Disorders of the Motor Unit Flashcards

1
Q

What are some of the symptoms of motor unit disease

A

Muscle weakness
Myalgia = muscle ache
Fasciculations = muscle twitch
Muscle wasting

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2
Q

How do you differentiate myopathy and neuropathy?

A

Clinical features
= In neurogenic there is loss of reflex, fasciculations, sensory loss
Lab
= Biopsy: Neurogenic- Fibre type grouping. Myopathy- Necrosis & Regeneration
= Enzymes: Myopathy has much more Ck
= EMG: Duration of Potentials: Neurogenic (Giant), Myopathy (Small). Fibrillation: Neurogenic (Yes), Myopathy (No).

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3
Q

What are some examples of Neurogenic diseases

A

MND
Guillane Barre Syndrome
Peripheral Neuropathy

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4
Q

What are some Myopathic Diseases

A

Inherited: DMD, Limb Girdle muscular dystrophy
Acquired: Dermatomyositis

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5
Q

Patient presents with Weakness, Atrophy, Fasciculations, Suppressed Reflexes. What is the diagnosis?

A

Lower Motor Neurone dysfunction in MND

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6
Q

What are the signs for UMN Dysfunction

A

Spasticity, Brisk reflexes, Babinski sign, Uncontrolled emotions

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7
Q

What are the causes of MND

A

Inherited
Toxicity
Pn Misaggregation and Abnormal Precipitation
Mutation

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8
Q

How do you treat MND?

A

Antispasticity, Relieve dry mouth, Aid respiration, Anti-glutamatergic

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9
Q

Patient presents with motor and sensory symptoms.
Sensory: Numbness
Motor: Weakness in hands, ankles, bulbar, facial

A

Guillaine Barre Syndrome

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10
Q

What are the causes of GBS

A

Infection of Upper Respiratory

Cross reacting antibodies attach myelin to periph nerves

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11
Q

What is the treatment of GBS

A

Plasma exchange

Immunoglobin to surppress immune response

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12
Q

What are the causes of Peripheral Neuropathy

A

Alcohol, B12, Carcinoma, Diabetes

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13
Q

Patient presents with invereted champagne bottle legs and Pes Cavus, muscle weakness and loss of reflexes/sensation

A

Heriditary Peripheral Neuropathy

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14
Q

How is DMD inherited

A

X Linked Recessive

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15
Q

How do you diagnose DMD

A

V. High Ck
No staining with Abs to dystrophin
Visible deletion in Xp21
Mutation in dystrophin gene

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16
Q

What are the signs of DMD

A

Progressive disease
Proximal muscle wasting
Calf pseudohypertrophy
Gowers manoeuvre

17
Q

How do you treat DMD

A

Symptomatic relief

Gene therapy - Direct muscle injection of recombinant DNA, myoblast implantation

18
Q

Patient presents with violaceous rash, proximal myopathy and inflammation of muscle

A

Dermatomyositis

19
Q

What are the causes of Dermatomyositis

A

Side effects of statins
Underlying malignant tumour
Autoimmune of small intramuscular bv