Muscle Weakness Flashcards

1
Q

Name the two different sites of motor lesion?

A

Upper Motor Neurone

Lower Motor Neurone

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2
Q

Name the three possibly affected areas of upper motor neurone lesion?

A

Cerebrum
Brainstem
Spinal Cord

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3
Q

Name the four possibly affected areas of lower motor neurone lesions

A

Anterior/Ventral horn cells
Peripheral nerve
Neuromuscular junction
Muscle

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4
Q

What are the cerebral signs of an upper motor neurone pathway lesion?

A

Unilateral (normally)

Dysphasia
Associated Sensory Symptoms
Seizure
Personality changes

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5
Q

What are the brainstem signs of an upper motor neurone pathway lesion?

A
Dysphagia
Diplopia
Dysarthria
Cranial nerve symptoms
Sensory symptoms
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6
Q

What are the spinal cord signs of an upper motor neurone pathway lesion?

A

Bilateral (usually)

Bladder/bowel/sexual dysfunction
Sensory symptoms

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7
Q

What are the peripheral nerve signs of a lower motor neurone lesion?

A

Specific distribution according to nerve / symmetrical distribution (according to myotome)
Sensory symptoms

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8
Q

What are the NMJ signs of a lower motor neurone lesion?

A

Specific distribution
Fatiguable weakness
Possible autonomic features

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9
Q

What are the muscle signs of a lower motor neurone lesion?

A

Wasting
Occasional pain
Systemic features
Proximal distribution (often)

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10
Q

What are the anterior horn cell signs of a lower motor neurone lesion?

A
Mixed UMN, LMN signs
Weakness
Wasting
Arreflexia
Fasciculations
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11
Q

Give a few examples of motor neurone diseases

A

Motor neuron disease
ALS - Amyotrophic lateral sclerosis
SMA - Spinal muscular atrophies
PLS - poliomyelitis

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12
Q

What are motor neurone diseases?

A

Disease causing wasting/atrophy of the anterior horn cells resulting with upper and lower motor neurone symptoms.

ALS also causes the corticospinal tract to sclerose

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13
Q

What is the incidence of Motor neurone disease?

A

Rare, approximately 2/100k

Mainly present age 50 - 70

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14
Q

What are the causes of Motor neurone diseases?

A

Unknown cause except in poliomyelitis where infection leads to MND

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15
Q

Describe the signs and symptoms of motor neurone diseases

A

Rapid, progressive course
Signs in several areas of body
UMN signs - Spastic paraparesis, dysarthria & brisk jaw jerk, brisk reflex

LMN signs - Weakness, wasting, fasciculations, dysarthria, dysphagia, respiratory failure

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16
Q

What is the treatment used for MND?

A

Riluzole - glutamate antagonist

It may slightly lengthen lifespan of those suffering with ALS

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17
Q

What does -paresis mean?

A

refers to weakness

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18
Q

What does -plegia mean?

A

refers to paralysed

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19
Q

What does hemi- mean?

A

refers to one side of the body

20
Q

What does para- mean?

A

refers to the two lower limbs

21
Q

What does quadra- mean?

A

All four limbs are affected

22
Q

What does myelo- refer to?

A

The spinal cord

23
Q

What does radiculo- refer to?

A

The nerve root

24
Q

What does neuro- refer to?

A

The peripheral nerve

25
Q

What does myo- refer to?

A

muscle

26
Q

Which part of the brain is responsible for the gait initiation?

A

Frontal lobes

Basal ganglia

27
Q

Which part of the nervous system is responsible for the movement?

A

Motor cortex
Spinal cord
Nerves
Muscle

28
Q

Which part of the nervous system is responsible for the control of posture?

A
Brainstem
Cerebellum
Vestibular system
Visual system
Muscle stretch reflexes
Joint position sense
29
Q

What is the general pattern of upper motor neurone problem?

A

Extensors weaker than flexors in arms
Flexors weaker than extensors in legs

Remember: Ext in A, Flex in legs

30
Q

What is the general muscle weakness distribution of muscle disease?

A

Proximal weakness

31
Q

What is the general muscle weakness distribution of polyneuropathy?

A

Distal weakness

32
Q

What is Muscular Dystrophy?

A

Group of inherited disorders of muscle structure characterised by progressive wasting and weakness

e.g. Duchenne, Becker’s, Congenital MD

33
Q

Incidence of Duchenne’s?

A

1/3500 - X-linked condition that usually only affects males

34
Q

What is the pathophysiology behind muscular dystrophy?

A

Genetic problem producing a flawed ‘dystrophin-glycoprotein complex’, which is normally responsible for anchoring muscle fibres to cell membranes/surrounding tissue

35
Q

What are the symptoms and signs of muscular dystrophy?

A

Childhood onset - normal at birth but delayed motor milestones
Progressive weakness and wasting
Proximal muscles affected more than distal
Can affect other organs (cardiomyopathy, resp muscle weakness, poor sphincter control)
Poor balance = frequent falls
Scoliosis
Ptosis

36
Q

What investigations are used to diagnose muscular dystrophy?

A

Serum: Elevated Creatinine Kinase - CK released on muscle breakdown
DNA testing
Muscle biopsy

37
Q

How does statin-induced myopathy develop?

A

Asymptomatic rise in serum Creatinine Kinase
Myalgia and myositis
Rhabdomyolysis (muscle breakdown)

38
Q

What are the signs symptoms of statin-induced myopathy?

A

Muscle pain/tenderness with nocturnal cramps

Proximal weakness

39
Q

How is statin-induced myopathy treated?

A

Stop drugs or reduce dose if symptoms are intolerable or CK is 10-fold higher than normal

40
Q

What is polymyositis?

A

Idiopathic inflammatory acquired condition where Cytotoxic T cells invade and destroy muscle fibres

Usually onset in adults with symptoms of proximal weakness, myalgia and raised creatinine kinase

41
Q

How is polymyositis and dermatomyositis diagnosed and treated?

A

Anti nuclear antibodies (ANAs) often present, but a muscle biopsy is needed to confirm diagnosis

Immunosuppresive treatment, e.g. Methotrexate, azathioprine

42
Q

What is Dermatomyositis?

A

Idiopathic inflammatory acquired condition where antibodies attack capillaries and small arterioles in muscle, skin etc.

Onset may be childhood or adults with symptoms of proximal weakness, myalgia and raised creatinine kinase as well as a characteristic rash and fever. Associated with inflammatory arthritis disease malignancy.

43
Q

What is Inclusion Body Myositis?

A

An idiopathic inflammatory acquired condition usually onset in the elderly characterised by slowly progressive weakness, especially of the knee, ankle and finger flexors and swallowing.

44
Q

How is Inclusion Body Myositis diagnosed and treated?

A

CK normal/mildly raised

Poor response to immunosuppression

45
Q

What is Cauda Equina Syndrome?

A

A serious neurologic condition in which damage to the cauda equina causes acute loss of function of the lumbar and sacral nerve roots which leads to bladder sphincter, anal sphincter, and sexual dysfunction, saddle and genital anaesthesia, and sometimes bilateral sciatica.