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Clinical Conditions II > Motor Neuron Disease > Flashcards

Motor Neuron Disease Flashcards

1
Q

What do pyramidal tracts consist of?

A

The direct motor pathways that lead to activation of alpha motor neurons in the spinal cord (primary and associated motor cortex)

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2
Q

What do extrapyramidal tracts consist of?

A

Motor pathways that are indirectly involved in movement (BG and cerebellum)

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3
Q

Lower Motor Neuron Lesion

A
  • weakness
  • flaccidity
  • fasciculations
  • wasting/ atrophy
  • areflexia
  • decreased coordination
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4
Q

Upper Motor Neuron Lesion

A
  • weakness
  • increased tone
  • spasticity
  • clonus
  • hyper reflexia
  • decreased coordination
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5
Q

What does motor neuron disease occur from?

A

Primarily from damage to an upper or lower motor neuron resulting in denervation of voluntary muscles

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6
Q

What occurs to denervated muscle?

A

Quickly atrophies and cannot respond to voluntary control

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7
Q

Signs and symptoms of motor neuron disease

A

Weakness, atrophy, loss of function

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8
Q

Lesions of what structures can result is motor neuron disease?

A 
Pyramidal tracts
Alpha motor neurons in spinal cord or brain stem
Nerve roots
Nerves
The neuromuscular junction
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9
Q

5 classifications of motor neuron diseases

A 
Myelopathies (spinal cord disorders)
Anterior horn cell disorders
Nerve root lesions
Peripheral nerve lesions
Neuromuscular junction lesions
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10
Q

Myelopathies

A

Cervical spondylosis, spinal cord tumors, trauma

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11
Q

Anterior horn cell disorders examples

A

ALS, progressive nuclear palsy, polio and GB

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12
Q

Nerve root lesions examples

A

Cervical rib syndrome, acute intervertebral disc prolapse

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13
Q

Peripheral nerve lesions examples

A

Poly and mono-neuropathies

Diabetic neuropathy

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14
Q

Neuromuscular junction lesions

A

Myesthenia gravis

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15
Q

Characteristics of MND caused by Anterior horn lesions

A
  • Affect anterior horn alpha motor neurons
  • Age of onset: 30-60 yo
  • Male predominance
  • 90-95% of cases “sporadic”
  • 5-10% of cases familial
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16
Q

5 Subclasses of Adult Anterior Horn Cell- MND

A
  • Progressive nuclear palsy
  • Pseudobulbar palsy
  • Progressive spinal muscular atrophy
  • Primary lateral sclerosis
  • Amyotrophic lateral sclerosis
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17
Q

Amyotrophic Lateral Sclerosis

A
  • Amyotrophy: Atrophy of muscle

- Lateral Sclerosis: hardening of lateral corticospinal tracts (lateral columns) due to gliosis

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18
Q

Population affected by ALS

A
  • Males > females
  • Caucasian
  • Majority diagnoses between 40-70 yo (average 55)
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19
Q

Average life expectancy of ALS at time of diagnosis

A

3-5 years

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20
Q

Signs and symptoms of anterior horn cell disease (ALS)

A
  • Muscle weakness (reduced power/force, pt complains of heaviness, clumsiness, fatigue)
  • Atrophy/wasting
  • NO CHANGE IN SENSATION
  • progressive course
  • fasciculations
  • Areflexia
  • Hypotonia
  • NO WEAKNESS OF EXTRAOCULAR MUSCLES OR SPHINCTERS
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21
Q

Cognitive function in ALS

A
  • Extent and level is uncertain and variable

- ranges from full frontotemporal dementia to neuropsychological, speech, or lingual deficits

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22
Q

Risk factors for ALS

A
  • Older age
  • Male gender
  • Caucasian race
  • Guamanian origin
    (only blood lipids were linked to ALS risk)
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23
Q

Majority of cases of ALS are part of a continuum that includes ….

A

Frontotemporal dementia

  • mutations in a DNA/RNA regulating protein C9ORF72
  • leads to accumulation of cellular RNA and/or proteins that are toxic
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24
Q

What is the primary protein product that accumulated with Frontotemporal dementia/ALS?

A

TDP-43

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25
Q

What do C9ORF72 mutations lead to?

A

dysregulation of RNA processing, leading to toxic cellular accumulations

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26
Q

Autophagy

A

Recent studies also indicate autophagy is important in diagnostic development

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27
Q

Role of Autophagy in Disease

A
  • up and down regulated in response to growth signals and environmental ques
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28
Q

Autophagy role in cancers

A
  • Protects against cancer by controlling growth

- down-regulation confers a survival advantage in some cancers

29
Q

Role of autophagy in neurogenerative disease

A
  • many NDDs are characterized by age-dependent neuronal death due to accumulation of normal proteo-metabolism products: Tau & beta amyloid, huntintin, and alpha-synuclein
  • Involves the tagging and clearance of these proteins and is the body’s only mechanism for disposal
30
Q

ALS is characterized by accumulation ….

A

TDP43

31
Q

3 general “forms” of ALS

A
  • Sporadic
  • Familial
  • Guamanian
32
Q

Sporadic ALS

A

most common (90%)

33
Q

Familial ALS

A
  • typically autosomal dominant inheritance pattern
  • accounts for only 10% of cases
  • individuals with inherited forms have 50% of passing it on
  • 20% have superoxide dismutase mutation
34
Q

Guamanian ALS

A

based on high incidence in Guam and Trust Territories in 1950s

35
Q

Potential Retrovirus Association & other Environmental Stimuli

A
  • HERV is + in many cases
  • Cyanobacteria, heavy metals, pesticides, intense physical activity, head injury, smoking, electromagnetic field or electric shock exposure
36
Q

Cyanobacteria are linked to the high incidence in Guam, secondary to ….

A

dietary factors

37
Q

Bulbar weakness (20% of cases)

A
  • difficulty chewing and swallowing
  • coughing
  • dysarthria and dysphagia
  • dyspnea
  • dasciculation
  • decreased respiratory muscle strength: often progressive and the primary cause of death
  • no sensory loss and extra ocular muscles and sphincters typically spared
38
Q

Limb weakness (80% of cases)

A
  • upper extremity muscle affected first in 50% of cases
  • fatigue-ability
  • weakness
  • cramping
  • muscle twitching
  • wasting
  • stiffness
  • speech, swallowing, respiration
  • cognitive function not typically affected
39
Q

Primary lateral sclerosis

A
  • exclusively upper motor neuron involvement

- improved survival compared to PMA

40
Q

Progressive muscular atrophy

A
  • exclusively lower LMN involvement
41
Q

Differential Diagnosis of ALS- Presentation

A
  • strength testing: mapping weakness allows differentiation of spinal/nerve or NMJ from anterior horn cell lesion
  • often involves muscles of chewing, speaking, swallowing, no involvement of extra-ocular muscles or sphincters
  • Sensation testing: normal
42
Q

Differential Diagnosis of ALS- Special Tests

A
  • MRI, CT, and X-Ray to rule out other conditions

- EMG: shows partial chronic partial denervations (few voluntary motor units, spontaneous activity in resting muscle)

43
Q

Most common misdiagnoses in ALS

A
  • Cerebral lesions: especially with C/S or L/S root involvement
  • Cervical spondylosis myelopathy
  • Conus medularis lesion
  • inclusion body myositis
  • Cramp/fasciculation syndromes
  • multifocal motor neuropathy
  • Kennedy’s disease
44
Q

Progression of ALS

A
  • progressive weakness leads to respiratory failure and/or aspiration pneumonia
  • pulmonary infections
  • malnutrition
  • fatal outcome within 2-5 years of DX
  • Bulbar involvement worse than spinal type
45
Q

Evaluation of ALS

A
  • Vital signs, dyspnea scale, SaO2
  • pulmonary function testing
  • careful muscle strength testing
  • sensation testing
  • cranial nerve testing
  • cognitive testing
  • mobility/transfer tests
  • QoL
46
Q

ALS Functional Rating Scale

A
  • Estimates the patients degrees of functional impairment
  • 10 questions require rater to give impression of patient’s level of functional impairment on performing common tasks
  • 5 point scale
  • scores summed with 40= best possible
  • shown to have satisfactory internal consistency and reliability
47
Q

Treatment of ALS

A
  • Edaravone
  • Antisense oligonucleotide silencing of FUS expression –> Jacifusen
  • miR-340
  • Riluzole
  • Anticholinerics
  • Mechanical ventilation
  • Feeding tube
  • Telbivudine for hepatitis
48
Q

PT related treatment of ALS

A
  • Active ROM
  • AAROM
  • PROM
  • Positioning
  • Respiratory/chest PT
  • Inspiratory muscle training
49
Q

Reparatory/Chest PT

A
  • Percussion, vibration, mechanical vibrators or precursors
  • Postural drainage
  • Cough assist technique
50
Q

Inspiratory muscle training

A

Weight versus inspiratory trainer or inspiratory spirometer

51
Q

What should be used to slow ALS disease progression

A

Riluzole

52
Q

What should be used to stabilize weight and prolong survival of ALS

A

PEG

53
Q

What should be considered to treat respiratory insufficiency

A

NIV

54
Q

Does Riluzole provide a beneficial effect on strength?

A

no but it does provide a small beneficial effect on other bulbar and limb function

55
Q

What is likely the most effective treatment for ALS

A

Riluzole

56
Q

Occupational therapy for ALS

A
  • increasing independence around the home

- equipment to assist in ADLs

57
Q

Physical therapy for ALS

A
  • assistive devises
  • braces
  • teach transfers
  • ROM to prevent contractures/DVT
58
Q

General principles for therapy for ALS

A
  • “text to speech” software devices allow pt to communicate
  • maximize function by adaptation/technology
  • instruct care giver in assisting patient
  • adapt environment for patient
59
Q

3 Classes of Neuromuscular Junction Disorders

A
  • Disorders involving Ca-mediated exocytosis of acetylcholine:
    1. Blockage of Ca channels: Lambert Eaton Syndrome
    2. Impaired Ca-mediated release of Ach: Botulism
  • Disorders of the Ach receptor
    3. Antibody induced down regulation of Ach receptors: Myasthenia Gravis
60
Q

Lambert Eaton Syndrome

A
  • Autoimmune disease in which the presynaptic neuromuscular junction Ca2+ channels are damaged or destroyed by antibodies
  • Decreased Ach release
  • Characterized by weakness and areflexia that improve with sustained contraction
  • Rx based on combination of K+ channel blocker and Ca2+ channel agonist
61
Q

Myasthenia Gravis

A

Immune mediated disease involving fluctuating weakness and easy fatigue ability of voluntary muscles

  • associated with thymus tumor, thyrotoxicosis, RA, Lupus erythmataosus
  • females > males
  • predilection for the external ocular and other cranial muscles
62
Q

myasthenia gravis presentation

A
  • diplopia
  • ptosis
  • dysarthria
  • LE weakness
  • generalized weakness
  • dysphagia
  • sustained muscular effort impaired, while brief effort is normal
  • reflexed normal and little atrophy
63
Q

Diagnosis of Myasthenia Gravis

A
  • Single fiber electromyography
  • E-stim facial nerve, observe rate of fatigue twitch
  • administer cholinesterase inhibitor: have patient do repeated voluntary contractures; if symptoms improve, test is +
  • check for thymoma or hyperthyroidism
  • immunological testing
  • nerve conduction velocity is normal
  • single fiber EMG shows increase variability in interval between twitches
  • pulmonary function test
64
Q

Myasthenia Gravis Etiology

A
  • patients develop antibodies to Ach receptors or to muscle specific kinase or LDL receptor related protein
  • may be due to thyme follicular hyperplasia
  • pro inflammatory environment contributes
  • leads to Ach receptor damage and loss, making it difficult to stimulate muscles
65
Q

Differential Diagnosis of MG- Critical Illness polyneuropathy

A

severe, systemic weakness, associated with critical illness, sepsis and multi organ failure

66
Q

Differential Diagnosis of MG- Critical Illness Myopathy

A
  • acute onset of severe diffuse weakness including respiratory muscles and loss of DTR
  • associated with steroid use and/or neuromuscular blockage
  • elevated serum CK and MG levels
67
Q

Disease characteristics of MG

A
  • insidious onset
  • Average age: 28 women, 42 males
  • slowly progressive, fluctuating course
  • managed with medications but difficult to cure
  • may ultimately be fatal- due to respiratory infections or complications
68
Q

Treatment of MG

A
  • Cholinesterase inhibitors
  • Thymectomy
  • Corticosteroids
  • Azathioprine and Mycophenolate (immunotherapy)
  • Plasmapheresis
  • IV immunoglobulins
69
Q

PT for MG

A
  • maximize function
  • adapt environment
  • educate/train caregiver
  • active exercise must be administered carefully and in small doses secondary to easily fatigued and may lead to exhaustion

Clinical Conditions II (11 decks)

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