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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
What are examples of chloride channel problems
Paramyotonia congenita Myotonia congenita
26
What kind of disease is Hyperkalemic/hypokalemic
Organic disease
27
What is the characteristic feature of myotonia congenita
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
28
Why is paramyotonia termed paradoxical
Because it becomes worse with exercise whereas as myotonia congenita, is alleviated by exercise
29
What is the cause of metabolic muscle disease
Enzyme defect in energy metabolism
30
Disorders of what metabolism can result in metabolic muscle disease, and how?
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
31
When do symptoms of Disorders of carbohydrate metabolism (CM) and lipid metabolism (LM) show
Weakness shows on significant (CM) or prolonged exertion (LM)
32
What occurs in mitochondrial myopathies
Prominent muscle weakness
33
How does endocrinopathy cause metabolic muscle diseease
Hormone abnormality can cause muscle weakness Or The treatment can cause a steroid induced myopathy
34
What is an example of causes of biochemical abnormalities resulting in metabolic muscle disease
Drugs resulting in low potassium levels
35
What is two Inflammatory muscle diseases
Polymyositis - just affects muscle Dermatomyositis - affects muscle and skin
36
What is Dermatomyositis associated with
Paraneoplastic - caused by underlying tumour
37
What is the overall presentation of Inflammatory muscle disease
Painful weak muscle Feeling unwell (due to system inflammation) Characteristic rash: eyes (red), around the knuckles
38
What is the investigations for of inflammatory muscle disease
CK blood test EMG, shows inflammation and myopathic cells Biopsy PM = CD8 cells DM = human mediate, be cells and CD4 cells
39
What is the treatment of inflammatory muscle disease
Immunosuppression - through high dose steroids
40
What is an Iatrogenic cause of muscle disease
Statins for cholesterol resulting in degeneration of muscle
41
What is a common neuromuscular junction disorder
Myasthenia gravis
42
What is the clinical presentation of myasthenia gravis
Fatiguable weakness: ``` Limbs Eyelids (ptosis) Muscles of mastication (chewing), swallow Talking SOB diplopia ```
43
When can myasthenia gravis become fatal
When the generalised weakness affects you breathing - can lead to aspiration pneumonia
44
What is the cause of myasthenia gravis
Due defect in the action of acetylcholine at neuromuscular junctions a
45
What muscle does myasthenia gravis not affect
Cardiac muscle
46
Why is the presentation if myasthenia gravis fatiguable weakness occur later on in the day
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
Investigations for myasthenia gravis
Antibody testing (AChR ab, Anti MuSK ab) Neurophysiology - Repetitive stimulation (contract muscle and see fatigue in Jitter) CT chest
48
What are you investigating for in a chest x ray for myasthenia gravis
Thyoma benign tumour arising from thymus tissue as is sometimes associated with myasthenia gravis
49
What is the different treatment management for myasthenia gravis
Symptomatic Immunosuppression Immunoglobulin/plasma exchange Thymectomy
50
What is used in symptomatic treatment of myasthenia gravis
Acetylcholinesterase inhibitor | - block ach breakdown, more ach available to overcome block
51
What is used in immunosupression treatment of myasthenia gravis
Prednisolone Azathioprine (steroid saving agent) stops antibody production
52
What is the function of immunoglobulin and plasma exchange treatment in myasthenia gravis
Plasma -Get rid of the antibody Immunoglobulin - bind to antibody and prevent it working
53
What is the function of a thymectomy in myasthenia gravis
Remove thymus, as overactive thyroid produces antibodies
54
What are the two antibodies playing a role in myasthenia gravis
anti-acetylcholine receptor (AChR) antibody anti-muscle-specific tyrosine kinase (MuSK) antibody
55
What is present in the periphery
Motor axons Sensory axons Autonomic fibres
56
Where is the two areas nerve disease can occur
Occurs proximal in the nerve (just as it leaves the spine) = root or Distally in the periphery nerve
57
What is is causes of root disease
Degenerative spine disease Inflammation Infiltration
58
What can cause lesion of individual peripheral nerve
Compressive /Entrapment neuropathy Vasculitic
59
What is the affect of entrapment neuropathy
Localised affect with signs an symptoms related to that nerve trapped
60
What occurs in vasculitic of peripheral nerves
Inflammation of blood vessels affects the blood supply to the nerve
61
What is it called when vasculitic happen to numerous peripheral nerves
mononeuritis multiplex
62
What is vasculitic also associated with
Other inflammatory and autoimmune disease
63
What is affected in generalised peripheral neuropathy
Motor, sensory axons and autonomic fibres
64
What is a characteristic feature of peripheral neuropathy pathology
Affects the longest nerves first | as they require more energy for the distant synapse to occur
65
What is the causes of generalised peripheral neuropathy
Metabolic: Diabetes, alcohol, renal, B12 Toxic: drugs Hereditary Infectious: Lyme, HIV, leprosy Paraneoplastic Inflammatory demyelinating
66
What is the commonest cause of peripheral neuropathy
Diabetes
67
How does alcohol cause peripheral neuropathy
Has a central affect, killing neuronal cells | leading to cerebellar degeneration
68
How does a paraneoplastic malignancy cause peripheral neuropathy
Sets of inflammation around to body (fighting tumour) which affects and attacks the nervous system
69
What is an acute inflammatory demyelinating causing peripheral neuropathy
Guillain barre syndrome | triggered by bacterial, viral infection, few days later experience weakness
70
What is a chronic inflammatory demyelinating causign peripheral neuropathy
Chronic inflammatory demyelinating polyneuropathy | Reoccurrence of autoimmune attack
71
How does inflammatory demyelinating cause peripheral neuropathy
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
What is the signs and symptoms of nerve root damage in nerve disease
Myotomal wasting and weakness Reflex change Dermatomal sensory change
73
What is the signs and symptoms of individual nerve disease
Wasting and weakness of innervated muscle Specific sensory change very local
74
What is signs and symptom in generalised peripheral neuropathy
Generalised symptoms Sensory and motor symptoms starting distally and moving proximally
75
What are the investigations used in nerve disease
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
What is the disease that affects your anterior horn cell
Motor neurone disease AKA Amyotrophic lateral sclerosis (ALS)
77
Where does ALS affect motor neurones
brain, brainstem, and spinal cord.
78
What is the usually pathway of progression in ALS
Usually limb onset, later bulbar respiratory involvement (respiratory failure is usually cause of death)
79
What is bulbar disease experienced in ALS patients
Impairment of function of the cranial nerves IX, X, XI and XII So patients show alterations in speech or swallowing abilities
80
What is the lower motor neurone signs see in ALS
muscle fasciculations, wasting, weakness
81
What is upper motor neurone signs seen in ALS
increased tone, brisk reflexes
82
What sensory signs are seen in ALS
No sensory involvement
83
What is the pathology of motor neurone disease ALS
Degeneration (wearing out of) of motor neurone | no inflammation
84
What is the prognosis of Motor neurone disease ALS
3-5 years from symptom onset 2-3 years from diagnosis 50% die within 14 months of diagnosis
85
What is used in the diagnosis of motor neurone disease (ALS)
Unique combination of UMN + LMN signs with no sensory features EMG - electrical myopathy (stick needles in muscles and check innervation)
86
What is the treatment of motor neurone disease (ALS)
Supportive (PEG, NIV, physiotherapist , Occupational Therapist, care) Riluzole
87
How does riluzole work in the treatment of motor neurone disease
Is anti glutamate antagonist blocks the affect of glutamate which is an excitatory neurotransmitter that over stimulates and damages cells
88
What is the affect of riluzole
Prolongs survival for a few months
89
In an EMG of Motor neurone disease what shouldn't be seen
A neuropathy - because the axon is in tact
90
When is PEG offered in ALS
When can no longer chew or swallow
91
When is non invasive ventilation offered in ALS
When patient has problems breathing

The Nervous System: Pathology (25 decks)

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