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Neurology-Week 4 & 5 > 4. Disorders of the PNS > Flashcards

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
Q

(Neuropathy/Myopathy)

Proximal > Distal
Normal reflexes
- Sensory symptoms
- autonomic symptoms
Elevated serum CK
A

Myopathy

26
Q

Neuromuscular disorder that causes weakness due to dysfunction of muscle fibers; includes muscular dystrophies, metabolic myopathies and inflammatory processes

A

Myopathy

27
Q

(Acquired/Hereditary) myopathies are often associated with skeletal deformities (scoliosis, hyperlordosis, pes cavus, scapular winging)

A

Hereditary

28
Q

Common Signs/Symptoms of Myopathies

A

Atrophy
Calf pseudohypertrophy
Scapular winging
Gower’s sign (getting up like 골룸)

29
Q

Lab tests for diagnosing Myopathies

A

Serum Creatine Kinase
Inflammatory markers
Genetic testing

30
Q

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

A

Electromyography

31
Q

Examples of acquired myopathies (Idiopathic Inflammatory Myopathies)

A

Polymyositis
Dermatomyositis
Inclusion Body Myositis

32
Q

Examples of drugs that can cause acquired myopathies

A

Steroids (chronic use)

Statins

33
Q

An inflammatory myopathy causing symmetric proximal muscle weakness; FEMALES more than males; CK elevated; responds to IMMUNOTHERAPY

A

Polymyositis

34
Q

An inflammatory myopathy with muscle and skin (rash) involvement; proximal muscle weakness with elevated CK; associated with MALIGNANCY; perifasicular atrophy on histology

A

Dermatomyositis

35
Q

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

A

Inclusion Body Myositis

36
Q

Examples of inherited myopathies (Muscular Dystrophies)

A
  • Duchenne
  • Becker
  • Myotonic Dystrophy
37
Q

Examples of inherited myopathies (Metabolic)

A
  • Pompe’s Disease

- McArdle’s Disease

38
Q

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

A

Dystrophinopathies

  • Duchenne’s (more severe; affects age 2-3)
  • Becker’s (milder; affects age 5-15)
39
Q

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

A

Myotonic Dystrophy

40
Q

Metabolic Myopathy; Acid Alpha-Glucosidase (AAG) deficiency; results in glycogen buildup in LYSOSOMES of skeletal and cardiac muscle tissue; Autosomal Recessive

A

Pompe’s Disease

41
Q

Enzyme deficiency in Pompe’s Disease

A

Acid Alpha-Glucosidase

42
Q

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

A

McArdle’s Disease

43
Q

Enzyme deficient in McArdle’s Disease

A

Glycogen Phosphorylase (a key enzyme in glycogenolysis)

44
Q

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

A

ALS (Lou Gherig’s Disease)

45
Q

(T/F) ALS involves sensory abnormality

A

False. ALS has no sensory involvement

46
Q

Treatment for ALS

A

Supportive
Riluzole (reduce glutamate excitotoxicity)

*“lu” = “lou” Gehrig

47
Q

ALS is largely a (sporadic/genetic) condition

A

Sporadic

*genetic is due to SOD1 mutation

48
Q

ALS motor abnormalites are often (uni/bi)lateral and (symmetric/asymmetric)

A

Bilateral; asymmetric

49
Q

ALS involves (upper/lower/mixed) motor abnormalities

A

Mixed

50
Q

An autosomal RECESSIVE disorder caused by ANTERIOR HORN degeneration; referred to as “Floppy Baby” syndrome; mutations in SMN1 gene

A

Spinal Muscular Atrophy

51
Q

Peripheral Motor Abnormality caused by an infection with polio virus; fecal-oral transmission

A

Poliomyelitis

52
Q

Examples of diseases of the NMJ

A

Myasthenia Gravis
LEMS
Botulism

53
Q

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

A

Myasthenia Gravis

54
Q

Treatment for Myasthenia Gravis

A

1st Line: Pyridostigmine(cholinesterase inhibitor)

55
Q

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

A

Lambert-Eaton Myasthenic Syndrome

56
Q

Myasthenia Gravis is (Pre/Post) synaptic, has (decrement/facilitation) on repetitive nerve stimulation and is associated with (small cell cancer/thymoma)

A

Postsynaptic
Decrement
Thymoma

57
Q

Lambert-Eaton Myasthetic Syndrome is (Pre/Post) synaptic, has (decrement/facilitation) on repetitive nerve stimulation, and associated with (small cell cancer/thymoma)

A

Presynaptic
Facilitation
Small Cell

58
Q

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)

A

Botulism

59
Q

What are the differential diagnosis for floppy baby

A
  1. Infant botulism

2. Spinal muscular atrophy

60
Q

What is the drug that blocks acetylcholinesterase

A

Pyridostigmine

  • Tx for Myasthenia Gravis

Neurology-Week 4 & 5 (14 decks)

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