4. Disorders of the PNS Flashcards

1
Q
  1. Disorder of cerebrum
  2. Disorder of spinal cord
  3. Disorder of the root
  4. Disorder of the plexus
  5. Disorder of many nerves
  6. Disorder of one nerve
  7. Disorder of muscle
A
  1. Encephalopathy
  2. Myelopathy
  3. Radiculopathy
  4. Plexopathy
  5. Polyneuropathy
  6. Mononeuropathy
  7. Myopathy
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2
Q

A process in which the nerve degenerates from the point of axonal damage outward; cell swelling, peripheral movement of nucleus, and chromatolysis (degradation of nissl substance)

A

Wallerian Degeneration

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3
Q

Focal degeneration of the Myelin Sheath with SPARING of axon; muscle atrophy does NOT occur

A

Segmental Demyelination

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4
Q

When the axon breaks down and “dies back”; often systemic and is progressive from distal to proximal; muscle atrophy DOES occur

A

Axonal Degeneration

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5
Q

A disorder of PNS; usually DISTAL and SYMMETRIC loss of tendon reflexes; both motor and sensory deficits; LOWER motor neuron findings; function and reflexes; include Arsenic poisoning, Guillian-Barre, Diabetic neuropathy, and CMT

A

Polyneuropathy

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6
Q

Overall, DISTAL motor problems are usually (nervous/muscular) in origin

A

Nervous

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7
Q

Examples of ACUTE Polyneuropathies (3 total)

A

Arsenic Poisoning
Porphyria
Guillian-Barre

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8
Q

Examples of CHRONIC Polyneuropathies (4 total)

A

Diabetic Neuropathy
B12 Deficiency
CIDP
Charcot-Marie Tooth Disease

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9
Q

ACUTE Polyneuropathy; LIFE-THREATENING immune-mediated demyelination of spinal nerve roots and PNS; DISTAL limb weakness, but rapidly ascending paralysis; loss of deep tendon reflexes; usually preceded by a “flu-like” illness or Campylobacter jejuni infection; Elevated CSF protein

A

Guillain-Barre Syndrome

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10
Q

Which Polyneuropathy is referred to as a “ascending” paralysis

A

Guillain-Barre Syndrome (aka. AIDP)

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11
Q

Causes of Guillain-Barre Syndrome

A

Preceding “flu-like” illness associated with Campylobacter jejuni infection

*causes immune-mediated damage to neurons

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12
Q

Guillain-Barre Syndrome is characterized by (ascending/descending) paralysis and associated with (Campylobacter/Clostridium)

A

Ascending; Campylobacter

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13
Q

CHRONIC Polyneuropathy; appearance of VERY THIN legs in proportion to proximal legs, pes cavus (high arches with hammer toes); histologic finding of onion bulb; patient are usually asymptomatic, but eventually complain of foot pain or dysfunction

A

Charcot-Marie Tooth Disease

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14
Q

Physical appearance of Charcot-Marie Tooth Disease

A

Thin lower legs (calf atrophy)
High arches
Hammer toes

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15
Q

CHRONIC Polyneuropathy; due to longstanding diabetes; report distal (feet before hands) SENSORY loss with a “burning” pain; high risk for amputations due to incidental damage; also results in autonomic dysfunction (orthostatic hypotension, dec. bladder emptying, etc.)

A

Diabetic Neuropathy

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16
Q

What is the common sensation patient with Diabetic Neuropathy complain of?

A

“Burning” pain

*usually distal

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17
Q

Autonomic dysfunction of diabetic neuropathy

A
  1. Orthostatic hypotension
  2. dec. bladder emptying
  3. post prandial nausea
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18
Q

A disorder caused by median nerve entrapment; C6 or C7 radiculopathy –> weak thumb abduction & opposition; thenar atrophy

A

Carpal Tunnel Syndrome

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19
Q

A disorder that occurs at elbow & wrist (Guyon’s Canal) –> weak finger/wrist flexion

A

Ulnar nerve palsy

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20
Q

A disorder that is most common at the spiral (radial) groove –> Saturday Night Palsy (weak finger/wrist extensors; wrist drop)

A

Radial nerve palsy

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21
Q

A disorder that is caused by compression just below the knee –> foot drop; steppage gait; weak foot dorsiflexion/eversion

A

Fibular (peroneal) nerve palsy

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22
Q

A disorder that is caused by compression and trauma –> weakness of leg and hamstrings; absent ankle jerk

A

Sciatic Nerve Palsy

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23
Q

A disorder caused by trauma/hematoma –> weak quads; numbness in anteriomedial thigh/shin; absent knee jerk; tight jeans can cause this (“meralgia paresthetica”)

A

Femoral Nerve Palsy

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24
Q

(Neuropathy/Myopathy)

Distal > Proximal
Reduced reflexes
\+ Sensory symptoms
\+/- autonomic symptoms
Normal serum CK
A

Neuropathy

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25
(Neuropathy/Myopathy) ``` Proximal > Distal Normal reflexes - Sensory symptoms - autonomic symptoms Elevated serum CK ```
Myopathy
26
Neuromuscular disorder that causes weakness due to dysfunction of muscle fibers; includes muscular dystrophies, metabolic myopathies and inflammatory processes
Myopathy
27
(Acquired/Hereditary) myopathies are often associated with skeletal deformities (scoliosis, hyperlordosis, pes cavus, scapular winging)
Hereditary
28
Common Signs/Symptoms of Myopathies
Atrophy Calf pseudohypertrophy Scapular winging Gower's sign (getting up like 골룸)
29
Lab tests for diagnosing Myopathies
Serum Creatine Kinase Inflammatory markers Genetic testing
30
Procedure where an electrode is inserted directly into a muscle to assess for any spontaneous discharges and evaluate voluntary Motor Unit Action Potentials; useful to help differentiate neurologic vs. muscular causes of weakness
Electromyography
31
Examples of acquired myopathies (Idiopathic Inflammatory Myopathies)
Polymyositis Dermatomyositis Inclusion Body Myositis
32
Examples of drugs that can cause acquired myopathies
Steroids (chronic use) | Statins
33
An inflammatory myopathy causing symmetric proximal muscle weakness; FEMALES more than males; CK elevated; responds to IMMUNOTHERAPY
Polymyositis
34
An inflammatory myopathy with muscle and skin (rash) involvement; proximal muscle weakness with elevated CK; associated with MALIGNANCY; perifasicular atrophy on histology
Dermatomyositis
35
An inflammatory myopathy that is most common AFTER age 50; MALES more than females; selective and ASYMMETRIC weakness of FINGER FLEXOR and quadriceps; normal CK; hallmark of inclusion bodies (clumps of cellular material and vacuoles) on histology
Inclusion Body Myositis
36
Examples of inherited myopathies (Muscular Dystrophies)
- Duchenne - Becker - Myotonic Dystrophy
37
Examples of inherited myopathies (Metabolic)
- Pompe's Disease | - McArdle's Disease
38
A muscular dystrophy caused by X-linked mutation (affects BOYS) for Dystrophin, which stabilizes the muscle membrane; can result in cardiomyopathy, calf hypertrophy, and Gower's sign
Dystrophinopathies - Duchenne's (more severe; affects age 2-3) - Becker's (milder; affects age 5-15)
39
Most common type of Muscular Dystrophy; AD inheritance and divided into Type 1 (distal) or Type 2 (proximal); characterized by Myotonia (delayed muscle relaxation or can't relax fist), cataracts, balding, and endocrinopathy
Myotonic Dystrophy
40
Metabolic Myopathy; Acid Alpha-Glucosidase (AAG) deficiency; results in glycogen buildup in LYSOSOMES of skeletal and cardiac muscle tissue; Autosomal Recessive
Pompe's Disease
41
Enzyme deficiency in Pompe's Disease
Acid Alpha-Glucosidase
42
Metabolic Myopathy; Glycogen Phosphorylase deficiency resulting in glycogen metabolism disorder; causes exercise induced pain and MYOGLOBINURIA (lots of myoglobin in the urine due to rhabdomyolysis); often cause "second wind" phenomenon
McArdle's Disease
43
Enzyme deficient in McArdle's Disease
Glycogen Phosphorylase (a key enzyme in glycogenolysis)
44
A neurodegenerative disease of BOTH upper and lower motor neuron due to loss of motor neurons in Anterior Horns; often associated with SOD1 gene; presents with ASYMMETRIC limb weakness, PRIMITIVE reflexes (babinski), and muscle wasting; patients usually die of respiratory failure or infection
ALS (Lou Gherig's Disease)
45
(T/F) ALS involves sensory abnormality
False. ALS has no sensory involvement
46
Treatment for ALS
Supportive Riluzole (reduce glutamate excitotoxicity) *"lu" = "lou" Gehrig
47
ALS is largely a (sporadic/genetic) condition
Sporadic *genetic is due to SOD1 mutation
48
ALS motor abnormalites are often (uni/bi)lateral and (symmetric/asymmetric)
Bilateral; asymmetric
49
ALS involves (upper/lower/mixed) motor abnormalities
Mixed
50
An autosomal RECESSIVE disorder caused by ANTERIOR HORN degeneration; referred to as "Floppy Baby" syndrome; mutations in SMN1 gene
Spinal Muscular Atrophy
51
Peripheral Motor Abnormality caused by an infection with polio virus; fecal-oral transmission
Poliomyelitis
52
Examples of diseases of the NMJ
Myasthenia Gravis LEMS Botulism
53
An autoimmune disease caused by antibodies against Ach receptors; POSTsynaptic; patients can present with fatigue, PROXIMAL weakness, ptosis, diplopia, and THYMOMA; diagnosed by amplitude DECREMENT (reduction) on EMG
Myasthenia Gravis
54
Treatment for Myasthenia Gravis
1st Line: Pyridostigmine(cholinesterase inhibitor)
55
An autoimmune disease caused by antibodies against VG Calcium channels (prevents Ach release); PREsynaptic; patients can present with autonomic symptoms (dry eyes/mouth); associated with Small Cell lung cancer; diagnosed by amplitude FACILITATION on EMG
Lambert-Eaton Myasthenic Syndrome
56
Myasthenia Gravis is (Pre/Post) synaptic, has (decrement/facilitation) on repetitive nerve stimulation and is associated with (small cell cancer/thymoma)
Postsynaptic Decrement Thymoma
57
Lambert-Eaton Myasthetic Syndrome is (Pre/Post) synaptic, has (decrement/facilitation) on repetitive nerve stimulation, and associated with (small cell cancer/thymoma)
Presynaptic Facilitation Small Cell
58
Peripheral Motor Abnormality caused by toxin produced by Clostridium bacteria prevents fusion of NT-containing vesicles with the presynaptic membrane; PREsynaptic; can infect babies through honey (spores); DESCENDING paralysis (floppy baby)
Botulism
59
What are the differential diagnosis for floppy baby
1. Infant botulism | 2. Spinal muscular atrophy
60
What is the drug that blocks acetylcholinesterase
Pyridostigmine * Tx for Myasthenia Gravis