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Clinical Conditions II > Lecture 1: Motor neuron diseases > Flashcards

Lecture 1: Motor neuron diseases Flashcards

1
Q

Myelopathy

A
  • Cervical spondolysis
  • Spinal cord tumor
  • Trauma
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2
Q

Anterior Horn cell disorders

A
  • ALS
  • Polio
  • Progressive nuclear palsy
  • GB
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3
Q

Nerve root lesions

A
  • Cervical rib syndrome
  • Acute intervertebral prolapse
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4
Q

Peripheral nerve lesions

A
  • Poly & mono neuropathy
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5
Q

Neuromuscular junction lesions

A
  • Myasthenia gravis
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6
Q

Characteristics of MND caused by anterior horn lesions

A
  • Affect anterior horn alpha-motor neurons
  • 30-60 y/o
  • Men
  • 95% = sporadic onset (may be genetic in subsequent generations)
  • 5-10% = familial
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7
Q

5 subclasses of adult anterior horn cell-MND

A
  • progressive nuclear palsy
  • pseudobulbar palsy
  • progressive spinal muscular atrophy (PMA)
  • primary lateral sclerosis (PLS)
  • amyotrophic lateral scelrosis (ALS)
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8
Q

ALS stands for:

A

Amytrophic: atrophy of muscle

Lateral Sclerosis: hardening of the lateral corticalspinal tracts

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9
Q

Population Affected by ALS

A
  • 60% of male
    • 20% more common in males
    • With increasing age, incidence in females approaches that of males
  • 93% caucasion
  • Majority diagnosed between 40-70
    • Diagnosis age = 55
    • Cases can occur in 20s-30s
  • Life expectancy:
    • 5yrs: 20%
    • 10yrs: 10%
    • 20yrs: 5%
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10
Q

2 things that stay in tact (symptom wise with ALS)

A
  • No changes in sensation
  • No extraocular or sphincter muscle involvement
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11
Q

Signs and Symptoms of ALS

A
  • Weakness (reduced power or force)
    • clumsiness, fatigue, heaviness
  • Atrophy/muscle wasting
  • Progressice
  • Fasiculations
  • Areflexia
  • Hypotonia
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12
Q

Cognitive function of ALS

A
  • Extent and level is highly variable
    • 50-60% develop frontotemporal function defecit
    • Range from full frontotemporal dimentia, neuropsychological, speech/language defecits
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13
Q

Risk factors for ALS

A
  • Older age
  • Male
  • Caucasian
  • Guamanian origin
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14
Q

Only ___ were linked to ALS risk

A

blood-lipids

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15
Q

Etiology of ALS

A
  • Complex genetic and environmental
  • Contiuum that includes frontotemporal dementia
  • Mutations in DNA/RNA regulating protein C9ORF72 → dysregulation in RNA processing & toxic cellular accumulations
    • 40% of famial and 20% of sporadic
      • Leads to accumulation of cellular RNA and/or proteins that are toxic:
        • TDP-43 = PRIMARY PRODUCT THAT ACCUMULATES
  • Mutations in SOD-1 (involved in free radical control)
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16
Q

Autophagy is up/down regulated in response to:

A

growth signals & environmental ques

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17
Q

Autophagy roll in cancer

A
  • Protects against cancer by controlling growth
    • Down-regulation confers a survival advantage in some caners
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18
Q

Autophagy in neurodegenerative diseases (NDDs):

Many NDDs are characterized by age-dependent:

A
  • Neuronal death
    • due to accumulation of normal proteo-metabolsim products
  • Autophagy tags and clears these proteins – body’s only mechanism for disposal
19
Q

Defect is autophagy pathway ___ , contribute to ___ accumulation and neurotoxicity

A

SOD-1 pathway

TDP43 build up

20
Q

Most common form of ALS

A

Sporadic (90%)

21
Q

Familal cases accout for ___% of ALS cases

A
  • 10% = familial cases
  • Autosomal dominence inheritance pattern
  • 50% change of passing it on
  • 20% have SOD mutation
22
Q

ALS and Retrovirus association

A
  • Human endogenous retrovirus (HERV) is + in many ALS pt’s
23
Q

other environmental stimuli for ALS

A
  • Cyanobacteria
  • Heavy metals
  • Pesticides
  • Intense PA
  • Head injury
  • Smoking
  • Electomagnetic field / electric shock
24
Q

20% of ALS patients present with ___ involvement

A

Bulbar

25
Q

Bulbar involvement (ALS) signs

A
  • Difficulty chewing/swallowing
  • Coughing
  • Dysarthia & dysphagia
  • Fasciulations
  • Decreased resp. strength: often primary cause of death
  • Still no sensory loss or extraoccular/sphincter loss
26
Q

80% of ALS cases are ____ involvement

A

spinal

27
Q

Spinal involvement (ALS) signs

A
  • UE affected first - 50% of pts
  • Fatigue
  • Weakness
  • Cramping
  • Wasting
  • Muscle twitching
  • Stiffness
  • Lesser extent of speech and swallowing
  • Cognitive function not typically affected
  • No senosry loss of extraocular or sphincter muscles
28
Q

Primarly lateral sclerosis is primarily ___ involvement

A

upper-motor neuron

-better outcomes than PMA

29
Q

Primary muscular atrophy is primarily in ___ involvement

A

lower-motor neuron

30
Q

which has worst outcomes: bulbar or spinal involvement?

A

Bulbar

31
Q

Treatment for ALS (PT)

A
  • NOT TO OVERTAX pt
  • Active ROM
  • Active-assisted
  • PROM
  • Respiratory/Chest PT:
    • Percussion, vibration, mechanical vibrators, precursors
    • Postural drainage
    • Cough assist
  • Inspiratory muscle training
    • Weights versus inspiratory trainer or spironmeter
32
Q

3 classes of disorders at the neuromuscular junction

A
  1. blockage of Ca+ channels: Lambert-eaton syndrome
  2. Impaired Ca+ mediated release of Ach: Botulism
  3. Antibody induced down regulation of Ach receptors: Myasthenia Gravis
33
Q

Lambert-Eaton Syndrome

A
  • Autoimmune disease which presynaptic NMJ Ca+ channels are destroyed by antibodies
    • Decreased Ach release
    • Weakness and areflexia that improve with sustained contraction
    • Treatment based on K+ channel blocker and Ca2+ channel agonist
34
Q

Myasthenia Gravis

A
  • Auto-immune down regulation of Ach receptors
  • Fluctuating weakness and easy fatigue
  • Thymus tumor, thyrotoxicosis, RA, lupus
  • Females > males
  • Biases: external ocular and other cranial nerves:
    • Muscles of mastication, swallowing, etc.
35
Q

Myasthenia Gravs Presentation

A
  • Diplopia (double vision) ~90%
  • Ptosis (drooping eye-lid)
    • Pupillary reflex not affected
  • Dysarthria (difficulty speaking)
  • LE weakness
  • General weakness
  • Dysphagia
  • Reflexes may be normal / little atrophy
36
Q

Myasthenia Gravis Classic Presentation

A

sustained muscular effort impaired

Brief effort is normal

37
Q

Diagnosis of Myasthenia Gravis

A
  • Administer a cholinesterase inhibitor
  • Tensilon test: have pt do repeated voluntary contractues: if symptoms improve, then test is (+)
38
Q

Etiology of Myasthenia Gravis

A
  • Patients develop antibodies to Ach receptors (most common) or to muscle specific kinase or LDL-receptor related protein
  • Leads to Ach receptor damage and loss, making it difficult to stimulate muscles
39
Q

Critical illness polyneuropathy

A
  • Severe
  • Systemic weakness associated with critical illness, sepsis and multi-organ failure
40
Q

Critical illness myopathy

A
  • Acute onset of severe diffuse weakness including respiratory muscles and loss of DTR
  • Associated with steroid use and/or neuromuscular blockade
  • Elevated serum CK & MG levels
41
Q

Characteristics of Myasthenia Gravis

A
  • Insidious onset
  • Avg age:
    • Women: 28
    • Males: 42
  • Slowly progressive, fluctuating course
  • Managed with medications, but difficult to cure
  • May ultimately be fatal
    • Respiratory infections or other complicaitons
42
Q

Treatment for Myasthennia Gravis

A

Immunotherapy

43
Q

Exercise with Myasthenia Gravis

A

Active exercise must be administered carefully and in small doses secondary to easily fatigued and may lead to exhuastion

Clinical Conditions II (5 decks)

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