What enzyme is commonly raised in muscle disease?
Creatinine kinase
How raised is CK in dystrophic muscle disease?
High
200-300x normal
How raised is CK in inflammatory muscle disease
Medium
20-30x normal
How raised is CK in neurogenic muscle disease?
Low
2-5x normal
Muscle pathology can be divided into 2 different groups. What are they?
Pathology affecting the muscle
Pathology affecting nerve supply to muscles
Describe some dystrophic muscle changes.
Varied muscle fibre size
Nuclei centrally placed
Endomysial fibrosis
Fatty infiltrate
Duchenne and Becker muscular dystrophy has which genetic inheritance?
X-linked recessive
What area of the body does Duchenne and Becker muscular dystrophy primarily affect?
Pelvic girdle
What age group are affected by Duchenne’s MD?
2-4 years
Die by 20
Pathology behind Duchenne’s MD
Alterations in anchorage of actin cytoskeleton to basement membrane
Fibres weakened without attachment
True/False: Becker MD is a genetic varient of Duchenne’s MD with earlier onset?
False - Later onset
Which MD is most common?
Myotonic
Which inheritence pattern does Myotonic MD follow?
Autosomal dominant Ch19 and Ch3
How many forms does myotonic MD exist in?
2 - MD1 and MD2
MD1 affects proximal/distal?
Distal
MD2 affects proximal/distal?
Proximal
Define polymyositis
Progressive MD caused by chronic inflammation of an unknown cause
Define dermatomyositis.
Polymyositis with skin changes
Associated with malignancy in 10% of cases
What is myasthenia gravis?
Autoimmune disorder where antibodies are made against ACh
Results in muscle weakening and dysfunction
Who does myasthenia graves usually affect?
Women between 20-40
Define rhabdomyolosis.
Breakdown of skeletal muscle
True/False: CT disorders usually affect men more than women?
False - Women are more affected than males