Muscle and Nerve Disease Flashcards

1
Q

Common symptoms of muscle disorders

A
Weakness of skeletal muscles 
Dyspnoea (respiratory muscles affected) 
Cardiomyopathy 
Poor feeding/failure to thrive in newborns 
Cramping and pain 
Myoglobinuria
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2
Q

Common signs of muscle disorders

A

Wasting/hypertrophy
Normal/reduced tone and reflexes
Motor weakness

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3
Q

Classifications of muscle diseases

A
Muscular dystrophies 
Channelopathies
Metabolic  muscle disease 
Inflammatory muscle disease 
Congenital myopathies
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4
Q

Examples of muscular dystrophies

A
Duchenne's 
Becker's 
Facioscapulohumeral 
Myotonic dystrophy 
Limb-Girdle
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5
Q

Examples of channelopathies

A
Disorders of Ca, Na and Cl channels 
Familial hypokalaemic periodic paralysis 
Hyperkalaemic periodic paralysis 
Paramyotonia congenital 
Myotonia congenital
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6
Q

Examples of metabolic muscle diseases

A

Disorders of carbohydrate/lipid metabolism

Mitochondrial myopathies/cytopathies

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7
Q

Examples of inflammatory muscle diseases

A

Polymyositis

Dermatomyositis

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8
Q

Presentation of inflammatory muscle diseases

A

Acute or subacute presentation
Painful, weak muscles
Characteristic rash if dermatomyositis

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9
Q

Treatment of inflammatory muscle disease

A

Immunosuppression

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10
Q

Common investigations for muscle disease

A
History and examination 
Creatine-kinase 
EMG 
Muscle biopsy for structure, biochemistry and inflammation 
Genetic testing
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11
Q

Investigations of inflammatory muscle diseases

A

Creatine-kinase - would be elevated
EMG - inflammation and myopathic
Biopsy

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12
Q

Clinical presentation of myasthenia gravis

A
Fatiguable weakness of limbs and muscles of mastication 
Ptosis 
Speech affected 
Dyspnoea 
Diplopia
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13
Q

Investigations of myasthenia gravis

A
AChR antibody 
Anti-MuSK antibody 
Neurophysiology - EMG, repetitive stimulation 
Tensilon test 
CT chest
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14
Q

Symptomatic treatment of myasthenia gravis

A

Acetylcholinesterase inhibitor

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15
Q

Immunosuppressive treatment of myasthenia gravis

A

Prednisolone

Steroid-sparing agent

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16
Q

Conditions associated with myasthenia gravis

A

Thymic hyperplasia in young people

Malignant thymoma in older people

17
Q

Under what circumstances might a thymectomy be done?

A

Malignant thymoma in females < 40

18
Q

Peripheral nerves consist of

A

sensory and motor axons

19
Q

Causes of peripheral nerve disease

A

Degenerative spine disease
Inflammation
Infiltration
Lesions

20
Q

Examples of lesions of individual peripheral nerves

A

Compressive/entrapment neuropathy

Vasculitic

21
Q

Causes of generalised peripheral neuropathy

A
Metabolic e.g. diabetes mellitus 
Toxic - drugs 
Hereditary 
Infectious e.g. Lyme disease 
Malignancy 
Inflammatory demyelinating conditions (acute or chronic) e.g. Guillain Barre syndrome
22
Q

Symptoms of pathology at the nerve root

A

Myotomal wasting and weakness
Reflex change
Dermatomal sensory change

23
Q

Symptoms of pathologies of an individual nerve

A

Wasting and weakness of the innervated muscle

Specific sensory change

24
Q

Symptoms of generalised peripheral neuropathy

A

Sensory and motor symptoms starting distally and moving proximally

25
Q

Investigation of nerve disease

A
Blood tests
Genetic analysis 
Nerve conduction study 
Lumbar puncture - CSF analysis 
Nerve biopsy
26
Q

Most common cause of Guillain-Barré syndrome

A

Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)

27
Q

Typical presentation of Guillain-Barré syndrome

A

2-4 weeks after relatively benign respiratory/GI illness
Complaints of finger dysesthesias and proximal muscle weakness of lower extremities
Weakness progressing to involve arms, trunk, cranial nerves and respiratory muscles

28
Q

Typical pattern of weakness in Guillain-Barré syndrome

A

Ascending and symmetrical

Lower limbs affected before upper limbs

29
Q

Symptoms of Guillain-Barré syndrome

A
Pain, tingling and numbness 
Progressive muscle weakness 
Co-ordination problems and unsteadiness 
Temporary paralysis of legs, arms, face or respiratory muscles 
Blurred vision/diplopia 
Difficulty speaking 
Dysphagia 
Difficulty with digestion/bladder control 
HR and BP fluctuations
30
Q

Clinical features of an upper motor neurone lesion

A
Muscle weakness 
Decreased control of active movement 
Spasticity 
Clasp-knife response 
Babinski sign 
Increased deep tendon reflex 
Pronator drift
31
Q

Clinical features of a lower motor neurone lesion

A
Muscle paresis/paralysis 
Fibrillations
Fasciculations 
Hypotonia/atonia 
Hyporeflexia 
Weakness limited to segmental or focal pattern 
Muscle wasting in end-stage
32
Q

What is myelopathy?

A

Disease of the spinal cord

33
Q

What is radiculopathy?

A

Condition due to compression of a nerve in the spinal cord

34
Q

Symptoms of radiculopathy

A

Pain
Numbness
Tingling
Weakness

35
Q

Causes of non-compressive spinal cord lesions

A
Myelitis 
Vascular
Metabolic 
Drugs and toxins 
Physical agents 
Degenerative
36
Q

Clinical presentation of motor neurone disease

A

Limb onset
UMN and LMN signs
Lower motor neurone - muscle fasciculations, wasting, weakness
Upper motor neurone - increased tone, brisk reflexes
No sensory involvement

37
Q

How is motor neurone disease diagnosed

A

Clinical presentation of combination of UMN and LMN signs, and EMG

38
Q

Treatment of motor neurone disease

A

Supportive - PEG, NIV

Riluzole