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The Nervous System: Pathology > Muscle and Nerve Disease > Flashcards

Muscle and Nerve Disease Flashcards

1
Q

Define muscle

A

An intricate machine designed to convert chemical energy to mechanical energy

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2
Q

What is the components of muscle to allow it to perform its function

A

Excitation-contraction coupling
The contractile mechanism
Structural components
The energy system

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3
Q

What is symptoms of muscle disease

A

Poor suck / feeding / failure to thrive / floppy

Weakness of skeletal muscle

Short of breath (respiratory muscles)

Poor swallow (aspiration)

Cardiomyopathy

Cramp, pain, myoglobinuria

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4
Q

What is signs of muscle disease

A

Wasting

Hypertrophy (due to overuse)

Normal or reduced tone and reflexes

Motor weakness
(not sensory)

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5
Q

What sign is seen in myoglobinura (breakdown of muscle into myoglobin)

A

Excreted urine is black

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6
Q

What investigations take place in muscle disease

A

History and examination

CK - blood test

EMG - electrical stimulation to check id muscle working

Muscle biopsy
(Structure, Biochemistry, Inflammation)

Genetic testing

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7
Q

Why do you test for CK in the blood

A

Is a muscle enzyme and elevation shows muscle disease is present

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8
Q

What is 6 different muscle disease classifications

A

Muscular dystrophies

Channelopathies

Metabolic muscle disease

Inflammatory muscle disease

Congenital myopathies

Iatrogenic - medication

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9
Q

Name all the proteins involved in the muscle muscular apparatus attaching the extracellular matrix to the intracellular matrix of the muscle in order to make myosin fibres contract

A

Laminin

Dystroglycan complex

Sacroglycan complex

Dystophin

Actin

Myosin fibrils

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10
Q

What is life threatening muscle disease symptoms and progressions

A

Respiratory muscles causing shortness of breath resulting in hypoventilation

Poor swallowing leading to aspiration pneumonia

cardiomyopathy leading to CVD and arrhythmias

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11
Q

What occurs if anything goes wrong with any of the protein apparatus linking the extracellular and intracellular matrix of the muscle

A

End up with a muscular disease

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12
Q

What is the cause of muscular dystrophies

A

Genetic,

something goes wrong with the components of the muscle

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13
Q

What is the affect of muscular dystophies

A

Affects the mechanism of the muscle

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14
Q

What are examples of muscular dystrophies

A 
Duchenne’s MD
Becker’s MD
Facioscapulohumeral MD
Myotonic dystrophy
Limb-Girdle MD
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15
Q

What is the cause of Duchennes MD

A

Absence of dystrophin protein that helps keeps muscle cells intact

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16
Q

What muscular dystrophy is a milder form of duchnennes MD

A

Beckers MD

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17
Q

What is typical symptoms and signs of duchesses and beckers MD

A

Hypertrophy

but Weakness of muscle

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18
Q

How does hypertrophy occur in muscular dystrophies

A

Due the fatty infiltration of muscle replacement

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19
Q

What symptoms are present inFacioscapulohumeral MD

A

Facial, scapula, and

bicep weakness

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20
Q

What does Limb- Girdle MD present with

A

Upper and lower limb proximal weakness

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21
Q

What is the cause of muscle channelopathies

A

Disturbed function of Ca, Na and Cl channels subunits or the proteins that regulate them

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22
Q

What is the result of muscle channelopathies

A

If something wrong with the channel can either not get the muscle to contract or cant get the muscle to relax

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23
Q

What is the patterns of symptoms in muscle channelopathties affecting potassium ion channels

A

Intermittent symptoms:

Normal then can have symptoms for a few days then they go away, then re-occcur at a later date

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24
Q

What are examples of Potassium channel problems

A

Familial hypokalemic periodic paralysis

Hyperkalemic periodic paralysis

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25
Q

What are examples of chloride channel problems

A

Paramyotonia congenita

Myotonia congenita

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26
Q

What kind of disease is Hyperkalemic/hypokalemic

A

Organic disease

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27
Q

What is the characteristic feature of myotonia congenita

A

Cant relax
Present with stiffness as muscle have a continual low level contraction

Once contract - cant relax e.g. shake hand and won’t let go

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28
Q

Why is paramyotonia termed paradoxical

A

Because it becomes worse with exercise whereas as myotonia congenita, is alleviated by exercise

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29
Q

What is the cause of metabolic muscle disease

A

Enzyme defect in energy metabolism

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30
Q

Disorders of what metabolism can result in metabolic muscle disease, and how?

A

Disorders of carbohydrate metabolism and lipid metabolism

As have problem with glycogen storage and fat storage which is the energy storage needed for muscle activity

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31
Q

When do symptoms of Disorders of carbohydrate metabolism (CM) and lipid metabolism (LM) show

A

Weakness shows on significant (CM) or prolonged exertion (LM)

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32
Q

What occurs in mitochondrial myopathies

A

Prominent muscle weakness

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33
Q

How does endocrinopathy cause metabolic muscle diseease

A

Hormone abnormality can cause muscle weakness

Or

The treatment can cause a steroid induced myopathy

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34
Q

What is an example of causes of biochemical abnormalities resulting in metabolic muscle disease

A

Drugs resulting in low potassium levels

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35
Q

What is two Inflammatory muscle diseases

A

Polymyositis - just affects muscle

Dermatomyositis - affects muscle and skin

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36
Q

What is Dermatomyositis associated with

A

Paraneoplastic - caused by underlying tumour

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37
Q

What is the overall presentation of Inflammatory muscle disease

A

Painful weak muscle

Feeling unwell (due to system inflammation)

Characteristic rash: eyes (red), around the knuckles

38
Q

What is the investigations for of inflammatory muscle disease

A

CK blood test

EMG, shows inflammation and myopathic cells

Biopsy
PM = CD8 cells
DM = human mediate, be cells and CD4 cells

39
Q

What is the treatment of inflammatory muscle disease

A

Immunosuppression - through high dose steroids

40
Q

What is an Iatrogenic cause of muscle disease

A

Statins for cholesterol resulting in degeneration of muscle

41
Q

What is a common neuromuscular junction disorder

A

Myasthenia gravis

42
Q

What is the clinical presentation of myasthenia gravis

A

Fatiguable weakness:

Limbs
Eyelids (ptosis)
Muscles of mastication (chewing), swallow
Talking
SOB
diplopia
43
Q

When can myasthenia gravis become fatal

A

When the generalised weakness affects you breathing - can lead to aspiration pneumonia

44
Q

What is the cause of myasthenia gravis

A

Due defect in the action of acetylcholine at neuromuscular junctions a

45
Q

What muscle does myasthenia gravis not affect

A

Cardiac muscle

46
Q

Why is the presentation if myasthenia gravis fatiguable weakness occur later on in the day

A

As Muscle is strong to begin with, but becomes weaker overtime throughout the day, this is because limited ACH to overcome barrier used up by the end of the day so symptoms appear later on

47
Q

Investigations for myasthenia gravis

A

Antibody testing
(AChR ab, Anti MuSK ab)

Neurophysiology
- Repetitive stimulation (contract muscle and see fatigue in Jitter)

CT chest

48
Q

What are you investigating for in a chest x ray for myasthenia gravis

A

Thyoma benign tumour arising from thymus tissue as is sometimes associated with myasthenia gravis

49
Q

What is the different treatment management for myasthenia gravis

A

Symptomatic

Immunosuppression

Immunoglobulin/plasma exchange

Thymectomy

50
Q

What is used in symptomatic treatment of myasthenia gravis

A

Acetylcholinesterase inhibitor

- block ach breakdown, more ach available to overcome block

51
Q

What is used in immunosupression treatment of myasthenia gravis

A

Prednisolone

Azathioprine (steroid saving agent)

stops antibody production

52
Q

What is the function of immunoglobulin and plasma exchange treatment in myasthenia gravis

A

Plasma -Get rid of the antibody

Immunoglobulin - bind to antibody and prevent it working

53
Q

What is the function of a thymectomy in myasthenia gravis

A

Remove thymus, as overactive thyroid produces antibodies

54
Q

What are the two antibodies playing a role in myasthenia gravis

A

anti-acetylcholine receptor (AChR) antibody

anti-muscle-specific tyrosine kinase (MuSK) antibody

55
Q

What is present in the periphery

A

Motor axons

Sensory axons

Autonomic fibres

56
Q

Where is the two areas nerve disease can occur

A

Occurs proximal in the nerve (just as it leaves the spine) = root

or

Distally in the periphery nerve

57
Q

What is is causes of root disease

A

Degenerative spine disease
Inflammation
Infiltration

58
Q

What can cause lesion of individual peripheral nerve

A

Compressive /Entrapment neuropathy

Vasculitic

59
Q

What is the affect of entrapment neuropathy

A

Localised affect with signs an symptoms related to that nerve trapped

60
Q

What occurs in vasculitic of peripheral nerves

A

Inflammation of blood vessels affects the blood supply to the nerve

61
Q

What is it called when vasculitic happen to numerous peripheral nerves

A

mononeuritis multiplex

62
Q

What is vasculitic also associated with

A

Other inflammatory and autoimmune disease

63
Q

What is affected in generalised peripheral neuropathy

A

Motor, sensory axons and autonomic fibres

64
Q

What is a characteristic feature of peripheral neuropathy pathology

A

Affects the longest nerves first

as they require more energy for the distant synapse to occur

65
Q

What is the causes of generalised peripheral neuropathy

A

Metabolic: Diabetes, alcohol, renal, B12

Toxic: drugs
Hereditary

Infectious: Lyme, HIV, leprosy

Paraneoplastic

Inflammatory demyelinating

66
Q

What is the commonest cause of peripheral neuropathy

A

Diabetes

67
Q

How does alcohol cause peripheral neuropathy

A

Has a central affect, killing neuronal cells

leading to cerebellar degeneration

68
Q

How does a paraneoplastic malignancy cause peripheral neuropathy

A

Sets of inflammation around to body (fighting tumour) which affects and attacks the nervous system

69
Q

What is an acute inflammatory demyelinating causing peripheral neuropathy

A

Guillain barre syndrome

triggered by bacterial, viral infection, few days later experience weakness

70
Q

What is a chronic inflammatory demyelinating causign peripheral neuropathy

A

Chronic inflammatory demyelinating polyneuropathy

Reoccurrence of autoimmune attack

71
Q

How does inflammatory demyelinating cause peripheral neuropathy

A

The body is attacks by infection which creates an auto-immune response
antibody cross reaction with antibodies on myelin sheath and damage the nerves

72
Q

What is the signs and symptoms of nerve root damage in nerve disease

A

Myotomal wasting and weakness

Reflex change

Dermatomal sensory change

73
Q

What is the signs and symptoms of individual nerve disease

A

Wasting and weakness of innervated muscle

Specific sensory change

very local

74
Q

What is signs and symptom in generalised peripheral neuropathy

A

Generalised symptoms

Sensory and motor symptoms starting distally and moving proximally

75
Q

What are the investigations used in nerve disease

A

Blood tests (Liver function)

Genetic analysis
Nerve Conduction Studies (stimuli and see nerve pattern)

Lumbar puncture (high protein in CSF analysis)

Nerve biopsy (rare and only do sensory nerve)

76
Q

What is the disease that affects your anterior horn cell

A

Motor neurone disease AKA Amyotrophic lateral sclerosis (ALS)

77
Q

Where does ALS affect motor neurones

A

brain, brainstem, and spinal cord.

78
Q

What is the usually pathway of progression in ALS

A

Usually limb onset,

later bulbar

respiratory involvement
(respiratory failure is usually cause of death)

79
Q

What is bulbar disease experienced in ALS patients

A

Impairment of function of the cranial nerves IX, X, XI and XII
So patients show alterations in speech or swallowing abilities

80
Q

What is the lower motor neurone signs see in ALS

A

muscle fasciculations, wasting, weakness

81
Q

What is upper motor neurone signs seen in ALS

A

increased tone, brisk reflexes

82
Q

What sensory signs are seen in ALS

A

No sensory involvement

83
Q

What is the pathology of motor neurone disease ALS

A

Degeneration (wearing out of) of motor neurone

no inflammation

84
Q

What is the prognosis of Motor neurone disease ALS

A

3-5 years from symptom onset

2-3 years from diagnosis

50% die within 14 months of diagnosis

85
Q

What is used in the diagnosis of motor neurone disease (ALS)

A

Unique combination of UMN + LMN signs with no sensory features

EMG - electrical myopathy
(stick needles in muscles and check innervation)

86
Q

What is the treatment of motor neurone disease (ALS)

A

Supportive
(PEG, NIV, physiotherapist , Occupational Therapist, care)

Riluzole

87
Q

How does riluzole work in the treatment of motor neurone disease

A

Is anti glutamate antagonist

blocks the affect of glutamate which is an excitatory neurotransmitter that over stimulates and damages cells

88
Q

What is the affect of riluzole

A

Prolongs survival for a few months

89
Q

In an EMG of Motor neurone disease what shouldn’t be seen

A

A neuropathy - because the axon is in tact

90
Q

When is PEG offered in ALS

A

When can no longer chew or swallow

91
Q

When is non invasive ventilation offered in ALS

A

When patient has problems breathing

The Nervous System: Pathology (25 decks)

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