PATH: Peripheral Nerves/Muscle Disorders Flashcards

1
Q

Mechanisms of Peripheral Nerve Pathology (Demylenation vs. Axonopathy)

A

Demyelination: Conduction Velocity Impacted

Axonopathy: Amplitude Impacted

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2
Q

Clinical Pattern of Peripheral Nerve Pathology?

Causes?

A
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3
Q

Clinical Pattern of Peripheral Nerve Pathology?

Causes?

A
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4
Q

Clinical Pattern of Peripheral Nerve Pathology?

Causes?

A
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5
Q

Autonomic Neuropathy Characteristics?

A
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6
Q

Proximal vs. Distal Motor Weakness?

A
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7
Q

Characteristics of Thiamine (B1) Deficiency?

A
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8
Q

Characteristics of Pyridoxine (B6) Deficiency?

A
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9
Q

Characteristics of Cobalamin (B12) Deficiency?

A
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10
Q

Diabetic Neuropathy

  • Modalities Impacted
  • Axonopathic/Demyelinating
  • Characteristics
A
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11
Q

Uremia

  • Modalities Impacted
  • Axonopathic/Demyelinating
  • Characteristics
A
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12
Q

Toxic causes of Peripheral neuropathy?

A
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13
Q

Spinocerebellar (Friedreich’s) Ataxia (SCA)

  • Modalities Impacted
  • Axonopathic/Demyelinating
  • Characteristics
  • Complications
A
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14
Q

Charcot-Marie-Tooth (CMT)

  • Modalities Impacted
  • Genetic Causes of each subtype (CMT 1A, CMT1B, CMT 1X, CMT2)
  • Axonopathic/Demyelinating
  • Presentation
  • Complications
A
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15
Q

Hereditary Causes of Neuropathy?

A

Spinocerebellar (Friedreich’s) Ataxia (SCA)

  • Sensory/Axonopathic
  • Inactivating GAA repeats in Frataxin gene
  • Impaired mitochondrial iron handling
  • Difficulty walking (before 20)
  • Pes Cavus & Kyphoscoliosis
  • Cardiomyopathy
  • Optic atrophy

Charcot-Marie-Tooth (CMT)

  • Sensory and Motor / Demylenating (CMT2 Axonopathic)
  • Autosomal Dominant or X-Linked (CMT 1X)
  • Foot drop & wasting
  • Champagne bottle/Stork legs
  • Pes Cavus & Scoliosis
  • Schwann cell hyperplasia and hypertrophic neuropathy (except CMT 2)
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16
Q

Infectious Causes of Neuropathy? How to Distinguish?

  • Modalities Impacted
  • Axonopathic/Demyelinating
  • Presentation
  • Complications
A
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17
Q

Paraneoplastic Neuropathy

  • Modalities Impacted
  • Axonopathic/Demyelinating
  • Cause
A
18
Q

Guillain-Barré Disease (Most Common Motor Neuropathy)

  • Axonopathic/Demyelinating
  • Pathogenesis
  • Prognosis
A
19
Q

Differences between LMN and UMN Disease?

A
20
Q

________________: Fatigable (worse with exercise) weakness

A

Myasthenic: Fatigable (worse with exercise) weakness

21
Q

_______________: inherited progressive muscular weakening and destruction/wasting

A

Dystrophies: inherited progressive muscular weakening and destruction/wasting

22
Q

_______________: Involuntary sustained contraction or impaired relaxation of muscles

A

Myotoias: Involuntary sustained contraction or impaired relaxation of muscles

23
Q

_______________: Ion channel disorders of muscle cells

A

Channelopathies: Ion channel disorders of muscle cells

24
Q

Motor neuron disease characterized by:

  • Focal muscle weakness and wasting
  • Fasciculations
  • Brisk reflexes
A
25
Q

Pure UPPER motor neuron disease characterized by muscle weakness and wasting?

A

Progressive Muscular Atrophy

26
Q

Pure UPPER motor neuron disease characterized by tetraperesis (RARE)

A

Primary Lateral Sclerosis

27
Q

Motor Neuron disease characterized by fasciculating tongue?

A

Progressive Bulbar/Pseudobulbar Palsy

28
Q

Myasthenia Gravis

  • Pathogenesis
  • Etiology
  • Complications
  • Treatment
A
29
Q

Lambert-Eaton Myasthenic-Myopathic Syndrome (LEMS)

  • Pathogenesis
  • Presentation
A
30
Q

Idiopathic inflammatory disease causing proximal skeletal muscle weakness and a characteristic rash probably mediated by autoimmunity?

  • Presentation
  • Pathogenesis
  • Diagnosis
A
31
Q

Dermatomyositis vs. Polymyositis?

A

Polymyositis

  • Like dermatomyositis but No Rash
  • Endomysial CD8+ cytotoxic T-cells directly injuring Myocytes

Dermatomyositis

  • CD4+ T-cell and B-cells => perimysial vessels => Perifascicular Atrophy
32
Q

Endocrine casues of Muscle Disease?

A
33
Q

Electrolyte (Calcium/Potassium) casues of Muscle Disease?

A
34
Q

Drug Mediated Muscle Toxicities

  • ____________: Type II fibre atrophy
  • __________ –proximal weakness
  • _________ -myalgia
  • _______________–rhabdomyolysis/necrotizing autoimmune myopathy
  • _____________: Malignant neuroleptic syndrome (rigidity and autonomic instability
  • _____________: Malignant Hyperthermia
A
35
Q
A

Duchenne Muscular Dystrophy

Becker Muscular Dystrophy (later, milder)

36
Q
A

Facioscapulohumeral Dystrophy

37
Q

Which type of muscle disease is poorly tolerant of general anesthesia?

A

Myotonias: Sustained involuntary contraction and Impaired relaxation

38
Q

Myotonia Congenita

  • Autosomal Dominant vs. Recessive
  • Exacerbating factors
A
39
Q

Mytonic Dystrophy

  • Autosomal Dominat or Recessive?
  • Presentation?
A
40
Q

Differences between the two Inhertied Paralytic Channelopathies?

A