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Neuro > Muscle Disorders > Flashcards

Muscle Disorders Flashcards

1
Q

What are muscular dystrophies and how do you Rx

A

Inherited muscle disorders that cause progressive weakness

Steroids
Ataluren - new drug which restores synthesis of dystrophin
OT / Physio
Treat other conditions

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2
Q

What should you think if rapid onset

A

Toxins
Drugs e.g. statin
Metabolic

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3
Q

What is Duchenne’s

A

X-linked recessive
Problem with dystrophin protein in muscle
Xp21 gene
Short survival

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4
Q

What are the symptoms of muscular dystrophies

What are later signs

A

Proximal muscle weakness first - shoulder / girdle
Muscle wasting
Pseudohypertrophy due to fatty infiltration of muscle
Gowers +ve (use hands on legs to help stand)
Waddling gait
Intellectular impairment
FTT

Late signs 
SOB due to resp muscle weakness 
Dysphagia 
Cardiomyoapthy + arrhythmia - screen 
Myoglobulinuria 
Scoliosis 
Tendon contractures 
Reduced tone and reflexes = late
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5
Q

What is Becker’s and how is it inherited

A

Milder form of DMD

X-linked

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6
Q

What is fascioscapulohumeral and how is it inherited

A 
Facial, scapular and biceps weakness
Weakened diaphragm
Bilateral sensorineural hearing loss
Retinal telangiectasis 
AD
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7
Q

What is limb girdle dystrophy

A

Upper and LL proximal weakness

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8
Q

How do you differentiate myopathy from neuropathy

A 
More gradual onset
Proximal weakness
Specific muscle groups
Preserved reflex
No parathesia or bladder
No fasiculations
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9
Q

What is myotonic dystrophy and what process does it follow and how do you Dx

A

Inherited AD affecting skeletal, cardiac and smooth muscle
Trinucleotide repeat disorder - anticipation
Most common muscle disease to present in adulthood

Dx = genetic analysis

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10
Q

What does it cause

A

Progressive muscle weakness
Prolonged contraction and unable to relax e.g. when shaking hand can’t let go
Cataract
Heart conduction - Cardiac arrhythmia / cardiomyopathy / heart block
Infertility due to testicular atrophy
Intellectual impairment
Peripheral neuropathy

Myotonic facies - ptosis / can't close mouth 
Frontal balding
Bilateral ptosis
Dysarthria
Myotonia
DM - type II 
Dysphagia
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11
Q

What are channelopathies

A

Abnormal fluxes of Ca, Na and Cl
Familial hypokalaemia periodic paralysis
Hyperkalaemia paralysis
Paramyotonia congenital

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12
Q

What are Sx

A

Episodic intermittent Sx
Unusual muscle contraction
Profound weakness
Measure K

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13
Q

What are metabolic muscle disorders

A

Disorders of carb / lipid metabolism
Mitochondria myopathy
Endocrinopathy
More significant weakness on exertion

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14
Q

What drugs can cause myopathy

A

Statins
Alcohol
Steroids

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15
Q

What are inflammatory muscle disease and what do they tend to have

A

Polymyositis - CD8
Dermatomyositites - CD4
Spontaneous pain at rest and local tenderness

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16
Q

What are the SX

A 
Painful weak muscles
Shoulder and pelvic girdle
Dysphagia
Resp
Characteristic rash - heliotrope vs gottron's papule
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17
Q

What do you do for all muscle disorders including inerhited

A 
Bloods - CK, ESR, AST and LDH 
- usually elevated 
EMG
Muscle Biopsy
Genetic test
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18
Q

How do you Rx

A

Steroids

Immunosuppresion

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19
Q

What is myasthenia gravis

A

Disorder of NMJ due to insufficient functioning Ach receptor
Ab against Ach receptor on post synaptic side of NMJ
Autoimmune

20
Q

What causes exacerbation

A 
Exertion
BB
Lithium 
Phenytoin
Ax
Pregnancy
Infecion
Hypokalaemia
21
Q

What are the Sx

A

Fatigable weakness in
Limbs
Ptosis at end of the day (droopy eyelid)
- Worsened prolonged upgaze
Extra-ocular muscle - diplopia
Proximal muscle wasting
Facial and bulbar weakness
- Dysphagia
- Weakness in muscles of mastication / speech
SOB due to weakness in resp muscles = dangerous
- Can go into type 2 resp failure

22
Q

What are associations

A 
Thymonas (20%)
Pernicious anaemia
Autoimmune thyroid 
Rheumatoid 
SLE
23
Q

How do you Dx

A 
AchR Ab = 80% 
Anti-MuSk Ab / tyrosine kinase Ab if -ve 
Neurophysiology / EMJ
- Decreased response to stimulation 
CT chest to look for thymoma
CK normal
Spirometry to check FVC
24
Q

How do you Rx

A 
Long acting acetylcholinesterase inhibitor - pyridostigmine 
Immunosuppression
- Initially prednisone if doesn't help 
- Azathioprine 
Bone protection if on steroid 
Thymectomy even if no thymoma
25
Q

What do you give in relapse

A

Prednisolone

26
Q

What do you give in life threatening resp crisis

A

Plasma exchange to filter Ab
IV IG
Treat trigger
May need NIV / intubation

27
Q

What is a thymoma

A

Tumour of anterior mediastinum

Death due to tamponade or airway compression

28
Q

What is it associated with

A

Myasthenia gravis
Red cell aplasia
Dermatomyositis
SIADH

29
Q

What is DDx

A

Polymyositis

Myopathy

30
Q

What are the SE anti-cholinesterase

A 
Salivation
Lacrimation
Sweating
Vomiting
Miosis
Diarrhoea
31
Q

What is Lambort Eaton

A

Ab against calcium channel in PNS

Pre-synaptic

32
Q

What causes

A

SCLC
Breast
Ovarian
Autoimmuen

33
Q

What are the symptoms

A

Repeated contraction = increased strength
Limb girdle weakness - LL
Diplopia
Ptosis
Hyporeflexia
Autonomic - dry mouth / poor peeing / impotence
Gait difficulty

34
Q

How do you Dx

A

EMG
Voltaged gated Ca Ab’s
CXR to look for lung cancer

35
Q

How do you Rx

A

Treat cancer
Immunosuppression
IV IG / plasma exchange may be beneficial

36
Q

What is not common in Lambort Eaton but common myasthenia

A

Opthalmoplegia

Ptosis

37
Q

How do you Dx muscle disorder like DMD

A

CK

38
Q

How do you Rx

A

Steroids

Physio

39
Q

What anaesthetic are people with MG resistant too and what do they require less

A

Suxamethonium as depolarising NMJ blocker
Myasthenia = Ab to post synaptic NMJ

Will require less of non-depolarising as MS already reduces the receptors

40
Q

What is spinal muscle atrophy

A

AR that causes loss of LMN leading to weakness of muscles used for movement affects anterior horn cell

  • Fasiculation
  • Reduced tone
  • Reduced power
  • Reduced reflexes
41
Q

How do you Rx

A 
MDT
Physio
NIIV 
Tracheostomy
PEG
42
Q

What Ax is CI in myasthenia graves

A

Gentamicin

43
Q

What is plasma exchange

A

Removes large molecular weight substances that are harmful e.g. Ab

44
Q

When is it used

A

MG
GBS
Devics
Demyelinating polyneuropathy

45
Q

What is IVIG and role

A

Give loads to Ab to dampen immune response

46
Q

When is it used

A

MG
GBS
Inflammatory myelopathy

47
Q

What are red flags for myopathy

A 
Floppy baby
Paucity of limb
Alert but less motor
Delayed motor
Frequent falls
Gower
Myopathic face
Myotonia
High foot arch
Hammer toe

Neuro (19 decks)

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