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Neuropathology > Lecture 2: Lower Motor Neuron Disorders > Flashcards

Lecture 2: Lower Motor Neuron Disorders Flashcards

1
Q

Weakness is exclusive to …
A. UMN lesions
B. LMN lesions
C. Both A and B

A

C. Both A and B

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2
Q

How does UMN sensory loss appear?

A

Adjacent Body Regions

Spinal Cord: Dermatomal Pattern

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3
Q

LMN signs of sensory loss present as?

A
  1. Spinal nerve roots: Dermatomal pattern
  2. Peripheral nerve pattern
  3. Stocking Glove
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4
Q

Cranial nerve involvement would be classified as
A. UMN
B. LMN
C. BOTH

A

B. LMN, Cranial nerves are exclusive to the peripheral nervous system. Except for Cranial nerve 2, which has its myelin sheet covered with oligodendrocytes.

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5
Q

Vision, Language, Cognition, Cerebellar, Basal Ganglia, and Corticobulbar abnormalities are involved with
A. UMN
B. LMN
C. BOTH

A

A. UMN

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6
Q

ALS, MG, and MD are …
A. Motor disorders
B. Sensory disorders
C. Both

A

A. Motor Disorders, No sensory involvement

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7
Q

UMN’s control the body with what nerve tract?

A

LCST

Lateral Corticospinal tract

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8
Q

UMNs control the face and head via what nerve tract?

A

Corticobulbar tract (CBT)

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9
Q

LMN Nuclei control the body via what tract?

A

Anterior horn cell in spinal cord

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10
Q

LMN Nuclei control the face and head motor via what tract?

A

Cranial nerve motor nuclei (Trigeminal, Facial, Ambiguus, Spinal Accessory, Hypoglossal)

**Reminder–CN 3, 4, and 6 are not part of the corticobulbar tract.

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11
Q

Which cranial nerves are not part of the Corticobulbar tract?

A

Cranial nerves 3, 4, and 6. These nerves are responsible for eye movement

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12
Q

LMN Axons controls he body via what tract?

A

Spinal nerve roots to Peripheral nerve roots

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13
Q

LMN Axons control the face and head motor via what tract?

A

Cranial nerves 5, 7, 9, 10, 11, 12

Intracranial segment, extra-cranial segment

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14
Q

LMN terminals controls the body motor via what tract?

A

Neuromuscular junction => Muscle

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15
Q

LMN Terminals control the face and head motor via what tract?

A

Neuromuscular junction => Muscle

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16
Q

What are some UMN signs for body motor involvement?

A
  1. Hyperreflexia
  2. Hypertonic
  3. Weakness
  4. (+) Babinski
  5. (+) Hoffmann;s
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17
Q

What are some face and head motor signs for UMN Involvement?

A

Hyperreflexia (Jawjerk, gag reflex)

Weakness

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18
Q 
Name the Cranial Nerves
I
II
III
IV
V
VI
VII
VIII
IX
X
XI
XII
A 
I Olfactory
II Optic
III Oculomotor
IV Trochlear
V Trigeminal
VI Abducens
VII Facial
VIII Vestibulochoclear
IX Glossopharyngeal
X Vagus
XI Spinal Accessory 
XII Hypoglossal
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19
Q

This nerve is responsible for tongue movement

A

CN XII Hypoglossal

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20
Q

This nerve is responsible for head turning

A

CN XI Spinal Accessory

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21
Q

This nerve is responsible for parasympathetic to most organs, Laryngeal muscles (voice) pharyngeal muscles (swallowing) and aortic arch reflexes

A

CN X Vagus

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22
Q

This nerve is responsible for pharyngeal muscles; carotid body reflexes; and salivation

A

CN IX Glossopharyngeal

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23
Q

This nerve is responsible for hearing and equilibrium sense

A

CN VIII Vestibulochochlear

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24
Q

This nerve is responsible for muscles of facial expression, taste, lacrimation, and salivation

A

CN VII Facial

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25
Q

This nerve is responsible for eye movements

A

CN III, IV, VI

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26
Q

This nerve is responsible for facial sensation; and muscles of mastication

A

CN V Trigeminal

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27
Q

This nerve is responsible for eye movements and pupil constriction

A

CN III Oculomotor

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28
Q

This nerve is responsible for scent/Olfaction

A

CN I Olfactory N

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29
Q

Classification of the four types of motor unit disorders is based on the part of the motor unit that is affected. What are the 4 regions?

A
  1. Primary muscle disease (Myopathies)
  2. Diseases of the Neuromuscular Junction
  3. Peripheral neuropathies (Axon and Myelin)
  4. Motor Neuron Disease (Cell Body)
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30
Q

Motor neuron diseases can be acquired of inherited.

Polio is an example of an?

A

Acquired infectious disease

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31
Q

Motor neuron diseases can be acquired of inherited.

Polymyositis is an example of an?

A

Acquired Autoimmune disease

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32
Q

Motor neuron diseases can be acquired of inherited.

ALS and PLS are examples of an?

A

Acquired Idopathic Disease

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33
Q

Motor neuron diseases can be acquired of inherited.

SMA is an example of?

A

Inherited LMN Disease

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34
Q

Motor neuron diseases can be acquired of inherited.

Hereditary spastic paraparesis is an example of?

A

Inherited UMN Disease

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35
Q

Motor neuron diseases can be acquired of inherited.

Familial ALS is an example of?

A

Inherited UMN and LMN Disease

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36
Q

ALS Stands for

A

A-Myo-Tropic Lateral Sclerosis

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37
Q

Breaking down Amyotropic lateral sclerosis.

A: 
Myo: 
Tropic: 
Lateral: 
Sclerosis:
A

-A: Means no or negative
-Myo: Refers to muscle
-Trophic: means nourishment
(No Muscle Nourishment)

Lateral: Is the area in the spine where the brain tells the muscles what to do

Sclerosis: Is hardening as the disease progresses. The lateral areas harden and the signals stop.

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38
Q

This is the most common adult motor neuron disease

A

ALS

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39
Q

What is the prevalence of ALS?

A

1-2 per 100,000 people

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40
Q

How many cases of ALS are documented in the US per year?

A

5000

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41
Q

Male to female ratio of ALS?

A

1.5 Males : 1 Female

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42
Q

2/3 of ALS patients experience
A. Spinal onset
B. Bulbar Onset
C. Both

A

A. Spinal Onset

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43
Q

1/3 of ALS patients experience
A. Spinal Onset
B. Bulbar Onset
C. Both

A

B. Bulbar Onset

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44
Q

What is true about the etiology of ALS?

A

Mostly unknown.

Sporadic/Unknown cause

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45
Q

What is the mean age of Developing ALS sporadically/unknown?

A

60 years

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46
Q

What percent of ALS patients acquire the disease genetically?

what is the age of onset?

A

5%

40 years

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47
Q

What are the 6 contributing factors that lead to ALS?

A
  1. SOD1 Mutations
  2. Oxidative Damage
  3. Aggregated Proteins
  4. Abnormal Mitochondria
  5. Excitoxicity
  6. TDP-43 Abnormal Astroglia
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48
Q

With ALS, what happens to the motor neuron?

A

Degeneration. Damage to motor neuron cell body in the Spinalcord/Brainstem/cortex

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49
Q

With ALS, what damages the motor axon?

A

Wallerian Degeneration

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50
Q

With ALS, some nuclei are usually spared, these include?

A
  1. Oculomotor
  2. Trochlear
  3. Abducens
  4. Nucleus of Onuf (Bowel And Bladder)
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51
Q

ALS is
A. Insidious
B. Known cause
C. Stable

A

A. Insidious

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52
Q

Describe the disease course of ALS

A
  1. Subtle signs: Loss of dexterity, stiffness, cramps, fatigue.
  2. Weakness, Slurred Speech, Atrophy, Spasticity
  3. Speech, swallowing, respiration involved
  4. Dependent for mobility and ADL’s
  5. AVG 3-5 year progression to death.
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53
Q

ALS presents with
A. UMN signs
B. LMN signs
C. Both

A

C. UMN and LMN signs

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54
Q

Between UMN and LMN involvement with ASL, what is spared from the disease?

A
  1. Extra ocular muscles
  2. Cognitive function
  3. Sensory Function
  4. Bowel and Bladder
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55
Q

Describe the Spinal form onset of ALS? 2/3 of cases

A
  1. Limb Onset
  2. Starts distally or proximally
  3. Usually asymmetrical early on
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56
Q

Describe the onset of bulbar form ALS

A
  1. Dysarthria and dysphagia

2. Simultaneous limb symptoms

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57
Q

This disease is a combination of UMN and LMN signs, typically asymmetric early on.

A

ALS

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58
Q

What are the spared components to ALS disease?

A
  1. Extraocular
  2. Cognitive
  3. Sensory
  4. Bowl/Bladder
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59
Q

What type of imaging would you get for ALS diagnosis?

A

MRI: R/O cortical, Brainstem, or cervical spine pathology

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60
Q

Why would you order lumbar puncture diagnosis for a patient with ALS?

A

To rule out Inflammatory causes

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61
Q

Why would you order a blood test for a patient with ALS?

A

R/O Toxic, metabolic, infections, inflammatory, and genetic causes

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62
Q

What are the 2 categories of testing with ENMG (Electroneuromyography)

A

NCV: Nerve conduction velocity
EMG: Electromyography

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63
Q

Why would you order NCV tests for a patient with ALS?

A
  1. To test for “Decreased compound motor unit action potential (CMAP)
  2. To test for “Normal or slow nerve conduction velocity (NCV)
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64
Q

During the acute phases of ALS, what is true of a nerve conduction velocity study?

A

Early on, the results might be normal. Need to wait for later progression of the disease to determine NCV.

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65
Q

Why would you order an EMG for a patient with ALS Disease? (3)

A

To look for…

  1. Fibrillations and (+) Sharp Waves
  2. Fasciculations
  3. Interference pattern reduced (Partial or Sparse)
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66
Q

ALS is a UMN or LMN Disorder?

A

Both

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67
Q

Key differential diagnosis for ALS,

UMN/LMN Disorders:

UMN Disorders:

LMN Disorders:

A

UMN/LMN Disorders: Brainstem/Spinal cord

UMN Disorders: Hereditary Disorders

LMN Disorders: Neuropathies/Myopathies

68
Q

What is the only FDA approved medicine for medical management of ALS?

A

Riluzole: 100mg/day

69
Q

For patients with ALS, what are the symptomatic managements?

A
  1. Sialorrhea (Excessive salivation)
  2. Muscle cramps
  3. Spasticity
  4. Dyspnea
  5. Depression
70
Q

For patients with ALS, What medical management must be considered for swallowing?

A
  1. Food modification

2. PEG: Percutaneous endoscopic gastrostomy tube

71
Q

For patients with ALS, what form of respiratory care is needed for them?

A
  1. Mechanical Ventilation

2. Pulmonary Function Tests

72
Q

Which form of ALS has a death rate within 2-3 years from respiratory failure?
A. Spinal form
B. Bulbar Form
C. Both

A

B. Bulbar Form 2-3 years till death

73
Q

Which form of ALS has a death rate due to respiratory failure at 3-5 years?
A. Spinal form
B. Bulbar Form
C. Both

A

A. Spinal form 3-5 years

74
Q

What percent of patients with ALS life greater than 10 years?

A

10%

75
Q

What are the prognostic indicators for ALS disease?

A
  1. Younger onset => Longer Duration

2. Early respiratory dysfunction => Poor Prognosis

76
Q

Clinical Management

What do you want to look for with a patient who may be presenting with ALS?

(5)

A
  1. UMN and LMN signs
  2. Sensory system intact
  3. No Bowl/Bladder Involvement
  4. Cognitive function still intact.
  5. Respiratory complications
77
Q

Clinical Management

What are the 4 ays therapists can intervene with a patient who presents with ALS?

A
  1. Explain the Psychological benefits associated with exercise
  2. Energy conservation and safety
  3. Caregiver training
  4. Equipment
78
Q

There are 4 distinct neuroanatomical regions of the muscle fibers and the motor unit attached to it.

Motor neuron diseases effect the _____________

Peripheral neuropathies effect the ____________

Diseases of the post/pre synaptic cleft effect the ____________

Diseases that effect the muscles are known as ____________

A

Motor neuron diseases effect the Cell Body

Peripheral neuropathies effect the Axon and Myelin

Disease of the Post/pre synaptic cleft effect the NMJ

Diseases that effect the muscles are known as Myopathies

79
Q

Myasthenia Gravis is a disorder that effects what region of the muscle?

A

The Neuromuscular Junction

80
Q

What is the most common neuromuscular junction disorder?

A

Myasthenia Gravis

81
Q

How many people develop Myasthenia Gravis?

A

1 in 10,000-20,000 people

82
Q

What age are women more commonly effected by Myasthenia Gravis?

A

20-30 years of age

83
Q

What age of men more commonly effected by Myasthenia Gravis?

A

50-60 years of age

84
Q

Myasthenia Gravis an … disease
A. Acquired
B. Inherited
C. Both

A

Acquired disease

85
Q

What is the etiology of Myasthenia Gravis disease?

A

Acquired Antibody-Mediated Autoimmune disease

86
Q

Myasthenia Gravis is an acquired antibody-mediated autoimmune disease. What percentage of people have …

Nicotonic Acetylcholine Receptor (AChR) antibodies?

A

90%

87
Q

Myasthenia Gravis is an acquired antibody-mediated autoimmune disease. What percentage of people have…

AChR antibodies in the ocular form?

A

50%

88
Q

What happens at the synaptic cleft with Myasthenia Gravis?

A

Post-synaptic receptor

Damage to the membrane

89
Q

What is happening at the synaptic cleft that is inhibiting the binding of Acetylcholine to the Acetylcholine receptors?

A

AChR Antibodies are binding to the ACh receptors, inhibiting the connection of ACh Neurotransmitters to the receptors.

90
Q

What is true of the disease course for Myasthenia Gravis?

A
  1. Insidious
  2. Slowly progressive initially and then stable
  3. May include episodic exacerbations and remissions.
91
Q

Describe the disease course of Myasthenia Gravis

A
  1. Subtle Onset
  2. Potential exacerbation with illness, pregnancy
  3. Fluctuating weakness and fatiguability with full body involvement within one year
  4. Progression to maximum severity in first 2 years.
92
Q

What is the general pattern of weakness for Myasthenia Gravis?

A

Proximal to Distal

Arms > Legs

Quads, Triceps, and Neck extensors more

93
Q

What are 6 contributing factors for fluctuating fatigue in patients with Myasthenia Gravis?

A
  1. Ptosis
  2. Diploplia
  3. Dysarthria
  4. Dysphagia
  5. Respiratory Weakness
  6. Limb Weakness
94
Q

What is the pattern and Spreading of Weakness for AChR MG?

A

Head to Lower extremities

95
Q

What is the Pattern and Spreading of weakness for MuSK MG?

A

Thoracic region down to lower extremity

96
Q

What is the pattern and Spreading of weakness for LEMS?

A

Thighs to upper extremities

97
Q

What are the 4 diagnostic tests to rule in/out Myasthenia Gravis?

A
  1. Tensilon
  2. Serologic Testing
  3. Slow repetitive nerve stimulation
  4. CT/MRI
98
Q

What are we looking for with a Tensilon test for Myasthenia Gravis?

A
  1. Positive Tensilon Test (A person is positive if after the injection, they are able to produce stronger muscle contractions) (This is preventing the breakdown of the chemical acetylcholine)
  2. Clinicall response within 30-60 seconds
  3. High sensitivity, Not specific to MG
  4. Risk: Cardiac Side Effect
99
Q

This drug is for patients with Myasthenia Gravis and it increases the concentration of Ach at the receptor.

A

Cholinesterase

100
Q

What are the side effects associated with Cholinesterase Inhibitors?

A
  1. Muscarinic

2. Nicotinic

101
Q

This is a side effect from Cholinesterase Inhibitors that causes diarrhea, cramping, and excessive secretions

A

Muscarinic

102
Q

This is a side effect from Cholinesterase Inhibitors that causes muscle fasciculations, and cholinergic crisis

A

Nicotinic

103
Q

What is a short term medical management for Myasthenia Gravis?

A
  1. Plasmapheresis

2. IVIG

104
Q

What are the benefits of taking Plasmapheresis and IVIG with Myasthenia Gravis?

A
  1. Clinical improvement within first week
  2. Benefits last for 1-2 months
  3. Complications uncommon
  4. Primarily used during Myasthenia crisis or to stabilize a patient prior to Thymectomy
105
Q

This is a medical management protocol for patients with Myasthenia Gravis to treat thymectomy

A

Immunosuppressive Corticosteroids

106
Q

What is the prognosis with Myasthenia Gravis?

A

80% of patients with focal disease eventually develop generalized MG

107
Q

Within _______ years of onset for Myasthenia Gravis, progression to maximal severity occurs

A

2 years

108
Q

Patients with Myasthenia Gravis with __________ have more aggressive disease course

A

Thymomas

109
Q

What is the lifespan of a patient with Myasthenia Gravis?

A

Normal life span

110
Q

What are the side effects associated with Myasthenia Gravis?

A
  1. Decreased quality of life
    - Limited efficacy and side effects of medication
    - Non-medicinal treatment
111
Q

Clinical management of Myasthenia Gravis

What are we trying to identify with the disease and clinicians?

A

Look for signs and symptoms

  1. Fluctuating and Fatiguable weakness
  2. Proximal > distal weakness
  3. UE > LE
    - Triceps, Quadriceps, neck extensors
  4. Bilateral asymmetric ocular muscle weakness
    - Pupillary sparing
  5. Nasal quality in voice
112
Q

With Myasthenia Gravis, how does the weakness generally present?

A

Proximal Greater than Distal Weakness

113
Q

With Myasthenia Gravis, is the UE or LE more effected?

A

UE effected > LE

114
Q

What are the major muscles mainly effected by Myasthenia Gravis? (3)

A
  1. Triceps
  2. Quadriceps
  3. Neck Extensors
115
Q

What is true of the ocular muscles with Myasthenia Gravis?

A

Bilateral Asymmetric Ocular muscle weakness

116
Q

What the ocular Bilateral asymmetric muscle weakness associated with Myasthenia Gravis, what is spared?

A

Pupillary function

117
Q

What happens to an individuals voice with Myasthenia Gravis?

A

Nasal Quality of voice is noticed

118
Q

Clinical Management of Myasthenia Gravis.

What are 2 things clinicians should be mindful of when working with these patients with respect to intervention?

A
  1. Consider Fatigue

2. Energy Conservation/Modification of tasks

119
Q

This is a Disorder with primary structural or functional impairments of the muscles.

A

Myopathy

120
Q

What are the signs and symptoms associated with Myopathy? (4)

A

Muscle weakness and Fatigability

  1. Generally proximal greater than Distal
  2. Low Tone
  3. Atrophy
  4. Pseudo Hypertrophy
121
Q

What is true about the sensation of a patient with Myopathy?

A

Sensation is intact

122
Q

What is true of sphincter function with Myopathy?

A

Normal Sphincter function

123
Q

What are the two ways someone can get Myopathy?

A
  1. Heredity

2. Acquired

124
Q

For the hereditary development of Myopathy, what are the (5) different ways?

A
  1. Congenital Myopathies
  2. Muscular dystrophies
  3. Myotonias & Channelopathies
  4. Primary metabolic myopathies
  5. Mitochondrial Myopathies
125
Q

For the acquired development of myopathy, what are the 5 ways individuals can attain this?

A
  1. Inflammatory myopathies
  2. Drug-induced and toxic
  3. Secondary metabolic myopathies
  4. Endocrine Myopathies
  5. Infectious myopathies
126
Q

What is the gold standard for assessing Myopathies?

A

Muscle Biopsy (Gold Standard for Myopathy)

127
Q

What is the most common and severe form of childhood muscular dystrophy?

A

Duchenne Muscular Dystrophy

128
Q

What is the proportion of children born with Duchenne’s muscular dystrophy?

A

1 in 3,500 male births

129
Q

What is the etiology of Duchenne’s muscular dystrophy

A
  1. Hereditary disease
    X-Linked Recessive
    30% spontaneous mutation
130
Q

What is the pathogenesis of Duchenne’s muscular dystrophy?

A
  1. Slowly progressive muscular weakness with histologic abnormalities
  2. Fibrosis, degeneration and regeneration of muscle, Proliferation of fatty and connective tissue
  3. Decrease in Dystrophin
131
Q

Patient’s who suffer from Duchenne’s muscular dystrophy have a decrease in __________

A

Dystrophin

132
Q

What is the disease course of Duchenne’s Muscular dystrophy?

A
  1. Insidious

2. Slowly progressive weakness

133
Q

Describe the disease course for Duchenne’s Muscular Dystrophy

A

Insidious, slowly progressive weakness

  1. Normal At birth
  2. Delayed motor milestones after 1 year
  3. 3-7 years old: Toe walking, towers maneuver, lordosis, scoliosis
  4. 7-12 years old: Lose ability to walk
  5. 20 years old: Death from fatty infiltration of heart and respiratory infections
134
Q

What are the clinical findings associated with Duchenne’s muscular dystrophy? (3)

A
  1. Proximal weakness > Distal
  2. Pseudohypertophy of the calves
  3. Gower’s maneuver
135
Q

Children with Duchenne’s muscular dystrophy experience what with respect to the musculature/ cardiovascular endurance

A

Easy Fatigability

136
Q

As Duchenne’s muscular dystrophy progresses, what clinical findings can be seen in the child? (7)

A
  1. Contracture
  2. Scoliosis/Lordosis
  3. Obesity
  4. Decreased Respiratory capacity
  5. Cognitive Defects
  6. Cardiac Dysfunction
  7. GI Dysfunction
137
Q

When diagnosing Duchenne’s muscular dystrophy, what clinical examination can a PT perform?

A

Assess for Progressive muscle weakness

-(Gower’s sign, excessive lordosis)

138
Q

When diagnosing Duchenne’s muscular dystrophy, what 4 diagnostic tests can be performed?

A
  1. Clinical examination looking for progressive muscle weakness
  2. Serum Enzyme levels: Elevated Creatine Kinase (50-100x normal values)
  3. Muscle Biopsy: looking for variation in size of muscle fibers, fat and connective tissue deposits, and dystrophin reducing or absence
  4. Genetic Testing: Defect in Dystrophin gene (on Chromosome Xp21)
139
Q

What is the medical management protocol with a patient suffering from Duchenne’s muscular dystrophy? (4)

A

Achieve and maintain a higher quality of life than expected for the disease process

  1. Slowing rate of progression
  2. Preventing/minimizing contracture and deformity
  3. Maintaining function and participation in spite of disease progression
  4. Need to allow compensations for function while protecting against secondary impairments that may result from compensation.
140
Q

With Duchenne’s muscular dystrophy, what 3 medical management protocols must be performed to promote longevity of life?

A
  1. Pharmaceutical intervention to slow strength decline (Prednisone 0.75mg/kg/day-prolongs ambulation up to 3 years)
  2. Maximizing cardiopulmonary function as disease progresses (Prevent pulmonary infections, secretion management, airway clearance, manage scoliosis, monitor cardiac function closely)
  3. Psychosocial and emotional support
141
Q

At what age do patients with Duchenne’s muscular dystrophy usually pass away?

A

Typically in 20’s

142
Q

Why causes termination of life with Duchenne’s muscular dystrophy?

A
  1. Fatty Infiltration of heart and respiratory infections

2. Improving mortality with advances in medical management (more now surviving into 30’s)

143
Q

At what age are those with Duchenne’s muscular dystrophy usually prescribed a wheelchair?

A

12 years of age

144
Q

Clinical Management of Duchenne’s muscular dystrophy

What are 5 indications of Duchenne’s muscular dystrophy?

A
  1. Progressive weakness
  2. Young Male
  3. Muscle atrophy with calf (Pseudohypertophy)
  4. Gowers maneuver, Sway back posture
  5. Unable to perform age appropriate motor skills (climbing, running, jumping)
145
Q

What are the 3 main interventions PT’s can perform with a patient who presents with Duchenne’s muscular dystrophy?

A
  1. Encourage: Assistive technology/orthotic as needed to increase function
  2. Discourage: Resistive and Eccentric exercises
  3. Stretching: Daily w/standing programs
146
Q

ALS, MG, and MD, are all __________ disorders with key presenting sign of ____________

A

Motor Disorders

Weakness

147
Q

Which motor disorder includes UMN and LMN complications?

A

ALS

148
Q

What is the Etiology for ALS?

A

95% Unknown

5% Hereditary

149
Q

What is the age onset for ALS?

A

Mean age: 60 years

150
Q

What are the signs and symptoms associated with ALS? (4)

A
  1. UMN and LMN Signs
  2. Intact Sensation
  3. Intact Cognition
  4. Intact Bowel and Bladder Function
151
Q

What diagnostic tests are best for ALS? (4)

A
  1. EMG
  2. MRI
  3. Lumbar Puncture
  4. Blood Tests
152
Q

What is the progression of ALS?
Spinal?
Bulbar?

A

Spinal: Limb Onset
Bulbar:
-Dysarthria (Difficult or unclear articulation of speech that is otherwise linguistically normal)
-Dysphagia (Difficulty or discomfort in swallowing)

153
Q

What is the typical lifespan of a patient with ALS
Spinal?
Bulbar?

A

Spinal: 3-5 years till death
Bulbar: 2-3 years till death

154
Q

What is the Etiology of Myasthenia Gravis?

A

Autoimmune disorder

155
Q

What is the Age of onset for Myasthenia Gravis?
Male?
Female?

A

Male: 50-60s
Female: 20-30s

156
Q

What are the sings and symptoms associated with Myasthenia Gravis? (5)

A
  1. Fluctuating and Fatigable weakness
    • Ptosis (Drooping or falling of the upper eye lid)
    • Dysarthria (Unclear speech)
    • Dysphagia (Difficulty/painful swallowing)
  3. Respiratory Weakness
  4. Nasally Speech
  5. Limb Weakness
    - Proximal > Distal
    - Quadriceps, Triceps, Neck Extensors
157
Q

What are the key diagnostic tests to perform for Myasthenia Gravis? (4)

A
  1. Tensilon Test (+)
  2. Serologic Testing: Blood serum exam
  3. Slow repetitive nerve stimulation (Single fiber EMG)
  4. Ct/MRI (Thymoma Screen)
158
Q

What is the general progression of Myasthenia Gravis? (2)

A
  1. Focal Disease progresses to generalized

2. 2 Years from onset to maximal severity

159
Q

What is the lifespan associated with one who has Myasthenia Gravis?

A

Normal

-Decreased Quality of life

160
Q

What is the etiology of Duchenne’s muscular dystrophy?

A

Hereditary

161
Q

What is the age of onset for Duchenne’s muscular dystrophy?

A

Primarily males

-First signs after 1 year

162
Q

What are the clinical signs and symptoms associated with Duchenne’s muscular dystrophy?(8)

A
  1. Delayed motor milestones
  2. Toe walking
  3. Proximal Weakness
  4. Pseudohypertrophy in claves
  5. Intact bowel and bladder
  6. Lordosis
  7. Gowers maneuver
  8. Intact Sensation
163
Q

What are the key diagnostic tests to perform with one who has Duchenne’s muscular dystrophy? (3)

A
  1. CK Levels
  2. Muscular Biopsy (Gold Standard)
  3. Genetic Testing
164
Q

What is the general progression of Duchenne’s Muscular Dystrophy?

A

3-7 years old: Toe walking

7-12 years old: Transition to wheelchair

165
Q

What is the lifespan of an individual with Duchenne’s muscular dystrophy?

A

Death around 20 years old

Neuropathology (7 decks)

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