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Neuro > Muscle and Nerve Disease > Flashcards

Muscle and Nerve Disease Flashcards

1
Q

Function of muscle

A

Convert chemical energy into mechanical energy

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2
Q

4 components of muscle

A
  1. Structural component
  2. Contractile mechanism
  3. Excitation-contraction coupling (ion channels)
  4. Energy system
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3
Q

Symptoms of muscle disease

A 
Weakness of skeletel muscle
Short of breath
Poor swallow - aspiration
Cardiomyopathy
Cramp, pain, stiffness, myoglobinuria
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4
Q

Symptoms of muscle disease in children

A

Failure to thrive
Floppy
Poor feeding

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5
Q

Signs of muscle disease

A

Wasting
Hypertrophy
Normal/reduced tone with change in reflexes
Motor weakness, sensory normal

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6
Q

Investigation of muscle disease

A 
History and Exam
Creatine Kinase
EMG
Muscle biopsy
Genetic testing
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7
Q

Why is creatine kinase tested when investigating muscle disease?

A

Degenerating muscle releases CK

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8
Q

Which type of muscle is CK testing not specific to? What should be tested instead?

A

Cardiac

Troponin

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9
Q

What elements are investigated when taking a muscle biopsy?

A

Structure
Biochemistry
Inflammation

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10
Q

4 categories of congenital/geneticc muscle disease

A

Structural
Contractile
Energy
Coupling

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11
Q

Examples of structural congenital/genetic muscle disease

A

Muscular dystrophies

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12
Q

Example of contractile congenital/genetic muscle disease

A

Congenital myotrophies

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13
Q

Examples of coupling congenital/genetic muscle disease

A

Channelopathies

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14
Q

Examples of energy congenital/genetic muscle disease

A

Enzymes/ mitochondrial disease

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15
Q

4 Categories of Acquired muscle disease

A

Metabolic - Ca, K
Endocrine - thyroid, adrenal, vitamin D
Inflammatory muscle disease
Iatrogenic -medication; steroids, statins

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16
Q

What symptoms may present with statin use to suggest muscle disease?

A

Aches
Unknown if psychological or organic
Some evidence of inflammatory myositis

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17
Q

Typical age Muscular dystrophies present

A

Younger or older onset

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18
Q

Character of disease course of muscular dystrophies

A

Progressive

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19
Q

One sign of cell degeneration in muscular dystrophies

A

High CK

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20
Q

5 examples of muscular dystrophy

A 
Duchenne's 
Beckers
Facioscapulohumeral
Myotonic dystrophy (cataracts)
Limb Girdle
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21
Q

Treatment for muscular dystrophies

A

Purely supportive, not curative
Managing respiration, swallowing
Provide support to children

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22
Q

Features of myotonic dystrophy

A

Multisystemic
Infertility
Cognitive decline

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23
Q

4 examples of channelopathies

A

Familial hypokalaemic periodic paralysis
Paramyotonia congenita
Hyperkalemic periodic paralysis
Myotonic congenita

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24
Q

What channels are affected in Familial hypokalaemic periodic paralysis?

A

Ca, Na, K

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25
Q

Which channels are affected in Paramyotonia congenita and Hyperkalemic periodic paralysis?

A

K

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26
Q

Which channels are afected in myotonia congenita?

A

Cl

27
Q

Two factors to distinguish in metabolic muscle disease

A

Exercise induced
or
Fixed weakness

28
Q

3 categories of metabolic muscle disease

A

Disorders of carbohydrate metabolism
Disorders of lipid metabolism
Mitochondrial myopathies/cytopathies

29
Q

Features of mitochondrial myopathies

A 
Genetic defect within mitochondrial gene
Mild or profound effects
Gut
Deafness
Diabetes
Seizures
Skeletal abnormalities
30
Q

2 types of inflammatory muscle disease

A

Polymyositis

Dermatomyositis

31
Q

Which inflammatory muscle disease is antibody associated?

A

Polymyositis

Needs aggressive immunosuppression to avoid fied weakness

32
Q

Which inflammatory muscle disease is paraneoplastic?

A

Dermatomyositis

33
Q

Onset of inflammatory muscle disease

A

Acute or subacute
Any age
Painful weak muscles
Characteristic rash of DM

34
Q

Investigations for inflammatory muscle disease

A

High CK
EMG, inflammation and myopathic
Biopsy

35
Q

Which cells are found in polymyositis on biopsy?

A

CD8 cells

36
Q

Which cells are found in dermatomyositis on biopsy?

A

Humeral mediated, B cells and CD4 cells

37
Q

Treatment of inflammatory muscle disease

A

Immunosuppression

38
Q

Disorder of the Neuromuscular Junction

A

Myasthenia Gravis

39
Q

Presentation of myasthenia gravis

A

Fatiguable weakness
Limbs, eyelids, muscles of mastication
–> Talking, SOB, diplopia

40
Q

Investigations for myasthenia gravis

A

Antibody serology - Anti MuSK, AChR ab
Neurophysiology
CT chest (thymoma)

41
Q

What percentage of patients with myasthenia gravis have underlying thymoma?

A

10%

Look for nodule - thymic hyperplasia

42
Q

Treatment for myasthenia gravis

A

Symptomatic - acetylcholinesterase inhibitor
Immunosuppression - prednisolone, steroid saving agent
Immunoglobulin.plasma exchange
Thymectomy

43
Q

4 components of the peripheral nerve

A

Sensory axons
Motor aons
Autonomic axons
Nerve sheaths (myelin)

44
Q

Small fibres of peripheral nerve communicate…

A

pain , temperature

45
Q

Large fibres communicate…

A

joint position, vibration

46
Q

3 origins of peripheral nerve disease

A 
Root disease
Lesion of individual peripheral nerve 
- Compression/entrapment neuropathy
-Vasculitic (mononeuritis multiplex)
Generalised peripheral neuropathy - Motor/Sensory/Both +/- autonomic features
47
Q

2 types of generalised peripheral neuropathy

A

Axonal v Demyelinating

48
Q

Causes of Generalised peripheral neuropathy

A 
Hereditary
Metabolic - diabetes, alcohol, renal B12
Toxic - drugs
Infectious - Lyme disease, HIV, Leprosy
Malignancy - paraneoplastic
Inflammatory demyelinating - Acute : Guillain Barre Syndrome , Chronic : Chronic inflammatory demyelinating polyneuropathy
49
Q

Signs and symptoms of Peripheral Nerve Disease - Root

A

Myotomal wasting and weakness
Reflex change
Dermatomal sensory change

50
Q

Signs and symptoms of Peripheral Nerve Disease - Lesion of individual nerve

A

Wasting and weakness of innervated muscle

Specific sensory change

51
Q

Signs and symptoms of Peripheral Nerve Disease - Gernealised peripheral neuropathy

A

Sensory and motor symptoms

Usually start distally and move proximally

52
Q

Investigations for peripheral nerve disease

A 
Blood tests - renal dysfunction, thyroid, liver
Genetic analysis
Nerve conduction  studies
Lumbar puncture (CSF analysis)
Nerve biopsy
53
Q

Motor Neuron disease - progression

A

Limb to bulbar to respiratory

54
Q

Signs of MND

A 
Upper and Lower Motor Neuron signs
Muscle fasciculations, weakness, wasting
Increased tone, brisk reflexes
Sensory sparing
10% have cognitive decline
55
Q

Prognosis of motor neuron disease

A

3-5 years from symptom onset, 2-3 from diagnosis

50% die within 14 months diagnosis

56
Q

Treatment for MND

A

Supportive (PEG feed, non invasive ventilation, physio, OT, SALT, care)
Riluzole - will extend life 3 months - slows down progression, no strength gained
Anticipatory, pallative care

57
Q

Genetic mutation in Duchenne Muscular Dystrophy

A

Dystrophin gene Xp21
X-linked recessive
Mothers to male children

58
Q

Signs and symptoms of Duchenne Muscular Dystrophy

A 
Frequent falls
Fatigue
Toe walking/difficulty walking - equinovarus foot
Muscle weakness
Muscle pseudohypertrophy
Muscle fibrosis
Positive Gower's test
59
Q

What is Gower’s sign

A

Patient starts in squatting position
Assume tripod position
Uses hands and arms to walk up body due to weakness in gluteus and quadriceps

60
Q

Which type of mutations in the dystrophin gene cause Duchenne and Becker’s?

A

Duchenne- nonsense, frameshift - more severe;presents before age 5
Becker’s - missense - milder - presents age 10-20

61
Q

Investigations for Duchenne’s MD

A

DNA testing
Creatine phosphokinase test - increased
Muscle biopsies - confirm mutation
EMG Electromyography - muscle destruction analysis

62
Q

Treatment for Duchenne’s

A

Prednisolone and creatinine replacement
OT
Physio
Patient and parent education

63
Q

Life expectancy for Duchenne’s

A

25-30 years

64
Q

Complications of Duchenne’s

A

Cardiomyopathy
Scoliosis
Respiratory complications, risk of infections
Osteoporosis

Neuro (9 decks)

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