

Most common presenting symptom of Progressive Muscular Atrophy

A

distal limb weakness with muscle atrophy

67
Q

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.

A

False

Arms > Legs

68
Q

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%.

A

56

69
Q

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

A

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

70
Q

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.

A

True

71
Q

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

A
  1. A
  2. B
  3. A
  4. A
72
Q

Risk factors: Sporadic Amyotrophic Lateral Sclerosis

  1. Cigarette smoking
  2. Increased ___ consumption
A

glutamate

73
Q

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

A

ambulate

74
Q

Longest surviving patient with ALS

A

Stephen Hawking

75
Q

Enumerate the CN not commonly affected in ALS

A

CN 3, 4, & 6 (oculomotor muscles)

76
Q

Enumerate the CN affected in ALS

A

CN V, VII, IX, X, and XII

77
Q

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

A

bulbar and facial

78
Q

In GBS, this is a common complaint even after recovery

A

Fatigue

78
Q

Immune-mediated disorder of the peripheral nervous system

A

Chronic Inflammatory Demyelinating Polyneuropathy

79
Q

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.

A

peroneal

79
Q

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

A

B. Hereditary Sensory Motor Neuropathy

80
Q

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

A
  1. B
  2. A
  3. A
  4. B
80
Q

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

A
  1. A
  2. B
  3. A
  4. B
80
Q

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

A

A. Class I

81
Q

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

A

C. Class IIa

82
Q

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

A

D. Class IIb

83
Q

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

A

A. Class IIIa

84
Q

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

A

B. Class IIIb

85
Q

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

A

E. Class IVb

86
Q

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

A

D. Class IVa

87
Q

Dropped head syndrome is common in what 3 conditions?

A

Amyotrophic lateral sclerosis, poliomyelitis, & myasthenia gravis

88
Q

most frequently affected trunk muscles in myasthenia gravis

A

erector spinae

88
Q

Initial manifestation of myasthenia gravis

A

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

89
Q

asymmetrical weakness of several muscles in both eyes

A

diplopia

90
Q

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

A

True

91
Q

Cardinal feature of myasthenia gravis

A

Myasthenia: fluctuating weakness of voluntary (skeletal) muscles

91
Q

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)___

A
  1. Antibodies
  2. Serum IgG
  3. post synaptic folds
92
Q

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.

A

True

93
Q

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

A

20 to 30

94
Q

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

A
  1. B
  2. A
  3. D
  4. C
95
Q

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

A

6

96
Q

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

A

Amyotrophic Lateral Sclerosis (ALS)

97
Q

Initial Presentation of Amyotrophic Lateral Sclerosis (ALS)

A

Painless, asymmetric limb weakness

98
Q

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

A

weakness, spasticity, muscular atrophy, and respiratory compromise

99
Q

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

A

primary motor cortex, brain stem, and spinal cord

100
Q

Onset of Amyotrophic Lateral Sclerosis

A

Insidious

101
Q

Familial Amyotrophic Lateral Sclerosis results from the _____ gene defect.

A

copper-zinc superoxide dismutase (SOD1)

102
Q

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

A

True

103
Q

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.

A

Juvenile Amyotrophic Lateral Sclerosis

104
Q

GBS Disability Scale: Minor symptoms and capable of running

A. 0
B. 1
C. 2
D. 3
E. 4
F. 5
G. 6

A

B. 1

105
Q

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.

A

Tactile fremitus / tactile vocal fremitus

106
Q

GBS Disability Scale: Healthy state

A. 0
B. 1
C. 2
D. 3
E. 4
F. 5
G. 6

A

A. 0

107
Q

GBS Disability Scale: Bedridden or chairbound

A. 0
B. 1
C. 2
D. 3
E. 4
F. 5
G. 6

A

E. 4

108
Q

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

A

F. 5

109
Q

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

A

D. 3

110
Q

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

A

C. 2

111
Q

Age range where Familial Amyotrophic Lateral Sclerosis is prevalent

A

30-40 y/o

Additional: Progression of FALS is more rapid than SALS

112
Q

Age range where Sporadic Amyotrophic Lateral Sclerosis is prevalent

A

40-50 y/o

113
Q

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

A

C. Both statements are true

114
Q

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

A

25

115
Q

GBS Disability Scale: Dead

A. 0
B. 1
C. 2
D. 3
E. 4
F. 5
G. 6

A

G. 6

116
Q

Age range where Amyotrophic Lateral Sclerosis is prevalent

A

40-60 y/o

117
Q

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

A
  1. A
  2. B
  3. A
  4. B
  5. B
118
Q

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

A
  1. A
  2. B
  3. A
  4. B
119
Q

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

A
  1. A
  2. A
  3. A
120
Q

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

A
  1. Progressive weakness in both arms and legs
  2. Areflexia
121
Q

Used by the MD to measure the severity of polio

A

Sharrad’s Index

122
Q

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

A

C. Both statements are true

123
Q

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.

A

True

124
Q

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.

A

Myasthenia gravis

125
Q

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

A

anterior tibialis, triceps, and portions of the face

126
Q

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.

A
  1. death
  2. 4 to 7
127
Q

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

A

Oat cell

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

128
Q

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.

A

True

129
Q
  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

A
  1. B
  2. B
  3. A
  4. A
130
Q

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

A
  1. B
  2. A
  3. A
  4. B
  5. A
131
Q

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

A
  1. A
  2. A
  3. B
  4. A
  5. B
132
Q
  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

A
  1. B
  2. A
  3. B
133
Q

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.

A
  1. postsynaptic receptor membrane
  2. calcium
134
Q

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

A

Spinal and Bulbar Muscular Atrophy

Note: SBMA also presents with sensory impairment, endocrinologic disturbances, and decreased reflexes.

135
Q

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

A

campylobacter jejuni

136
Q

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

A

C. Both statements are true

137
Q

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

A

D. Definite ALS

138
Q

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

A

C. Probable ALS

139
Q

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

A

B. Probable ALS laboratory suspected

140
Q

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

A

A. Possible ALS

141
Q

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

A

E. Definite Familial ALS laboratory suspected

142
Q

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.

A

True

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

143
Q

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

A

Myasthenia gravis

144
Q

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

A

C. Both statements are true

145
Q

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

A

Chronic inflammatory demyelinating polyneuropathy

(3) Neurological Examination (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions